Primary Myelofibrosis (PMF)

Primary Myelofibrosis with Myeloid Metaplasia (MMF) is a chronic malignant hematological disorder characterized by an enlarged spleen, varying degrees of anemia and low platelet counts, red cells in the peripheral blood that resemble tear drops, the appearance of small numbers of immature nucleated red cells and white cells in the blood, varying degrees of fibrosis of the marrow cavity (myelofibrosis) and the presence of marrow cells outside the marrow cavity (extramedulary hematopoieses or myeloid metaplasia). MMF and marrow fibrosis are thought to be a response to a malignant proliferation of hematopoietic stem cells. The syndrome ultimately leads to marrow failure characterized by severe anemia and frequently low platelet counts.

Optimal care for primary myelofibrosis is at present supportive and palliative but new strategies including stem cell transplantation show promise.  A gene marker, JAK2, is present in approximately 50% of MF patients.

Symptoms of Primary Myelofibrosis

Nonspecific symptoms of primary myelofibrosis include fatigue, weight loss and night sweats.  Symptoms due to an enlarging spleen are also common as the primary myelofibrosis progresses.