Blastic OKT6-positive proliferation preceding malignant histiocytosis

A 45-year old male presented latero-cervical lymphoadenopathy. Biopsy revealed a malignant proliferation of immature "lymphoid" cells bearing T6 antigen and HLA-DR but negative for other lymphoid markers, suggesting a phenotype similar to Langerhans cells. The patient did not receive any therapy and six months later developed a histologically typical malignant histiocytosis, involving spleen and liver. Other reported cases of lymphoid malignancies (two bearing the T6 antigen on blast cells) preceding malignant histiocytosis were found and compared with ours. Most of these cases were characterized by the pediatric age of the patients and were presented as acute leukemias exhibiting, in at least some of them, reliable T-cell markers. Our case appears to represent, on the other hand, a blastic proliferation of precursors of both histiocytes and Langerhans dendritic cells which eventually progressed to malignant histiocytosis. The relevance of this observation in the debate on the origin of Langerhans cells and the relationships existing between macrophages and dendritic cells is discussed.