ISSN: 1300-7777 E-ISSN: 1308-5263
Primary autoimmune myelofibrosis: a report of three cases and review of the literature [Turk J Hematol]
Turk J Hematol. 2009; 26(3): 146-150

Primary autoimmune myelofibrosis: a report of three cases and review of the literature

Rakhee Kar, Shyamali Dutta, Seema Tyagi
Department Of Hematology, All India Institute Of Medical Sciences, New Delhi, India

Myelofibrosis in association with autoimmune disorders has been consistently recognized in sporadic case reports over a number of years. Autoimmune myelofibrosis has been described most commonly in association with systemic lupus erythematosus (SLE). In addition, myelofibrosis presenting as cytopenias and showing clinical response to immunosuppressant drugs, notably steroids, has been reported with a wide range of immune-mediated disorders, including Sjögren’s syndrome, polyarteritis nodosa, rheumatoid arthritis, ulcerative colitis, and primary biliary cirrhosis. Attempts have been
made to define a syndrome of primary autoimmune myelofibrosis (PAIMF), as a distinct steroid-responsive clinicopathologic entity with excellent prognosis. Herein, we describe three cases of autoimmune myelofibrosis with a review of the literature.

Keywords: Myelofibrosis, autoimmune, leukoerythroblastosis, splenomegaly, autoantibodies


Primer otoimmün miyelofibrozis-üç olgunun sunumu ve literatürün gözden geçirilmesi

Rakhee Kar, Shyamali Dutta, Seema Tyagi
Department Of Hematology, All India Institute Of Medical Sciences, New Delhi, India

Otoimmün bozukluklar ile ilişkili miyelofibrozis birkaç yıldan bu yana, sürekli olarak sporadik olgu sunumlarında göze çarpmaktadır. Otoimmün miyelofibrozis en yaygın olarak sistemik lupus eritematozus (SLE) ile ilişkili olarak tanımlanmaktadır.
Ayrıca, sitopeni ile kendini gösteren ve immün sistemi baskılayan ilaçlara, özellikle steroidlere, klinik yanıt oluşturan miyelofibrozis çok sayıda immun-aracılı bozuklukla beraber bildirilmektedir. Bunlar Sjögren sendromu, poliarteritis nodosa, romatoid
artrit, ülseratif kolit ve primer biliyer sirozu içermektedir. Çok iyi bir prognoza sahip, steroide yanıt veren farklı bir klinikopatolojik antite olarak primer otoimmün miyelofibrozis (POIMF) sendromunu tanımlama girişimlerinde bulunulmuştur.
Biz, burada literatürü gözden geçirerek, üç otoimmün miyelofibrozis olgusunu tanımlamaktayız.

Anahtar Kelimeler: Miyelofibrozis, otoimmün, lökoeritroblastozis, splenomegali, otoantikorlar


Rakhee Kar, Shyamali Dutta, Seema Tyagi. Primary autoimmune myelofibrosis: a report of three cases and review of the literature. Turk J Hematol. 2009; 26(3): 146-150

Corresponding Author: Seema Tyagi, India


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