Hematology Exam 1 Flashcards

Question

What is the cascade pathway known now as?

Answer 1

Now known that the primary pathway for the initiation of blood coagulation is the tissue factor pathway.

Answer 2

The contact activation pathway (aka intrinsic pathway) and the tissue factor pathway (aka extrinsic pathway).

Answer 3

Prothrombin ---XaVa---> thrombin (a protease) meets with fibrinogen to become fibrin

Answer 4

TF --> VIIa --> XaVa --> Thrombin a process by which thrombin, the most important constituent of the coagulation cascade in terms of its feedback activation roles, is released very rapidly. FVIIa circulates in a higher amount than any other activated coagulation factor. Following damage to the blood vessel, FVII leaves the circulation and comes into contact with tissue factor (TF) expressed on tissue-factor-bearing cells (stromal fibroblasts and leukocytes), forming an activated complex (TF-FVIIa).

Answer 5

XIIa --> XIa --> IXa -VIIa --> XaVa --> Thrombin <-- Prothrombin. The contact activation pathway begins with formation of the primary complex on collagen by high-molecular-weight kininogen (HMWK), prekallikrein, and FXII (Hageman factor). Prekallikrein is converted to kallikrein and FXII becomes FXIIa. FXIIa converts FXI into FXIa. Factor XIa activates FIX, which with its co-factor FVIIIa form the tenase complex, which activates FX to FXa. The minor role that the contact activation pathway has in initiating clot formation can be illustrated by the fact that patients with severe deficiencies of FXII, HMWK, and prekallikrein do not have a bleeding disorder. Instead, contact activation system seems to be more involved in inflammation.

Answer 6

Fibrinogen --> Fibrin (soft clot) -- XIIIa --> Fibrin (hard clot)

Answer 7

Plasmin is released as a zymogen called plasminogen (PLG) from the liver into the systemic circulation. Plasminogen is the inactive precursor of the trypsin-like serine protease plasmin. It is normally found circulating through the blood stream. When plasminogen becomes activated and is converted to plasmin, it unfolds a potent enzymatic domain that dissolves the fibrinogen fibers that entgangle the blood cells in a blood clot. This is called fibrinolysis.

Answer 8

In circulation, plasminogen adopts a closed, activation resistant conformation. Upon binding to clots, or to the cell surface, plasminogen adopts an open form that can be converted into active plasmin by a variety of enzymes, including tissue plasminogen activator (tPA), urokinase plasminogen activator (uPA), kallikrein, and factor XII (Hageman factor). Fibrin is a cofactor for plasminogen activation by tissue plasminogen activator. Urokinase plasminogen activator receptor (uPAR) is a cofactor for plasminogen activation by urokinase plasminogen activator. The conversion of plasminogen to plasmin involves the cleavage of the peptide bond between Arg-561 and Val-562.

Answer 9

Fibrin---> fibrin degradation products

Answer 10

Extrinsic=damaged endothelial cells --> t-PAI --> plasminogen. Intrinsic=damaged endothelial cells --> Factor XIIa --> urokinase --> plasmin --> fibrin --> fibrin degradation products

Answer 11

Personal history: miscarriages, menorrhagia, nose bleeds, bleeding with dental work. Family history and genetic risk: miscarriages. Known liver, immunologic or hematologic conditions. Chronic health problems. Medications (prescription, OTC and herbals). Occupations, hobbies (exposure to hematologically damaging agents). Dietary history. Socioeconomic status.

Answer 12

skin, mucous membranes, lymph nodes, cardiopulmonary, renal, MSK, abdominal, CNS

Answer 13

CBC, bleeding time, prothrombin/INR, partial thrombin time, active PTT (aPTT)

Answer 14

Iron, ferritin, transferrin, total iron binding capacity, direct and indirect coomb's test, reticulocyte count

Answer 15

Measures the coagulation effectiveness of prothrombin, fibrinogen, factors V, VII, X and vitamin K (extrinsic and common pathway). Deficiency of clotting factors=PT is increased!

Answer 16

Same regardless of testing reagents or methods INR therapeutic ranges from 1.5 to 3.5 depending on the problem being treated. International Sensitivity Index (ISI), Usually between 1.0-2.0.

Answer 17

DVT prophylaxis=1.5-2.0. DVT treatment=2.0-3.0. Orthopedic Surgery=2.0-3.0. Embolus Prevention Atrial Fibrillation=2.0-3.0. Pulmonary Embolism Treatment=2.5-3.5. Prosthetic Valve Embolus Prevention=2.5-3.5.

Answer 18

Coagulation effectiveness of clotting factors of the intrinsic pathway. Factors II,V, VIII, IX, XI and XII. Factors II, IX and X depend on vitamin K- liver disease can decrease their levels-prolonging PTT. Normal 60-70 seconds. Critical >100 seconds.

Answer 19

Impairs platelet ability to clump together to control bleeding= Vit K, ASA, NSAIDS, Heparin, Coumadin, Warfarin, tPA.

Answer 20

scurvy, easy bruising, henoch-schonlein purpura

Answer 21

Quantitative-thrombocytopenia, Qualitative-platelet function disorders

Answer 22

Congenital- haemophilia (A, B), von willebrands. Acquired- vitamin K deficiency, liver disease. Mixed/consumption: DIC

Answer 23

Most common type of hemophilia. AKA ‘classic hemophilia.’ Primarily an inherited disorder in which one of the proteins needed to form blood clots is missing or reduced. Approximately one in 5,000 males in the US.

Answer 24

Patient does not bleed harder or faster than a person without hemophilia, they bleed longer. Normal plasma levels of FVIII range from 50% to 150%. Different levels of hemophilia: mild, moderate, and severe.

Answer 25

6% up to 49% of the normal clotting factor in their blood

Answer 26

15% of the hemophilia population- 1% up to 5% of the normal clotting factor in their blood

Answer 27

60% of the hemophilia population- <1% of the normal clotting factor in their blood

Answer 28

family pedigree

Answer 29

X chromosome

Answer 30

1. A girl who is not a carrier, 2. A girl who is a carrier, 3. A boy without hemophilia, 4. A boy with hemophilia

Answer 31

Desmopressin acetate (DDAVP) to treat small bleeds. Deep cuts or internal bleeding ,bleeding into the joints or muscles, require more complex treatment. The clotting factor missing (VIII or IX) must be replaced.

Answer 32

Factor VIII concentrate. Prophylaxis w/ plasma or recombinant factor IV ( can be self injected).

Answer 33

Second most common, protein needed to form clots reduced/missing, 70% have family history, 30% spontaneous mutation, 1 in 25,000 male births, bleeds longer.

Answer 34

plasma and recombinant

Answer 35

Also known as: Plasma Thromboplastin Antecedent (PTA) Deficiency, Rosenthal Syndrome. 1 in 100,000 people, Autosomal dominant, males=females, More common in Ashkenazi Jewish descent.

Answer 36

Bleeding time test, platelet function tests, prothrombin time (PT), activated partial thromboplastin time (aPTT) tests. Specific Factor XI assay is extremely useful in ruling out combined deficiencies. There is variability in the presentation of this disease. XI part of clotting cascade.

Answer 37

Not likely to bleed spontaneously, Hemorrhage normally occurs after trauma or surgery. Joint bleeds are uncommon. Prone to bruising, nosebleeds, or blood in the urine. Woman may experience menorrhagia and prolonged bleeding after childbirth.

Answer 38

In the US no factor XI concentrates available until this year. Fresh-frozen plasma is normally used for treatment. But since Factor XI is not concentrated in fresh frozen plasma, considerable amounts of plasma may be required to maintain the factor level. In the case of mouth bleeds antifibrinolytic products such as Amicar are used.

Answer 39

I, II, V, X, XII, XIII

Answer 40

fibrinogen

Answer 41

prothrombin

Answer 42

proaccelerin, labile factor

Answer 43

thromboplastin antecedent

Answer 44

Hageman factor

Answer 45

fibrin-stabilizing factor

Answer 46

stable factor

Answer 47

antihemophilic factor

Answer 48

christmas factor

Answer 49

Stuart-prower factor

Answer 50

plasma factor

Answer 51

afibrinogenemia, dysfibrinogenemia, or hypofibrinogenemia, 1 per 2 million people, autosomal recessive, females and males equally

Answer 52

Often at birth, spontaneous abortion, more disposed to thrombosis. Labs: measure the amount of fibrinogen in the blood, prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin clotting time (TCT) test.

