AI Summary & Key Takeaways
AI InsightAlthough Ham’s test and sugar water test of our patient were negative, with the results of bone marrow aspiration-biopsy, abdominal MRI and flow cytometry tests, we found out that pancytopenia was due to PNH with type II cells dominancy and CRF was due to hemosiderosis.
📌 3 Key Takeaways
Abstract
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare and acquired stem cell disease which is characterized with chronic intravasculer hemolysis and diffuse venous thrombosis. In PNH, renal failure is rare and acute renal failure, which is caused by hemolytic attacks that are usually trigerred by infection and surgical intervention, may happen. A 48-years old male patient who had chronic renal failure (CRF) and has been hemodialysing for about 11 years was admitted to our hospital with the symptoms of pancytopenia. Although Ham’s test and sugar water test of our patient were negative, with the results of bone marrow aspiration-biopsy, abdominal MRI and flow cytometry tests, we found out that pancytopenia was due to PNH with type II cells dominancy and CRF was due to hemosiderosis.
Özet
Paroksismal nokturnal hemoglobinüri (PNH) kronik intravasküler hemoliz ve yaygın venöz trombozlarla karekterize yaygın, kazanılmış bir kök hücre hastalığıdır. PNH’de böbrek yetmezliği nadirdir ve genellikle enfeksiyonun ve cerrahi müdahalelerin tetiklediği hemolitik ataklar sonucunda gelişen akut böbrek yetmezliği görülmüştür. Kronik böbrek yetmezliği (KBY) olan ve yaklaşık 11 yıldır hemodiyalize giren 48 yaşındaki erkek hasta pansitopeni semptomlarıyla hastanemize başvurdu. Hastanın yapılan şeker su ve Ham’s testleri negatif gelmesine rağmen, yapılan kemik iliği aspirasyon-biyopsisi, batın MR ve akım sitometri testleri neticesinde pansitopeninin, tip II hücrelerin çoğunlukta olduğu PNH’e ve KBY’nin ise hemosiderosise bağlı olduğu saptandı.