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Laparoscopic Endoscopic Surgical Science Tans Olduka Gecikmi Bir mmn Yetmezlik Olgusu: Good's Sendromu [Respir Case Rep]
Respir Case Rep. 2017; 6(1): 36-40 | DOI: 10.5505/respircase.2017.48243

Tans Olduka Gecikmi Bir mmn Yetmezlik Olgusu: Good's Sendromu

Soner Umut Kver1, Fatma Tokgz Akyl1, Ercan Nain2, Meltem oban Aca1, Aya Kykm2, Aylin Gngr1, Tlin Sevim1
1Sreyyapaa Gs Hastalklar ve Gs Cerrahisi Eitim ve Aratrma Hastanesi, Gs Hastalklar Klinii, stanbul
2niversitesi Tp Fakltesi Hastanesi, ocuk Alerji ve mmnolojisi Hastalklar, stanbul

Goods sendromu (GS), timoma ve immn yetmez-liin birliktelii ile karakterize olduka nadir bir hastalktr. Altm alt yanda kadn hasta alt solunum yolu enfeksiyonu ve hipoksemik solunum yetmezlii tanlaryla kliniimize yatrld. On iki yl nce mediastinal kitle nedeniyle opere edilmi ve iyi diferansiye timik karsinom saptanmt. Son yllarda sk alt solunum yolu enfeksiyonlar yks mevcuttu. Eski akcier grntlemelerinde tekrarlayan akcier enfeksiyonlarn dndren bronektazi, peribronial kalnlamalar ve yer deitiren nodller saptand. Hastann yks, klinik ve radyolojik bulgular sonucunda immn yetmezlik n tans ile istenen immnglobulin (Ig) paneli ve lenfosit aktivitesi deerlendirmesinde, IgG seviyesi ve T lenfosit aktivitesinde dklk saptand ve timoma ve immn yetmezlik birliktelii olarak Goods sendromu tans konuldu. On drt gn antibiyoterapi sonunda dzelme salanan hasta immnoloji kliniine ynlendirilerek taburcu edildi. mmnoloji tarafndan intravenz immnglobulin ve proflaktik trimetoprim sulfametoksazol tedavisi balanan hastann enfeksiyon ataklar azald. Tedavinin ikinci ylnda stabil olarak takip edilmektedir.

Anahtar Kelimeler: Good's sendromu, immun yetmezlik, timoma

A Case Report with a Considerably Delayed Diagnosis of Immunodeficiency: Good's Syndrome

Soner Umut Kver1, Fatma Tokgz Akyl1, Ercan Nain2, Meltem oban Aca1, Aya Kykm2, Aylin Gngr1, Tlin Sevim1
1Department of Chest Diseases, Sreyyapaa Chest Diseases and Thoracic Surgery Training and Research Hospital, stanbul, Turkey
2Department of Pediatric Allergy and Immunology, Marmara University Hospital, stanbul, Turkey

Goods syndrome is a rare disease which is char-acterized by association of a thymoma and immunodeficiency. A 66-year-old woman was admitted with the diagnosis of lower respiratory tract infection and hypoxemic respiratory failure. Twelve years ago, she was operated for a mediastinal mass, and a well-differentiated thymic carcinoma was detected. She had a history of frequent recurrent lower respiratory tract infections. There were bronchiectasis, peribronchial thickening, and migratory nodules in past chest radiographs, suggesting recurrent pulmonary infections. Immunoglobulin (Ig) panel and lymphocyte activity were assessed with a preliminary diagnosis of immune deficiency. De-creased IgG levels and T lymphocyte activity were detected, and she was diagnosed with Good's syndrome. The patient showed clinical improvement at the end of a 14-day antibiotic therapy and was discharged to be referred to the immunology clinic. The infection episodes were reduced follow-ing intravenous immunoglobulin treatment, and prophylactic sulfamethoxazole trimethoprim treatment was initiated by the immunology clinic. She is still stable in the second year of follow-up.

Keywords: Good's syndrome, immunodeficiency, thymoma

Soner Umut Kver, Fatma Tokgz Akyl, Ercan Nain, Meltem oban Aca, Aya Kykm, Aylin Gngr, Tlin Sevim. A Case Report with a Considerably Delayed Diagnosis of Immunodeficiency: Good's Syndrome. Respir Case Rep. 2017; 6(1): 36-40

Sorumlu Yazar: Soner Umut Kver, Trkiye
Makale Dili: Trke
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