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.

Pancytopenia

An abnormal reduction in numbers of all blood cell types (red blood cells, white blood cells, and platelets).

Total: 11

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PMID (PMCID)
27628559	MALE
	[Zoledronic Acid Administration is Effective for Hematopoiesis Management in a Patient with Primary Myelofibrosis].
	Sugama Y, Kuroda H, Horiguchi H, Yamauchi N, Sato K, Yamada M, Hamaguchi K, Nakamura H, Abe T, Fujii S, Maeda M, Fujita M, Koguma M, Koguma T, Kato J. Gan To Kagaku Ryoho. 2016;43(9):1131-4.
	An 80-year-old man received a diagnosis of anemia and a decreased platelet count from his physician in 2008, and was referred to our department where primary myelofibrosis was diagnosed.The patient was classified into the lower risk group according to the International Prognostic Scoring System(IPSS), and was followed up without treatment.In June 2010, pancytopenia progressed, and the patient was reclassified into the higher risk group.Blood count did not increase with metenolone treatment, but increased with zoledronic acid(ZA)that he started receiving from January 2012.
20886427	FEMALE	Middle Aged
	[A rare cause of variceal bleeding].
	Naderi J, Lohse AW, Galle PR, Schramm C. Z Gastroenterol. 2010;48(10):1215-8.
	Agnogenic myeloid metaplasia (AMM) is a clonal stem cell disease, which is characterized by myelofibrosis, osteosclerosis and pancytopenia.
18224414	FEMALE	Middle Aged
	Dramatic remission of anemia after thymectomy in a patient of idiopathic myelofibrosis with thymoma.
	Shih YY, Hsiao LT, Yang CF, Wu YC, Chiou TJ. Int J Hematol. 2008;87(1):56-9.
	Anemia is one of the characteristics of idiopathic myelofibrosis (IMF), and malignant thymoma is usually associated with various hematologic disorders, including anemia, pancytopenia, and hypogammaglobulinemia.
18538711	MALE	Adult
	[Histopathologic pattern of hyperplasia of bone marrow hematogones (medullar b lymphoid cell precursors) occurring after treatment of idiopathic myelofibrosis].
	Lebwaze BM, Le Tourneau A, Rio B, Perrot JY, Heuberger L, Kabongo JM, Kalengayi RM, Molina T, Diebold J, Audouin J. Ann Pathol. 2008;28(1):27-31.
	We report the case of a patient presenting idiopathic myelofibrosis with minimal myeloid blastic transformation causing severe pancytopenia, treated by allograft and showing in a bone marrow biopsy, a hyperplasia of B-lymphoid cells.
11863080	MALE	Middle Aged
	Clodronate in myelofibrosis: a case report.
	Froom P, Elmalah I, Braester A, Aghai E, Quitt M. Am J Med Sci. 2002;323(2):115-6.
	A 59-year-old man had well-documented agnogenic myeloid metaplasia (AMM) with pancytopenia.
1635179	FEMALE	Middle Aged
	[Improvement of bone marrow hematopoiesis in idiopathic myelofibrosis with prednisolone].
	Tanosaki R, Uchida H, Masuda Y, Michikawa N, Ogawa T, Kubo A, Ikeda Y. Rinsho Ketsueki. 1992;33(2):256-8.
	A 64 year-old female patient of idiopathic myelofibrosis (IMF), who had had rapidly progressing massive splenomegaly and severe pancytopenia refractory to blood transfusion, was treated with PSL 0.6 mg/kg/day for a month, being significantly improved not only symptomatically and hematologically but also in bone marrow hematopoiesis.
1469787	MALE	Adult
	[Primary myelofibrosis successfully treated with allogeneic bone marrow transplantation].
	Iwata N, Inoue N, Tamura A, Miyazaki E, Fujimori Y, Okamoto T, Takemoto Y, Kosaki M, Kanamaru A, Kakishita E, et al.. Rinsho Ketsueki. 1992;33(11):1703-7.
	A 31-year-old man with primary myelofibrosis initially received low dose Ara C. Splenomegaly decreased but pancytopenia continued.
7436463	MIXED_SAMPLE	Infant
	Familial myelofibrosis.
	Sieff CA, Malleson P. Arch Dis Child. 1980;55(11):888-93.
	The condition closely resembles acute idiopathic myelofibrosis of infancy, but the early onset with severe pancytopenia and the histological appearances may arouse suspicion of the possible familial nature of the condition.
545285	MALE
	[Malignant myelofibrosis. One case (author's transl)].
	Lefevre P, Ricard MF, Imbert M, Reyes F, Pinaudeau Y, Sultan C. Nouv Presse Med. 1979;8(43):3545-7.
	In a 75-year-old man, the rapid development of a pancytopenia as a result to total marrow failure, in the absence of tumour or extramedullary myelopoiesis, but with a histological appearance of the marrow identical to that seen in agnogenic myeloid metaplasia led to a diagnosis of malignant myelofibrosis.
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.
937347	MALE	Middle Aged
	Spontaneous hematologic remission in agnogenic myeloid metaplasia.
	Shreiner DP. Am J Med. 1976;60(7):1014-8.
	A 60 year old man had well documented agnogenic myeloid metaplasia with pancytopenia.