Answer 53

cryoprecipitate

Answer 54

2%-50%, >50% little or no bleeding disorders, Rare (26 cases), Autosomal recessive males=females

Answer 55

prothrombin time (PT), activated partial thromboplastin time (aPTT)

Answer 56

Fresh Frozen Plasma, Prothrombin complex concentrates (PCCs) (May precipitate thromboembolic events)

Answer 57

Owren’s disease, labile factor deficiency, proaccelerin deficiency’ parahemophilia. (-Not to be confused with Factor V Leiden- a type of thrombophilia). 1 per million get it. Autosomal recessive : males=females. Catalyst or “accelerator” in the process to convert prothrombin to thrombin.

Answer 58

easy bruising, nose or mouth bleeds, bleeding after trauma or surgery

Answer 59

joint bleeding, gastrointestinal bleeds, hematomas

Answer 60

heavy menstrual bleeding (menorrhagia), first trimester miscarriage other complications during pregnancy and delivery

Answer 61

Diagnosis is made through personal & family history. Labs: prothrombin time (PT) test, partial thromboplastin time (PTT), activated partial thromboplastin time (APTT) test, confirmed by a factor X functional activity (FX:C) or Russell viper venom (RVV) time assay.

Answer 62

Incidence:1 in 500,000-1,000,000 world wide, Autosomal recessive, Affects males/females equally, Factor X activates enzymes that form clot

Answer 63

There is no factor X concentrate currently available in the US. Fresh-frozen plasma or plasma-derived Prothrombin Complex concentrates (PCCs) are normally used as treatment. Fibrin glue may relieve bleeding symptoms. Mild: antifibrinolytic agents, Aminocaproic acid or tranexamic acid topical therapies.

Answer 64

Normally do not require treatment. Having a low factor XII level has little to no clinical significance.

Answer 65

Incidence of Factor XII deficiency: 1 in 1 million. Autosomal recessive fashion, males=females Usually do not experience bleeds.

Answer 66

Fibrin Stabilizing Factor deficiency, Factor XIII. Autosomal recessive : males=females. Protein responsible for stabilizing the formation of a blood clot. Usually associated w/ trauma. High risk of head bleeds w/ or w/out trauma. Delayed bleeding after surgery,

Answer 67

normal coagulation screening tests, detailed family history., specific factor XIII assays can confirm the diagnosis

Answer 68

Goal is to limit trauma that can lead to bleeding. Use soft-bristled toothbrush, electric shaver, avoid aspirin or aspirin-containing products. No contact sports or activities that may result in impact, scratches or abrasions. If bumped, apply ice to area for 1 hour. Avoid straining for BM – use stool softener, no enemas, rectal suppositories or anal sex. Avoid blowing nose.

Answer 69

Defined as a platelet count of less than 150 × 103 per μL.

Answer 70

Often discovered incidentally when obtaining a CBC. The etiology usually is not obvious. Requires additional investigation.

Answer 71

Platelet counts > 50 × 103 per μL rarely have symptoms.

Answer 72

Platelet count from 30 to 50 × 103 per μL may manifest as purpura.

Answer 73

10 to 30 × 103 per μL may cause bleeding with minimal trauma.

Answer 74

< 5 × 103 per μL may cause spontaneous bleeding and constitutes a hematologic emergency.

Answer 75

emergent thrombocytopenia does, nonemergent just needs outpatient treatment

Answer 76

drug-induced thrombocytopenia, immune thrombocytopenic purpura (ITP) (Idiopathic thrombocytopenia purpura), pseudothrombocytopenia

Answer 77

acute infection, heparin-induced thrombocytopenia, liver disease, thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, disseminated intravascular coagulation (DIC) or a hematologic disorder.

Answer 78

gestational thrombocytopenia, preeclampsia, HELLP (hemolysis, elevated liver enzymes, and low platelet count)

Answer 79

Easy bruising, petechiae, melena, rashes fevers and bleeding. Medications, immunizations, recent travel, recent hospitalization, transfusion history, family history, medical history, and acute/chronic alcohol use.

Answer 80

visual symptoms, headaches, abdominal pain, influenza-like symptoms

Answer 81

Skin-petechiae, purpura, bruising. Eyes- hemorrhage is suggestive of central nervous system bleeding. Lymph nodes, Abdomen-splenomegaly, hepatomegaly. Neurologic system.

Answer 82

CBC, a peripheral blood (white blood cells disorders, hemolytic anemias, thrombocytopenia), Coagulation studies, LFTs, kidney function

Answer 83

Bone marrow failure (aplastic anemia, paroxysmal nocturnal hemoglobinuria, shwachman-diamond syndrome). Bone marrow suppression (medication, chemotherapy, irradiation), chronic alcohol abuse, congenital macrothrombocytopenias (alport syndrome, bernard -soulier syndrome, fancoli anemia, platelet-type or pseudo-von willebrand disease, wiskott-aldrich syndrome), Myelodysplastic syndrome, neoplastic marrow infiltration, nutritional deficiencies (vitamin B12, folate).

Answer 84

Alloimmune destruction (posttransfusion, neonatal, posttransplantation), Autoimmune syndromes (antiphospholipid syndrome, SLE, sarcoidosis, Disseminated intravascular coagulation/severe sepsis, Drug-induced thrombocytopenia.

Answer 85

chronic alcohol abuse, dilutional thrombocytopenia (hemorrhage, excessive crystalloid infusion), gestational thrombocytopenia, mechanical destruction (aortic valve, mechanical valve, extracorporeal bypass), preeclampsia/HELLP syndrome, Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome, Hypersplenism, distributional thrombocytopenia, Liver disease, cirrhosis, fibrosis, portal hypertension, Pseudothrombocytopenia, Pulmonary emboli and Pulmonary hypertension.

Answer 86

autoimmune thrombocytopenia- body forms antibodies to platelets and then begins to destroy them

Answer 87

Abrupt onset (childhood ITP), Gradual onset (adult ITP), Purpura, Menorrhagia, Epistaxis, Gingival bleeding, Recent live virus immunization -childhood ITP, Recent viral illness and Bruising tendency.

Answer 88

Cytomegalovirus, Epstein-Barr virus, hepatitis C virus, HIV, Mumps, parvovirus B19, Rickettsia rubella, and varicella-zoster virus.

Answer 89

It depends on the suspicion, but smear and coagulation studies and liver/kidney function tests could help.

Answer 90

Referral to hematologist, interventions are corticosteroids, IV immunoglobulin, and chemotherapy.

Answer 91

Acute or subacute onset of symptoms: Neurologic dysfunction, Anemia, Thrombocytopenia, Severe bleeding is unusual, petechiae are common, Fever - 50% of Pts, Dark urine (hemoglobinuria).

Answer 92

hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever and renal disease.

Answer 93

Less CNS involvement than TTP, more severe renal involvement in HUS. In HUS they will have a hx of diarrhea.

Answer 94

CBC, Platelet count, Blood smears, Coagulation studies, BUN creatinine, Serum bilirubin and Lactate dehydrogenase.

Answer 95

red blood cell fragments

Answer 96

Admit them, stabilize them, refer to hematologist, plasma exchange with frozen fresh plasma, octaplas (octapharma), corticosteroids

Answer 97

a protein that acts like glue to help the platelets stick together and form a blood clot

Answer 98

clotting factor VIII

Answer 99

Low levels of von willebrand factor and may have low levels of factor VIII. Type 1 is the mildest and most common form of VWD. About 3/4 people who have VWD have type 1.

Answer 100

2A, 2B, 2M and 2N. This factor doesn't work well. Type 2 is based on different gene mutations.

Answer 101

NO von willebrand factor levels and low levels of factor VIII. It is the most serious form and it is very rare.

Answer 102

Frequent, large bruises from minor bumps or injuries, Frequent or hard-to-stop nosebleeds Prolonged bleeding from the gums after a dental procedure, Heavy or prolonged menstrual bleeding in women, Blood in stools, Blood in urine, Heavy bleeding after a cut or other accident, Heavy bleeding after surgery and Heavy bleeding after delivery.

