A Challenging Diagnosis of IgG4-Related Disease When Understanding Limitations of Laboratory Testing Was Pivotal
| Autor: | Kevin Higgins, Janey Hsiao, Arthur Bookman, Pak Cheung Chan, Victoria Y. Y. Xu, Alireza Zahirieh, Mary J. Bell, Zeina Ghorab |
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| Rok vydání: | 2017 |
| Předmět: |
Pathology
medicine.medical_specialty lcsh:Medicine Case Report Lacrimal gland Asymptomatic Lymphocytic Infiltrate 03 medical and health sciences 0302 clinical medicine Lymphoplasmacytic Infiltrate parasitic diseases Biopsy medicine 030203 arthritis & rheumatology medicine.diagnostic_test business.industry lcsh:R General Medicine medicine.disease medicine.anatomical_structure 030220 oncology & carcinogenesis Concomitant IgG4-related disease Bone marrow medicine.symptom business |
| Zdroj: | Case Reports in Medicine Case Reports in Medicine, Vol 2017 (2017) |
| ISSN: | 1687-9635 1687-9627 |
| DOI: | 10.1155/2017/8748696 |
| Popis: | A 76-year-old man was incidentally found on a CT scan to have lymphadenopathy and bilateral kidney enlargement suggestive of infiltrative renal disease. He was largely asymptomatic but had bilateral salivary and lacrimal gland enlargement. A grossly elevated serum IgG (>70 g/L) with concomitant suppression of other immunoglobulins, a small IgG restriction, and a parotid biopsy revealing lymphoplasmacytic infiltrate with slight kappa light chain excess all suggested a lymphoproliferative disorder (LPD). The diagnostic workup was further confounded by a normal serum IgG4 concentration. Moreover, bone marrow and renal biopsies did not reveal evidence of LPD. Discussion with the laboratory not only clarified that the markedly increased total IgG could not be accounted for by the small IgG restriction, but also identified a discrepancy in the IgG4 measurement. Repeat analysis of a follow-up sample revealed an elevated IgG4 of 5.94 (reference interval: 0.039–0.864) g/L, which prompted a repeat parotid biopsy that showed predominant IgG4+ lymphocytic infiltrates. Despite the deluding presentations, a final diagnosis of IgG4-related disease (IgG4-RD) was made based on elevated serum IgG4 concentrations and histopathological findings. This case highlights the importance of recognizing limitations of laboratory testing and the benefit of close communications among clinical subspecialties and the laboratory. |
| Databáze: | OpenAIRE |
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