A Solitary Variant Of Congenital Self-healing Reticulohistiocytosis: Solitary Hashimoto-pritzker Disease

Abstract

A three month old male infant presented with pallor, hepatomegaly (4.5 centimeters), splenomegaly (1.5 centimeters), anaemia (Hb 6 gm/dl) and thrombocytopenia (16 x 109/l). A liver biopsy was diagnostic for Langerhans cell histiocytosis (histiocytosis X). The patient’s lymphocytes, co-cultured with neonatal lymphocytes, were positive for virus-like particles without reverse transcriptase activity. The hepatomegaly diminished after 6 months and a second liver biopsy showed decreased histiocytic infiltration. No treatment was given. A second viral blood culture remained negative. After 14 months, the hepatomegaly had disappeared completely and there were no more abnormal haematological or clinical findings.