Delayed Treatment Response in a Neonate with Multisystem Langerhans Cell Histiocytosis Case report and review of literature
Journal Title: Sultan Qaboos University Medical Journal - Year 2017, Vol 17, Issue 2
Abstract
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, which are part of the mononuclear phagocytic system. Te disorder varies in terms of the extent of the disease, its natural course and patient outcomes. While skin rashes are a common presentation of neonatal LCH, other systems or organs may also be involved. Delays in the diagnosis of neonatal LCH may occur due to its non-specifc presentation and a lack of awareness of the condition among doctors. We report a two-month-old male neonate who presented to the Chacha Nehru Bal Chikitsalya hospital, New Delhi, India, in 2016 after the onset of pulmonary symptoms. He had been noted to have a generalised rash which had progressively worsened from 15 days of age. Following a skin biopsy and chest imaging, he was diagnosed with multisystem LCH with risk organ involvement. Tere was a delayed response to combined chemotherapy with no major side-effects.
Authors and Affiliations
Amitabh Singh| Departments of Pediatrics, Chacha Nehru Bal Chikitsalya, New Delhi, India, Anirban Mandal| Department of Pediatrics, Sitaram Bhartia Institute of Science and Research, New Delhi, India, Lavleen Singh| Departments of Pathology, Chacha Nehru Bal Chikitsalya, New Delhi, India, Sataroopa Mishra| Departments of Pediatrics, Chacha Nehru Bal Chikitsalya, New Delhi, India, Ankita Patel| Departments of Pediatrics, Chacha Nehru Bal Chikitsalya, New Delhi, India
Keywords
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- EP ID EP14778
- DOI 10.18295/squmj.2016.17.02.016
- Views 334
- Downloads 15
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