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.
A severe form of respiratory insufficiency characterized by inadequate gas exchange such that the levels of oxygen or carbon dioxide cannot be maintained within normal limits.
Pulmonary fibrosis and sea-blue histiocyte infiltration in a patient with primary myelofibrosis.
Yamauchi K, Shimamura K. Eur Respir J. 1995;8(9):1620-3.
The authors present the case of a 73 year old man with primary myelofibrosis, pulmonary fibrosis, and acquired sea-blue histiocytosis, who died of respiratory failure.
