Robbins CH 14 RBCs

Question 1

What does basophilic stippling of RBCs suggest?

Answer 1

A marrow injury–drug/toxin

Question 2

Hypochromic RBCs are seen with what 2 disorders?

Answer 2

Iron deficiency and Thalassemias

Associated with reduced Hb synthesis

Question 3

What do Schistocytes suggests?

Answer 3

Microangiopathic hemolytic anemia–shock/sepsis

Question 4

What is seen with acute and chronic blood loss?

Answer 4

Reticulocytosis and marrow hyperplasia

Marrow responding to a decrease in RBCs

Question 5

Very hypo cellular marrow and unable to respond to anemia–associated with pancytopenia

Answer 5

Aplastic marrow

Question 6

What does the Coombs test, test for?

Answer 6

Testing for autoimmune hemolytic anemia

Question 7

Impairs the ability of the marrow to mount a significant and sustained reticulocytosis. Typically microcytic and hypo chromic

Answer 7

Iron deficiency anemia

Question 8

What is haptoglobin

Answer 8

Serum protein that binds to free Hb

Question 9

How can SLE cause decreased haptoglobin levels?

Answer 9

SLE can result in hemolysis which causes a decrease in haptoglobin

Question 10

What does an elevated D-dimer suggest?

Answer 10

microangiopathic hemolytic anemia

Question 11

A condition in which a mutation affects one of several membrane cytoskeletal proteins–important in maintaining RBCs shape (spectrin, ankyrin, band 4.2 and 3, Protein 4.1, glycophorin A). The RBCs lack central pallor on peripheral blood smear…

Answer 11

Hereditary spherocytosis

Question 12

What is suggested by the lack of central pallor on blood smear of RBCs?

Answer 12

Hereditary spherocytosis

Question 13

What disease may lead to an aplastic crisis precipitated by a parvovirus infection?

Answer 13

Hereditary spheocytosis

Question 14

What is the treatment of hereditary spheocytosis?

Answer 14

Splenectomy–beneficial because the spherocytes are no longer detained by the spleen

Question 15

What are the 3 characteristics of disseminated intravascular coagulation?

Answer 15

Gives rise to thrombocytopenia
Bleeding
Appearance of fragmented RBCs on blood smear

Question 16

B-thalassemia major causes a-globin chain aggregation causing apoptosis. This leads to anemia and iron overload. What can the iron overload cause?

Answer 16

Results in hemochromatosis with infiltrative cardiomyopathy, hepatic cirrhosis, and bronze diabetes from pancreatic islet dysfunction

Question 17

Why are maxillofacial deformities seen with B-thalassemia major?

Answer 17

Severe anemia–increases EPO production which leads to the expansion of the marrow

Question 18

What group of individuals has the highest incidence of developing B-thalassemia major?

Answer 18

patients from mediterranean descent

Question 19

How can sickle cell anemia lead to a risk for infection with encapsulated bacterial organisms?

Answer 19

Cumulative ischemic damage to the spleen results in autosplenectomy – impaired splenic function and resultant inability to clear bacteria from the bloodstream can occur in childhood

Question 20

What individuals are most likely to have alpha-thalassemia major?

Answer 20

southeast Asian ancestry

Question 21

What is alpha-thalaseemia major?

Answer 21

Complete lack of a-globin chains precludes formation of Hb A1, A2 and F

Only tetramer of gamma chains (Bart’s Hb) en be made–severe fetal anemia

Question 22

Sensitivity to oxidizing agents causing a hemolytic anemia–X-linked disorder that affects about 10% of african-american males

Answer 22

G6PD deficiency

Question 23

What are the drugs that subject Hb to damage by oxidants seen with G6PD deficiency?

Answer 23

Primaquine
Sulfonamides
Nitrofurantioin
Phenacetin
Aspirin

Question 24

What are bite cells?

Answer 24

Result from the attempts of overeager splenic macrophages to pluck out the heinz bodies–addingto the hemolysis seen with G6PD deficiency

Question 25

Abnormal HFE gene...

Answer 25

seen in hereditary hemochromatosis--iron overload

Question 26

What is the mechanism of action of hydroxyurea therapy given for sickle cell anemia?

Answer 26

increases the concentration of HbF in RBCs Interferes with polymerization of HbS

Question 27

How is B-thalassemia minor and iron deficiency anemia differentiated since both are characterized by hypo chromic and microcytic RBCs?

Answer 27

With iron deficiency anemia there is no increase in HbA2 and there would a a decreased ferritin level as well

Question 28

Anemia of chronic disease is associated with an increase in____.

