Philadelphia-negative chronic myeloproliferative neoplasms
Big picture
Philadelphia-negative chronic myeloproliferative neoplasms (Ph-negative MPNs) are clonal disorders of the myeloid hematopoietic stem cell in which mature blood cells are overproduced. They are “chronic” because the cells are usually relatively mature, but they are malignant because they are clonal, progressive, and may transform into myelofibrosis or acute myeloid leukemia (AML).
The three classic Ph-negative MPNs are:
- Polycythemia vera (PV) → too many red cells
- Essential thrombocythemia (ET) → too many platelets
- Primary myelofibrosis (PMF) → marrow fibrosis, splenomegaly, cytopenias/extramedullary hematopoiesis
The textbook separates the major MPNs by genetics: PV, ET, and myelofibrosis are BCR::ABL1-negative, while chronic myeloid leukemia (CML) is BCR::ABL1-positive / Philadelphia-positive.
The examiner usually wants you to explain:
Clonal myeloid proliferation
→ high blood counts or marrow fibrosis
→ thrombosis/bleeding/splenomegaly
→ diagnose with CBC + smear + JAK2/CALR/MPL + bone marrow
→ treat according to thrombosis risk and symptoms
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