The influence of clinical features mimicking primary immunodeficiency diseases (mPID) on children with Langerhans cell histiocytosis (LCH) - Four with mPID among 39 LCH children from one referral center during 18-year period
| Autor: | Liang-Shiou Ou, Jing-Long Huang, Tsung-Yen Chang, Tang-Her Jaing, Wen-I Lee, Tsung-Chieh Yao, Iou-Jih Hung, Shiuan-Chen Lin, Shih-Hsiang Chen, Li-Chen Chen, Chao-Ping Yang |
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| Rok vydání: | 2019 |
| Předmět: |
0301 basic medicine
Male Pediatrics medicine.medical_specialty Primary Immunodeficiency Diseases Immunology 03 medical and health sciences 0302 clinical medicine Refractory Langerhans cell histiocytosis Recurrence medicine Immunology and Allergy Humans In patient Child Referral and Consultation Retrospective Studies Medical review business.industry Infant Hematology medicine.disease Prognosis Transplantation Histiocytosis Langerhans-Cell 030104 developmental biology medicine.anatomical_structure Child Preschool Primary immunodeficiency Referral center Female Bone marrow business 030215 immunology |
| Zdroj: | Immunobiology. 225(2) |
| ISSN: | 1878-3279 |
| Popis: | Background Recurrent or refractory infections can be a warning sign of primary immunodeficiency diseases (PID). Such mimicking PID (mPID) can occur in patients with Langerhans cell histiocytosis (LCH). Because some cases with refractory molluscum contagiosum-like lesions and persistent otorrhea are finally diagnosed with LCH, we wondered whether such mPID can occur in LCH children and affect on their prognosis. Methods We retrospectively reviewed all children with LCH at our institute from 2001 to 2018. A complete medical review of sex, age, symptoms, treatment course, and outcome comparison was performed. Results Of 39 enrolled LCH patients, three had persistent otorrhea and one had refractory molluscum contagiosum-like lesions despite aggressive antibiotic therapy. These four cases with mPID had significantly higher rates of multi-system involvement, recurrence and 5-month more lag time, but no risk organ (liver, spleen and bone marrow) involvement compared to those without mPID, although bone and skin were the most involved in both groups. Overall, the lag-time in multi-system was longer than that in single-system involvement (median 2.5 vs. 1.0 months; p = 0.003). The diagnosis-age of risk organ involvement was younger than those without (median 8 vs. 43 months; p = 0.004). There were no significant differences in diagnosis-age, single/multi-system and risk organ involvement between remission and recurrence groups. All were alive excluding four who were lost to follow-up. Conclusions The LCH children with mPID had greater lag time, multi-system involvement, recurrence and more refractory treatment including transplantation despite the ratio of bone and skin lesions equal to those without mPID. |
| Databáze: | OpenAIRE |
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