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Internal Medicine Board Review > Hematology > Flashcards

Hematology Flashcards

1
Q

List some extrinsic survival defects that cause anemia.

A

Examples of extrinsic (acquired) red cell survival defects include autoimmune hemolytic anemia, malaria, DIC, TTP, HUS, HELLP.

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2
Q

Is reticulocytosis increased or decreased for each of these: Production defect? Maturation defect? Survival defect?

A

Reticulocytosis is decreased in production and maturation defects and increased in survival defects.

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3
Q

When are Howell-Jolly bodies seen?

A

Howell Jolly bodies are seen in anatomically and functionally asplenic patients.

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4
Q

Characterize the lab values in iron deficiency anemia vs. anemia of chronic disease.

A

IDA causes a microcytic, hypochromic anemia. The iron level is low in both IDA and anemia of chronic disease. However, in IDA the TIBC is high (low in ACD), transferrin saturation is low (low to normal in ACD), ferritin is low (high to high-normal in ACD), and soluble transferrin receptor is high (normal in ACD).

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5
Q

What are the common causes of iron deficiency anemia?

A

The common causes of IDA are chronic blood loss, pregnancy, lack of dietary iron, malabsorption, and chronic low-grade intravascular hemolysis.

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6
Q

Characterize the CBC in patients with alpha-thalassemia minor.

A

Patients with alpha-thalassemia minor are typically asymptomatic with significant microcytosis with little to no anemia.

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7
Q

What is the characteristic hemoglobin electrophoresis finding in patients with beta-thalassemia minor?

A

In beta-thalassemia there is an increased A2 component on hemoglobin electrophoresis.

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8
Q

How many lobes must a neutrophil have to be considered “hypersegmented”?

A

Neutrophils are considered hypersegmented if there are 5 lobes in >5% of PMNs or if any PMNs have 6 lobes in their nuclei.

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9
Q

Which megaloblastic anemia is associated with neurologic disease?

A

B12 deficiency is associated with neurologic disease.

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10
Q

What are the MMA and homocysteine levels in B12 and folate deficiencies?

A

MMA and homocysteine are both elevated in B12 deficiency. In folate deficiency only the homocysteine level is increased with normal MMA.

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11
Q

Which general lab tests are abnormal in hemolytic states?

A

Bilirubin and LDH tend to be elevated in hemolytic states. Haptoglobin is low in both intravascular and extravascular hemolysis. Reticulocytes are elevated in hemolysis.

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12
Q

What cells are seen in the peripheral blood of patients with G6PD deficiency?

A

Bite cells are seen in the peripheral smear of patients with G6PD deficiency.

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13
Q

Which virus is implicated in the development of aplastic crisis or worsening of anemia in patients with sickle cell disease?

A

Aplastic crisis or worsening anemia in a patient with sickle cell disease may be caused by infection with parvovirus B19.

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14
Q

Which cells are seen on peripheral smear in patients with hereditary spherocytosis?

A

Spherocytes are seen in the peripheral smear of patients with hereditary spherocytosis. Coombs test is negative.

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15
Q

What is the clinical presentation of PNH?

A

PNH leads to variable degrees of intravascular hemolysis, which can lead to hemoglobinuria and iron deficiency.

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16
Q

Which tests are used to diagnose PNH?

A

Diagnosis of PNH is now made with flow cytometry demonstrating loss of CD55 and CD59 from the cell membrane.

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17
Q

Which leukemia/lymphoma is associated with autoimmune hemolytic anemia?

A

CLL and lymphoma are associated with autoimmune hemolytic anemia.

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18
Q

What is the most common neurologic manifestation of porphyria?

A

The most common neurologic manifestation of porphyria is neurovisceral abdominal pain.

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19
Q

What is the typical presentation of hereditary hemochromatosis?

A

Patients with hemochromatosis usually present in middle age with early signs and symptoms of iron overload including fatigue and weakness, abnormal LFTs, bronzing of the skin, diabetes mellitus, joint pain +/- crystalline arthropathy (CPPD), and erectile dysfunction.

