An Asian variant of intravascular large B-cell lymphoma: clinical, pathological and cytogenetic approaches to diffuse large B-cell lymphoma associated with haemophagocytic syndrome

Diffuse large B-cell lymphoma with haemophagocytic syndrome (BCL-HS) has be en reported mainly in Asia and is regarded as a distinct variant of intrava scular lymphoma (IVL). However, it is unclear whether all cases of BCL-HS f all within the framework of IVL and available clinical information is limit ed. We analysed 25 cases with BCL-HS, including 11 autopsied cases (median, 66 years; male-female ratio, 1.1:1). The patients presented with fever, an aemia, thrombocytopenia, hepatosplenomegaly, haemophagocytosis, bone marrow invasion, respiratory disturbance and disseminated intravascular coagulopa thy, but usually lacked lymphadenopathy, mass formation, neurological abnor malities and skin lesions. The clinical course was aggressive with a median survival of 7 months. The morphological findings were uniform: large lymph oid cells infiltrated vessels and/or sinusoids of the liver, marrow, lung, kidney and other organs. They were positive for CD19, CD20, CD79a and HLA-D R, but negative for CD10, CD23 and CD30. CD5 was positive in five out of 17 cases. Our critical review indicates that BCL-HS is the equivalent of the Asian variant of IVL.