Presents with reactive hemophagocytic syndrome with anemia, thrombocytopenia, hepato-splenomegaly, absence of peripheral lymphadenopathy or mass formation
Rare CNS and skin involvement
Frequently involves liver, marrow, lung, kidney
Immunophenotype same as conventional intravascular lymphoma
Nearly all cases reported to date in Asians
Rare non-Asian cases reported
Poor prognosis, same as conventional intravascular lymphoma
Alternate names in literature
Malignant histiocytosis-like B-cell lymphoma
Diffuse large B-cell lymphoma with haemophagocytic syndrome
Intravascular lymphoma with haemophagocytic syndrome
The precise classification of this disorder or variant is not clear as apparently not all cases of Asian diffuse large B-cell lymphoma with haemophagocytic syndrome exhibit intravascular features
