Polycythemia Vera

POLYCYTHEMIA VERA

Q. 1

Which among the following tumor is associated with Polycythemia vera?

 A

Osteosarcoma

 B

Ovarian tumor

 C

Cerebellar haemangioblastoma

 D

Pitutary adenoma

Q. 1

Which among the following tumor is associated with Polycythemia vera?

 A

Osteosarcoma

 B

Ovarian tumor

 C

Cerebellar haemangioblastoma

 D

Pitutary adenoma

Ans. C

Explanation:

Tumors associated with polycythemia vera are Hypernephroma, Hepatoma, Cerebellar hemangioblastoma, Uterine myoma, Adrenal tumors, Meningioma, Pheochromocytoma.

 
The JAK2 gene located on the short arm of chromosome 9 and loss of heterozygosity on chromosome 9p due to mitotic recombination is the most common cytogenetic abnormality in Polycythemia Vera.
 
Ref: Harrison’s Internal Medicine, 18th Edition, Pages 898-900

Q. 2

Which of the following is not commonly seen in Polycythemia Vera?

 A

Thrombosis

 B

Hyperuricemia

 C

Prone for acute leukemia

 D

Spontaneous severe infection

Q. 2

Which of the following is not commonly seen in Polycythemia Vera?

 A

Thrombosis

 B

Hyperuricemia

 C

Prone for acute leukemia

 D

Spontaneous severe infection

Ans. D

Explanation:

In polycythemia vera splenomegaly may be the initial presenting sign.

In some patients venous or arterial thrombosis may be the presenting manifestation of PV.

Due to the large turnover of hematopoietic cells, hyperuricemia with secondary gout and uric acid stones are seen in PV.

Incidence of acute nonlymphocytic leukemia is increased in PV.

Ref: Current Medical Diagnosis and Treatment, 2012, Chapter 13 ; Harrison’s Principle of Internal Medicine, 18th Edition, Pages 898-900


Q. 3

Choose the TRUE/FALSE statements regarding polycythemia vera:

a) The JAK2 gene is located on the short arm of chromosome 12
b) Splenomegaly will be the initial presenting sign in PV
c) Venous and arterial thrombosis may be the presenting manifestation of PV
d) Red cell mass and plasma volume determinations are mandatory to establish the presence of an absolute erythrocytosis

e) A bone marrow aspirate and biopsy provide specific diagnostic information

 A

b,c,d-True & a,e-False

 B

a,b,c,e-True & d-False

 C

a,c,d,e-True & b-False

 D

b,c,e-True & a,d-False

Q. 3

Choose the TRUE/FALSE statements regarding polycythemia vera:

a) The JAK2 gene is located on the short arm of chromosome 12
b) Splenomegaly will be the initial presenting sign in PV
c) Venous and arterial thrombosis may be the presenting manifestation of PV
d) Red cell mass and plasma volume determinations are mandatory to establish the presence of an absolute erythrocytosis

e) A bone marrow aspirate and biopsy provide specific diagnostic information

 A

b,c,d-True & a,e-False

 B

a,b,c,e-True & d-False

 C

a,c,d,e-True & b-False

 D

b,c,e-True & a,d-False

Ans. A

Explanation:

The JAK2 gene is located on the short arm of chromosome 9. A bone marrow aspirate and biopsy provide no specific diagnostic information unless there is a need to exclude some other disorder, these procedures need not be done.

 
Ref: Harrisons Principles of Internal Medicine, 18th Edition, Page 898, 899

Q. 4

Most common postoperative complication in polycythemia vera –

 A

Infection

 B

Cardiopulmonary complication

 C

Hemolysis

 D

Uremia

Q. 4

Most common postoperative complication in polycythemia vera –

 A

Infection

 B

Cardiopulmonary complication

 C

Hemolysis

 D

Uremia

Ans. B

Explanation:

Ans is . ‘b’ i.e., Cardiopulmonary complication

o Many patients of polycythemia experience a post-operative complication, most often bleeding or thrombosis. o So, none of the given options is correct.

o Amongst the given options, I would like to prefer cardiopulmonary complication because thrombosis (DVT) is the most common cause of pulmonary embolism.

Quiz In Between


Q. 5

In Polycythemia vera, all the following are seen except:

 A

Thrombocytopenia

 B

Increased GI bleed

 C

Thrombosis

 D

Transient visual loss

Q. 5

In Polycythemia vera, all the following are seen except:

 A

Thrombocytopenia

 B

Increased GI bleed

 C

Thrombosis

 D

Transient visual loss

Ans. A

Explanation:

Answer is A (Thrombocytopenia)

Polycythemia vera is associated with thrombocytosis and not thrombocytopenia

Polycythemia vera is associated with thrombocytosis and not thrombocvtopenia

Polycythemia Vera is an acquired myeloproliferative disorder that is characterised by overproduction of three hematopoitic cell linesQ with predominant elevation in RBC’s0.

Thus there is overproduction of granulocytes, red cells and platelets. Overproduction of platelets results in thrombocytosis. Thrombocytopenia is not seen.