Answer 28

serum ferritin

Question 29

Disorder that results from an acquired stem cell membrane defect produced by a PIGA gene mutation that prevents the membrane expression of certain proteins that require a glycolipid anchor?

Answer 29

Paroxysmal nocturnal hemoglobinuria (PNH)

Question 30

Complement distraction of RBCs, Granulocytes, and Platelets. Patient presents with hemoglobinuria (dark urine) is at an increased risk of venous thrombosis due to platelet defects and has recurrent infections caused by impaired leukocyte fucntions?

Answer 30

Paroxysmal nocturnal hemoglobinuria

Question 31

What 2 disorders by results from paroxysmal nocturnal hemoglobinuria?

Answer 31

Acute leukemia | Aplastic anemia

Question 32

Spectrin mutations...

Answer 32

Hereditary spherocytosis

Question 33

What cause the dark urine with G6PD deficiency?

Answer 33

Intravascular hemolysis

Question 34

Postive Coombs test

Answer 34

Indicates the presence of anti-RBC antibodies

Question 35

IgM binds RBCs at low temperature at peripheral body sites and fix complement--within increased temp the IgM is dissociated from the cell and leaves C3b behind--extravascular hemolysis of RBCs...

Answer 35

Cold agglutinin disease

Question 36

What are disorders/infections that can cause cold agglutinin disease?

Answer 36

``` Lymphoid neoplasms Mycoplasma EBV HIV Influenza CMV ```

Question 37

What phenomenon is seen with cold agglutinin disease?

Answer 37

Raynauds

Question 38

What lab values are increased in cold agglutinin disease and what causes this?

Answer 38

Increased MCV and Bilirubin caused by increased RBC turnover

Question 39

What immunoglobin is involved in warm antibody hemolytic anemia?

Answer 39

IgG--chronic and not triggered by cold

Question 40

Spherocytes are seen with paroxysmal nocturnal hemoglobinuria, what causes this?

Answer 40

Incrased osmotic fragility of RBCs

Question 41

Increased serum ferritin -mild and marked?

Answer 41

Anemia of chronic disease--mild increase Hemochromatosis-- marked increase

Question 42

What type of malaria are sickle cell trait ind. resistant to?

Answer 42

P. Falciparum

Question 43

What is erythroblastosis fetalis?

Answer 43

Maternal antibodies coating fetal RBCs--sensitization occurred during previous delivery--IgG made crossing placenta, attaching to fetal RBCs, causing hemolysis

Question 44

Immune-mediated mechanism caused, in many causes, by cephalosporin therapy?

Answer 44

Drug-induced hemolytic anemia

Question 45

What is hepcidin?

Answer 45

Liver-derived plasma peptide Iron absorption regulator

Question 46

When are hepcidin levels increased?

Answer 46

when iron stores are high

Question 47

When are hepcidin levels decreased?

Answer 47

hereditary and acquired hemochromatosis

Question 48

Iron transporter that moves nonheme iron from he gut lumen to the duodenal epithelium?

Answer 48

DMT-1

Question 49

What is hemosiderin?

Answer 49

Aggregated from of ferritin Does not circulate like ferritin and is found in tissue

Question 50

HFE gene mutation

Answer 50

Leads to excessive absorption of dietary iron and hemochromatosis

Question 51

What is transferrin?

Answer 51

Transports iron btw plasma, iron stores, and developing RBCs

Question 52

What are the characteristics of anemia of chronic disease?

Answer 52

Increased ferritin with reduced total iron-binding capacity

Question 53

What is the pathophysiology of anemia of chronic disease?

Answer 53

Chronic inflammatory or neoplastic disease increase the secretion of cytokines--IL-1, TNF, Interferon-g--promote sequestration of iron in storage compartments and depress EPO production

Question 54

Disease often seen with chronic alcohol abuse, Is a megaloblastic anemia. Often presents with neutrophils showing defective segmentation--extra nuclear lobes?

Answer 54

Folate deficiency

Question 55

What is indicated by tear drop RBCs?

Answer 55

Myelophthisic disorder--immature RBCs and WBCs and splenomegaly with extensive fibrosis

Question 56

What are the two best know causes of high MCV-- macrocytosis?

Answer 56

Vit B12 deficiency | Folate deficiency

Question 57

What are causes of aplastic anemia?

Answer 57

Exposures to chemo drugs or chemicals Viral infections--ALMOST NEVER Bacterial infection

Question 58

ADAMTS13 deficiency

Answer 58

accumulation of large von willebrand multimers--results in thrombotic thrombocytopenia purpura (TTP)

Question 59

Heat stroke caused by hyperthermia and loss of perspiration from dehydration leads to...

Answer 59

Hemoconcentration

Question 60

Can cause arterial and DVTs with increased risk of cerebral artery thrombosis. Patient often false positively tests for syphilis? A thrombocythopenia often present. Women with multiple miscarriages should be consider for this disease?

Answer 60

Antiphospholipid syndrome false + caused by anticardiolipin antibody

Question 61

With DIC what lab values are increased and what are decreased?

Answer 61

PT/PTT increased Thrombocytopenia Low fibrinogen concentration

Question 62

Helmet cells

Answer 62

Typical of conditions that den produce a microangiopathic hemolytic anemia--DIC, thrombocytopenia purpura, SLE, hemolytic-uremic syndrome, lamignant HTN

Question 63

Platelets destroyed in spleen after being coated with antibodies to platelet membrane glycoproteins IIb/IIIa or Ib-IX

Answer 63

Immune thrombocytopenic purpura (ITP)

Question 64

Does giving a blood transfusion to a patient with immune throbocytopenic purpura help?

Answer 64

No--antibodies coat both the patient's platelets and any transfused platelets

Question 65

What is the the treatment for immune throbocytopenic purpura if corticosteroid therapy fails?

Answer 65

Splenectomy

Question 66

Seen with Glanzmann thrombasthenia and Chronic immune throbocytopenic purpura?

Answer 66

Glycoprotein IIb/IIIa dysfunction/deficiency

Question 67

Von willebrand factor metalloproteinase deficiency is a feature of what disorder?

Answer 67

Thrombotic thrombocytopenia purpura

Question 68

What is the pathophysiology of heparin-induced thrombocytopenia?

Answer 68

Formation of IgG antibodies to heparin-platelet factor 4 complexes that bind to Fc receptors on the surface of platelets--causes platelet activation and thrombosis

Question 69

What isn't given if a patient present with a protein C deficiency?

Answer 69

Warfarin

Question 70

What is the classic pentad of Thrombotic thrombocytopenia purpura?

Answer 70

