Onc - MDS and Leukemias

Question 0

Distinguishing between polycythemia vera and renal cell carcinoma as a cause for increased hematocrit?

Answer 0

Low versus high erythropoietin

Question 1

Features of polycythemia vera?

Answer 1

#Headache, blurred vision, tinnitus
#Hypertension
#Splenomegaly
#Thrombosis and bleeding

Question 2

Suspected polycythemia vera – non-CBC lab findings? Most accurate test? Increased risk to develop which cancer?

Answer 2

#Elevated B12
#Low iron

JAK2 mutation

Conversion to AML

Question 3

Treatment for polycythemia vera?

Answer 3

#Phlebotomy and aspirin (to prevent thrombosis)
#Hydroxyurea (to lower cell count)
#allopurinol or rasburicase (to protect against uric acid rise)
#Antihistamines (to prevent pruritis following warm showers)

Question 4

Essential thrombocytosis – clinical sign? Treatment?

Answer 4

Erythromelalgia – Painful red hands

If under 60, asymptomatic, and platelet count under 1.5 million no treatment

If patients over 60, thromboses or platelet count over 1.5 million:
#Hydroxyurea
#aspirin for Erythromelalgia

Question 5

Patient with essential thrombocytosis presents after beginning hydroxyurea – now has red blood cell suppression. Switch to which medication?

Answer 5

Anagrelide (inhibits platelet maturation)

Question 6

Teardrop shaped cells and nucleated RBCs suggest what diagnosis? Treatment?

Answer 6

Myelofibrosis

Thalidomide and Lenalidomide (TNF-a inhibitors that increase bone marrow production)

Question 7

Patient presents with infection and pancytopenia after a long history of myelodysplastic syndrome – need to rule out? Best initial test? Most accurate test?

Answer 7

Acute leukemia

Blood smear will show blasts

Flow cytometry

Question 8

Leukemia patient presents with Eosinophilic inclusions in white blood cells – type of leukemia? Associated associated with what genetic mutation?

Answer 8

AML (auer rods)

Translocation between chromosomes 15 and 17

Question 9

Best indicator of prognosis in acute leukemia?

Answer 9

Cytogenetics (specific chromosomal characteristics)

Question 10

Treatment for patients with promyelocytic leukemia?

Answer 10

All-trans-retinoic acid

Question 11

Complication of promyelocytic (M3) leukemia?

Answer 11

DIC

Question 12

ALL treatment?

Answer 12

Chemotherapy + intrathecal methotrexate to prevent CNS relapse

Question 13

Patient presents with pruritis, splenomegaly, night sweats, fever and high WBC count with increased basophils - suspected diagnosis? Most accurate test? Best initial treatment? Can be cured with? If untreated, can result in?

Answer 13

CML

PCR or FISH for BCR-ABL

Tyrosine kinase inhibitors (imantinib)

Bone marrow transplant

Blast crisis

Question 14

Patient presents with acute leukostasis reaction (extremely high white blood cell count) – Next step?

Answer 14

Leukapheresis to remove excess WBCs (more important than getting a diagnosis)

Question 15

Patient over 60 presents with asymptomatic pancytopenia on CBC. CBC shows bi-lobed neutrophils – suspected diagnosis? Other lab findings? Usual cause of death?

Answer 15

Myelodysplastic syndrome (pre- leukemia disorder)

#Increased MCV
#Nucleated RBCs
#ringed sideroblasts

Bleeding and infection

Question 16

Myelodysplastic syndrome – treatment?

Answer 16

#Transfusions (azacitadine decreases transfusion dependence)
#Erythropoietin
#lenalidomide if patients have 5Q deletion (better prognosis)

Question 17

Smudge cell seen on smear – suspected diagnosis? Clonal proliferation of?

Answer 17

CLL

Abnormal B-cells

Question 18

Stages of CLL? (And treatment?)

Answer 18

Stage 0 – elevated WBC (none)
Stage 1 – lymphadenopathy. (None)

Stage 2 – Hepatosplenomegaly (fludarabine + rituximab)
Stage 3 – anemia (fludarabine + rituximab)
Stage 4 – thrombocytopenia (fludarabine + rituximab)

Question 19

Patient with refractory CLL – treat with?

Mild CLL?

Severe infection?

Autoimmune thrombocytopenia or hemolysis?

Answer 19

Cyclophosphamide

Chlorambucil

IVIg

Prednisone

Question 20

Antibiotic prophylaxis in CLL?

Answer 20

PCP

Question 21

Middle aged man presents with pancytopenia, massive splenomegaly. BM tapped is attempted but dry. Suspected diagnosis? Best initial test? Most accurate test? Treatment?

Answer 21

Hairy cell leukemia

Smear showing hairy cells

Flow cytometry for CD11c

Cladribine, pentostatin