Chronic Myeloproliferative Disorders (CMPD)

 

CMPD includes CML, polycythemia vera (PCV), myelofibrosis with myeloid metaplasia (MMM), and essential thrombocythemia (ET). Each is a clonal proliferation of a pluripotent stem cell that can differentiate along granulocytic, erythroid, and megakaryocytic lines. Each has a chronic course that often terminates as acute leukemia or myelofibrosis. CMPD is more common in adults.

  • PCV is characterized by excessive proliferation of all three cell lines resulting in high red cell mass, leukocytosis, and thrombocytosis.
  • MMM is characterized by some degree of fibrosis of the marrow, extramedullary hematopoiesis causing massive splenomegaly, and circulating normoblasts, immature granulocytes, and abnormal platelets.
  • ET is characterized by very high and persistent thrombocytosis, including presence of abnormal platelets. Bleeding problems or coagulopathy occur.

 


 

CMPD Abnormalities

 

 +1q PCV, MMM
-7 MMM
+8 PCV, MMM
+9 PCV,MMM
del(13q) interstital or terminal PCV,MMM
del(20)(q11.2) or (q11.2q13.1) PCV,MMM
t(9;22)(q34;q11.2) ET

ALL / AML / CLL-CML / MDS / MPD / Lymphoma / Solid Tumor