Hereditary Spherocytosis In An Adult [Tepecik Eğit Hast Derg]
Tepecik Eğit Hast Derg. 2012; 22(1): 63-65 | DOI: 10.5222/terh.2012.60804  

Hereditary Spherocytosis In An Adult

Cengiz Ceylan1, Selda Çakın1, Ülkü Ergene1
Tepecik Eğ. Ve Araşt. Hastn. İç Hastalıkları Kliniği İzmir

A 58 year old male complaining from fatigue and enlargement of the spleen during last 3 years was admitted.His uncle had been undergone a splenectomy in his family history.There were the enlargement of spleen (15 cm from arcus costarum)and the liver (3cm from arcus costarum)on his examination.His laboratory findings were: Hb: 5.2gr,reticulocyte 41 %, 30 percent spherocytosis in periferal blood smear.Direct and indirect Coombs tests were negative.Glucose 6 phosphate dehydrogenase level was normal.There was increased activity of erythropoiesis in his bone marrow exam.Osmotic fragility of his erythrocytes has increased.He experienced no complication after splenectomy.

Keywords: Adult,familiary,hemolytic anemia,hepato-splenomegaly,spherocytosis


Erişkinde Ailesel Sferositoz

Cengiz Ceylan1, Selda Çakın1, Ülkü Ergene1
Tepecik Eğ. Ve Araşt. Hastn. İç Hastalıkları Kliniği İzmir

58 yaşındaki erkek hastada 3 yıldır halsizlik ve dalak büyüklüğü öyküsü vardı.Aile öyküsünde dayısının dalağı ameliyatla alınmıştı.Fizik bakıda Kosta kavsini dalak 15cm,karaciğer 3cm aşıyordu.Hemoglobin 5.2gr,retilolosit%41 ve periferik yaymada %30 sferositoz saptandı.Total bilirübin %2.6mg ve CRP 30.5 mg/dl idi.Direk ve indirek Coombs testleri olumsuzdu.Glukoz 6 fosfat dehidrogenaz normal bulundu.Kemik iliği aspirasyonunda artmış hemopoez saptandı.Eritrositlerin osmotik frajilitesinin arttığı saptandı.Vitamin B12 düzeyi düşüktü.Splenektomi sonrası hemolitik kriz görülmedi.

Anahtar Kelimeler: Erişkin,familyer, hemolitik anemi, hepatosplenomegali,sferositoz


Cengiz Ceylan, Selda Çakın, Ülkü Ergene. Hereditary Spherocytosis In An Adult. Tepecik Eğit Hast Derg. 2012; 22(1): 63-65


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