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20
Q

Which test is used to diagnose hereditary hemochromatosis?

A

The most sensitive test to diagnosis hemochromatosis is transferrin saturation. If iron saturation is >45% consider a ferritin and genetic testing.

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21
Q

What disorder(s) cause a high ferritin? A high ferritin with multiple cytopenias and hemophagocytosis in the bone marrow?

A

Very high ferritin levels are seen in hemochromatosis, hemophagocytic lymphohistiocytosis, and adult Still disease.

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22
Q

Explain how aspirin and NSAIDs work to decrease platelet function.

A

Aspirin irreversibly acetylates cyclooxygenase and suppresses thromboxane A2. NSAIDs bind reversibly to cyclooxygenase.

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23
Q

At the bedside, how can you tell whether a hemostatic problem is primary or secondary?

A

Primary hemostatic problems generally involve platelet dysfunction or low platelet counts. They present with multiple, tiny, superficial hemorrhages leading to petechiae, purpura, ecchymoses, and mucocutaneous bleeding. Secondary hemostatic problems involve clotting factors and cause deep tissue bleeding including hematomas and hemarthroses.

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24
Q

What are the 4 tests initially used to evaluate a bleeding disorder? What do they measure?

A

Four tests to initially assess coagulation and platelet status include PT, (extrinsic and common pathways), PTT (intrinsic pathway), platelet count, and platelet function tests.

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25
Q

What is the usual clinical presentation of ITP in an adult patient?

A

The typical adult presentation of ITP is bruising, petechiae, and purpura.

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26
Q

What are the 1st and 2nd line treatments for ITP?

A

The 1st line treatment for ITP is steroids. Second line treatment is IVIg.

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27
Q

TTP is due to problems with which enzyme?

A

TTP is due to a deficiency of or inhibitor to ADAMTS13, the enzyme responsible for cleavage of ultra-long multimers of von Willebrand factor.

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28
Q

Which 5 clinical manifestations occur with TTP? With aHUS?

A

The five clinical manifestations of TTP are thrombocytopenia, microangiopathic hemolytic anemia, fever, renal failure, and neurologic changes. Atypical HUS has all of the same manifestations and lab results, except the renal manifestations occur more and neurologic manifestations occur less than in TTP.

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29
Q

What is the treatment of choice for TTP? For aHUS?

A

The treatment of choice for TTP is plasmapheresis. The treatment of aHUS is eculizumab, a monoclonal antibody that inhibits the terminal complement cascade.

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30
Q

Which bacterial infection is associated with diarrhea-associated HUS?

A

E. coli O157:H7 is associated with diarrhea-associated HUS.

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31
Q

What do the letters in HELLP syndrome stand for? In which patients doe it occur?

A

HELLP stands for hemolysis, elevated liver enzymes, and low platelets. This syndrome occurs in 20% of woman with preeclampsia and 10% of patients with eclampsia.

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32
Q

Does HIT type II cause bleeding or thrombosis?

A

HIT type II causes thrombocytopenia but is associated with thrombosis not bleeding.

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33
Q

What is the treatment for HIT-II?

A

The treatment of HIT-II is to stop all heparin products first. Then you must treat with direct thrombin inhibitors, lepirudin or argatroban.

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34
Q

When does post-transfusion purpura occur?

A

Post-transfusion purpura is rare and occurs primarily in women sensitized by pregnancy. It occurs ~1 week after transfusion and can last days to weeks.

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35
Q

What are the 2 most common types of vWD?

A

The two most common types of vWD are type 1 and type 2. Type 1 is a quantitative defect in von Willebrand factor. Type 2, which has multiple subtypes, is a qualitative defect.

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36
Q

What are Factor 8 activity levels in patients with vWD?

A

von Willebrand factor is a carrier for factor 8 and protects it from degradation. Therefore, in vWD the factor 8 levels are low.

