CLINICAL PRACTICE GUIDELINE FOR POLYCYTHEMIA VERA



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ELEVATED HEMOGLOBIN AND RBC MASS

SERUM EPO IN NONPHLEBOTOMIZED PATIENTS

< OR = 5 IU/ l

> OR = 5 IU/ l

Answer if the following:

Acquired polycythemia, splenomegaly, thrombocytosis, leukocytosis

YES

NO

Polycythemia Vera

Do BM aspirate and Biopsy with cytogenetics (Optional –if available)

(Jak2 =)

Primary Familial and Congenital Polycythmia

- Screen first degree member

CYANOSIS

YES

CHD or Chronic Lung Disease

NO

YES

NO

P50

Methemoglobinemia

- Methemoglobin determination

- Evaluate Hemoglobin M

- Evaluate deficiency of Cytochrome b5 or (if available)

YES

NO

Congenital polycythemia due to actual affinity of Hemoglobin for Oxygen

Evaluate 2-3 BPG

?

?

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