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)
29866231	MALE	Middle Aged
	Lenalidomide Induced Toxic Epidermal Necrolysis and Del (5q): Co-occurrence of Rarities.
	Chaudhry QUN, Iftikhar R, Satti TM, Mahmood SK, Khan MA, Shahbaz N. J Coll Physicians Surg Pak. 2018;28(6):S89-S90.
	Primary myelofibrosis (PMF) is a clonal, BCR-ABL1 negative myeloproliferative neoplasm characterised by splenomegaly, leukoerythroblastic peripheral blood picture and bone marrow fibrosis.
29350220 (5715205)	OTHER
	Nephrotic syndrome in primary myelofibrosis with renal extramedullary hematopoiesis and glomerulopathy in the JAK inhibitor era.
	Del Sordo R, Brugnano R, Covarelli C, Fiorucci G, Falzetti F, Barbatelli G, Nunzi E, Sidoni A. Clin Nephrol Case Stud. 2017;5:70-77.
	Primary myelofibrosis (PMF) is an uncommon form of myeloproliferative neoplasm (MPN) characterized by a proliferation of predominantly megakaryocytes and granulocytes in the bone marrow that, in fully-developed disease, is associated with reactive deposition of fibrous connective tissue, extramedullary hematopoiesis (EMH), and splenomegaly.
28775247 (5551928)	MALE	Middle Aged
	Spinal Abscess Caused by Salmonella Bacteremia in a Patient with Primary Myelofibrosis.
	Fareed S, Nashwan AJ, Abu Jarir S, Husain A, Suliman DS, Ibrahim F, Moustafa A, Akhter MS, Yassin MA. Am J Case Rep. 2017;18:859-864.
	CASE REPORT We are reporting the case of a 50-year-old male with primary myelofibrosis and long-standing splenomegaly with back pain as a presenting symptom who was found to have spinal cord compression.
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.
25034748	MALE
	Acute respiratory distress syndrome in a patient with primary myelofibrosis after ruxolitinib treatment discontinuation.
	Beauverd Y, Samii K. Int J Hematol. 2014;100(5):498-501.
	We present a case of a 76-year-old man diagnosed with primary myelofibrosis who presented with constitutional symptoms and symptomatic splenomegaly.
25741217 (4347479)	OTHER
	Translocation t(3;12)(q26;q21) in JAK2(V617F) Point Mutation Negative Chronic Idiopathic Myelofibrosis: A Case Report.
	Meanovic S, ahovic H, Peric M. Balkan J Med Genet. 2014;17(1):63-8.
	Chronic idiopathic myelofibrosis (CIMF) is a stem cell defect characterized by splenomegaly with multiorgan extramedullary hematopoiesis, immature peripheral blood granulocytes and erythrocytes and progressive bone marrow fibrosis.
23039051 (3515333)	MALE
	Disseminated tuberculosis in a patient treated with a JAK2 selective inhibitor: a case report.
	Colomba C, Rubino R, Siracusa L, Lalicata F, Trizzino M, Titone L, Tolomeo M. BMC Res Notes. 2012;5:552.
	Primary myelofibrosis is a myeloproliferative disorder characterized by bone marrow fibrosis, abnormal cytokine expression, splenomegaly and anemia.
21393891	MIXED_SAMPLE	Middle Aged
	JAK2-positive Philadelphia-negative myeloproliferative neoplasms.
	Sharma A, Buxi G, Marwah S, Yadav R. Indian J Pathol Microbiol. 2011;54(1):117-20.
	Here are two cases which incidentally presented with splenomegaly and moderate leukocytosis, and were diagnosed as MPN-primary myelofibrosis (PMF) in prefibrotic phase and polycythemia vera (PV), respectively, using revised World Health Organization (WHO) 2008 criteria.
20181038 (2830976)	OTHER
	Acute variceal bleeding in a patient with idiopathic myelofibrosis successfully treated with endoscopic variceal band ligation and chemotherapy: a case report.
	Tamaki K, Otaka M, Sakamoto N, Matsumoto K, Yamashina S, Watanabe S. J Med Case Rep. 2010;4:25.
	Idiopathic myelofibrosis is a chronic myeloproliferative disorder characterized by leukoerythroblastosis, massive splenomegaly, and increases in the reticular and collagen fibers in the bone marrow.
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.
