Evans sendromlu olguda tekrarlayan derin ven trombozu atakları ve faktör V leiden heterozigot mutasyon birlikteliği: nadir bir olgu
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Date
2012-08-10
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Uludağ Üniversitesi
Abstract
Kanamaya meyil oluşturan Evans sendromu ile tromboza meyil oluşturan trombofilik durum birlikteliği oldukça nadir görülmektedir. Olgumuz Mart 2009’ da İTP tanısı almış olup (trombosit: 5710), takiplerinde 3 ay sonra derin ven trombozu (DVT) gelişmesi üzerine incelenip; aile öyküsü ve edinsel risk faktörleri dışlandıktan sonra heterozigot faktör V leiden mutasyonu saptandı. Düşük molekül ağırlıklı heparin (DMAH) ile 1 yıl antikoagülan tedavi verildi. Olguya İTP nüksleri nedeni ile tanıdan 7 ay sonra ikinci basamak tedavi olarak splenektomi yapıldı. Splenektomiye yanıtlı olan olgunun takiplerinde anemi gelişmesi üzerine tetkik edilip Evans sendromu tanısı kondu. Splenektomiden 16 ay sonra ikinci DVT atağı gelişen olguya uzun süreli DMAH tedavisi planlandı. Olgu splenektomiden 19 ay sonra hemogramı normal olup, herhangi kanama diyatezi yokken muhtemel tromboza bağlı bir komplikasyon sonucu ani ölüm ile kaybedildi.
The association of Evans sydrome which has tendency to bleeding with thrombophilic condition which has tendency to thrombosis is too rare. Our 40 years male case was diagnosed with immun thrombocitopenic purpura (İTP) in March 2009 (thrombocyte: 5710). On his follow-up after three mounts deep venous thrombosis (DVT) developed. Then upon investigation of history, clinic and laboratory of the case we found heterozygous factor V mutation. Treatment with low molecule weight heparine (LMWH) was administered during one year. After 7 mounts of diagnosis splenectomy was performed as a second line therapy because of recurren İTP relapses and afterward he remained in remission. After 7 mounts of splenectomy our case was diagnosed with Evans sydrome while researching the anemia which was detected just at that time. After 16 mounts of splenectomy second deep venous thrombosis event occured, than we planned long term treatment with DMWH. Our patient was lost with a sudden death after 19 mounts of splenectomy probably due to a new event associated with thrombosis.
The association of Evans sydrome which has tendency to bleeding with thrombophilic condition which has tendency to thrombosis is too rare. Our 40 years male case was diagnosed with immun thrombocitopenic purpura (İTP) in March 2009 (thrombocyte: 5710). On his follow-up after three mounts deep venous thrombosis (DVT) developed. Then upon investigation of history, clinic and laboratory of the case we found heterozygous factor V mutation. Treatment with low molecule weight heparine (LMWH) was administered during one year. After 7 mounts of diagnosis splenectomy was performed as a second line therapy because of recurren İTP relapses and afterward he remained in remission. After 7 mounts of splenectomy our case was diagnosed with Evans sydrome while researching the anemia which was detected just at that time. After 16 mounts of splenectomy second deep venous thrombosis event occured, than we planned long term treatment with DMWH. Our patient was lost with a sudden death after 19 mounts of splenectomy probably due to a new event associated with thrombosis.
Description
Uludağ Üniversitesi Tıp Fakültesi 8. İç Hastalıkları Kış Kongresi’nde sunulmuştur.
Keywords
Evans syndrome, Tekrarlayan derin ven trombozu, Heterozigot faktör V leiden mutasyonu, Recurrent deep venous thrombosis, Heterozygous factor V leiden mutation
Citation
Keni, N. vd. (2012). "Evans sendromlu olguda tekrarlayan derin ven trombozu atakları ve faktör V leiden heterozigot mutasyon birlikteliği: nadir bir olgu". Uludağ Üniversitesi Tıp Fakültesi Dergisi, 38(3), 207-209.