MKSAP HemeOnc V

Question 1

Clinical features of fever, change in mental status, thrombocytopenia, and features of hemolytic anemia (low haptoglobin level, elevated lactate dehydrogenase level, elevated reticulocyte count). What test do you need to initiate therapy for presumed thrombotic thrombocytopenic purpura (TTP)?

Answer 1

Schistocytes on peripheral blood smear

Confirm the diagnosis of a microangiopathic hemolytic anemia (MAHA). TP is fatal in 90% of patients without therapy. Patients require emergent treatment with plasma exchange. Don’t need to wait for ADAMTS13. Coagulation studies are almost always normal in patients with TTP.

Question 2

dyspnea, hypoxia, fever, chills, and hypotension within 6 hours of transfusion. chest radiograph shows diffuse bilateral pulmonary infiltrates.

Answer 2

TRALI (treatment is supportive)

Question 3

___ deficiency should be suspected in patients with macrocytic anemia, malnutrition, and alcohol dependence.

Answer 3

Folate

Alcohol dependence is not a risk factor for cobalamin (B12) deficiency.

Question 4

Patients with pancytopenia, macrocytic erythrocytes, hypersegmented neutrophils, and findings consistent with intramedullary hemolysis should have ____ assessed to determine the cause of megaloblastic anemia.

Answer 4

vitamin B12 and folate levels

Question 5

___ typically presents in the chronic phase with elevated neutrophils or platelets, or both.

Answer 5

CML (chronic myeloid leukemia)

Question 6

macrocytic anemia and other cytopenias and a peripheral blood smear showing dysplastic, hypogranular neutrophils and nucleated erythrocytes.

Answer 6

Myelodysplastic syndrome. A bone marrow biopsy specimen showing hypercellular marrow with dysplastic myeloid progenitor cells and lack of orderly maturation is diagnostic.

Other causes of dysplasia, including chronic infections (HIV), vitamin B12 deficiency, alcohol use, and medications such as sulfamethoxazole, must be ruled out.

Question 7

Factors that contribute to High probability scores for HIT are (4)

Answer 7

1. associated with a 50% decrease in platelet count
2. onset between days 5 and 10 of heparin exposure
3. new thrombosis
4. no other cause of thrombocytopenia.

Question 8

What is the most appropriate managment of lytic bone lesions caused by multiple myeloma?

Answer 8

Zolendronic acid

Question 9

Treatment for cold agglutinin disease?

Answer 9

Cold avoidance and rituximab (not glucocorticoids, splenectomy, and IVIG)

Question 10

Signs of intravascular hemolysis with positive direct antiglobulin testing showing C3 (no IgG) on erythrocytes with agglutinated erythrocytes on peripheral smear is consistent with ___.

Answer 10

cold agglutinin autoimmune hemolytic anemia

Question 11

Coomb’s test with +IgG or IgG and C3

Answer 11

warm agglutinin hemolytic anemia

Question 12

_____ is a cause of heparin resistance.

Answer 12

Antithrombin deficiency (aPTT does not increase despite increasing doses of heparin)

Question 13

A prolonged aPTTthat does not fully correct with a mixing study suggests the presence of a ____.

Answer 13

Factor inhibitor and the diagnosis of acquired hemophilia A

Treatment is with activated factor VII, activated prothrombin complex concentrate, or recombinant porcine factor VIII.

Question 14

____ which can be recognized by a normal activated partial thromboplastin time and an elevated prothrombin time, should be expected in patients who have poor oral intake and are taking prolonged courses of antibiotics.

Answer 14

Vitamin K deficiency

Question 15

With the exception of testing for genetic mutations, thrombophilia testing should not be performed in the acute setting or while receiving anticoagulant therapy and should be delayed at ____ to minimize diagnostic error.

Answer 15

least 2 weeks after discontinuation of anticoagulant therapy (ideal testing strategy is to temporarily stop AC in 1 year after treatment for DVT is started and wait 2 weeks)

Question 16

CD55/59 deficiency is associated with

Answer 16

paroxysmal nocturnal hemoglobinuria

Question 17

Patients with abdominal thrombosis, splenomegaly, and portal hypertension should be evaluated for the presence of a myeloproliferative neoplasm, specifically the ____, even in the absence of erythrocytosis or thrombocytosis.

Answer 17

JAK2 V617F gene mutation

Question 18

Cold agglutinin disease is associated with which infections?

Answer 18

EBV and Mycoplasma pneumoniae

Question 19

positive direct antiglobulin test (DAT) and spherocytes on the peripheral blood smear should further raise suspicion for hemolysis from an IgG autoantibody with exposure to new drug (CTX)

Answer 19

drug-induced hemolytic anemia

Question 20

____ is a transfusion-transmissible pathogen that can be responsible for malaria-like symptoms, hemolytic anemia, thrombocytopenia, and abnormal liver chemistry study results. The incubation period for transfusion-related babesiosis ranges from 11 to 176 days.

Answer 20

Babesia microti

Question 21

Pregnant women with immune thrombocytopenic purpura can be safely monitored during their pregnancy without intervention as long as they are asymptomatic and the platelet count remains greater than ____.

Answer 21

30,000

Question 22

For patients with small cell lung cancer who have large centrally located tumors that are not amenable to resection, standard management is ____, which can result in cure for about 20% to 30% of patients.

Answer 22

combined chemotherapy and radiation

Question 23

____ and ____therapy for up to 2 to 3 years results in an improvement in 10-year disease-free and overall survival and is the standard care for patients with high-risk and very-high-risk prostate cancer.

Answer 23

Combination therapy with radiation and a gonadotropin-releasing hormone agonist

Question 24

The standard treatment for locally advanced head and neck cancer is a combination of radiation with systemic therapy, most commonly ____.

Answer 24

cisplatin (contraindicated in renal disease with Cr >1.5) or cetuximab.