Answer 103

All of the same signs and symptoms for type 1 and 2, plus severe bleeding episodes for no reason. Can be fatal if not treated right away.

Answer 104

Bleeding from a small wound that lasted more than 15 minutes or started up again within the first 7 days following the injury. Prolonged, heavy, or repeated bleeding that required medical care after surgery or dental extractions. Bruising with little or no apparent trauma, there may be a lump under a bruise. Nosebleeds for no reason and lasted more than 10 minutes despite pressure on the nose. Any blood in stools for no reason. Heavy menstrual bleeding (bleeding with clots or lasts longer than 7-10 days). History of muscle or joint bleeding.

Answer 105

Aspirin, nonsteroidal anti-inflammatory drugs (NSAIDs), clopidogrel (Plavix), Warfarin, heparin. Liver or kidney disease, blood or bone marrow disease, high or low blood platelet counts.

Answer 106

Skin - petechiae, purpura, bruising, Eyes- hemorrhage is suggestive of central nervous system bleeding, lymph nodes, Abdomen-splenomegaly, hepatomegaly, neurologic system.

Answer 107

based on suspicion, presentation and history. No single test can diagnose VWD. There is a VWB factor antigen. VWB factor ristocetin (ris-to-SEE-tin) cofactor activity. Factor VIII clotting activity. VWB factor multimers. Platelet function test. Test more than once to confirm a diagnosis. Refer to a hematologist to confirm the diagnosis and followup.

Answer 108

Based on the type of VWD and how severe it is. Most cases of VWD are mild, and may need treatment only for surgery, tooth extraction, or an accident. Medications-Desmopressin, Antifibrinolytic medicines, Fibrin glue placed directly on a wound to stop bleeding. Von Willebrand factor replacement therapy. IV infusion of concentrated von Willebrand factor and factor VIII.

Answer 109

Oral contraceptives, A levonorgestrel intrauterine device. Contains the hormone progestin. Aminocaproic acid or tranexamic acid. antifibrinolytic medicines reduce bleeding by slowing the breakdown of blood clots. Desmopressin. Women who are done having children or don't want children=endometrial ablation.Hysterectomy=Not an indication but if done for other reasons, but there is a possible bleeding complication during surgery.

Answer 110

Factor V Leiden thrombophilia and Prothrombin G20210A mutation

Answer 111

Protein C and protein S. Antithrombin III deficiencies.

Answer 112

Factor V Leiden Thrombophilia. Inherited disorder of blood clotting. Factor V Leiden is the name of a specific gene mutation that results in increased tendency to form abnormal blood clots (DVTs are common). Most common inherited form of thrombophilia esp in European descent.

Answer 113

DVTs occur most often in the legs or pelvis (also occur in the brain, eyes, liver, and kidneys). Increased risk of clots will break away, (travel through the bloodstream, PE could form).

Answer 114

Increased risk of spontaneous abortion (Three x more likely to have recurrent miscarriages in 2 or 3 trimester), More likely to develop clots on combination oral contraceptives, May be associated w/ preeclampsia, slow fetal growth, and abruption.

Answer 115

Same presentation and risks as Factor V Leiden thrombophilia. They are both detected by PCR. Screening is not cost effective for either.

Answer 116

Excessive clotting that uses up all platelets and clotting factors resulting in internal and/or external bleeding

Answer 117

Sepsis, Hemorrhage, Cancer, Complications of pregnancy or childbirth, Trauma, Surgery, Poisonous snakes, Burns and Frostbite.

Answer 118

Acute-develops quickly and must be treated ASAP. Chronic-develops slowly, it is the most common cause (cancer) and there is more clotting than bleeding.

Answer 119

stores WBC for fighting infection, filters bacteria, destroys old or imperfect RBCs

Answer 120

produces clotting factors, stores blood cells, converts bilirubin to bile, stores extra iron

Answer 121

Uses supravital stain which stains cells in the living state.

Answer 122

retics per 1,000 RBC's/10= Retic %, % retics x pt. HCT/45= Corrected retic

Answer 123

Inadequate mixing of specimen, Hemolyzed specimens, Lipemic specimens, Cold agglutinins, Clotted specimens, Platelet clumps, Diluted specimens

Answer 124

carries carbon dioxide and helps with acid-base balance

Answer 125

Supplied by the diet and absorbed in the duodenum and proximal jejunum

Answer 126

Carried by protein transferrin to the cells and binds to the heme portion of the hemoglobin and it is recycled

Answer 127

erythrocytes 4-6 million

Answer 128

In man and in all mammals, erythrocytes are devoid of a nucleus and have the shape of a biconcave lens. In the other vertebrates (e.g. fishes, amphibians, reptilians and birds), they have a nucleus.

Answer 129

carried by plasma in the form of soluble carbonates

Answer 130

Lack of nucleus allows more room for hemoglobin and the biconcave shape of these cells raises the surface and cytoplasmic volume ratio. Characteristics make more efficient the diffusion of oxygen by these cells.

Answer 131

mean life of erythrocytes is about 120 days. When they come to the end of their life, they are retained by the spleen where they are phagocyted by macrophages

Answer 132

red cells can have different shapes: normal (discocyte), berry (crenated), burr (echinocyte), target (codocyte), oat, sickled, helmet, pinched, pointed, indented, poikilocyte

Answer 133

whole blood occupied by intact red blood cells

Answer 134

Blood loss -which is the most common cause, can be short term or chronic. Iron deficiency anemia, heavy menstrual periods, surgery, trauma, GI/GU bleed and cancer, obvious bleeding, occult bleeding, repeated diagnostic tests. Lack of red blood cell production (acquired from poor diet -lack of Vit C, Fe, folic acid, Vit B12, Zn (or malabsorption) abnormal hormone levels- erythropoietin).

Answer 135

Hb, Hct, and RBC are all concentrations dependent on red blood cell mass as well as plasma volume. All 3 values will be reduced if the red blood cell mass (RBCM) is reduced or the plasma volume is increased. In acute bleeding there is acute volume depletion. It is not until the fluid is moved into the intravascular space that lab results will reflect blood loss- 36-48hrs later. Volume depletion may mask low Hb/Hct until volume depletion is corrected.

Answer 136

Women of childbearing age because they have no iron stores therefore any bleeding puts them at risk, children, older adults and the malnourished.

Answer 137

Pregnancy -the plasma volume increases by 25-50%

Answer 138

Microcytic, normocytic, and macrocytic. Microcytic anemia is defined by a MCV of < 80. The differential diagnosis of a microcytic anemia includes iron deficiency anemia (IDA), thalassemias, anemia of chronic disease (ACD) and sideroblastic anemias, including lead poisoning.

Answer 139

Depend on the severity and rate of development- Symptoms are less likely if it evolves slowly due to compensatory mechanisms. Can result from decreased oxygen delivery and/or hypovolemia. Headache, dizziness, shortness of breath, pale skin, coldness in the feet and hands, chest pain, bounding pulse, roaring in the ears, because the heart has to work harder to pump oxygen rich blood.

Answer 140

Erythropoietin is an endocrine hormone made in the kidneys and enhances the maturity of normoblasts which turn into reticulocytes. After roughly 4 days the reticulocyte turns into a mature RBC. This RBC circulates for 110 to 120 days until macrophages remove them.

Answer 141

Look for signs of organ or multisystem involvement Tachycardia, dyspnea, fever or postural hypotension. Jaundice may be difficult to detect under artificial lighting. Pallor in the palms, nail beds, face or conjunctivae as a predictor varies from 19-70%. Watch for weight loss, wasting, poor muscle tone/strength to show severity/chronicity of disease. Lymphadenopathy, hepatosplenomegaly, bone tenderness especially over the sternum and bone pain. Petechiae, ecchymoses and other signs of coagulopathy. When ordering labs (CBC) order them STAT which should get results in 4 to 6 hours. Have the patient call you back in 24 hours for plan.

Answer 142

Call the consultant for referral in advance of the patient appointment (i.e. Mrs. Jones with low WBC, axillary node, weight loss is coming in 3 days) and send results.

