Delayed Treatment Response in a Neonate with Multisystem Langerhans Cell Histiocytosis Case report and review of literature.

Anirban Mandal, Amitabh Singh, Lavleen Singh, Sataroopa Mishra, Ankita Patel

Journal: Sultan Qaboos University medical journal 2018;17(2):e225-e228

PMID: 28690898

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Abstract

Langerhans cell histiocytosis (LCH) is a rare proliferative disorder of the Langerhans cells, which are part of the mononuclear phagocytic system. The 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-specific 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. There was a delayed response to combined chemotherapy with no major side-effects.

Address: Department of Pediatrics, Chacha Nehru Bal Chikitsalya, New Delhi, India.; Department of Pediatrics, Sitaram Bhartia Institute of Science and Research, New Delhi, India.; Department of Pathology, Chacha Nehru Bal Chikitsalya, New Delhi, India.

DOI: 10.18295/squmj.2016.17.02.016

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MeSH Terms: Anti-Inflammatory Agents, Antineoplastic Agents, Phytogenic, Biopsy, Delayed Diagnosis, Disease Progression, Drug Therapy, Combination, Exanthema, Histiocytosis, Langerhans-Cell, Humans, India, Infant, Langerhans Cells, Lung, Male, Prednisone, Skin, Vinblastine