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 |
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| Institution: | 1. Primary Immunodeficiency Care and Research (PICAR) Institute, Taiwan;2. Department of Pediatrics, Division of Allergy, Asthma and Rheumatology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan;3. Department of Pediatrics, Division of Hematology/Oncology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan;1. Shiraz Institute for Cancer Research, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran;2. Department of Immunology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran;3. Neurosurgery Department, Shiraz University of Medical Sciences, Shiraz, Iran;1. Unidad de Investigación Biomédica. Delegación Zacatecas. Instituto Mexicano del Seguro Social, IMSS, C.P. 98000, Mexico;2. Centro de Investigación en Ciencias de la Salud y Biomedicina. CICSaB Universidad Autónoma de San Luis Potosí, UASLP, C.P. 78000, Mexico;3. Unidad Regional de Reumatología y Osteoporosis Hospital Central Dr. Ignacio Morones Prieto. San Luis Potosí, SLP, C.P. 78290, Mexico;4. Cátedras CONACYT- Unidad de Investigación Biomédica de Zacatecas-IMSS, Zacatecas, C.P. 98000, Mexico;5. Facultad de Medicina y Ciencias de la Salud, Universidad Autónoma de Zacatecas, Hospital Rural No. 51 IMSS Bienestar, Villanueva, Zacatecas, C.P. 99559, Mexico;1. School of Veterinary Medicine and Biomedical Sciences, University of Nebraska-Lincoln, Lincoln, NE, United States;2. Department of Biochemistry, University of Nebraska-Lincoln, Lincoln, NE, United States;3. Center for Biotechnology, University of Nebraska-Lincoln, Lincoln, NE, United States;1. Dipartimento di Medicina Veterinaria, Università degli Studi di Milano, Via Celoria 10, 20133, Milano, Italy;2. Dipartimento di Produzioni Animali, Università degli Studi della Tuscia, Via San Camillo de Lellis, 01100, Viterbo, Italy;1. Medical Sciences Faculty of Minas Gerais, Post-Graduate Program in Health Sciences, Minas Gerais, Brazil;2. Department of Physiology and Biophysics, INCT-Nanobiofar, ICB, Federal University of Minas Gerais, Brazil;3. Department of Biological Sciences, Laboratory of Experimental Pathophysiology, Federal University of Ouro Preto, Minas Gerais, Brazil |
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| Abstract: | BackgroundRecurrent 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.MethodsWe 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.ResultsOf 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.ConclusionsThe 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. |
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| Keywords: | Primary immunodeficiency disease Langerhans cell histiocytosis Persistent otorrhea Molluscum contagiosum-like lesion |
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