chronic myeloproliferative disorders

Question 1

shared characteristics of myeloproliferative neoplasms/disorders

Answer 1

1. Increased numbers of cells from one or more cell lines
2. Hepatosplenomegally (extramedullary hematopoiesis)
3. Clonal marrow expansion
4. Hypercatabolism (fevers, sweats, weight loss)
5. Predisposition to evolve into acute myeloid leukemia
6. Loss of control of normal proliferation - for example, erythroid cells from patients with P. vera can grow in the absence of erythropoietin, while normal cells cannot.

Question 2

myeloproliferative disorders that affect granulocytes

Answer 2

• chronic myeloid leukemia
• idiopathic myelofibrosis
• chronic neutrophilic leukemia

Question 3

myeloproliferative disorders that affect RBCs

Answer 3

polycythemia vera

Question 4

myeloproliferative disorders that affect platelets

Answer 4

essential thrombocytosis

Question 5

growth factor for RBCs

Answer 5

EPO

Question 6

growth factor for PMNs

Answer 6

G-CSF/GM-CSF

Question 7

growth factor for monocytes

Answer 7

GM-CSF

Question 8

growth factor for platelets

Answer 8

thrombopoietin

Question 9

growth factors that take stem cells to myeloid precursor

Answer 9

stem cell factor (SCF) and IL-3

Question 10

CML required translocation

Answer 10

(9;22), abl/bcr genes

Question 11

BMBx in CML

Answer 11

• hyper cellular with increased myeloid:erythroid ratio

- no fat

Question 12

typical CML labs

Answer 12

• elevated WBC
• possibly elevated platelets and Hgb
• elevated LDH
• smear with few blasts but immature myeloid cells that run the gambit

Question 13

PE for CML

Answer 13

splenomegaly

Question 14

why early satiety in some disorders?

Answer 14

enlarged spleen compressing stomach

Question 15

contrast CML and AML

Answer 15

CML - many maturity levels of cells and high proliferation in smear
AML - very high blasts, differentiation block to more mature forms

Question 16

common PE findings in polycythemia vera

Answer 16

• splenomegaly
• hepatomegaly
• plethora

Question 17

typical labs in polycythemia vera

Answer 17

• elevated Hgb/Hct
• possible elevated WBC and/or platelets
• blood smear normal
• EPO low

Question 18

molecular diagnosis of polycythemia

Answer 18

95% have V617F JAK2 (Janus Activating Kinase 2) mutation

Question 19

major treatment for polycythemia vera

Answer 19

phlebotomy

Question 20

typic PE for essential thrombocythemia

Answer 20

mild splenomegaly

Question 21

major complication of thrombocythemia

Answer 21

thrombosis

Question 22

treatment of thrombcythemia

Answer 22

hydroxyurea in severe cases

Question 23

typical diagnostic labs in thrombocythemia

Answer 23

• platelets above 450
• increased and abnormal megakaryocytes in BMB
• JAK or MPL mutation
• normal iron, normal chromosomes

Question 24

typical PE in primary myelofibrosis

Answer 24

• splenomegaly “hallmark”
• hepatomegaly
• bruising

Question 25

typical diagnostic lab findings in primary myelofibrosis

Answer 25

• anemia
• teardrop RBCs in smear
• platelet and WBC can be up or down
• fibrosis due to collagen deposition in bone marrow