277b myeloproliferative neoplasms Flashcards Preview

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Flashcards in 277b myeloproliferative neoplasms Deck (18):
1

myeloproliferative disorders

JAK2 is involved in hematopoietic growth factor signaling.

Mutations are implicated in myeloproliferative disorders other than CML.

2

JAK2 fxn

GF --> JAK P self --> downstream signals via STAT

mutation --> active without GF

3

CML - mutation?

(no JAK2 mutation)

philadelphia chromosome: bcr-abl transformation --> tyrosine kinase activity --> increased cell division, inhibition of apoptosis

4

CML -Rx?

Rx imatinib

5

CML findings -
RBCs
WBCs
Platelets

RBC decreased

WBC increased

platelets increased

6

Essential thrombocytosis - findings?

increased platelets (little purple dots on smear)

BM have enlarged megakaryocytes that are clustered together

7

Essential thrombocytosis - what most be excluded?

anything that can cause increased platelet accounts which is more common than ET

post-surgery, splenectomy, infection, etc.

8

Essential thrombocytosis - clinical presentation?

thrombus --> stroke, PVT, PE

bleeding if ET bad enough

9

Essential thrombocytosis - mutations

JAK2 or CALR

10

polycythemia vera - RBC, WBC, Platelets, EPO, mutation

increased x 3 (Hct > 55%) --> suppresses EPO

JAK2 mutation

11

polycythemia vera - clinical presentation?

gout from increased uric acid

intense itching after a hot shower

erythromelalgia --> severe, burning pain and red color due to episodic blood clot in vessels of extremities

12

Myelofibrosis - cause?

fibrotic destruction of BM

can arise from ET, PV

13

Myelofibrosis - smear findings?

leukoerythroblasosis (young WBCs should never see)

tear drop RBCs

14

Myelofibrosis - BM biopsy?stain?

hypercellularity w/ clustering of abnomral megakaryocytes

stain with reticulin for fibrosis

15

Myelofibrosis - RBCs? WBCs? platelets?

RBC - decreased --> huge liver and spleen
WBC/Platelets - variable

16

Myelofibrosis - mutation

JAK2 or CALR

17

CML - smear findings?

neutrophils, metamyelocytes, basophiles

18

CML - mutation

9;22 translocation (Rx imatinib)