Answer 143

You can do a chest x-ray, US/CT of the abdomen/pelvis to check for mediastinal/hilar nodes, enlarged liver or spleen or abdominal/pelvic nodes to aid in diagnosis.

Answer 144

CBC which includes Hb, Hct, RBC, RBC indices and WBC. A WBC differential, PLT and reticulocyte count are not part of the routine CBC and would need to be ordered. Many machines will report RBC distribution width (RDW) but this is not enough to form any diagnosis. Blood smears should be evaluated by experienced examiners, not machines for differential diagnosis.

Answer 145

Mean corpuscular volume (MCV) (High values are almost exclusively seen in vitamin B12 or folate deficiency, High values also seen with medications and increased reticulocyte count, False positive results can be due to cold agglutins. Repeat tests should be brought to body temp before running). Mean corpuscular hemoglobin (MCH) (Low values are seen in thalassemia and iron deficiency, High values in macrocytosis of any cause), and Mean corpuscular hemoglobin concentration, (high values are almost exclusive to congenital or acquired spherocytosis or other congenital diseases where RBC’s are abnormally desiccated- SCD, Hb C disease, xerocytosis)

Answer 146

high level with anemia reflects increased erythropoietic response to hemolysis or blood loss

Answer 147

low level with anemia is strong evidence for deficient production (reduced marrow response)

Answer 148

Low count with pancytopenia suggests aplastic anemia. Extremely low count suggests pre red cell aplasia.

Answer 149

Low WBC and anemia is indicative of bone marrow suppression or deficiencies of cobalamin or folate

Answer 150

High WBC and anemia may indicate infection, inflammation or malignancy

Answer 151

Their presence may mean malignancy, hematologic disease or severe sepsis or heart failure.

Answer 152

Low counts can indicate hypersplenism, autoimmune plt destruction, sepsis or folic acid/cobalamin deficiency

Answer 153

High counts may reflect chronic iron deficiency, myeloproliferative or myelodysplastic disorders

Answer 154

Myelodysplasias, fibrosis, neoplasm, aplastic anemia, autoimmune – SLE, rheumatoid arthritis Infectious – EBV, CMV, Chemotherapy and other medications, Vitamin deficiency- Vit B12 and folate

Answer 155

follow up with a blood smear

Answer 156

If Hb was not being replaced the resultant decrease would be 1% per day because there is only 1% reticulocytes in the blood. Therefore if the rate of fall is 15g/dl in 1 week that is a greater drop and therefore the patient must be bleeding or RBC are being destroyed.

Answer 157

Has a rapid fall in Hb, reticlocytosis, abnormally shaped RBC on smear, increased LDH and reduced haptoglobin are 90%specific for diagnosing hemolysis.

Answer 158

It is not useful in anemia diagnosis. It is used in aplastic anemia, abnormal cells such as blasts, and pancytopenia.

Answer 159

glucocorticoids

Answer 160

CHF and renal failure

Answer 161

Diminished or absent hematopoietic cells in the marrow, (injury to pluripotent stem cells, it involves more than RBC’s, it is associated with pancytopenia bone marrow failure is a more appropriate term)

Answer 162

Normocytic, because the cells are normal size and shape, just inadequate in numbers. Pancytopenia in the absence of splenomegaly.

Answer 163

fanconi's anemia

Answer 164

Exposure to drugs, viruses, radiation or toxins, Malignancy, Autoimmune, or Pregnancy

Answer 165

NSAIDS- phenylbutazone which is now used for horses. Chloramphenicol – used for resistant infection. Gold – arthritis. Antiepileptics – felbamate, carbamazepine, valproic acid, phenytoin and Nifedipine.

Answer 166

Exposure to solvents, degreasing agents, pesticides and radiation are all factors (Landscapers, shoe repair, benzene exposure, rubber factories, oil refineries, bus depot Reduced ability to detoxify our environment may be a contributing factor, Use of non-bottled water increases risk)

Answer 167

Epstein- Barr, Seronegative hepatitis, HIV, Herpes virus

Answer 168

Hypothesis is damage from drugs, radiation etc. stimulates lymphocyte activity which results in hematopoietic inhibitory response. This triggers cytokine cascade leading to apoptopic death of pluripotent cells. Stressed, dying and immunologically activated target cell population is seen.This is T cell mediated.

Answer 169

immunosuppressive therapy

Answer 170

Paroxysmal nocturnal hemoglobinuria (These patients are prone especially after immunotherapy), Fanconi’s anemia. (Diagnosed before 16yrs, Has increased chromosomal breakage in lymphocytes on bone marrow examination) Telomerase mutations, abnormal thrombopoietin

Answer 171

Related to pancytopenia, not just anemia, fatigue and cardiopulmonary symptoms, recurrent infections due to leukopenia, mucosal hemorrhage and increased menstrual flow from thrombocytopenia, pallor and petichiae, liver, spleen and lymph nodes are not enlarged

Answer 172

CBC revealing pancytopenia and reduced absolute number of reticulocytes, smear shows normocytic cells, with a few macrocytic cells and almost absent reticulocytes. Bone marrow aspiration, biopsy and cytogenic analysis must be performed. Bone marrow space consists mostly of fat cells and marrow stroma- decrease in all elements.Marrow-malignant cells or fibrosis are not present. Residual hematopoietic cells are morphologically normal. Rule out myelodysplastic syndrome- the precursor to leukemia.

Answer 173

CBC revealing pancytopenia and reduced absolute number of reticulocytes, smear shows normocytic cells, with a few macrocytic cells and almost absent reticulocytes. Bone marrow aspiration, biopsy and cytogenic analysis must be performed. Bone marrow space consists mostly of fat cells and marrow stroma- decrease in all elements.Marrow-malignant cells or fibrosis are not present. Residual hematopoietic cells are morphologically normal. Rule out myelodysplastic syndrome- the precursor to leukemia.

Answer 174

bone marrow cellularity < 30%, absence of severe pancytopenia, depression of at least 2 of the 3 blood elements

Answer 175

Bone marrow cellularity <20

Answer 176

All criteria is met in moderate and severe aplastic anemia, ANC <200

Answer 177

Bone marrow transplant patients. We want to kill all of the cells. They are "naked" to the environment and most at risk for infection.

Answer 178

Pancytopenia without spleenomegaly can be seen in: Acute leukemias, myelodysplastic syndrome (MDS), Fibrosis or tumor, Severe megaloblastic anemia, Paroxysmal nocturnal hemoglobinuria (PNH), Overwhelming HIV infection. Examination of bone marrow , biopsy and smear should distinguish these as rule outs.

Answer 179

Depends on the severity of pancytopenia and patient age, unless severe and very severe cases are successfully treated 70% will be dead in 1 year. Increased age causes increased mortality in all classes of disease mainly from bleeding.

Answer 180

``` Withdrawal of offending agent, supportive care – fluids, antibiotics, definitive therapy- HCT, immunosuppressive agents. Depends on age , under 45- HCT over 45- immunosuppression Antithymocyte globulin (ATG) is immunotherapy , solu medrol, cyclosporine. Transfusions should be minimized. Place a central line for ease of blood draws and therapy. Add G-CSF for neutropenia. Mycophentolate did not improve survival. High dose cyclophosphamide without stem cell infusion. ```

Answer 181

``` Withdrawal of offending agent, supportive care – fluids, antibiotics, definitive therapy- HCT, immunosuppressive agents. Depends on age , under 45- HCT over 45- immunosuppression Antithymocyte globulin (ATG) is immunotherapy , solu medrol, cyclosporine. Transfusions should be minimized. ```

Answer 182

Macrocytic, this kind of anemia is associated with liver disease. The enlarged red blood cells are sometimes the only sign a patient is experiencing when they're deficient in B12 and folate. Vitamin B!2 and folate are 2 important nutrients for the health of the nervous system. This condition can also be a side effect of chemotherapy drugs, and is a common consequence of heavy alcohol use.

Answer 183

Macrocytic, this kind of anemia is associated with liver disease. The enlarged red blood cells are sometimes the only sign a patient is experiencing when they're deficient in B12 and folate. Vitamin B!2 and folate are 2 important nutrients for the health of the nervous system.

