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.

Immune dysregulation

Altered immune function characterized by lymphoid proliferation, immune activation, and excessive autoreactivity often leading to autoimmune/inflammatory complications.

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PMID (PMCID)
15160919	FEMALE	Adult
	Autoimmune myelofibrosis: report of three cases and review of the literature.
	Rizzi R, Pastore D, Liso A, Liuzzi GM, Dalena AM, Specchia G, Ricco R, Liso V. Leuk Lymphoma. 2004;45(3):561-6.
	Among these, the most relevant differential diagnosis is with chronic idiopathic myelofibrosis, particularly when disclosing autoimmune clinical and/or laboratory features as epiphenomenon related to a secondary immune-dysregulation.