Journals / Respiratory Case Reports / 2017 / Cilt: 6 - Sayı: 1

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

Tanısı Oldukça Gecikmiş Bir İmmün Yetmezlik Olgusu: Good's Sendromu

Pages
36–40
DOI
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

She is still stable in the second year of followup.

📌 3 Key Takeaways

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Objective & Hypothesis:Good's syndrome is a rare disease which is characterized by association of a thymoma and immunodeficiency.
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Key Findings & Data:A 66-year-old woman was admitted with the diagnosis of lower respiratory tract infection and hypoxemic respiratory failure.
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Conclusion & Impact:She is still stable in the second year of followup.
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

Good's syndrome is a rare disease which is characterized 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 welldifferentiated 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. Decreased 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 following intravenous immunoglobulin treatment, and prophylactic sulfamethoxazole trimethoprim treatment was initiated by the immunology clinic. She is still stable in the second year of followup.

Özet

Good's sendromu (GS), timoma ve immün yetmezliğin birlikteliği ile karakterize oldukça nadir bir hastalıktır. Altmış altı yaşında kadın hasta alt solunum yolu enfeksiyonu ve hipoksemik solunum yetmezliği tanılarıyla kliniğimize yatırıldı. On iki yıl önce mediastinal kitle nedeniyle opere edilmiş ve iyi diferansiye timik karsinom saptanmıştı. Son yıllarda sık alt solunum yolu enfeksiyonları öyküsü mevcuttu. Eski akciğer görüntülemelerinde tekrarlayan akciğer enfeksiyonlarını düşündüren bronşektazi, peribronşial kalınlaşmalar ve yer değiştiren nodüller saptandı. Hastanın öyküsü, klinik ve radyolojik bulguları sonucunda immün yetmezlik ön tanısı ile istenen immünglobulin (Ig) paneli ve lenfosit aktivitesi değerlendirmesinde, IgG seviyesi ve T lenfosit aktivitesinde düşüklük saptandı ve timoma ve immün yetmezlik birlikteliği olarak 'Good's sendromu' tanısı konuldu. On dört gün antibiyoterapi sonunda düzelme sağlanan hasta immünoloji kliniğine yönlendirilerek taburcu edildi. İmmünoloji tarafından intravenöz immünglobulin ve proflaktik trimetoprim sulfametoksazol tedavisi başlanan hastanın enfeksiyon atakları azaldı. Tedavinin ikinci yılında stabil olarak takip edilmektedir.

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Cite This Article

KÜVER, S. U., AKYIL, F. T., NAİN, E., AGCA, M. C., KIYKIM, A., GÜNGÖR, A., SEVİM, T. (2017). A Case Report with a Considerably Delayed Diagnosis of Immunodeficiency: Good's Syndrome. Respiratory Case Reports, Cilt: 6 - Sayı: 1, 36–40. https://ajindex.com/articles/302475

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References (1)

  1. Kelesidis T, Yang O. Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence. Clin Immunol 2010; 135:347-63. [CrossRef] 2. Kelleher P, Misbah SA. What is a Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol 2003; 56:12-6. 3. Tarr PE, Sneller MC, Mechanic LJ, Economides A, Eger CM, Strober W, et al. Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. Medicine (Baltimore) 2001; 80:123-33. 4. Souadjian JV, Enriquez P, Silverstein MN, Pepin JM. The spectrum of diseases associated with thymoma. Coincidence or syndrome? Arch Intern Med 1974; 134:374-9. [CrossRef] 5. Rosenow EC, Hurley BT. Disorders of the thymus. A Review. Arch Intern Med 1984; 144:763-70. [CrossRef] 6. Tarr PE, Lucey DR. Good's syndrome: the association of thymoma with immunodeficiency. Clin Infect Dis 2001; 33:585-6. [CrossRef] 7. Joven MH, Palalay MP, Sonido CY. Case report and literature review on Good's syndrome, a form of acquired immunodeficiency associated with thymomas. Hawaii J Med Public Health 2013; 72:56-62. 8. Kitamura A, Takiguchi Y, Tochigi N, Watanabe S, Sakao S, Kurosu K, et al. Durable hypogammaglobulinemia associated with thymoma (Good syndrome). Inter Med 2009; 48:1749-52. 9. Ohuchi M, Inoue S, Hanaoka J, Igarashi T, Tezuka N, Ozaki Y, et al. Good Syndrome coexisting with leukopenia. Ann Thorac Surg 2007; 84:2095-7. [CrossRef]