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

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

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.

References

  • Castleman B, Iverson L, Menendez VP. Localized mediasti- nal lymphnode hyperplasia resembling thymoma. Cancer 1956;9(4):822-30.
  • Baserga M, Rosin M, Schoen M, Young G. Multifocal Castle- man disease in pediatrics: case report. J Pediatr Hematol Oncol 2005;27(12):666-9.
  • Parez N, Blader-Meunier B, Roy CC, Dommergues JP. Paediatric Castleman disease: report of seven cases and review of the litera- ture. Eur J Pediatr 1999;158(8):631-7.
  • Akyüz C, Kale G, Emir S, Akçören Z, Büyükpamukçu M. T-cell lymphoma in a child with multicentric castleman disease. Med Pe- diatr Oncol 2000;35(2):133-4.
  • Keven K, Nergizoğlu G, Ateş K, Erekul S, Orhan D, Ertürk S, et al. Remission of nephrotic syndrome after removal of localized Castleman’s disease. Am J Kidney Dis 2000;35(6):1207-11.
  • Mandreoli M, Casanova S, Vianelli N, Pasquali S, Zucchelli P. Remission of nephrotic syndrome due to AA amyloidosis and ini- tiation of glomerular repair after surgical resection of localized Castleman’s disease. Nephron 2002;90(3):336-40.
  • Chan TM, Cheng IK, Wong KL, Chan KW. Resolution of mem- branoproliferative glomerulonephritis complicating angiofol- licular lymph node hyperplasia (Castleman’s disease). Nephron 1993;65(4):628-32.
  • Bowne WB, Lewis JJ, Filippa DA, Niesvizky R, Brooks AD, Burt ME, et al. The management of unicentric and multicentric Castle- man’s disease: a report of 16 cases and a review of the literature. Cancer 1999;85(3):706-17.
  • Nishimoto N, Sasai M, Shima Y, Nakagawa M, Matsumoto M, Shi- rai T, et al. Improvement in Castleman’s disease by humanized an- ti-interleukin-6 receptor antibody therapy. Blood 2000;95(1):56- 61.

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

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

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

References

  • Castleman B, Iverson L, Menendez VP. Localized mediasti- nal lymphnode hyperplasia resembling thymoma. Cancer 1956;9(4):822-30.
  • Baserga M, Rosin M, Schoen M, Young G. Multifocal Castle- man disease in pediatrics: case report. J Pediatr Hematol Oncol 2005;27(12):666-9.
  • Parez N, Blader-Meunier B, Roy CC, Dommergues JP. Paediatric Castleman disease: report of seven cases and review of the litera- ture. Eur J Pediatr 1999;158(8):631-7.
  • Akyüz C, Kale G, Emir S, Akçören Z, Büyükpamukçu M. T-cell lymphoma in a child with multicentric castleman disease. Med Pe- diatr Oncol 2000;35(2):133-4.
  • Keven K, Nergizoğlu G, Ateş K, Erekul S, Orhan D, Ertürk S, et al. Remission of nephrotic syndrome after removal of localized Castleman’s disease. Am J Kidney Dis 2000;35(6):1207-11.
  • Mandreoli M, Casanova S, Vianelli N, Pasquali S, Zucchelli P. Remission of nephrotic syndrome due to AA amyloidosis and ini- tiation of glomerular repair after surgical resection of localized Castleman’s disease. Nephron 2002;90(3):336-40.
  • Chan TM, Cheng IK, Wong KL, Chan KW. Resolution of mem- branoproliferative glomerulonephritis complicating angiofol- licular lymph node hyperplasia (Castleman’s disease). Nephron 1993;65(4):628-32.
  • Bowne WB, Lewis JJ, Filippa DA, Niesvizky R, Brooks AD, Burt ME, et al. The management of unicentric and multicentric Castle- man’s disease: a report of 16 cases and a review of the literature. Cancer 1999;85(3):706-17.
  • Nishimoto N, Sasai M, Shima Y, Nakagawa M, Matsumoto M, Shi- rai T, et al. Improvement in Castleman’s disease by humanized an- ti-interleukin-6 receptor antibody therapy. Blood 2000;95(1):56- 61.
There are 9 citations in total.

Details

Other ID JA68DH42UZ
Journal Section Research Article
Authors

Suna Emir This is me

Ümmühan Çay This is me

Nermin Uncu This is me

Emrah Şenel This is me

Sema Apaydın This is me

Publication Date December 1, 2011
Submission Date December 1, 2011
Published in Issue Year 2011 Volume: 5 Issue: 1

Cite

Vancouver Emir S, Çay Ü, Uncu N, Şenel E, Apaydın S. MULTICENTRIC CASTLEMAN DISEASE AND PROTEINURIA; CASE REPORT OF A 12 YEAR OLD BOY. Türkiye Çocuk Hast Derg. 2011;5(1):44-8.


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