Answer 184

maturing properly...cells lose their nucleus and decrease in size during the normal growth process.... when this doesn't happen it is a sign of B12 and folate deficiency anemia

Answer 185

maturing properly... this is a sign of B12 and folate deficiency anemia

Answer 186

B12 and folate deficiency anemia

Answer 187

vitamin is absorbed in the small intestine with the help of intrinsic factor, produced in the stomach lining

Answer 188

If intrinsic factor is not present, Vitamin B12 cannot be absorbed. This condition is known as pernicious anemia, and is treated with B12 injections.

Answer 189

If intrinsic factor is not present, Vitamin B12 cannot be absorbed. This condition is known as pernicious anemia, and is treated with B12 injections. Alcohol interferes with the absorption of B12, and chronic heavy drinkers are at increased risk for macrocytic anemia.

Answer 190

Alcohol interferes with the absorption of B12, and chronic heavy drinkers are at increased risk for macrocytic anemia.

Answer 191

Folate is abundant in green leafy vegetables, liver and mushrooms. Food must be cooked to make folate available, but too much cooking destroys this vitamin. Absorption of folate takes place in the small intestine.

Answer 192

lack of folate in the diet, problems absorbing folate, difficulty utilizing the vitamin, increased requirement

Answer 193

Patients with celiac disease have trouble absorbing folate because this disease damages the cells where absorption takes place. It's also important to note that several drugs interfere with absorption, including birth control pills. Alcohol also interferes with the use of folate.

Answer 194

Severe anemia with macrocytic cells with or without neurologic disturbances. Hyper segmented neutrophils on blood smear. Pancytopenia of uncertain cause. Unexplained neurologic symptoms- dementia, weakness, sensory ataxia, parasthesias.

Answer 195

Severe anemia with macrocytic cells with or without neurologic disturbances. Hyper segmented neutrophils on blood smear. Pancytopenia of uncertain cause. Unexplained neurologic symptoms- dementia, weakness, sensory ataxia, parasthesias.

Answer 196

Deficiency of both causes megaloblastic anemia, but only lack of B12 causes neurologic changes

Answer 197

Classic clinical picture of PA is a prematurely grey, Northern European person with lemon colored skin, mentally sluggish with a shiny tongue and a shuffling broad base gait. Subtle presentation is hard to characterize, neuropsychiatric problems, iron deficiency anemia or thalessemia. Neuropathy affects the legs more than arms. Loss of vibration and position sense.

Answer 198

Classic clinical picture of PA is a prematurely grey, Northern European person with lemon colored skin, mentally sluggish with a shiny tongue and a shuffling broad base gait. Subtle presentation is hard to characterize, neuropsychiatric problems, iron deficiency anemia or thalessemia. Neuropathy affects the legs more than arms.

Answer 199

Onset of folate deficiency can take 4-5 months (pregnancy). Symptoms are associated with anemia.

Answer 200

Older adults – less absorption, alcoholics- cannot absorb- Wernicke’s encephalopathy, malnourished vegans, bariatric surgery, pregnant and breast feeding women

Answer 201

Measure serum cobalamin , RBC and folate levels – before any meals, metabolic intermediates (Methylmalonate (MMA) and homocysteine accumulate in deficiency state). Antibodies to intrinsic factor which would diagnose pernicious anemia. Schilling test- radioactive cobalamin is traced. Metabolite testing (MMA) should be reserved for those with borderline levels. Bone marrow examination is unnecessary.

Answer 202

Measure serum cobalamin , RBC and folate levels – before any meals, metabolic intermediates (Methylmalonate (MMA) and homocysteine accumulate in deficiency state). Antibodies to intrinsic factor which would diagnose pernicious anemia. Schilling test- radioactive cobalamin is traced.

Answer 203

Folic acid 1mg orally once a day for one to four months, until labs improve. Vitamin B12 deficiency must be ruled out before starting treatment since folate can mask megaloblastic anemia. Hematologic abnormalities can be partially reversed but neurologic symptoms will progress. When in doubt give both drugs until test results come back. Maintenance therapy is used for congenital abnormalities. General use of high dose folic acid without an indication is not recommended, especially at higher doses. It can be used with chemotherapy that are folate reductase inhibitors. Cholestyramine binds it in the stomach- space doses.

Answer 204

Folic acid 1mg orally once a day for one to four months, until labs improve. Vitamin B12 deficiency must be ruled out before starting treatment since folate can mask megaloblastic anemia. Hematologic abnormalities can be partially reversed but neurologic symptoms will progress. When in doubt give both drugs until test results come back.

Answer 205

X, so it affects males more because they can show signs and symptoms

Answer 206

Some are unaffected, some have episodic anemia while others have chronic hemolysis. Acute hemolytic anemia, Favism- hemolytic response to eating broad beans,congenital nonspherocytic hemolytic anemia, neonatal hyperbilirubinemia. There may be no symptoms except shorter life span of RBC until a trigger causes hemolysis. 2 to 4 days after exposure you see sudden jaundice, pallor, dark urine with or without back pain.

Answer 207

200-400 million people world wide

Answer 208

infection, DKA can also trigger it

Answer 209

Hyperbilirubinemia is not present at birth in G6PD and there is more jaundice than anemia

Answer 210

Hyperbilirubinemia is not present at birth in G6PD and there is more jaundice than anemia