``` Transient neurologic problems Fever Thrombocytopenia Microangiopathic hemolytic anemia Acute renal failure ```

Question 71

The activity of what is decreased in thrombotic thrombocytopenia purpura?

Answer 71

ADAMTS13--which acts as a von willebrand factor multiuser protease

Question 72

Patients with liver disease have defects in what clotting factors?

Answer 72

Extrinsic pathway coagulation--causing changes in PT

Question 73

Inherited bleeding disorder with normal platelet count and prolonged bleeding time?

Answer 73

Von willebrand disease

Question 74

Which clotting factor will be decreased with von willebrand disease?

Answer 74

Factor VIII-because vWF acts as a carrier for this factor

Question 75

What is the pathophysiology of von willebrand disease?

Answer 75

Reduction in quantity of vWF impairing platelet adhesion to damaged vessel walls

Question 76

HUS is related to what?

Answer 76

preceding infectious gastroenteritis with diarrhea E. coli infection

Question 77

What clotting factor is decreased with Hemophilia A?

Answer 77

Decreased factor VIII activity

Question 78

What is the characteristic finding of hemophilia A?

Answer 78

Bleeding into soft tissue--joints

Question 79

If gone untreated what can hemophilia A lead to?

Answer 79

Hemathroses

Question 80

What lab value is prolonged due to hemophilia A?

Answer 80

PTT--intrinsic pathway affected

Question 81

If a patient with hemophilia A gets a transfusion to + factor VIII and their PTT is not corrected then what has occur?

Answer 81

An inhibitor of factor VIII is present If there is no inhibitor then the PTT will be corrected with transfusion

Question 82

What deficiency is involved in hemophilia B?

Answer 82

Factor IX deficiency

Question 83

A rare autosomal recessive disorder that involves defective platelet aggression from deficiency or dysfunction of glycoprotein IIb/IIIa?

Answer 83

Glanzmann thrombasthenia

Question 84

Caused by antibodies to platelet membrane glycoproteins IIb/IIIa or Ib/IX?

Answer 84

Immune thrombocytopenic purpura

Question 85

If a patient receives a platelet transfusion what are they more likely to contract?

Answer 85

More likely to cause sepsis due to the storage at room temp making platelets more susceptible to contamination

Question 86

Caused when donor plasma contains HLA or granulocyte-specific antibodies--granulocyte enzymes are released and increase capillary permeability and result in sudden pulmonary edema and respiratory distress.

Answer 86

Transfusion-related acute lung injury (TRALI)