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37
Q

What are the differences between Bernard-Soulier syndrome and Glanzmann thrombasthenia?

A

Bernard-Soulier has giant platelets which exhibit decreased platelet adhesion. These platelets cannot bind vWF. Glanzmann thrombasthenia patients have a normal platelet count but deficient 2b/3a complex causing fibrinogen to not cross-connect.

38
Q

Characterize the PT and PTT in patients with antiphospholipid syndrome.

A

In antiphospholipid syndrome the PT is normal and the PTT is high.

39
Q

What are mixing studies, and when are they used?

A

Mixing studies are useful to differentiate a factor deficiency from the presence of an inhibitor. With a factor deficiency the PT/PTT will correct when mixed with normal plasma. In the presence of an inhibitor, the PT/PTT do not correct.

40
Q

What is the usual cause when a mixing study shows the PTT initially normalizing, but 2 hours later it is again prolonged?

A

Some factor inhibitors are time-dependent. Factor 8 inhibitor in particular may be normal with initial mixing study but prolonged after incubation for 2 hours.

41
Q

What determines the risk of bleeding in a patient with Factor 8 deficiency (hemophilia A)?

A

The risk of bleeding with factor 8 deficiency depends on the factor 8 level.

42
Q

When do you begin the treatment of a bleeding episode in a patient with Factor 8 deficiency?

A

Hemophilia patients with factor 8 levels <1% have severe risk of bleeding and patients with >5% factor 8 activity have mild disease. Treatment of bleeding should be done early to prevent complications such as arthropathy. For patients with mild deficiency and bleeding, desmopressin can be given to release vWF and factor 8 from endothelial cells.

43
Q

What is the clinical presentation of Factor 11 deficiency?

A

Patients with factor 11 deficiency tend to bleed at mucosal sites.

44
Q

How do patients present if they have Factor 12 deficiency?

A

Patients with factor 12 deficiency have normal PT and very prolonged PTT. However, they tend not to bleed and can even undergo surgery without high risk of bleeding.

45
Q

Which special diagnostic test is used to diagnose Factor 13 deficiency?

A

Coagulation studies are normal in factor 13 deficiency. This is diagnosed using a special clot lysis assay.

46
Q

How does PT and PTT differ in DIC vs. TTP?

A

PT and PTT are prolonged in DIC and normal in TTP.

47
Q

Which disease states are associated with chronic DIC?

A

Chronic DIC is virtually always associated with solid tumors.

48
Q

How do certain cephalosporins cause vitamin K deficiency? How do broad-spectrum antibiotics cause vitamin K deficiency?

A

Certain cephalosporins, like cefotetan and cefoperazone, have side chains which block vitamin K epoxide reductase. Broad spectrum antibiotics cause vitamin K deficiency by killing organisms in the gut which produce vitamin K.

49
Q

Patients with which deficiencies are more likely to experience a venous thrombosis during initial anticoagulation with warfarin?

A

Patients with protein C and S deficiency are more likely to become hypercoagulable and experience venous thrombosis during the initiation of warfarin.

50
Q

What are the most common genetic mutations that cause venous thrombosis?

A

The most common genetic mutations that cause venous thrombosis are factor 5 Leiden and prothrombin gene mutation (G20210A).

51
Q

What battery of tests is done on patients with possible inherited thrombophilia?

A

In patients suspected of having a thrombophilia the workup includes factor 5 Leiden mutation, prothrombin G20210A mutation, antithrombin deificiency, protein C and S, and antiphospholipids.

52
Q

What are the classes of oral anticoagulant drug therapies available for treatment of deep venous thrombosis?

A

Drugs that can be used to treat deep venous thrombosis include warfarin, LMWH, unfractionated heparin, fondaparinux, and the oral factor 10a inhibitors rivaroxaban and apixaban.

53
Q

What are established indications for an IVC filter in a patient with deep venous thrombosis?

