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.

Poikilocytosis

The presence of abnormally shaped erythrocytes.


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PMID (PMCID)
20949707
 MALE Middle Aged
Bilateral ureteral obstruction due to primary myelofibrosis caused hyperuricaemia.
Cvetkovic ZP, Cvetkovic BR, Celeketic D, Milenkovic D, Perunicic-Pekovic G.
Acta Chir Iugosl. 2010;57(2):79-83.
Primary melofibrosis (Agnogenic myeloid metaplasia-AMM) is a chronic myeloproliferative disease characterized by splenomegaly, a leukoerythroblastic blood picture, teardrop poikilocytosis and varying degrees of marrow fibrosis.
7871986
 MALE Infant
Agnogenic myeloid metaplasia in childhood: a report of two cases and efficiency of intravenous high dose methylprednisolone treatment.
Cetingul N, Yener E, Oztop S, Nisli G, Soydan S.
Acta Paediatr Jpn. 1994;36(6):697-700.
Myelofibrosis with myeloid metaplasia, or agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder characterized by fibrosis of the bone marrow accompanied by aniso- and poikilocytosis, leukoerythroblastosis and hepatosplenomegaly with extramedullary hematopoiesis.
7871986
 MALE Infant
Agnogenic myeloid metaplasia in childhood: a report of two cases and efficiency of intravenous high dose methylprednisolone treatment.
Cetingul N, Yener E, Oztop S, Nisli G, Soydan S.
Acta Paediatr Jpn. 1994;36(6):697-700.
Myelofibrosis with myeloid metaplasia, or agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder characterized by fibrosis of the bone marrow accompanied by aniso- and poikilocytosis, leukoerythroblastosis and hepatosplenomegaly with extramedullary hematopoiesis.
851094
 MALE Middle Aged
Acute myelofibrosis: a report of three cases.
Fabich DR, Raich PC.
Am J Clin Pathol. 1977;67(4):334-8.
This report describes three cases of an atypical variant of agnogenic myeloid metaplasia characterized by pancytopenia, high peripheral blast cell count, lack of significant splenomegaly or tear-drop poikilocytosis, and diffuse marrow fibrosis with an abnormal proliferation of megakaryocytes.
318917
 MIXED_SAMPLE Adult
Idiopathic acquired sideroblastic anemia terminating in acute myelofibrosis: case report and review of leterature.
Yeung K, Trowbridge AA.
Cancer. 1977;39(1):359-65.
Acute myelofibrosis is a rare but distinct accelerated variant of agnogenic myeloid metaplasia that is characterized by marked anemia, peripheral blood myeloblastosis and normoblastosis, a lack of teardrop poikilocytosis, and prominent myelofibrosis.