A rare myeloproliferative neoplasm characterized by stem-cell derived clonal over proliferation of mature myeloid lineages, such as erythrocytes, leukocytes, and megakaryocytes, with variable degrees of megakaryocyte atypia, associated with reticulin and/or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis, and abnormal cytokine expression.
Reduced ability of the right ventricle to perform its function (to receive blood from the right atrium and to eject blood into the pulmonary artery), often leading to pitting peripheral edema, ascites, and hepatomegaly.
Refractory right ventricular failure due to granulocytic sarcoma.
Tillawi IS, Variakojis D. Arch Pathol Lab Med. 1990;114(9):983-5.
This is, to our knowledge, the first case report of granulocytic sarcoma diffusely involving the heart and causing refractory right ventricular failure in a patient with a long-standing history of agnogenic myeloid metaplasia.