Polvcythemia vera is associated with an increased incidence of both thrombosis and bleeding Both arterial and venous thrombosis may be seen in Polycythemia vera.

Paradoxically patients may show a tendency of bleeding and bruising, mainly due to platelet of dysfunction.

Transient visual loss may be seen in polycythemia vera as a manifestation of associated hyperviscosity syndrome.


Q. 6

Polycythemia vera may transform into:

March 2013 (b)

 A

ALL

 B

AML

 C

CML

 D

Renal cell carcinoma

Q. 6

Polycythemia vera may transform into:

March 2013 (b)

 A

ALL

 B

AML

 C

CML

 D

Renal cell carcinoma

Ans. B

Explanation:

Ans. B i.e. AML

Polycythemia vera

  • It is a stem cell disorder characterized as a panhyperplastic, malignant, and neoplastic marrow disorder.
  • The most prominent feature of this disease is an elevated absolute red blood cell mass because of uncontrolled red blood cell production.
  • This is accompanied by increased white blood cell (myeloid) and platelet (megakaryocytic) production, which is due to an abnormal clone of the hematopoietic stem cells with increased sensitivity to the different growth factors for maturation
  • Progression to acute myeloid leukemia/ myelodysplastic syndromes (AML/MDS) is a possible evolution of polycythemia vera (PV)

Q. 7

Following are seen in polycythemia vera except:

September 2011

 A

Most common cause of polycythemia

 B

Increased erythropoietin

 C

Erythropoietin independent growth of red cell progenitors

 D

Intrinsic abnormality of hematopoietic precursors

Q. 7

Following are seen in polycythemia vera except:

September 2011

 A

Most common cause of polycythemia

 B

Increased erythropoietin

 C

Erythropoietin independent growth of red cell progenitors

 D

Intrinsic abnormality of hematopoietic precursors

Ans. B

Explanation:

Ans. B: Increased erythropoietin

The combination of iron deficiency anemia, glossitis and esophageal dysphagia usually related to the webs is known as the Plummer Vinson syndrome or Paterson Kelly syndrome.

Plummer-Vinson syndrome/PVS/Paterson-Brown-Kelly syndrome/sideropenic dysphagia

It presents as a triad of dysphagia (due to esophageal webs), glossitis, and iron deficiency anemia.

  • In primary polycythemia, there may be 8 to 9 million and occasionally 11 million erythrocytes per cubic millimeter of blood (a normal range for adults is 4-6), and the hematocrit may be as high as 70 to 80%.
  • In addition, the total blood volume sometimes increases to as much as twice normal.

Q. 8

All are true about Polycythemia vera except 

 A

Increased vit B12 

 B

Decrease LAP score

 C

Leucocytosis

 D

Increased platelets

Q. 8

All are true about Polycythemia vera except 

 A

Increased vit B12 

 B

Decrease LAP score

 C

Leucocytosis

 D

Increased platelets

Ans. B

Explanation:

Ans. is ‘b’ i.e., Decrease LAP score 

Polycythemia vera

  • Polycythemia vera is a neoplasm arising in a multipotent myeloid sterm cell that is characterized by increased marrow production of erythroid, granulocytic and megakaryocytic elements.
  • This leads to erythrocytosis (polycythemia), granulocytosis, and thrombocytosis in the peripheral blood.
  • Polycythemia is responsible for most of the clinical symptoms of polycythemia vera.
  • Polycythemia vera progenitor cells have markedly decreased requirements for erythropoietin and other hematopoietic growth factors. Accordingly serum erythropoietin levels in polvuthemia vera are very low, whereas almost all other forms of absolute polycythemia are caused by elevated ervthropoietin levels.

Clinical manifestations

1. Symptoms due to polycythemia

  • Most symptoms are related to the increased red cell mass and hematocrit, i.e., Polycythemia.
  •  The elevation of hematocrit is usually accompanied by increased total blood volume, and together these two promote abnormal blood flow, particularly on the low pressure venous side of the circulation, which becomes greatly distended. That results in –
  • Plethora or cynosis owing to stagnation of deoxygenated blood in peripheral vessels.
  • Headache, diziness, hypertension and
  • Dininshed vision from blockade of retinal vessels.

2. Symptoms due to granulocytosis

  • Basophils secrete histamine that results in –
  • Intese pruritis
  • Peptic ulceration
  • Increased vit 13,,, binding capacity because of increase in transcobalmine I & II.

3. Symptoms due to Thrombocytosis

  • There is increased risk of both thrombosis and major bleeding episodes.

Thrombosis                 DVT, MI, Stroke, Budd chiari syndrome.

Bleeding                     Upper GI bleeding from peptic ulcer.

Laboratory features of Polvcythemia vera. 

Increased Red cell count (Polycythemia) Increased Vit B 12 binding capacity
Increased WBC count (Leucocytosis) Increased blood viscocity
Increased platelet count (Thrombocytosis) Decreased ESR
Increased Leucocyte alkaline phosphate (LAP) score

Hyperuricemia due to increased turnover of cells.

Increased hemoglobin

Increased histamine level


Quiz In Between



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