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.

Splenomegaly

Abnormal increased size of the spleen.

Total: 38

(per page)

PMID (PMCID)
7563614	FEMALE	Middle Aged
	[Aplastic anemia with giant splenomegaly and myelofibrosis successfully treated with antilymphocyte globulin].
	Kaito K, Otsubo H, Ogasawara Y, Sekita T, Saeki A, Nishiwaki K, Masuoka H, Shimada T, Yoshida M, Kobayashi M, et al.. Rinsho Ketsueki. 1995;36(8):786-91.
	Splenomegaly and myelofibrosis are characteristic of primary myelofibrosis and although the relationship is uncertain, there is no previous report on aplastic anemia with splenomegaly and myelofibrosis.
8531337	MALE
	[Successful treatment of splenomegaly and anemia by VP therapy in idiopathic myelofibrosis complicated with acromegaly: a case report].
	Imai Y, Akiyama N, Hanazono Y, Inamori K, Mitani K, Hirai H, Nagai R, Yazaki Y. Rinsho Ketsueki. 1995;36(10):1233-6.
	[Successful treatment of splenomegaly and anemia by VP therapy in idiopathic myelofibrosis complicated with acromegaly: a case report].
8190037	MIXED_SAMPLE	Adult
	Autoimmune myelofibrosis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus.
	Paquette RL, Meshkinpour A, Rosen PJ. Medicine (Baltimore). 1994;73(3):145-52.
	Conversely, the absence of splenomegaly in a patient with presumed idiopathic myelofibrosis may suggest an autoimmune etiology.
8155190	MALE
	[Splenoma with accumulation of megakaryocytes during the course of an idiopathic myelofibrosis].
	Brouland J, Molina T, Delmer A, Zittoun R, Diebold J. Ann Pathol. 1994;14(1):32-5.
	The diagnosis was done on surgical specimen of splenectomy performed for a painful splenomegaly occurring in a 71-year-old man presenting a primary idiopathic myelofibrosis.
1636666	MIXED_SAMPLE	Infant
	Agnogenic myeloid metaplasia in children.
	Mallouh AA, Sa'di AR. Am J Dis Child. 1992;146(8):965-7.
	Agnogenic myeloid metaplasia is a chronic myeloproliferative disorder characterized by splenomegaly, leukoerythroblastosis, extramedullary hematopoiesis, teardrop-shaped red blood cells, and fibrosis of the bone marrow.
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.
1758057	MALE
	[Primary myelofibrosis transforming into multiple subcutaneous monoblastoma--a case report].
	Hamamoto K, Taniguchi H, Ohga S, Nagano T, Kishimoto Y, Kitajima H, Fujimoto M, Kimura T, Fujitake H, Yasunaga K. Rinsho Ketsueki. 1991;32(11):1481-5.
	A 83-year-old man was diagnosed with primary myelofibrosis based on the presence of leukoerythroblastosis, splenomegaly, chromosome 46 XY, a dry tap bone marrow aspiration and fibrosis on bone marrow biopsy, when he was admitted for herpes zoster in June 1987.
2313911	FEMALE
	[Blastic crisis of primary myelofibrosis associated with multiple myeloblastomas ].
	Fukushima T, Uchida M, Iwasaki H, Kamiya K, Tanaka T, Yoshimura T, Sasaki K, Tsutani H, Wano Y, Takayama H, et al.. Rinsho Ketsueki. 1990;31(1):95-9.
	The patient is a 71-year-old female who underwent splenectomy due to splenomegaly 32 months after diagnosed as having primary myelofibrosis.
2313911	FEMALE
	[Blastic crisis of primary myelofibrosis associated with multiple myeloblastomas ].
	Fukushima T, Uchida M, Iwasaki H, Kamiya K, Tanaka T, Yoshimura T, Sasaki K, Tsutani H, Wano Y, Takayama H, et al.. Rinsho Ketsueki. 1990;31(1):95-9.
	A rapid development of splenomegaly in a patient with primary myelofibrosis seems to be associated with leukemic transformation.
2216813	FEMALE	Middle Aged
	[Immunoscintigraphy of the hematopoietic bone marrow in osteomyelofibrosis].
	Bartenstein P, Kirchner B, Scheidhauer K, Schober O. Nuklearmedizin. 1990;29(4):180-2.
	In planning the treatment of splenomegaly in primary or secondary osteomyelofibrosis it is necessary to know whether there is any significant hematopoiesis outside the spleen.
3178046	MIXED_SAMPLE	Adult
	Preoperative splenic artery occlusion as an adjunct for high risk splenectomy.
	Fujitani RM, Johs SM, Cobb SR, Mehringer CM, White RA, Klein SR. Am Surg. 1988;54(10):602-8.
	The massive splenomegaly in this group resulted from chronic myelogenous leukemia (n = 2), isolated splenic lymphoma (n = 1), and agnogenic myeloid metaplasia (n = 1).
3982370	FEMALE	Adult
	Secondary myelofibrosis with metastatic breast cancer simulating agnogenic myeloid metaplasia: report of a case and review of the literature.
	Yablonski-Peretz T, Sulkes A, Polliack A, Weshler Z, Okon E, Catane R. Med Pediatr Oncol. 1985;13(2):92-6.
	These findings are rare and myeloid metaplasia with striking extramedullary hematopoiesis causing massive splenomegaly and mimicking agnogenic myeloid metaplasia is seldom seen in patients with metastatic carcinoma.
6695146	MALE	Middle Aged
	Polycythaemia following splenectomy in myelofibrosis with myeloid metaplasia. A reorganization of erythropoiesis.
	Barosi G, Baraldi A, Cazzola M, Fortunato A, Palestra P, Polino G, Ramella S, Spriano P. Scand J Haematol. 1984;32(1):12-8.
	3 patients with myelofibrosis with myeloid metaplasia were splenectomized because of anaemia and disturbing splenomegaly.
6680005	MIXED_SAMPLE
	[Idiopathic splenomegalic myelofibrosis. Report of 2 clinical cases].
	Grandis P. Ann Osp Maria Vittoria Torino. 1983;26(7-12):293-308.
	A description of the clinical and haematological features of splenomegalic idiopathic myelofibrosis is followed by the presentation of two personal cases, one with a classic picture of the advanced stage and marked, progressive anaemia, leukopenia, frank splenomegaly and renal alterations, the second in the initial stage, with liver and spleen enlargement, very marked thrombocytosis and high white and red cell counts.
7315844	MALE	Middle Aged
	Extramedullary blast transformation in the central nervous system in idiopathic myelofibrosis.
	Barnes HM, Prchal JT, Scott CW. Am J Hematol. 1981;11(3):305-8.
	Idiopathic myelofibrosis (IM) is a myeloproliferative syndrome characterized by anemia, fibrosis of the bone marrow, variable proliferation of megakaryocytes, myelocytic and erythrocytic precursors, and splenomegaly.
445365	FEMALE
	Idiopathic myelofibrosis without splenomegaly.
	Cheng DS. Cancer. 1979;43(5):1761-5.
	The rarity of the absence of significant splenomegaly and the presence of large numbers of circulating myeloblasts in idiopathic myelofibrosis are reviewed.
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.