Polycythemia vera: Difference between revisions
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+ | == Clinical Manifestations == |
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+ | * Elevated hemoglobin and hematocrit |
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+ | ** Possibly mild leukocytosis and thrombocytosis |
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+ | * Hypertension (45%) |
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+ | * Splenomegaly (35%) |
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+ | * Pruritus (35%), particularly after water exposures |
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+ | * [[Erythromelalgia]] (30%) |
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+ | * Arterial (15%) and venous (5-10%) thrombosis |
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+ | * Also: facial plethora, hepatomegaly |
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+ | == Investigations == |
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+ | * CBC |
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+ | ** Hemoglobin greater than 185 (73%) |
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+ | ** WBC greater than 10.5 (49%) |
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+ | ** Platelet count greater than 450 (53%) or 1000 (4%) |
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+ | ** [[Leukoerythroblastic reaction]] on blood film (6%) |
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+ | * Elevated serum LDH (50%) |
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+ | * ''JAK2'' mutation positive (98%) |
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+ | * Abnormal karyotype (12%) |
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+ | * Low serum erythropoietin level (81%) |
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* Major criteria |
* Major criteria |
Latest revision as of 16:00, 23 August 2023
Clinical Manifestations
- Elevated hemoglobin and hematocrit
- Possibly mild leukocytosis and thrombocytosis
- Hypertension (45%)
- Splenomegaly (35%)
- Pruritus (35%), particularly after water exposures
- Erythromelalgia (30%)
- Arterial (15%) and venous (5-10%) thrombosis
- Also: facial plethora, hepatomegaly
Investigations
- CBC
- Hemoglobin greater than 185 (73%)
- WBC greater than 10.5 (49%)
- Platelet count greater than 450 (53%) or 1000 (4%)
- Leukoerythroblastic reaction on blood film (6%)
- Elevated serum LDH (50%)
- JAK2 mutation positive (98%)
- Abnormal karyotype (12%)
- Low serum erythropoietin level (81%)
WHO Diagnostic Criteria
- Major criteria
- Polycythemia, as evidenced by one of:
- Hemoglobin >16.5 g/dL in men or >16.0 g/dL in women
- Hematocrit >49% in men or >48% in women
- Increased red cell mass more than 25% above reference range
- Bone marrow biopsy showing hypercellularity for age with trilineage growth (panmyelosis) including prominent erythroid, granulocytic, and megakaryocytic proliferation with pleomorphic, mature megakaryocytes (differences in size)
- Presence of JAK2 V617F or JAK2 exon 12 mutation
- Polycythemia, as evidenced by one of:
- Minor criterion
- Subnormal serum erythropoietin level
- Diagnosis of PV requires meeting either all 3 major criteria, or the first 2 major criteria and the minor criterion¶