Abstract
A 3-year-old boy presented with irregular fever, multiple painless swelling over neck, axilla and inguinal region for one and half year. The child developed progressive pallor for one to two months. Examination revealed generalized lymphadenopathy including massive cervical lymphadenopathy, hepatosplenomegaly, severe anaemia and absence of jaundice. Here, a case of sinus histiocytosis with massive lymphadenopathy complicated by a rare immune dysregulation in the form of autoimmune haemolytic anaemia is reported.
Highlights
Sinus histiocytosis with massive lymphadenopathy (SHML) is a nonneoplastic, usually self limiting disease of unknown aetiology [1]
SHML most commonly presents as painless cervical lymphadenopathy with frequent involvement of other lymph nodes
Langerhans cell histiocytosis (LCH) was excluded in the present case by the absence of birbeck granules and CD1a positivity
Summary
Sinus histiocytosis with massive lymphadenopathy (SHML) is a nonneoplastic, usually self limiting disease of unknown aetiology [1]. SHML most commonly presents as painless cervical lymphadenopathy with frequent involvement of other lymph nodes. The authors report here one uncommon interesting case of generalised lymphadenopathy with autoimmune haemolytic anaemia. The neck swelling started with involvement on left side followed by right side. Fine needle aspiration cytology of cervical lymph node showed sheets of sinus histiocytes with some evidence of emperipolesis which means active penetration by a smaller cell into a larger cell.
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