Hematologic and oncologic disorders

Question 1

Normal lab values for RBCs

Answer 1

RBCs: 4-6
MCV: 80-100
-Microcytic <80
-Normocytic 80-100
-Macrocytic >80
MCHC: 32%-36%
MCH: 26-34

Question 2

Reticulocyte count

Answer 2

Immature red blood cells
Normal: 0.5-2.5
High: Erythropoeitc response to anemia and probable blood loss of hemolysis.
Low: Anemia of marrow failure, most common cause of anemia.

Question 3

Mean Corpuscular Volume (MCV)

Answer 3

Espression of the average volume and size of individual erythrocytes.
Normal: 80-100

Question 4

Total iron binding capacity (TIBC)

Answer 4

Normal 250-450
>450 indicates low levels of iron in the blood.
<250 indicates high levels of iron in the blood.

Question 5

Mean corpuscular hemoglobin conentration (MCHC)

Answer 5

Normal: 32%-36%
-Expression of the average hgb concentration or proportion of each RBC occupied by hgb as a percentage.
-More accurate measure than MCH
-How red the blood cell is.
-Low: less hgb=less red
-High: high hgb=more red

Question 6

Microcytic, hypochromic anemias

Answer 6

Iron deficiency anemia & Thalassemia

Question 7

Iron deficiency anemia

Answer 7

Microcytic, hypochromic anemia due to overall deficiency of iron.
Most common cause of anemia (excpet elderly pts which would be anemia of chronic disease)
Caused by: blood loss, inadequate iron intake, impaired absorption of iron.
**S/S: **Usually slow in onset and few symtpoms are seen with hct >30, but as hct falls will see pica, dyspnea, headache, palpitations, weakness, tachycardia, postural hypotension, pallor.
Labs/diagnostics: Low MCV, low MCHC, low ferritin, low serum iron, low hgb/hct, low RBCs, high TIBC, high RDW
Management: Oral ferrous sulfate (300-325mg) 1-2 hours after meals (food decreased iron absorption by 50%).
-Iron shouldn’t be taken with antacids as they decreased absorption
-Iron taken with Vitamin C increases absorption

Question 8

Thalassemia

Answer 8

Genetically inherited disorders resulting in abnormal hgb production and microcytic, hypochromic anemia.
Found mainly in mediterranean, african, middle eastern, indian and asian populations. Asians with anemia, think thalassemia
**S/S: **general physical findings are unremarkable unless severe thalassemia.
-Beta thalassemia is the most familiar type of thalassemia (reduced or absent production of beta globin chains),
Two forms of beta thalassemia: Minor and major
-Thalassemia minor: Most common. Heterozygous (underproduced beta chains) for beta thalassemia. Minor anemia.
-Thalassemia major: Homozygous (zero beta chains) for beta thalassemia. More severe anemia.
**Labs/diagnostics: **low hgb, low MCV, low MCHC, normal TIBC, normal ferritin.
Management: No treatment for mild to moderate forms. RBC transfusion/splenectomy for more severe forms.
-Iron is contraindicated as iron overload can result.

Question 9

Macrocytic, normochomic anemia

Answer 9

Folic acid deficiency, pernicious anemia, B12 deficiency

Question 10

Folic acid deficiency

Answer 10

A macrocytic, normochromic anemia due to folic acid deficiency (<2). Inadequate intake/malabsorption of folic acid.
S/S: fatigue, dyspnea on exertion, pallor, HA, tachycardia, anorexia, glossitis. No neurological signs, which diffierentiates this from B12 deficiency
Lab/diagnostics: Hgb/hct/RBC low, serum folate low, MCV elevated, MCHC normal.
Management: Folate 1mg daily (always give B12 due to potential neurological symptoms)

Could be medication induced by methotrexate, alcohol, phenytoin, oral contraceptives, isoniazid

Question 11

Pernicious anemia

Answer 11

A macrocytic, normochromic anemia due to deficiency of intrinsic factor(produced by parietal cells in stomach), which results in malabsorption of B12. Autoimmune inflammation in stomach that affects the inability to absorb B12.
**S/S: **Weakness, glossitis, palpitation, dizziness, anorexia, POSITIVE NEURO FINDINGS: paresthesia, loss of vibratory sense, loss of fine motor control, positive romberg and babinski signs.
Lab/diagnostics: Low hgb/hct/RBCs, MCV increased, MCHC normal, low serum B12 (<200), Anti IF and antiparietal cell antibody test is used to affirm a defficiency.

Management: B12 100mcg IM daily x1 week. Maintenance therapy requires continuous lifelong monthly administration. (Watch for hypokalemia)

Question 12

Anemia of chronic disease

Answer 12

Chronic normocytic, normochromic anemia associated with chronic inflammation, infection, renal failure and malignancy. Most common in elderly. 2nd most common cause of anemia.
**S/S: **Fatigue, weakness, dyspnea on exertion, anorexia.
Lab/diagnostics: Low hgb and hct, low serum iron and TIBC, MCV and MCHC normal, high ferritin.
Management: Treat associated disease and provide nutritional support.
Erythropoetin for pts with chronic diseases such as kidney failure or RA.

