Polycythemia Vera PV

Question 1

Neoplastic proliferations of mature myeloid cells -RBCs ?

Answer 1

Polycythemia Vera

Question 2

PV which mutation ?

Answer 2

JAK2 kinase

Question 3

PV which cells inc ?

Answer 3

1. Granulocytes
2. RBC
3. Platelets

Question 4

Clinical symptoms of PV ?

Answer 4

1. Blurry vision
2. Headache
3. Inc risk of thrombosis
4. Flushed face - Congestion - Plethora
5. Itching after bath - Histamine release from inc mast cells

Question 5

Tx of PV ?

Answer 5

1. Phlebotomy
2. HydroxyUrea

Without Tx death wihtin 1 year

Question 5

PV vs Reactive Polycythemia ?

Answer 5

In PV -
1. Erythropoietin level dec -EPO dec
2. SaO2 normal

In Reactive Polycythemia
1.EPO- Inc
2.SaO2 low
3.Renal cell Ca

Question 6

Essential thrombocytopenia which mutation ?

Answer 6

JAK2 kinase

Pathoma-59

Question 7

Symptoms of ET ?

Answer 7

1. Rare prognosis to amrrow fibrosis or Acute Leukemia
2. No risk of Gout or Hyperuricemia

Question 8

Myelofibrosis same PV - CML

Answer 8

JAK2 KINASE mutation

Question 9

Marrow fibrosis cause ?

Answer 9

Megakaryocytes produce excess Platelet derived growth factor-PDGF

Question 10

Tear drop RBC ?

Answer 10

Myelofibrosis

Question 11

Clinical features of Myelofibrosis /

Answer 11

1. Splenomegaly
2. Extramedullary hematopoiesis
3. Leukoerythroblastic smear
4. Tear drop RBC
5. Nucleated RBC
6. Immature granulocytes
7. Inc risk - infection _ thrombosis - bleeding

pathoma-59