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Medicine

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Hematology

Question
12 out of 34
 

True about polycythemia rubra vera is all Except: (LQ)



A Visual disturbance
B Thrombosis

C ed ESR
D Infection

Ans. D

Infection

Polycythemia Vera (Ref. Hari- 18th ed., Pg.- 898)

It is a myeloproliferative disorder with an absolute increase in red blood cell mass.

A mutation is JAK-2 plays an important role

Extra Edge:

Clinical Features

Clinical symptoms are due to increased (Ref. Hari-18th ed., Pg.-899)

1. Blood Viscosity

2. Vascular stasis

3. Thrombotic tendency

4. Hemorrhagic diathesis (uncommon).

Features are:

a. Patients are plethoric and cyanotic (owing to stagnation and deoxygenation of blood in periphery)

b. Angina, CNS disturbance, gout (hyperuricemia), headache, dizziness, hypertension, Visual disturbance

c. GI symptoms, peptic ulcer disease, hematemesis, melena, abdominal pain, (splenic or renal infarction)

d. Pruritus exaggerated by a warm bath due to release of histamine from basophils may also occur.

e. Massive Splenomegaly is present.

f. Digital ischemia, easy bruising, epistaxis, acid-peptic disease, or gastrointestinal hemorrhage may occur due to

g. vascular stasis or thrombocytosis.

h. Erythema, burning, and pain in the extremities, a symptom complex known as erythromelalgia, is another

i. complication of the thrombocytosis of PV due to increased platelet stickiness.

j. Given the large turnover of hematopoietic cells, hyperuricemia with secondary gout, uric acid stones, and

k. symptoms due to hypermetabolism can also complicate the disorder.

Criteria for Diagnosis of Polycythemia Vera

Category A

1. Total red cell mass:

2. Male ≥ 36 ml/kg

3. Female ≥ 32 ml/kg

4. Arterial oxygen saturation ≥ 92%

5. Splenomegaly.

Category B

1. Thrombocytosis (platelets > 4 lacs/mL)

2. Leukocytosis (white blood cells > 1200/mL)

3. Increased leukocyte alkaline phosphatase (LAP) score

4. Serum B12 > 900 pg/ml or B12 binding capacity > 2200 pg/ml.

5. PV is diagnosed when A1 + A2 + A3 or A1 + A2 and any 2 from category B are present

Investigations

1. PCV is elevated > 60%

2. Elevated RBC count 7 – 10 million/μL

3. Hb level > 18 gm/dL in males and > 16 gm/dL in females

4. Elevated white cell and platelet counts

5. Hypercellular marrow with erythropoiesis or granulopoiesis and megakaryocytes

6. Absent iron stores.

7. Elevated serum B12 levels > 900 pg/ml

8. Elevated neutrophil alkaline phosphatase score.

9. Hyperuricemia, pseudohyperkalemia (due to release of potassium from platelets during in vitro coagulation).

10. O2 saturation > 92% at room temperature.

11. Serum iron may be reduced.

12. erythropoietin ,

13. Microcytic with erythrocytosis

14. Thrombosis or bleeding both can happen.

Extra Edge:

Causes of Microcytosis with erythrocytosis

1. Beta thalassemia

2. Chronic Hypoxia

3. PCV

Extra Edge:

1. Infection is not a feature.

2. O2 saturation > 92% at room temperature

3. ESR is not raised

4. Pruritus and hypertension are the feature

5. LAP score is increased

Extra Edge: Causes of decrease ESR = PCV, CHF, SSA, Afibrogenemia

Hematology Flashcard List

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