PV

Question 0

This mutation ( V617F) a change of valine to phenylalanine at the 617 position, appears to render hematopoietic cells more sensitive to growth factors such as erythropoietin and thrombopoietin because the receptors for these growth factors require JAK2 for signal transduction

Answer 0

JAK2 Mutation

Question 1

Provides instruction for making a protein that promotes the growth and division( proliferation ) of cells.

Answer 1

JAK2 Gene

Question 2

Hyper cellular marrow with variable degree of reticulin fibrosis

Answer 2

PV

Question 3

Increase histamine levels and gastric acidity or possible Budd- Chiari syndrome( hepatic portal vein thrombosis ) or mesenteric vein thrombosis.

Answer 3

Peptic ulcer disease

Question 4

Early satiety because of gastric filling being impaired by the enlarged spleen

Answer 4

Splenomegaly

Question 5

Increase myeloproliferative activity of the abnormal clone.

Answer 5

Weight loss

Question 6

Increase the histamine levels released from increased basophils and mast cells and can be exacerbated by a warm bath or shower

Answer 6

Pruritus

Question 7

Red blood cells in patients with PV

Answer 7

Normochromic normocytic

Question 8

WBC is elevated particularly

Answer 8

Neutrophils

Question 9

PV also should be suspected in patients with:

Answer 9

Portal venous thrombosis and splenomegaly with or without thrombocytosis and leukocytosis

Question 10

What happen to Fe stores?

Answer 10

D or absent bec of the I rbc mass

Question 11

Serum Epo is D or I in PV?

Answer 11

D

Question 12

Major criteria in DDx PV?

Answer 12

Elevated rbc mass, N o2 saturation and palpable splenomegaly

Question 13

High turn over rate of bone marrow cell releasing DNA metabolites

Answer 13

Hyperuricemia

Question 14

What is the minor criteria?

Answer 14

Thrombocytosis plt ct > 400 000 ml, leukocytosis, inc aklaline phosphatase, serum b12 >900

Question 15

Megakaryocytes, leukocytosis, normal rbc count, normal to mildly hyper cellular marrow

Answer 15

Essential thrombocytosis

Question 16

Treatment et

Answer 16

Hydoxyurea aspirin anagrelide interferon p32 busulfan

Question 17

Peripheral leukoerthyroblastosis, teardrop cells, often with anemia

Answer 17

Myelofibrosis

Question 18

Splenomegaly, hypermetabolic symptoms ( wt loss, fever and night sweat), bleeding problems, bone pain, gout, can transform to acute leukemia, high WBC, bone marrow aspiration

Answer 18

Primary myelofibrosis