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A.
Polycythaemia is an increase in red blood cell mass which may be due to an actual increase in red
blood cell mass (true polycythaemia) or a spurious laboratory value (apparent polycythaemia). True
polycythaemia is classified as either primary or secondary. Primary polycythaemia is caused by a
myeloproliferation (uncontrolled proliferation of red cell precursors in the bone marrow) and is
not mediated by excess erythropoietin. In contrast, secondary polycythaemia can be caused by an external source, such as smokers polycythaemia, or disorders like renal cancer. Erythropoietin levels in secondary polycythaemias vary depending on the cause.
Polycythaemia vera can be diagnosed when a patient has all three major criteria:
Increased red blood cell mass [at least 36 mL per kg for men, at least 32 mL per kg for women
Normal arterial oxygen saturation [at least 92 percent], and
Splenomegaly (increase in the size of spleen)
The first two major criteria plus any two of the minor criteria:
Platelet count of more than 400 X 103 per µl
Leukocyte count of more than 12 X 103 per µl
Alkaline phosphatase level of more than 100 U per L
Vitamin B12 level of more than 900 pg per ml or an unbound vitamin B12 binding capacity of more than 2,200 pg per ml.
The disease can have life-threatening complications, including stroke and evolution into either myelofibrosis with myeloid metaplasia or acute leukaemia. Treatment is directed according to patient risk stratification and frequent phlebotomy (removal of blood) and chemotherapy are the
cornerstones of treatment. Haematocrit (PCV) levels should be maintained below 45 percent in men and 42 percent in women. Chemotherapy using hydroxyurea, busulfan, or pipobroman is indicated for high-risk patients. Interferon-alfa is another chemotherapeutic option for reducing red blood
cell burden and treating PV-associated pruritus. Non-lifethreatening sequelae include microvascular complications and aquagenic pruritus. Microvascular complications may appear clinically as
headache, light-headedness, transient neurologic abnormality, transient ocular disturbance, tinnitus, atypical chest pain, paraesthesias, and erythromelalgia. Low-dose aspirin can be used to treat these disorders. Aquagenic pruritus is a generalised body itching often brought on by a hot
bath. Treatment options include either selective serotonin reuptake inhibitors or interferon-alfa.
You need to consult an Oncologist who will do a complete physical examination and the required tests so that appropriate treatment can be started.
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