Question 13

Sickle cell anemia

Answer 13

Chronic hemolytic anemia that is genetically transmitted. Typically african americans.
A crisis is periodic exacerbations of vessel obstructions. Cellular hypoxia results in acidosis and tissue ischemia. Pain occurs from tissue ischemia and blood hyperviscocity. Factors which precipitate sickling include hypoxia, infections, high altitudes, dehydration, physical or emotional stress, surgery, blood loss and acidosis.
S/S: Signs of disease develop in infancy or childhood. Deleyed growth development, increased susceptibility to infections. In crisis pts experience: sudden onset of severe pain in extremities/back/chest/abdomen, aching joints, weakness, dyspnea.
Lab/diagnostics: low hgb, peripheral smear shows classic distorted sickle shaped RBCs.
**Management: **Treat both acute and chronic complications of the disease. For acute exacerbation give fluids for dehydration, pain meds, and oxygen for hypoxia.

Question 14

Von Willebrand Disease

Answer 14

A genetic disorder that results in the reduced ability to create blood clots. Caused by mutation of deficiency in von Willebrand factor and clotting factor 8.
S/S: Frequent, prolonged or severe episodes of bleeding. Easy bruising.
Management: Desmopressin (DDAVP).

Question 15

Idiopathic thrombocytopenia purpura (ITP)

Answer 15

Thrombocytopenia resulting from autoimmune destruction of platelets with or without suppression of thrombopoiesis.
Normal platelets 150-400
S/S: in adults, ITP is usually chronic and causes mild to severe thrombocytopenia that my be asymptomatic.
Thrombocytopenia may be incuded by: Heparin, sulfonamides, thiazides, quinine, zosyn, gold.
Lab/diagnostics: Bone marrow analysis, low platelet count with other causes of thrombocytopenia ruled out.There may be a history of easy brusing and bleeding (gums and kidneys bleed first).
Management: May not be needed until platelets are <20,000.
-IV steroids and IV gammaglobulin increase platelet count. Platelet transfuion may be beneficial.
IV gammaglobulin is prefered over steroids in HIV-ITP
Splenectomy is highly effective

Question 16

Heparin induced thrombocytopenia (HIT)

Answer 16

Thrombocytopenia induced by heparin. Causes arterial and venous thrombosis.
Most common type of drug induced thrombocytopenia and most common cause of thrombocytopenia in hospitalized patients. Occurs more frequently in srugical patients due to recieving heparin pre and post surgery.
S/S: Often initially asymptomatic. May develop symptoms related to arterial or venous thrombosis. Skin necrosis at injection site. Anaphylactic reaction after administration.
Lab/diagnostics: Suspect HIT if platlets fall >50% from previous value.
-Heparin-induced platelet aggregation assay along with serotonin release assay is considered HIT testing reference standard.
Management: Early recognition is key due to high risk of thrombosis.
-Direct thrombin inhibitors used for substitution for anticoagulants: Argatroban, apixaban

Question 17

Disseminated intravascular coagulation (DIC)

Answer 17

Acquired coagulation disorder, with hemorrhage and thrombosis, that occurs as a secondary process concomitant with a pathphysiologic disease or clinical state such as malignant neoplasms, infections/sepsis, liver disease, massive trauma, extensive burns, shock, OB complications, acute leukemia.
High mortality rate.
S/S: DIC varies greatly in clinical severity and usually demonstrated disturbance in hemostasis with ecchymosis, oozing from venipuncture sites and petechiae.
**Lab/diagnostics: **thrombocytopenia, low fibrinogen, low RBCs, increased fibrin degradation products (FDPs), prolonged PT/PTT and positive d-dimer (most sensitive).
Management: diagnose and correct underlying cause. PRBCs, platelet transfusion, cryo (to maintain fibrinogen levels), plasma.
Goal of therapy is cessation of bleeding, increasing plasma fibrinogen and the platelet count, and decreasing fibrin degredation products (FDPs).

Question 18

Acute lymphocytic leukemia (ALL)

Answer 18

Malignancy that causes hematopoietic cells to lose their ability to mature normally and differentiate. The cells proliferate in uncontrolled fashion and ultimately replace normal bone marrow, leading to decreased production of normal RBCs, WBCs, and platelets.
-More common in males.
-Circulatory blasts are hallmark of disease.
Confirm diagnosis with bone marrow biopsy.
Bone pain d/t expanding bone mass.