A

An IVC filter should be considered when anticoagulation is contraindicated and when there is a failure of anticoagulation.

54
Q

Which patients, except in the case of life-threatening bleeding, are not given platelet transfusions, regardless of the degree of thrombocytopenia?

A

Patients with HIT and with TTP/HUS should not be transfused platelets as they can increase the platelet consumption and thrombosis.

55
Q

What is the most common reason for an acute hemolytic transfusion reaction? What is the clinical presentation?

A

Acute hemolytic transfusion reaction is most commonly due to ABO incompatibility due to an error in the blood bank.

56
Q

Which type of transfusion is most associated with transfusion-related bacterial infections? Why?

A

Platelet transfusions are most commonly associated with bacterial infections because platelets are stored at room temperature.

57
Q

What are the lab findings in a patient with aplastic anemia?

A

In aplastic anemia patients have pancytopenia and a hypocellular marrow.

58
Q

How can you tell the difference between acute and chronic leukemias?

A

The presence of blast differentiates acute from chronic leukemias. They are present in acute leukemias. In chronic leukemias there is overproduction of more mature cell lines but not of blasts.

59
Q

What is the treatment for leukemic patients who have hyperviscosity symptoms due to a large number of circulating blasts?

A

Hyperviscosity due to increased circulating blasts is treated with leukapheresis or hydroxyurea followed by chemotherapy.

60
Q

What percentage of marrow blasts defines AML?

A

In AML there are >20% blasts in the bone marrow.

61
Q

What is the characteristic peripheral blood finding in a patient with AML?

A

Auer rods in the peripheral blood is a very specific finding of AML.

62
Q

Characterize the AML M3 type genetic translocation, and describe how it affects treatment. What is the prognosis for aPML?

A

AML M3, or acute promyelocytic leukemia, is caused by a t(15:17) mutation. It has a favorable prognosis. The treatment involves use of all-trans retinoic acid. Although it has a favorable prognosis, it is frequently associated with DIC.

63
Q

Which FAB category of AML is associated with DIC?

A

M3 AML, or acute promyelocytic leukemia, is associated with DIC due to release of procoagulant granules from cytoplasmic granules.

64
Q

What physical findings do patients with ALL often present with?

A

Patients with ALL often present with cytopenias, constitutional symptoms, and neurologic symptoms.

65
Q

Which ALL cytogenetic abnormalities are associated with a poor prognosis?

A

Unfavorable prognostic indicators in ALL include age >60, WBC >100,000, mature B- and T-cell types, persistent minimal residual disease, t(9:22) mutation, and t(4:11) mutation.

66
Q

What is 5q deletion syndrome?

A

5q deletion syndrome is a form of myelodysplastic syndrome with a favorable prognosis which occurs more commonly in women.

67
Q

What are the 4 most common MPDs?

A

The 4 most common myeloproliferative diseases are CML, essential thrombocytosis, polycythemia vera, and primary myelofibrosis.

68
Q

JAK2 is found in what disease states?

A

JAK2 is found in almost all patients with polycythemia vera and in ~50% of patients with essential thrombocythemia and myelofibrosis.

69
Q

CML is associated with which cytogenetic abnormality in >90% of cases?

A

CML is most commonly associated with t(9:22) mutation, Philadelphia chromosome.

70
Q

What is the current standard of care for treating chronic-phase CML?

A

Chronic-phase CML is treated with a tyrosine kinase inhibitor such as imatinib, dasatinib, or nilotinib.

71
Q

What side effects are associated with tyrosine kinase inhibitors?

A

Side effects of tyrosine kinase inhibitors include edema, hepatotoxicity, cytopenias, hemorrhage, worsening of LV dysfunction, and exacerbations of heart failure.

72
Q

What symptoms necessitate treatment in patients with ET?

A

Treatment of patients with essential thrombocythemia should be limited to those with erythromelalgia.

73
Q

Which peripheral blood finding is seen in patients with myelofibrosis?

