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.

Neoplasm

An organ or organ-system abnormality that consists of uncontrolled autonomous cell-proliferation which can occur in any part of the body as a benign or malignant neoplasm (tumour).

Total: 3

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PMID (PMCID)
23117264	MIXED_SAMPLE	Middle Aged
	Splenic irradiation as a component of a reduced-intensity conditioning regimen for hematopoietic stem cell transplantation in myelofibrosis with massive splenomegaly.
	Ito T, Akagi K, Kondo T, Kawabata H, Ichinohe T, Takaori-Kondo A. Tohoku J Exp Med. 2012;228(4):295-9.
	Primary myelofibrosis is a hematologic neoplasm characterized by bone marrow fibrosis and extramedullary hematopoiesis.
19167611	MALE	Adult
	JAK2 V617F/C618R mutation in a patient with polycythemia vera: a case study and review of the literature.
	Yoo JH, Park TS, Maeng HY, Sun YK, Kim YA, Kie JH, Cho EH, Song J, Lee KA, Suh B, Choi JR. Cancer Genet Cytogenet. 2009;189(1):43-7.
	The frequency of JAK2 V617F mutation is about 90% in patients with PV, 50-60% in patients with essential thrombocythemia (ET), primary myelofibrosis (PMF), and less in patients with other myeloid neoplasms, while extremely rare in lymphoid malignancies.
18607123 (2627188)	MALE	Adult
	Intrahepatic extramedullary hematopoiesis mimicking a hypervascular hepatic neoplasm on dynamic- and SPIO-enhanced MRI.
	Lee IJ, Kim SH, Kim DS, Lee JM, Han JK, Choi BI. Korean J Radiol. 2008;9 Suppl:S34-8.
	We present a rare case of a focal intrahepatic extramedullary hematopoiesis (EMH) that mimicked a hypervascular hepatic neoplasm in a 33-year-old woman with idiopathic myelofibrosis.