Multisentrik Castleman Hastalığı ve Proteinüri; 12 Yaşında Bir Çocuk Vakanın Takdimi

Year 2011, Volume: 5 Issue: 1, 44 - 48, 01.12.2011

Suna Emir Ümmühan Çay Nermin Uncu Emrah Şenel Sema Apaydın

Abstract

Multisentrik Castleman hastalığı, çocuklarda nadir görülen ve nedeni bilinmeyen lenfoproliferatif bir hastalıktır. Castleman hastalığında renal tutulum sık olmayıp erişkinlerde multisentrik Castleman hastalığıyla ilişkili proteinüri nadiren bildirilmiştir.Bu yazıda, tekrarlayan karın ağrısı ve ishal şikayeti ile başvuran ve laboratuvar incelemelerinde anemi, yüksek eritrosit sedimentasyon hızı (ESH) ve fibrinojen seviyesi saptanarak başlangıçta familial Mediterranean fever (FMF) tanısı alan 12 yaşında bir erkek hasta sunuldu. Kolşisin tedavisi başlanan hastanın klinik ve laboratuvar bulgularında düzelme olmadı. İzleminde, 26 ay sonra, masif proteinüri gelişen hastanın böbrek biyopsisi normal bulundu. Tekrarlanan abdominal ultrasonografide (USG), 5x4cm. boyutunda intraabdominal kitlenin saptanması ve beraberinde anemi, hipergamaglobulinemi, akut faz göstergelerinin yüksekliği ve proteinüri olması nedeniyle tanıda Castleman hastalığı olabileceği düşünüldü. Cerrahi olarak çıkarılan kitlenin patolojik incelemesi Castleman hastalığı mikst tip olarak yorumlandı. Kitlenin total çıkarılmasından bir ay sonra anemi ve ESH yüksekliği düzeldi, masif proteinüri dramatik olarak geriledi.Olgumuz, Castleman hastalığının çocuklukda nadir görülmesi ve beraberinde proteinürinin bildirildiği ilk çocuk vaka olması nedeniyle önem taşımaktadır.

Keywords

Multisentrik, Castleman hastalığı, proteinüri, çocukluk çağı, renal tutulum, angiofolliküler, lenf nodu hiperplazisi

MULTICENTRIC CASTLEMAN DISEASE AND PROTEINURIA; CASE REPORT OF A 12 YEAR OLD BOY

Abstract

Multicentric Castleman disease is a rare lymphoproliferative disorder of unknown cause in children. Renal involvement of Castleman disease is uncommon. Proteinuria in association with multicentric Castleman disease has been reported to occur infrequently in only adult patients. We report a 12-year-old boy that presented with recurrent abdominal pain, diarrhea, anemia, high sedimentation rate (ESR) and fibrinogen level. He was suspected to have familial Mediterranean fever (FMF) and treated with colchicine. Unfortunately, he showed no marked improvement in the clinical and laboratory findings. Despite a comprehensive evaluation, his diagnosis remained elusive for several years. Eventually, three years later, he was found to have 42 gr/day of proteinuria. Renal biopsy was reported as normal. During the laboratory work-up for the etiology of proteinuria, an intraabdominal mass sized 5x4cm was found by abdominal ultrasonography. The patient’s clinical and laboratory findings such as anemia, pergamaglobulinemi, increased ESR and excessive proteinuria led us to think the diagnosis of Castleman disease. He underwent to the surgery with the presumptive diagnosis of Castleman disease. Pathologic examination was confirmed as Castleman disease mixed type. After removal of the mass, one month later, all the abnormal laboratory findings including anemia, excessive proteinuria, and increased ESH, fibrinogen and immunoglobulin levels disappeared. In this case, the proteinuria appears to be related to Castleman disease and removal of the mass relieved proteinuria. To our knowledge, this interesting case is the first report of the occurrence of proteinuria in a in a child with Castleman disease

Keywords

Multicentric Castleman disease, proteinuria, children, renal involvement, angiofollicular, lymph node hyperplasia

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