Leukemias and Myeloproliferative Disorders

Question 1

What are blast indicative of?

Answer 1

AML or ALL

Question 2

What are band indicative of?

Answer 2

infection

Question 3

What if neutrophils are high and lymphs are low with no abnormal cells

Answer 3

corticosteroids

Question 4

Causes of polycythemia

Answer 4

• dehydration
• tobacco abuse
• Pickwickian
• JAK2 mutation
• High erythropoetin levels

Question 5

Myeloproliferative neoplasms vs Myelodysplastic Syndrome

Answer 5

MPN: too much blood

MDS: not enough blood, cytopenias, and BM hyperplasia

–>As dysplasia increases, the less functional blood is made

Question 6

Chronic myeloproliferative neoplasms

Answer 6

1. Chronic myeloid leukemia (CML)

2. Polycythemia vera

Question 7

Polycytemia: primary versus secondary

Answer 7

primary: acquired or inherited mutation
Ex. polycythemia vera

secondary: due to elevated EPO
Ex. Hypoxia, EPO secreting tumor like RCC

combined: due to decreased plasma volume and increased RBC mass
Ex. tobacco use

Question 8

Which mutation is associated with Polycythemia vera?

Answer 8

JAK2 mutation

Dx: BM bx

• hypercellular (increased RBC mass, high hematocrit)
• absent Fe stores
• Epo is low

Question 9

Polycythemia vera: presentation/labs

Answer 9

• pruritis following a warm bath**
• Erythromelalgia of hands/feet (burning, redness, warmth)
• Facial plethora
• splenomegaly

labs:
Hgb ~18 (lil high)

Question 10

Polycythemia vera: treatment

Answer 10

• phlebotomy (goal is Hct <45%)

- ASA, low dose

Question 11

Essential Thrombocythemia

Answer 11

Median platelet count is >1,000,000 (>450,000 is abnormal)

BM bx: megakaryocytes are prominent and in clusters

Tx:

1. ASA, low dose
2. Hydroxyurea (if med to high risk)

Question 12

Chronic Myeloid Leukemia

Answer 12

• Philadelphia chromosome**
• WBC (100-200K)
• Blast phase (or blast crisis) with either lymphoid blasts, or myeloid blasts.**

Question 13

CML: labs

Answer 13

• HIGH WBC

- Bone marrow biopsy (strongly recommended)

Question 14

CML: treatment

Answer 14

Cure:
allogenic stem cell transplant

Disease control:
Imatinib (Gleevec)
tyrosine kinase inhibitors

Palliative therapy
Hydroxyurea

Question 15

Can all myeloproliferative neoplasms and myelodysplastic syndromes transform to acute leukemia?

Answer 15

yes

-worse prognosis if leukemia arises from these

Question 16

Acute Myelogenous Leukemia (AML)

Answer 16

-older patients
-Auer rods**
Tx: allogenic stem cell transplant

Question 17

Acute Lymphoblastic Leukemia (ALL)

Answer 17

• mostly affects kids (3-7 years)
• higher incidence of CNS involvement

Tx: chemotherapy (danurubicin, vincristin, prednisone, asparginase)

Question 18

Similarities of AML and ALL

Answer 18

• pancytopenia with circulating immature cells (blasts)

- MARKED leukocytosis >200K*

Question 19

Chronic Lymphocytic Leukemia (CLL)

Answer 19

• Malignancy of mature B lymphocytes
• MC lymphoid leukemia in adults*

-SMUDGE CELLS*
CBC: elevated WBC with lymphocytosis

Question 20

Aplastic Anemia

Answer 20

Causes: drugs (chloramphenicol [antibiotic], benzene, toluene), radiation, infection, or inherited (Ex. Fanconi anemia)

Signs/Sx:

• pallor
• purpura, petechiae
• NO spleen enlargement

Tx:
Allogenic stem cell transplant

Question 21

Hairy cell leukemia

Answer 21

• Men*
• Middle age

Signs/Sx:
-palpable spleen**

labs:
-pancytopenia (low RBC, WBC, platelet)

peripheral smear:
hairy b cells**

Question 22

LAP score

Answer 22

=leukocyte alkaine phosphatase

-This is ordered when WBC is elevated and want to differentiate reactive process from CML

IF LAP score is low –> CML