22958626 (3436631)	OTHER
	Pulmonary hypertension diagnosed by echocardiography during idiopathic myelofibrosis. A case report and a brief review of the literature.
	Perrone C, Cartolari R, Lupi B, Morelli S. Multidiscip Respir Med. 2010;5(4):267-70.
	Idiopathic myelofibrosis is a rare chronic myeloproliferative disease leading to extramedullary hematopoiesis (myeloid metaplasia) with splenomegaly.
20700988	FEMALE	Adult
	[Agnogenic myeloid metaplasia in pregnancy. Case report].
	Manole I, Costachescu G, Aldea MJ, Gavrilut M, Dumitrascu I. Rev Med Chir Soc Med Nat Iasi. 2010;114(2):465-9.
	Idiopathic myelofibrosis is a rare myeloproliferative disorder characterized by excessive accumulation of connective tissue in the bone marrow in association with anemia, splenomegaly and extramedullary hematopoiesis.
16321815	MALE
	Agnogenic myeloid metaplasia with pulmonary hematopoiesis.
	Boula A, Mantadakis E, Xilouri I, Foudoulakis A, Samonis G. Hematology. 2005;10(6):501-3.
	Agnogenic myeloid metaplasia (AMM) is characterized by bone marrow fibrosis, splenomegaly and leukoerythroblastic anemia and is frequently accompanied by extramedullary hematopoiesis (EMH).
14629130	FEMALE	Middle Aged
	Reversal of marrow fibrosis in agnogenic myeloid metaplasia by allogeneic peripheral blood stem cell transplantation.
	Udomsakdi-Auewarakul C, Visuthisakchai S, Sangruchi T, Issaragrisil S. Asian Pac J Allergy Immunol. 2003;21(2):121-6.
	Agnogenic myeloid metaplasia (AMM) is a clonal hematopoietic stem cell disorder characterized by bone marrow fibrosis, extramedullary hemopoiesis, splenomegaly and a leukoerythroblastic blood picture.
12884816	MALE	Middle Aged
	[Fatal septic shock and rhabdomyolysis following transfusion of platelet concentrates contaminated with Streptococcus pneumoniae].
	Katayama T, Kamiya M, Hoshina S, Masuoka H, Nishiwaki K, Sano K, Hagino T, Kobayashi M. Rinsho Ketsueki. 2003;44(6):381-5.
	The initial diagnosis of primary myelofibrosis was established by splenomegaly, leukoerythroblastosis and bone marrow fibrosis.
11167703	FEMALE	Middle Aged
	A case of porphyria cutanea tarda in association with idiopathic myelofibrosis and CREST syndrome.
	Lee SC, Yun SJ, Lee JB, Lee SS, Won YH. Br J Dermatol. 2001;144(1):182-5.
	She had presented with splenomegaly 3 years before the development of PCT, and was diagnosed as having idiopathic myelofibrosis, based on bone marrow biopsy.
11293952	MALE	Middle Aged
	Idiopathic myelofibrosis complicated by portal hypertension treated with a transjugular intrahepatic portosystemic shunt (TIPS).
	Belohlavek J, Schwarz J, Jirasek A, Krajina A, Polak F, Hruby M. Wien Klin Wochenschr. 2001;113(5-6):208-11.
	The authors report a 56-year-old man with idiopathic myelofibrosis and splenomegaly complicated by hepatopathy, severe portal hypertension and recurrent variceal bleeding.
8846142	MALE	Child
	Successful allogeneic bone marrow transplantation for agnogenic myeloid metaplasia in a 3-year-old boy.
	Rossbach HC, Grana NH, Chamizo W, Barrios NJ, Barbosa JL. J Pediatr Hematol Oncol. 1996;18(2):213-5.
	Agnogenic myeloid metaplasia (AMM) is a myeloproliferative disorder characterized by marrow fibrosis, extramedullary hematopoiesis, splenomegaly, and leukoerythroblastosis with abnormalities of red blood cell morphology.
8667677	FEMALE	Middle Aged
	[Splenomegalic pancytopenia of probable immune origin: cure after treatment with glucocorticoids].
	Fuentes A, Feliu E, Blade J, Campo E, Ribera JM, Cervantes F, Rozman C. Med Clin (Barc). 1996;106(7):263-6.
	The origin of the process was attributed probably to an immune etiology and differential diagnosis with idiopathic myelofibrosis and non tropical idiopathic splenomegaly must be considered.
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.