Answer 211

``` Exposure to drugs with a high redox potential can initiate severe hemolysis (Primaquine, sulfa drugs) Fava beans can trigger a reaction. Severity of symptoms depends on level of enzyme deficiency. Separated according to class 1 through 5 with class 1 being most severe. ```

Answer 212

Should be considered in any nonimmune hemolytic anemia. Should be considered in any newborn with unexplained hyperbilirubinemia. Finding decreased activity of G6P. Fluorescent spot test is the simplest most reliable form. Methemoglobin reduction test is another useful tool. False negative results occur when the most severely deficient red cells have been removed by hemolysis and retesting is needed.

Answer 213

Methemoglobinemia is a disease resulting in hemoglobin that is less likely to allow o2 to diffuse to the tissues. Methylene blue is a treatment used, but G6P is needed for it to work therefore deficiency makes this treatment ineffective. In addition, methylene blue can induce hemolysis because it has a high redox potential.

Answer 214

Avoid exposure to known triggers, pregnant and nursing women who are heterozygous should avoid drugs with a high redox potential, avoid fava beans, treat the underlying anemia. Spleenectomy and Vitamin E have occasionally helped. Transfusions are only used for severe hemolysis.

Answer 215

Dapsone, Nitrofurantoin, Primaquine, Methylene blue, Phenazopyridine, Dimercaprol, Uricase, Toluine blue and all sulfa drugs.

Answer 216

Aniline dyes, Napthalene ( moth balls), Henna compounds, Fava beans, Red wine, blueberries, soy and tonic water

Answer 217

Neonatal jaundice can occur with irreversible brain damage. Phototherapy is used and in extreme cases exchange transfusions. Screening is not performed.

Answer 218

especially after drug exposure, eating fava beans or infection

Answer 219

A shortened survival of red blood cells less than 100 days.

Answer 220

Compensatory increased erythropoietin secretion limits the anemia

Answer 221

An increase in reticulocyte is seen as long as iron and nutrients are available. The normal reticulocyte count is 1-2%, here you can see 4-5%.

Answer 222

In mild disease you will not see anemia unless compensatory mechanism cannot match the rate of hemolysis

Answer 223

hereditary

Answer 224

Mature RBC has lost it’s nucleus, mitochondria and RNA. It is left with Hb, RBC membrane and the metabolic machinery to make ATP. Hemolysis can be due to the formation of Heinz bodies, membrane injury, unstable or missing proteins, hydration abnormalities, defective glycolysis.

Answer 225

Direct trauma, as in bongo drummers and march hemoglobinuria (runners' or foot-strike hemolysis), shear stress, (as in defective mechanical heart valves), Heat damage, (as in thermal burns) Complement-induced lysis, (as in paroxysmal cold hemoglobinuria), osmotic lysis (following infusion of hypotonic solutions), lysis (from bacterial toxins) (eg, clostridial sepsis) Lysis (from exposure to high concentrations of copper).

Answer 226

The severity and type depend on the cells site of destruction. Incompletely hemolyzed RBCs, which have lost some of their membrane, can reform and become spherocytes or microspherocytes, while others are destroyed outright. Red cell "ghosts", which are red cell membranes devoid of hemoglobin (Hgb), may be seen under extreme circumstances.

Answer 227

Any form of hemolysis can lead to renal damage due to the heme proteins and breakdown products. Hematology should always be consulted since the hemolysis can suddenly turn life threatening.

Answer 228

Severely damaged RBC’s are destroyed in the liver or phagocytosed in the spleen

Answer 229

Rapid onset of pallor and anemia, jaundice with increased indirect bilirubin concentration, history of pigmented (bilirubin) gallstones, splenomegaly, presence of circulating spherocytic red cells (eg, autoimmune hemolytic anemia, congenital spherocytosis), other informative red cell shape changes.

Answer 230

Increased serum lactate dehydrogenase (LDH) and indirect bilirubin concentration, reduced or absent level of serum haptoglobin, A positive direct antiglobulin test (Coombs test), increased reticulocyte percentage or absolute reticulocyte number (not specific for hemolysis), indicating the bone marrow's response to the anemia. Abnormal smear.

Answer 231

erythropoietin; anemia

Answer 232

Diagnosing hemolysis — The combination of an increased serum lactate dehydrogenase (LDH) and a reduced haptoglobin is 90 percent specific for diagnosing hemolysis, while the combination of a normal serum LDH and a serum haptoglobin >25 mg/dL is 92 percent sensitive for ruling out the presence of hemolysis.

Answer 233

Hemolysis without high reticulocyte may mean precursor cells may also be affected or the bodies response is not sufficient-Damaged marrow or chemotherapy

Answer 234

There is no generic treatment for hemolytic anemia. Appropriate management of a patient with hemolytic anemia requires that the underlying cause be determined, and may include red cell transfusion if the anemia is severe.

Answer 235

erythopoiesis

Answer 236

An imbalance between iron uptake and demand (usually increased demand). Absolute deficiency is when iron stores are absent due to malnourishment, malabsorption or blood loss. Functional deficiency is insufficient iron due to anemia of chronic disease (inflammation) or epogen use.

Answer 237

Hemorrhage, hematemesis, melena, hemoptysis, menorrhagia, hematuria, blood donations.

Answer 238

Usually GI, Always rule out a bleed first because this diagnosis requires immediate attention

Answer 239

Decreased absorption due to achlorhydria, food/med interactions, celiac disease, demands of pregnancy, infancy and early childhood, increased demand due to epoetin alpha use to produce more RBC, dialysis and chemotherapy patients

Answer 240

iron deficiency anemia

Answer 241

In the absence of iron for hemoglobin the cells shrink and become microcytic (small) and hypochromic (pale). Reduced o2 carrying capacity causes listlessness, fatigue, pallor In severe cases tachypnea, dyspnea and angina result

Answer 242

Behavioral and neuropsychiatric manifestations, pica (pagophagia) specifically for ice, angular stomatitis ( sores on the corners of the mouth), glossitis, esophageal webs and strictures, koilonychia (spoon nails), Beeturia (ingestion of beets leads to red urine), restless leg syndrome, (responds to iron therapy even if labs are normal), fatigue and exercise intolerance, headache, irritability

Answer 243

Multigravid woman in her forties presents with fatigue and heavy periods, Hb low at 8, MCV low at 75, MCH is low, blood smear shows microcytic, hypochromic cells. Serum iron low at 10. Total iron binding capacity (TIBC) is high- meaning there is plenty of ability to bind iron. Iron stores are absent in bone marrow. We typically do not see the classic presentation though.

Answer 244

Many have normal RBC indices and a normal blood smear. Onset is insidious and presents with symptoms of other diseases- CAD, dementia, CHF.

Answer 245

Ferritin level between 10-15. Microcytic, hypochromic erythrocytes. Absence of hemosiderin- an iron storing complex within cells. Reduced RBC and reticulocyte Hb, Hb, Hct. Increased iron binding capacity. Once a diagnosis is made finding the cause is imperative. Usually seen with ESA's.

Answer 246

Is extensive when anemia is mild and red cell indices are normal. Anemia of chronic inflammation (ACI) is the most common. Alternative diagnosis followed by anemia of renal failure and endocrine disorders. Most important differential diagnosis is ACI and thalassemia.

Answer 247

In anemia of chronic disease (ACD) serum iron and TIBC will be low with a normal or increased ferritin. Thalassemia may have a positive family history, splenomegaly, target cells and tear drop cells on peripheral smear, normal iron and ferritin levels and normal RBC.

Answer 248

Ferrous sulfate 325mg po three times a day. It is cheap and effective. GI side effects decrease compliance-NVD, black stools.Therapy is usually 4 to 6 months. Liquid preparations may stain teeth. Space doses if taking TCN, flouroquinolones, levothyroxine, vit E and cholestyramine. Transfusions are reserved for the hemodynamically unstable.

Answer 249

Ferrous salts are absorbed better than ferric. Ascorbic acid increases absorption and toxicity. Iron is absorbed best on an empty stomach. Iron should not be given with antacids. Iron polysaccharide complex (Niferex) seems to be better tolerated than other iron salts.

Answer 250

Iron dextran, ferric gluconate complex (Ferrlecit), iron sucrose (Venofer), Ferumoxytol (Feraheme)

Answer 251

Pica or restless leg syndrome usually disappear immediately upon treatment. Moderate anemia will see results in 7-10 days. Failure to respond could mean: Incorrect diagnosis, non-compliance, co-morbidity, underdose or malabsorption.

Answer 252

Decreased stimulation of RBC production in bone marrow, (Beta thalassemia, Anemia of chronic disease, Chronic renal insufficiency). Isolated decrease in RBC precursors (Red cell aplasia). Bone marrow damage, (Fibrosis Stem cell damage, infiltration with tumor/infection). Intrinsic bone marrow disease, (Myelodysplasia/sideroblastic anemia).

Answer 253

'Gastrointestinal sideropenia'= celiac disease, atrophic body gastritis, h. pylori infection, and gastric bypass surgery.

Answer 254

Iron travels in the bloodstream bound to transferrin. If there are more binding sites available, iron stores are low.

Answer 255

iron is stored in the spleen, liver & bone marrow as ferritin or hemosiderin. Serum ferritin measures the free iron in the serum.

