Histiocytosis in Nigerian children: A report of two variants

Abstract

Histiocytoses are a rare group of proliferative disorders with very similar clinical and histological pictures. We present a case report of two variants seen in an eight-month-old female and five-month-old male in a tertiary hospital in southern Nigeria. They both presented with painless neck swellings and fever, leucocytosis, neutrophilia and lymphopenia. Initial histologic examinations of the cervical lymph nodes biopsy posed a diagnostic conundrum. However, Immuno-histochemical analysis done on both sample showed CD1a, positive S100 in keeping with Langerhans cell histiocytosis in the former. While, that of the latter showed strongly positive CD68, positive S-100 in 30% cells in keeping with Sinus histiocytosis with massive lymphadenopathy (SLMH) in the latter. Clinicians should have a high index of suspicion for histiocytosis in children presenting with generalised lymphadenopathy. Also, apart from the routine histology, immunohistochemistry analysis is recommended for all cases