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.

Myelofibrosis

Replacement of bone marrow by fibrous tissue.

Total: 60

(per page)

PMID (PMCID)
30089953 (6060584)	OTHER
	Cytodiagnosis of Coexistence of Leukemic Infiltration and Extramedullary Hematopoiesis in a Cervical Lymph Node, in T Cell Leukemia Patient.
	Bothale A, Bothale K, Mahore S, Dongre T. J Cytol. 2018;35(3):189-192.
	Although EMH is known to occur in agnogenic myeloid metaplasia with myelofibrosis, chronic myelogenous leukemia, thalassemia, and infiltrative disorders, such as lymphomas, it is rare in acute leukemias.
29745886	FEMALE
	Primary myelofibrosis but not autoimmune myelofibrosis accompanied by Sjogren's syndrome and primary biliary cirrhosis in a patient with trisomy 8 mosaic: a case report and literature review.
	Lu C, Wu X, Wen H, Gao H, Wang C, Yang B, Liang Z, Gao C, Li X. Clin Exp Rheumatol. 2018;36 Suppl 112(3):234-236.
	Primary myelofibrosis but not autoimmune myelofibrosis accompanied by Sjogren's syndrome and primary biliary cirrhosis in a patient with trisomy 8 mosaic: a case report and literature review.
27874969	FEMALE
	Idiopathic myelofibrosis accompanied by peritoneal extramedullary hematopoiesis presenting as refractory ascites in a dog.
	Rautenbach Y, Goddard A, Clift SJ. Vet Clin Pathol. 2017;46(1):46-53.
	Although idiopathic myelofibrosis is a relatively rare condition in dogs, this case demonstrates that ascites caused by peritoneal implants of hematopoietic tissue may be the initial manifestation of myelofibrosis.
27734802	MALE	Middle Aged
	An usual cause of elliptocytosis.
	Broseus J, Roth-Guepin G, D'Aveni-Piney M, Perrot A, Lesesve JF, Perrin J. Ann Biol Clin (Paris). 2016;74(6):704-707.
	The objectification of elliptocytosis accompanied by splenomegaly, a collagen myelofibrosis and the presence of the mutation JAK2V617F allowed the diagnosis of primary myelofibrosis with atypical initial presentation.
26153565	FEMALE
	JAK2-positive cutaneous myelofibrosis presenting as sclerosing extramedullary hematopoietic tumors on the scalp: case presentation and review of the literature.
	LeBlanc RE, Lester L, Kwong B, Rieger KE. J Cutan Pathol. 2015;42(11):858-62.
	Our patient died of disease several months following the appearance of her lesions, which is in keeping with other reports that suggest that cutaneous myelofibrosis may serve as an independent poor prognostic sign in otherwise advanced primary myelofibrosis.
26153565	FEMALE
	JAK2-positive cutaneous myelofibrosis presenting as sclerosing extramedullary hematopoietic tumors on the scalp: case presentation and review of the literature.
	LeBlanc RE, Lester L, Kwong B, Rieger KE. J Cutan Pathol. 2015;42(11):858-62.
	We report the second case of cutaneous myelofibrosis with a documented JAK2 activating mutation involving the scalp of a 67-year-old woman with primary myelofibrosis in her marrow.
23117264	MIXED_SAMPLE	Middle Aged
	Splenic irradiation as a component of a reduced-intensity conditioning regimen for hematopoietic stem cell transplantation in myelofibrosis with massive splenomegaly.
	Ito T, Akagi K, Kondo T, Kawabata H, Ichinohe T, Takaori-Kondo A. Tohoku J Exp Med. 2012;228(4):295-9.
	The first patient was a 60-year-old woman who received peripheral blood stem cell transplantation for post-essential thrombocythemia myelofibrosis from an HLA-identical sibling; the second patient was a 60-year-old man who received unrelated bone marrow transplantation for primary myelofibrosis.
21188553	MALE	Infant
	Idiopathic myelofibrosis in an infant.
	Bavikar RR, Kulkarni RK, Rathod AD, Hastak MS. Indian J Pediatr. 2011;78(6):734-6.
	Cases of secondary myelofibrosis associated with Vitamin D deficiency and Systemic Lupus Erythematosus have been reported from India .In this case report, the authors describe clinical signs, laboratory findings and histologic features in a 6 month old infant with Idiopathic myelofibrosis.
19785025	MALE	Child
	Chronic idiopathic myelofibrosis with myeloid metaplasia presenting as refractory ascites.