Answer 256

Amount of iron in the blood. A low serum iron level and increased TIBC indicate iron deficiency.

Answer 257

Characterized by vaso-occlusive phenomena and hemolysis. Vaso-occlusion causes pain crises and organ dysfunction leading to life long disabilities and early death. Causes a very poor quality of life.

Answer 258

Homozygous genetic disease of varying severity

Answer 259

Results from a substitution of a valine for glutamic acid as the sixth amino acid of the globulin chain that creates a tetramer of hemoglobin that is poorly soluble when deoxygenated

Answer 260

cell membrane structure changes, it adheres to endothelium better than normal blood cells, the sickle shape cannot squish through vessels

Answer 261

Sickle cell anemia describes the homozygous Hb S trait. It is most severe for homozygous Hb S. Intermediate intensity for Hb SC disease. Benign for those who have the trait. When Thalassemia co-exists the anemia is less severe.

Answer 262

Dactylitis- pain in hands and feet that is excruciating. Waxing and waning of acute pain on top of chronic pain.

Answer 263

Spleen sequestration- acute painful enlargement of the spleen due to intrasplenic trapping of RBC’s. An emergency- hypovolemic shock can occur. Hemoglobin levels will drop by 2.

Answer 264

May be precipitated by weather, wind, low barometric pressure, pollutants, menses, illness, dehydration, stress, alcohol consumption and infection. Pain episodes last 2-7 days typically.

Answer 265

Therapy is affected by opioid clearance, hyperalgesia and pseudo addiction

Answer 266

24% of patients will have an overt stroke by age 45, 25% of children will have ischemic lesions that will impair cognitive function. As they age there is greater risk of neurocognitive decline and intracranial hemorrhage. Epilepsy is 2-3 times more common. Psychosocial issues and depression. Delayed puberty and growth development are seen. Reduced or absent spleen function due to splenic infarction.

Answer 267

May be reversed by prompt reverse transfusion therapy

Answer 268

Especially encapsulated organisms like Strep. Pneumoniae and H. influenza. Parvovirus and H1N1 are more virulent in this population. Bacteremia can occur by Strep pneumo that has 20-50% mortality. Occurs less often in adults. Prophylactic antibiotics are given.

Answer 269

Meningitis (infants and children more commonly), bacterial pneumonia. acute chest syndrome, osteomyelitis, vasooclusion causes leg ulcers, priapism, pulmonary complications are the most common causes of death. Renal failure, retinopathy, MI, increased cardiac output (heart failure).

Answer 270

Acute hepatic ischemia, benign cholestasis, hepatic sequestration crisis, transfusional iron overload, acute and chronic cholelithiasis secondary to pigmented gallstones, acute and chronic liver disease secondary to hepatitis C virus infection (HCV) complicating blood transfusion, drug toxicity.

Answer 271

Anemia with reticulocytosis, hyperbilirubinemia, elevated LDH, low haptoglobin, blood smear reveals sickled cells that are normochromic and normocytic.

Answer 272

preventative care with hematologist and internist, prophylactic antibiotics (penicillin at least until age 5), vaccines (pneumovax and flu), transfusion for stroke risk. Hydroxyurea is used to reduce Hb polymerization.Chronic transfusion is the treatment for CNS injury like a stroke. Morphine and methadone for pain.

Answer 273

Folic acid, Vit D, MVI without iron and calcium

Answer 274

HCT has been used in patients under 16 years of age and is the only cure. Patient education of the signs and symptoms of complications. Fever needs to be treated as a medical emergency. Genetic counseling. Avoid anything that stimulates bone marrow. Maintain adequate oxygen saturations. Monitor BP, retinal changes, renal function, depression, skin for ulcers and pain control.

Answer 275

Has improved from a fatal childhood illness to a chronic disease with progressive deterioration for those with access to comprehensive care The median age at death for those with homozygous sickle Hgb (HgbSS) was 42 years for men and 48 years for women.The median age at death for those with HgbSC disease was 60 years for men and 68 years for women. (this was before hydroxyurea was used).

Answer 276

Is made with DNA testing, screening helps to identify while in utero, heel blood is used to test for sickle cell and thalassemia at birth, sickled cells are seen on blood smear. It iIs diagnosed in childhood.

Answer 277

A genetic disorder where a there is a reduction in one of the globin chains of hemoglobin. May be a problem of the alpha or beta portion of the globin. It is a homozygous disease. Defect can be alpha or beta chain with beta occurring more commonly, (Beta chains are reduced but alpha stays the same and therefore accumulate in RBC and shortens its life cycle, Hypochromic microcytic). Confirmed by electrophoresis of Hb. Minor disease can be misdiagnosed as iron deficiency anemia.

Answer 278

May have minor or major manifestations. | Usually apparent in infancy – pale, listless, poor appetite, slow growth, jaundice. Enlarged heart, spleen and liver.

Answer 279

Thalassemia minor can be asymptomatic. If the patient is heterozygous the anemia is mild and therefore termed thalassemia minor.

Answer 280

If the patient is homozygous the anemia is chronic and severe and is termed thalassemia major and it is usually fatal in childhood

Answer 281

Symptoms start at 6 months of agem diagnosis occurs 6-12 mos-Chronic anemia, hemolysis –profound, noxious effects of massive ineffective erythropoiesis, pallor, irritability growth retardation, hepatosplenomegaly – abdominal swelling, jaundice. Major symptoms: Skeletal changes are profound (chipmunk face, ribs and bones become box-like), Liver and gall bladder problems (hepatospenomegaly, gall stones) and kidneys are enlarged and urine is dark, Endocrine symptoms (growth problems hypothyroidism and gonadism), Cardiac problems (heart failure, arrhhythmias), aplastic crisis (parvovirus), and pain!

Answer 282

Profound hypochromic microcytic anemia, Bizarre red cell morphology, WBC strikingly high, reticulocyte level low, PLT normal, Iron levels are high, Ferritin is high, Serum is icteric (yellow), bone marrow shows erythroid hyperplasia.

Answer 283

80% of children will die in the first 5 years- | Manifested by organ damage from iron overload

Answer 284

Spleenectomy , Iron chelation, Folic acid supplement, Hypertransfusion.

Answer 285

Thalassemia A major= still birth, major, incompatible with life.Thalassemia A minor= only a carrier, you have no symptoms.Thalassemia B major= the only ones you will treat. Thalassemia B minor=only carriers, no symptoms.

Answer 286

Myeloid neoplasms- derived from bone marrow progenitors that are restricted to developing into erythrocytes, granulocytes (neutrophils, basophils, and eosinophils), monocytes, or megakaryocytes.

Answer 287

granulocyte precursor

Answer 288

Acute myeloid leukemias, myeloproliferative neoplasms, and myelodysplastic syndromes.

Answer 289

AML is very aggressive and requires immediate attention-Defined as >20% myeloid blasts in the bone marrow or peripheral blood.

Answer 290

It is a solid tumor composed of immature white blood cells called myeloblasts. A chloroma is an extramedullary manifestation of acute myeloid leukemia; in other words, it is a solid collection of leukemic cells occurring outside of the bone marrow.

Answer 291

AML with myelodysplasia-related changes (Myelodysplasia=in which the normal process of making mature blood cells (red blood cells, white blood cells, and platelets) – known as hematopoiesis – is impaired).

Answer 292

heterogeneous group of clonal hematopoietic stem cell disorders that are directly related to previous cytotoxic chemotherapy and/or radiation therapy.

Answer 293

Two predominant and clinically significant types of therapy-related myeloid neoplasms (t-MN) have been defined, those arising after treatment with alkylating chemotherapy and/or radiation therapy and those arising after therapy with topoisomerase II inhibitors.

Answer 294

AML with translocations between chromosome 8 and 21, AML with inversions in chromosome 16, AML with translocations between chromosome 15 and 17 and AML with translocations in chromosomes 9 and 11. There are also myeloid proliferations related to down syndrome.

Answer 295

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination.

Answer 296

erythrocytes

Answer 297

basophils, eosinophils and neutrophils (these are all granulocytes, a type of leukocyte)

Answer 298

lymphocyte (agranulocyte, a type of leukocyte)

Answer 299

monocyte (agranulocyte, a type of leukocyte)

Answer 300

thrombocyte

Answer 301

Chronic myeloid leukemia (CML) — CML demonstrates overproliferation of the granulocytic lineage at all stages of maturation.

Answer 302

It is always associated with the presence of a BCR-ABL fusion gene, which is usually created by a reciprocal translocation that results in the formation of the Philadelphia chromosome. It is reported as Ph chromosome +. It does affect treatment.

Answer 303

It refers to disorders that exhibit dysplasia and ineffective blood cell production with a variable risk of transformation to acute leukemia. It is a precursor to leukemia.

Answer 304

Bone marrow cellularity is often increased, but may be normocellular or hypocellular. The percentage of blasts in the bone marrow is normal or increased, but less than 20 percent.

Answer 305

Maturation is present, but dysplasia is noted in one or more myeloid lineages. Hematopoiesis is ineffective and results in cytopenias.

Answer 306

