Primary myelofibrosis

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.

Pallor

Abnormally pale skin.


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PMID (PMCID)
23997458
 OTHER
Chronic Childhood Idiopathic Myelofibrosis in Down's Syndrome: A Case Report.
Singh S, Sharma S, Tejwani N, Chandra J.
Indian J Hematol Blood Transfus. 2012;28(3):184-6.
A case of chronic idiopathic myelofibrosis in an 8year old boy with Down's syndrome is reported here, who presented with progressive pallor and hepatosplenomegaly.