A

The peripheral smear of patients with myelofibrosis often shows teardrop cells.

74
Q

What are the common physical findings on initial presentation of NHL?

A

Patients with NHL often present with palpable lymphadenopathy and constitutional symptoms.

75
Q

An NHL that produces an IgM monoclonal gammopathy, and is associated with hyperviscosity, is termed what?

A

Waldenstrom’s macroglobulinemia is a NHL that produces IgM monoclonal gammopathy and is associated with hyperviscosity.

76
Q

What are the 2 general types of NHL?

A

NHLs can be divided into indolent and aggressive lymphomas.

77
Q

NHL prognosis depends on which 2 patient-specific factors?

A

Prognosis in NHL is dependent on patient age and performance status.

78
Q

Which drug is incorporated into treatment for most cases of NHL - whether indolent or aggressive?

A

Rituximab is used in the treatment of most cases of NHL, whether indolent or aggressive.

79
Q

What is the treatment for asymptomatic follicular lymphoma?

A

Asymptomatic patients with follicular lymphoma do not require treatment.

80
Q

Marginal zone lymphoma is associated with which chronic infection?

A

Marginal zone lymphomas are most commonly associated with H. pylori infection.

81
Q

What is the difference between CLL and SLL?

A

CLL and SLL are essentially the same disease. In CLL the neoplastic cells are in the circulation. In SLL, the malignant cells are found in lymph nodes. The treatment is the same.

82
Q

What does rapid enlargement of previously stable lymph nodes in a patient with CLL/SLL suggest?

A

About 10% of patients with CLL/SLL transform to a diffuse large cell lymphoma. This transformation is heralded by fever, rapid enlargement of lymph nodes and elevated LDH.

83
Q

What is the treatment of CLL/SLL without symptoms?

A

Asymptomatic patients with CLL/SLL do not require treatment.

84
Q

What is the clinical presentation of hairy cell leukemia?

A

Patients with hairy cell leukemia present with splenomegaly and cytopenias.

85
Q

What is the standard chemo regimen for DLBCL?

A

Standard treatment of DLBCL is with R-CHOP.

86
Q

Characterize the clinical presentation of Burkitt’s. What cytogenetic abnormality is associated?

A

Patients with Burkitt’s lymphoma often present with enlarging abdominal mass and tumor lysis syndrome. Burkitt’s is associated with C-MYC oncogene.

87
Q

Which diseases are seen after radiation therapy to the chest during treatment of Hodgkin lymphoma?

A

Radiation to the chest is associated with future solid tumors including lung and breast cancer.

88
Q

Primary CNS lymphoma in patients with HIV/AIDS is associated with which virus?

A

Primary CNS lymphoma in HIV/AIDS patients is associated with Epstein-Barr virus.

89
Q

Which virus is associated with post-transplant lymphoma?

A

Epstein-Barr virus is associated with post-transplant lymphoma.

90
Q

Which diseases are associated with an increased risk of lymphoma?

A

Certain disease have an increased risk of lymphoma including chronic autoimmune thyroiditis, Sjogren’s, celiac disease, chronic H. pylori, and HIV/AIDS.

91
Q

What are the typical symptoms of multiple myeloma? The lab findings?

A

The typical symptoms of multiple myeloma are bone pain, fatigue, and frequent infections.

92
Q

Characterize the differences between MGUS, smoldering myeloma, and multiple myeloma.

A

Multiple myeloma, smoldering myeloma, and MGUS are all diseases on the same spectrum. In MGUS the monoclonal protein is <3 g/dL, bone marrow plasma cells are < 10%, and there is no end organ damage. In smoldering myeloma there are >3 g/dL of monoclonal protein and >10% plasma cells in the bone marrow but no end organ damage. In multiple myeloma there if >10% plasma cells in marrow, >3 g/dL of monoclonal protein and end organ damage.

Internal Medicine Board Review (13 decks)

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