POLYCYTHEMIA VERA

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

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

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.

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.

Answer is A (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 lines^Q 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.

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)

Polycythaemia vera (PV) is a clonal disorder characterized by increased production of all myeloid elements resulting in pancytosis (i.e increased red cells, granulocytes, platelets) in the absence of any recognizable cause.

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.