``` T lymphocytes (cytotoxic T lymphocytes, helper T lymphocytes, or regulatory T lymphocytes) and B lymphocytes (lymphocytes or plasma cells). In general, the lymphoid neoplasms are divided into neoplasms derived from lymphoid precursors and neoplasms of mature lymphocytes and plasma cells. ```

Answer 307

Historically, lymphoid neoplasms that present with bone marrow and blood involvement (leukemia) have been segregated from those that present as a mass (lymphoma).

Answer 308

not on the anatomic location of tumor cells, but rather on the cell of origin of the tumor.

Answer 309

Indolent, aggressive and highly aggressive. These are all uncommon.

Answer 310

Survival of untreated indolent lymphoid neoplasms is generally measured in years. Indolent lymphomas represent 35 to 40 percent of the non-Hodgkin lymphomas (NHLs).

Answer 311

Survival of untreated aggressive lymphoid neoplasms is usually measured in months. About half of the NHLs are aggressive.

Answer 312

Survival of untreated highly aggressive lymphoid neoplasms is measured in weeks.The highly aggressive lymphomas can arise from B cells or T cells.

Answer 313

The precursor B lymphoblastic leukemia/lymphomas are neoplastic disorders of immature lymphoblasts committed to the B cell lineage. The precursor T lymphoblastic leukemia/lymphomas are neoplastic disorders of immature lymphoblasts committed to the T cell lineage that may arise within the thymus or the bone marrow.

Answer 314

A mature B cell neoplasm, chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL), — is the most common leukemia in Western countries, accounting for approximately 30 percent of all leukemias in the United States.

Answer 315

A type of b cell neoplasm, chronic lymphocytic leukemia/small lymphocytic lymphoma

Answer 316

— comprises about 7 percent of adult NHLs in the United States and Europe.

Answer 317

Follicular lymphoma (FL) — is the second most common lymphoma in the United States and Western Europe. Patients usually present with painless peripheral adenopathy.

Answer 318

Hodgkin’s lymphoma- formerly called Hodgkin's disease, arises from germinal center or post-germinal center B cells. HL has a unique cellular composition, containing a minority of neoplastic cells (Reed-Sternberg cells and their variants) in an inflammatory background.

Answer 319

Hodgkin lymphoma, although a lymphoid malignancy, is pathologically and clinically distinct from the other lymphoid neoplasms and is considered a distinct entity with a generally excellent prognosis

Answer 320

A significant minority of cases of classical HL are EBV-associated, particularly the lymphocyte depleted and mixed cellularity types

Answer 321

nodular lymphocyte-predominant HL is never EBV-associated

Answer 322

There are numerous cutaneous T cell lymphomas. Mycosis fungoides (MF) is the most common of these. Sézary syndrome is an erythrodermic, leukemic variant of MF.

Answer 323

Refers to a neoplasm of hematopoietic tissue as opposed to a solid tumor

Answer 324

Leukemic cells infiltrate the bone marrow and lymphoid tissue and spill into the bloodstream and infiltrate the bodies organs.

Answer 325

The cells may be mature or immature.

Answer 326

The overproduction of WBC’s can be seen on CBC, unless disease is confined to the bone marrow.

Answer 327

Classified according to cell type and maturity

Answer 328

Any cell can cause leukemia, but it is usually granulocytic, monocytic or lymphocytic, (there are subtypes based on cell membrane characteristics and genotype).

Answer 329

acute; chronic

Answer 330

In chronic granulocytic leukemia most of the circulating cells are maturing granulocytes and neutrophils but there are few primitive cells.

Answer 331

In most cases the WBC will be profoundly elevated (30).

Answer 332

Aleukemic leukemia is when the disease is confined to the bone marrow and blood counts appear normal.

Answer 333

Because the leukemic cells crowd out the bone marrow there is anemia due to ineffective erythopoiesis, infection and bleeding from thrombocytopenia.

Answer 334

Leukemic cells make it to the bone, liver, spleen and lymph nodes.

Answer 335

Chronic disease proceeds at a slow pace and can be well controlled for long periods of time.

Answer 336

rapidly progressive

Answer 337

the disease relapses and proves fatal

Answer 338

Philadelphia + cancers can be successfully treated with tyrosine kinase inhibitors.

Answer 339

Your own cells are collected during remission for you to use later if you need them. It can be used if there is no match. Patients cells are collected and treated with leukemia killing antibodies.

Answer 340

Less chance of GVHD more chance of relapse

Answer 341

More of a chance of GVHD less chance of relapse. All cells must be destroyed by chemotherapy.

Answer 342

Graft vs host disease (GVHD)- the donor lymphocytes attack the hosts cells (transplant rejects the patient)

Answer 343

Transplants are not always successful. Infection | and a relapse could occur if all cells were not destroyed.

Answer 344

New cells transfused and “move in” to the marrow

Answer 345

Immunosuppression is necessary only in allogenic

Answer 346

leukemia; may take several months to several years for leukemia to appear

Answer 347

Moderate leukopenia, thrombocytopenia, anemia

Answer 348

No specific treatment, watch and wait.

Answer 349

bone marrow

Answer 350

May form discrete tumors that weaken the bone and cause spontaneous fractures. Lytic lesions, "CRAB" (calcium, renal, anemia, bone)

Answer 351

Ca, Scr, and CBC

Answer 352

Neoplastic cells cause large amounts of blood protein to increase and therefore blood viscosity.

Answer 353

Protein is usually IgG, proteins can accumulate in tissues and organs and block function. Protein is usually IgG. Proteins can accumulate in tissues and organs and block function.

Answer 354

This is a chronic disease that will wax and wane

Answer 355

BMT- improves survival but does not cure the disease. Thalidomide (remember toxicities) suppresses blood vessel proliferation that support myeloma cells. bortezomib inj. Watch if dose is SC or IV localized radiation to affected bone.

Answer 356

asymptomatic period before myeloma presents, lasts years, Happens in adults over 65, patients may not live long enough for the disease to appear

Answer 357

asymptomatic period before myeloma presents, it happens in adults over 65, some patients may not live long enough for the disease to appear

Answer 358

Malignant neoplasms are the leading cause of disability and death. Cancer is second only to heart disease for US cause of death (23%). Lung in men and breast in women are the most common solid tumors. Survival depends on early diagnosis and treatment before the disease has spread (metastasis).

Answer 359

Curability is assessed in terms of 5 year survival rates 95% for thyroid cancer, 5% for pancreatic cancer

Answer 360

Breast and melanoma, this means the disease “hides” and is kept at bay until the immune system cannot contain it anymore

Answer 361

A complete blood count with differential examination of the peripheral smear immunophenotypic analysis of the circulating lymphocytes. Bone marrow aspirate and biopsy and lymph node biopsy are not among the required elements of the diagnostic work-up. Chromosomal changes seen in CLL are also not diagnostic features of the disease

Answer 362

chronic lymphoproliferative disorder (lymphoid neoplasm) characterized by a progressive accumulation of functionally incompetent lymphocytes, which are monoclonal in origin.

Answer 363

The disorder is considered to be identical (i.e., one disease at different stages) to the mature B cell neoplasm small lymphocytic lymphoma

Answer 364

lymphocytosis

Answer 365

The smear of chonic lymphocytic anemia. It often contains "smudge" cells that are lymphocytes that appear to have been flattened or smudged in the process of being spread on the glass slide.

Answer 366

Immunophenotypic analysis, usually by flow cytometry, is a key component to the diagnosis of CLL

Answer 367

lymphocytosis

Answer 368

The smear contains "smudge" cells that are lymphocytes that appear to have been flattened or smudged in the process of being spread on the glass slide

Answer 369

Expression of B cell associated antigens including CD19, CD20, and CD23.

Answer 370

Expression of CD20 is usually weak. Expression of CD21 and CD24 can be seen, but is not required for diagnosis. There is also expression of CD5, a T cell associated antigen.

Answer 371

Absolute B lymphocyte count in the peripheral blood ≥5000/microL. Demonstration of clonality of the circulating B lymphocytes by flow cytometry of the peripheral blood. Extremely low levels of SmIg. Expression of B cell associated antigens (CD19, CD20, and CD23); and expression of the T cell associated antigen CD5.

Answer 372

Lymphocytosis rises concern over CLL as well as: | Mononucleosis, pertussis, toxoplasmosis and other neoplasms. Flow cytometry confirms the diagnosis.

Answer 373

Not all patients require treatment at the time of diagnosis. CLL is an extremely heterogeneous disease with certain subsets of patients having survival rates w/o treatment that is similar to the normal population. With the possible exception of allogeneic hematopoietic cell transplantation (HCT), CLL cannot be cured by current treatment options.

Answer 374

Prospective, randomized trials evaluating immediate versus delayed treatment strategies have found no improvement in long-term survival with early treatment. Spontaneous regression of variable duration is a rare occurrence.

Answer 375

Asymptomatic early disease Watch and wait. No proven benefit for early treatment although this waiting period may be emotionally stressful for the patient

Hematology Exam 1 Flashcards

(412 cards)