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.

Hepatomegaly

Abnormally increased size of the liver.


Total: 2

                       


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PMID (PMCID)
15641221
 MALE
Idiopathic myelofibrosis associated with renal extramedullary hematopoiesis and nephrotic syndrome: case report.
Liu TT, Chen JB, Chen WJ, Kuo CY, Lee CT.
Chang Gung Med J. 2000;23(3):169-74.
Herein, we report the case of a patient with idiopathic myelofibrosis who initially presented with hepatomegaly, anemia, and leukoerythroblastosis.
2232372
 FEMALE Middle Aged
Primary myelofibrosis terminating in megakaryoblastic crisis.
Kurashima K, Kawamura Y, Ohka T, Hayashi M, Matsuda T.
Jpn J Med. 1990;29(2):226-9.
The patient, a 62-year-old female, having had primary myelofibrosis for 13 years and a splenectomy 4 years before, was admitted because of high fever, hepatomegaly, thrombocythemia and leukocytosis.