Myeloproliferative Diseases MPD

Question 1

The most common of the MPNs

Myeloproliferative neoplasms

Answer 1

PV

Question 2

PV

Answer 2

activation of JAK2 → erythropoietin hypersensitivity, erythropoietin-independent erythroid colony formation,
حساسية عالية للأريثروپويتن و كذلك الأنتاج مستقلا عنه .

More than 95% of PV patients express this mutation, as do ∼50% of PMF and ET patients.

Question 3

Bartter’s syndrome

Answer 3

فد مرض كلوي ، حيث يخسر الكثير من الملح وأحد اسباب ال

Excess erythropoiesis

Question 4

Tumors that may cause excess erythropoiesis

Familial causes

Answer 4

Cerebellar hemangioblastoma
Uterine myoma
Meningioma

VHL mutation (Chuvash polycythemia)

Question 5

With the exception of aquagenic pruritus, ………………or……………… , no symptoms distinguish PV from other causes of erythrocytosis.

Answer 5

aquagenic pruritus

erythromelalgia
Erythromelalgia is a rare clinical syndrome characterized by a triad of redness, warmth, and burning pain, most notably affecting the extremities due to increased platelet stickiness.

Question 6

Hepatic venous thrombosis (Budd-Chiari syndrome) is particularly common in

Answer 6

young women

Question 7

………….should be suspected in any patient who develops hepatic vein thrombosis

Answer 7

PV

Question 8

In PV , Vascular stasis or thrombocytosis can lead to

Answer 8

Digital ischemia
easy bruising,
epistaxis,
acid-peptic disease,
gastrointestinal hemorrhage

Question 9

Given the large turnover of hematopoietic cells

Answer 9

hyperuricemia
secondary gout
uric acid stones

Question 10

PV cause …………….. von Willebrand’s disease.

Answer 10

acquired

Question 11

Most PV patients who do not express JAK2 V617F express a mutation in………….

Answer 11

Exon 12

Question 12

If flebotomy lead to iron deficiency in PV

Answer 12

Just required every 3 months

Question 13

Treatment of PV

Answer 13

Phlebotomy
ساليسيلةيت وة تةنيا خطرة

allopurinol should be administered to avoid further elevation of the uric acid

chemotherapy is used to reduce splenomegaly or leukocytosis or to treat pruritus.

Question 14

Why thrombocytosis can cause bleeding

Answer 14

Due to vonWillberand disease

Question 15

Pruritis

Answer 15

antihistamines
antidepressants ( doxepin)
/ JAK1/2 inhibitor (ruxolitinib),
pegylated interferon α (IFN-α), /
psoralens with ultraviolet light in the A range (PUVA) therapy, /
hydroxyurea

Question 16

Symptomatic splenomegaly can be treated with either

Answer 16

ruxolitinib or pegylated IFN-α

Question 17

……………: produced complete hematologic and molecular remissions in ∼20% of PV patients

Answer 17

Pegylated IFN-α

Question 18

In some patients with end-stage disease, pulmonary hypertension may develop due to

Answer 18

fibrosis or extramedullary hematopoiesis.

حساوكة ،مقصدي انه بوده myelofibrosis

Question 19

The least common MPN

Answer 19

PMF

Question 20

PMF

Just this among MPD ,give abnormal red cell morphology

Answer 20

marrow fibrosis, extramedullar hematopoiesis,

Spleen enlargement can cause splenic infarction with fever and pleuritic chest pain.

Abnormal blood count

night sweats, fatigue, and weight loss

teardrop-shaped red cells, nucleated red cells, myelocytes, and promyelocytes; myeloblasts

Hyperuricemia and secondary gout may ensue

Question 21

PMF

Answer 21

JAK2 V617F is present in ∼55% of PMF patients

Mutations in the thrombopoietin receptor, MPL, occur in ~4%

Most of the rest have mutations in the calreticulin gene (CALR)

Question 22

occurrence of autoimmune abnormalities such as immune complexes, antinuclear antibodies, rheumatoid factor, or a positive Coombs’ test

number of circulating CD34+ cells is markedly increased in …………. .

Answer 22

PMF

Question 23

Survival in PMF →

Answer 23

shorter than in PV and ET patients.

Question 24

About 10% of …………..patients spontaneously transform to an aggressive form of acute leukemia for which therapy is usually ineffective.

Answer 24

PMF

Question 25

Splenomegaly in PMF can lead to

Answer 25

Splenomegaly → abdominal pain, portal hypertension, easy satiety, and cachexia

splenectomy also increases the risk of blastic transformation.

Question 26

ET

Answer 26

associated with mutations in JAK2 (V617F), MPL, or CALR and manifested clinically by overproduction of platelets without definable cause

PT & PTT → normal

prolonged bleeding time and impaired platelet aggregation can be present.

Question 27

Hindering morrow aspirations

Answer 27

PMF
ET

Question 28

Differential diagnosis of ET

Answer 28

absence of stainable iron demands an explanation because :
iron deficiency alone can cause thrombocytosis,
and absent marrow iron in the presence of marrow hypercellularity is a feature of PV.

Significant splenomegaly should suggest the presence of another MPN, and in this setting, a red cell mass determination should be performed because splenomegaly can mask the presence of erythrocytosis.

Question 29

Erythromelalgia

Answer 29

PV + ET