Question 19

Leukemias

Answer 19

Neoplasms arising from hematopoietic cells in the bone marrow, causing anemia, low platelets and low WBCs.
More common in males.
**S/S: **May be asymptomatic, generalized lymphadenopathy, weight loss, anorexia, fatigue, weakness.
Lab/diagnostics: CBC with subnormal RBCs and neutrophils, ESR, bone marrow aspirate to confirm diagnosis.
Management: Chemotherapy (initiate allopurinol to reduce tumor lysis syndrome), bone marrow transplant, and control of symptoms.

Question 20

Acute myelogenous leukemia (AML)

Answer 20

Malignancy of hematopoietic cells. Increased risk of developing after chemo or radiation.
-Presence of Auer rods

Question 21

Chronic lymphocytic leukemia (CLL)

Answer 21

A chronic leukemia characterized by abnormal B lmphocytes and often generalized lymphadenopathy.
Most common leukemia in adults.
-Lymphocytosis is hallmark of disease (usual range is 40,000-150,000).
-Lymphadenopathy present

Question 22

Chronic myeloid leukemia (CML)

Answer 22

Distinguished by the presence of Philadelphia chromosomes in the leukemic cells and elevated WBCs (hallmark of disease)

Question 23

Lymphoma

Answer 23

Lymphocytic malignancy. Diagnosed by biopsy of enlarged lymph nodes.
Staging:
1. Disease loaclized to singly lymph node.
2. More than one lymph node group involved on same side of diaphragm.
3. Lymph nodes or the spleen involved on both sides of diaphragm
4. Liver or bone marrow involvement.

Types: Non-hodgkins lymphoma (NHL) and Hodgkinds lymphoma (HL)

Because lymphomas can be extensive at diagnosis, associated oncological emergencies (superioir vena cava syndrome, tumor lysis syndrome, and cardiac tamponade) should be anticipated, diagnosed and treated

Question 24

Non-Hodgkins lymphoma (NHL)

Answer 24

Malignant cells form in the lymph system. Lacks reed-sternbern cells.
Cause is unknown but may have a viral etiology.
S/S: Often present with lymphadenopathy, typically painless, widespread or isolated. Group A and B symptoms with abdominal fullness, skin ulcers, splenomegaly, hepatomegaly.
Less predictable pattern spread than Hodgkins lymphoma.
Advanced stage disease is usually apparent.
Lab/diagnostics: CT, x-rays, US, MRI to located and stage disease.
-Tissue biopsy of largest node or accessible involved organ is required, but not definitive in diagnosis.
**Management: **Radiation, chemo, bone marrow transplant.

Question 25

Hodgkins Lymphoma (HL)

Answer 25

A group of cancers characterized by the presence of reed-sternberg cells.
S/S: Manifests as painless enlarged lymph nodes in the upper body. May have no symptoms (Group A) or fever, night sweats, weight loss, pruritus, fatigue (group B).
-Usually presents with cervical adenopathy and spreads in a predictable fashion along lymph node groups.
Labs/diagnostics: Hall amrk is presence of Reed-Sternberg cells on lymph node biopsy. Excisional biopsy required, no needle aspirate.

Question 26

Tumor lysis syndrome (TLS)

Answer 26

Oncologic emergency in which rapid destruction of tumor cells ead to electrolyte abnormalities such as hypocalcemia, hyperkalemia and hyperphosphatemia and hyperuricemia. If left untreated it can progress to renal failure and subsequent multiorgan failure.
Typically seen with initiation of chemo.
S/S: due to metabolic abnormalitites.
Management:: Aggressive IV hydration to improve renal perfusion and GFR from rumor cell destruction.
Uric acid inhibitors (allipurinol)

Question 27

Superioir vena cava syndrome

Answer 27

Any obstruction of blood flow through SVC. In oncological setting frequently caused by tumor obstruction or direct invasion of SVC. In malignant setting most frequetly associated with lung cancer or NHL.
S/S: chest pain, dyspnea, stridor, cough, dysphagia, face and neck swelling, sycope, dilated veins in chest/neck/arms.
-May see mediastinal enlargement, malignant pleural effusion.
**Management: **treatment of underlying malignancy.

Question 28

Lung cancer

Answer 28

Leading cause of cancer death in U.S.
Non-small cell lung cancers (NSCLC, most common form) can grow slowly and be asymptomatic. Having symptoms signifies advanced disease S/S: persistent coughing, hemoptysis chest pain or painful breathing, difficulty breathing or SOB, fatigue, hoarness or wheezing, frequent upper respiratory infections, bone pain.
Lab/diagnostics: only definitive test is biopsy, Chest x-ray presentation varies with cell type, CT of chest to facilitate staging, CMP to check for SIADH and hypercalcemia.

Question 29

Coagulation labs

Answer 29

Prothrombin time (PT): 10-14
Partial thrombin time (PTT): 60-70
Activated partial thrombin time (aPTT): 30-40
Platelets: 150,000-440,000