	Patra B, Maheshwari A, Chandra J, Aneja S, Agarwal S, Nangia A, Seth A. Pediatr Blood Cancer. 2010;54(1):151-3.
	Chronic idiopathic myelofibrosis (CIM) with myeloid metaplasia is a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extra-medullary hematopoesis (EMH), and varying degree of myelofibrosis.
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.
18243425	FEMALE	Middle Aged
	[Pleural extramedullary hematopoiesis].
	Mrabti H, Chelghoum M, Odier L, Chassagne-Clement C, Pavic M, Devaux Y. Rev Med Interne. 2008;29(7):573-6.
	Agnogenic myeloid metaplasia, associated with myelofibrosis, is a myeloproliferative disorder.
17762725	MALE	Middle Aged
	A meningioma with islets of extramedullary myeloid metaplasia: case report.
	Zona G, Spena G, Sbaffi PF, Spaziante R. Neurosurgery. 2007;61(2):E418-9; discussion E419.
	Idiopathic myelofibrosis is a clonal stem cell disorder that leads to ineffective erythropoiesis accompanied by reactive myelofibrosis (bone marrow fibrosis).
17694960	FEMALE	Adult
	Extensive myelofibrosis responsive to treatment for acute erythroblastic leukaemia.
	Fadilah SA, Raja-Zahratul-Azma RS, Leong CF. Malays J Pathol. 2006;28(1):55-8.
	AML associated with significant myelofibrosis developing both de novo or secondary to primary (idiopathic) myelofibrosis is characterised by a fulminant course and extremely poor prognosis, primarily due to treatment-resistant disease.
15978949	MALE
	Hairy cell leukemia (HCL) with extensive myelofibrosis responds to thalidomide.
	Strupp C, Fenk R, Kundgen A, Gattermann N, Haas R, Germing U. Leuk Res. 2005;29(8):967-9.
	The diagnosis of idiopathic myelofibrosis was suggested by the bone marrow trephine biopsy, which was hypocellular with myelofibrosis.
16186683	MALE	Child
	Juvenile rheumatoid arthritis with myelofibrosis with myeloid metaplasia.
	Jain V, Maheshwari A, Gulati S, Kabra M, Kalra V. Indian J Pediatr. 2005;72(9):789-91.
	Myelofibrosis with myeloid metaplasia is defined as a myeloproliferative disorder characterized by leukoerythroblastosis, tear drop erythrocytes, extramedullary hematopoesis and varying degree of myelofibrosis.
15864476	MALE	Middle Aged
	Paravertebral pseudoaneurysm thrombosed after percutaneous thrombin injection.
	Lujan S, Puras E, Cancer S, Perera M. Ann Vasc Surg. 2005;19(3):421-4.
	We present a case of a lumbar pseudoaneurysm in a 62-year-old patient with myelofibrosis (agnogenic myeloid metaplasia).
11999361	FEMALE	Middle Aged
	A pleural effusion caused by fibrous hematopoietic tumor successfully treated with prednisolone in a patient with agnogenic myeloid metaplasia with myelofibrosis.
	Hirayama Y, Koyama R, Nagai T, Matsunaga T, Kogawa lK, Sakamaki S, Kokai Y, Niitsu Y. Int J Hematol. 2002;75(3):305-8.
	A pleural effusion caused by fibrous hematopoietic tumor successfully treated with prednisolone in a patient with agnogenic myeloid metaplasia with myelofibrosis.
12803124	FEMALE	Adult
	Autoimmune haemolytic anaemia as the presenting manifestation of agnogenic myeloid metaplasia.
	Mohite U, Pathare A, al Kindi S, Hiwase D, Vadakkepat N, Knox-Macaulay H. Haematologia (Budap). 2002;32(4):495-9.
	These findings confirmed the concomitant diagnosis of agnogenic myeloid metaplasia with myelofibrosis.
11583029	FEMALE	Adult
	Secondary myelofibrosis in visceral leishmaniasis--case report.
	Suvajdzic N, Pavlovic M, Misic S, Cemerikic V, Atkinson HD, Colovic M. Haematologia (Budap). 2001;31(2):167-71.
	A diagnosis was difficult to establish as VL is rarely encountered in the continental parts of Yugoslavia, and with the presence of associated myelofibrosis it could easily have been mistaken for chronic idiopathic myelofibrosis.
11426569	FEMALE
	Subcutaneous extramedullary hematopoiesis in a patient with secondary myelofibrosis following polycythemia vera.
	Tanaka M, Kanamori H, Yamaji S, Mishima A, Yamazaki E, Taguchi J, Aoba M, Fujisawa S, Mohri H, Ishigatsubo Y. Leuk Lymphoma. 2001;40(3-4):437-40.
	Although extramedullary hematopoiesis has been generally observed in primary myelofibrosis, in this case it was seen in myelofibrosis secondary to polycythemia vera.