Rosai-Dorfman Disease: A previously unreported association with Sickle Cell Disease
1 Specialist Registrar in Pediatrics, Guy's and St Thomas' Hospital, Lambeth Palace Road, London, SE1 7EH, UK
2 Consultant Histopathologist, Guy's and St Thomas' Hospital, Lambeth Palace Road, London, SE1 7EH, UK
3 Consultant Pediatrician with special interest in hemoglobinopathies, Guy's and St Thomas' Hospital, Lambeth Palace Road, London, SE1 7EH, UK
BMC Clinical Pathology 2007, 7:3 doi:10.1186/1472-6890-7-3Published: 2 April 2007
Rosai-Dorfman Disease is an uncommon benign systemic histio-proliferative disease. This is the first time the disease, although more common in people of African descent, is described in association with Sickle cell disease.
A Nigerian boy born started a complex medical history with post-natal anemia of unknown origin. Subsequently he was diagnosed with Sickle Cell Anemia (Hb SS). At age 3 during a routine review, he was noted to have generalised massive lymphadenopathy. He had further reoccurrences of this lymphadenopathy, but investigations did not reveal the cause until age five. At this point, because of the progressive lymph node enlargement, a biopsy was performed, and he was diagnosed with Rosai-Dorfman Disease. Since that time, the child has had further episodes of intermittent massive lymphadenopathy, particularly associated with Sickle Crisis. His medical history has been further complicated by development of complications from Sickle Cell Disease, cardiomyopathy and an autoimmune hemolytic anemia with multiple alloantibodies.
This case for the first time presents the co-existence of two diseases, of increased prevalence in those of African descent, but to date not described in the literature to occur concurrently.