mpnmds

Question 1

what is the defeinition of a myeloproliferative neoplasms

Answer 1

group of clonol myeloid neoplasma characterized by proliferation of one or more cell type in peripheral blood with minimal dysplasia.

Question 2

what are some common feature of myeloproliferative neoplasms

Answer 2

basophilia and splenomegaly

Question 3

what is the median age of CML diagnosis

Answer 3

46-53 years of age

Question 4

what percent of people with CML are asymptomatic

Answer 4

40.00%

Question 5

what are the pathological features of the chronic phase of CML

Answer 5

peripheral blood left shifted more granulocytosis in all states netrophilia with myelocyte bulge.

Question 6

what do you see in the chronic phase of CML in the Bone marrow aspirate or biopsy

Answer 6

markedly hyper-cellular

Question 7

what is the difference between the major minor and kilodaltion breakpoints for BCR ABL of CML

Answer 7

major p210 vast majority, minor p190, kilodaltion p230 cml with thrombosis

Question 8

what is CML in the accelerated phase

Answer 8

peripheral blood or BM blasts in less than 20% PB basophilia less than 20%

Question 9

what is CML in the blast phase

Answer 9

PB or BM greater than 20%

Question 10

tell me about CML in general

Answer 10

tyrosine kinase overactivity due to a translocation of chormosones 9 and 22

Question 11

what is the treatment of CML

Answer 11

imatanib or stem cell tranplantation for younger patients

Question 12

what is the prognosis of CML

Answer 12

4-6 years in the chornic phase then in the terminal blast phase with imatinib prolonged survival.

Question 13

what do you see in leukemoid reactions

Answer 13

Nml resonse to infection segs and bands no basophilia or splenomegaly LAP score elevated normal megas

Question 14

what do you see in the CML

Answer 14

clonol MPN myelocyte bulge basophilia and splenomegaly LAP score decreased dwarf megas

Question 15

what is polycythemia vera

Answer 15

increased RBC mass RBC HGB and HCT, medain age about 60

Question 16

what are the clinical features of polycythemia VERA PV

Answer 16

hyperviscosity related symptoms pruritus after warm shower.

Question 17

what is the diagnosis criteria for PV

Answer 17

2 major and 1 minor or I major and 2 minor, major criteria HGB >18.5 men 16.5 women JAK2 mutation, minor hypercellular marrow with panmyelosis low serum epo endogenous erythroid colony formation in vitro.

Question 18

what are the pathological features of polycythemic phase

Answer 18

peripheral blood erythrocytosis and in the bone marrow hyperceulluar with panmyelosis erythroid predominance variable enlarged megakaryocytes and clustered.

Question 19

what are the pathological features of the spent phase of PV

Answer 19

leukoerytrhoblastosis left shifrted neutrophils and tear drob shaped RBC bone marrow often a dry tap increasing fibrosis

Question 20

what is the pathophysiology of PV

Answer 20

JAK2 tyrosine kinase point mutation accounts for more than 95%

Question 21

what is the treatment of PV

Answer 21

blood letting myelosuppresive drugs 15 year survival of 65% worse with history of thrombosis

Question 22

what are some differnentail diagnosis of r PV

Answer 22

secondary polycythemina due to smoking high altitude ECT>

Question 23

how can you differnatial between secondary polycythemia and PV

Answer 23

clonol MPN decreased EPO splenomegaly penlarged megas in PV

Question 24

what is Essential thrombocytosis

Answer 24

increased platelat production more than 450k

Question 25

what are the clinical feature of essential thrombocytosis

Answer 25

mary are asymptomatic can lead to thrombosis splenomegaly hemmorhage

Question 26

how can you diagnose ET

Answer 26

need all four more than 450K plateltes bone marrow megakaryocytic hyperplasia, JAK2 V617F mutation and not meeting criteria for other MPN MDS and myeloid neoplasm