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.

Hepatosplenomegaly

Simultaneous enlargement of the liver and spleen.


Total: 8

                       


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PMID (PMCID)
29945135
 FEMALE Adult
Gaucher Disease and Myelofibrosis: A Combined Disease or a Misdiagnosis?
Mariani S, Palumbo G, Cardarelli L, Santopietro M, Foa R, Giona F.
Acta Haematol. 2018;139(4):240-242.
Gaucher disease (GD) and primary myelofibrosis (PMF) share similar clinical and laboratory features, such as cytopenia, hepatosplenomegaly, and marrow fibrosis, often resulting in a misdiagnosis.
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.
12436302
 MALE Middle Aged
[Coincidence of chronic idiopathic myelofibrosis and chronic lymphocytic leukaemia. A rare phenomenon?].
Bohm J, Schaefer HE, Fisch P.
Pathologe. 2002;23(6):480-5.
In 1986 we diagnosed chronic idiopathic myelofibrosis (CIMF) in a 45-year-old asymptomatic patient with hepatosplenomegaly.
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.
1540883
 MALE Middle Aged
Plasmacytoid monocyte proliferation associated with myeloproliferative disorders.
Baddoura FK, Hanson C, Chan WC.
Cancer. 1992;69(6):1457-67.
One patient also had hepatosplenomegaly and bilateral renal enlargement concomitantly with myelofibrosis with myeloid metaplasia that progressed within months to acute myelogenous leukemia.
1873957
 FEMALE Middle Aged
Case report: mammographic and sonographic demonstration of extramedullary haematopoiesis of the breast.
Zonderland HM, Michiels JJ, ten Kate FJ.
Clin Radiol. 1991;44(1):64-5.
A female patient with agnogenic myeloid metaplasia presented with hepatosplenomegaly and a palpable mass in the right breast.
2252428
 MALE Middle Aged
Extramedullary hematopoiesis in a bronchial carcinoid tumor. An unusual complication of agnogenic myeloid metaplasia.
Lara JF, Rosen PP.
Arch Pathol Lab Med. 1990;114(12):1283-5.
The patient had a 6-year history of agnogenic myeloid metaplasia and hepatosplenomegaly.