2001 Volume 40 Issue 9 Pages 878-882
Objective B-cell lymphoma-associated hemophagocytic syndrome (B-LAHS) has been increasingly reported in Asia and is regarded as a variant of intravascular lymphomatosis (IVL). Recently CDS was reported to be expressed in some cases of diffuse large cell lymphoma and IVL. We therefore examined the expression of CDS on lymphoma cells in B-LAHS and compared the clinical and laboratory data between CD5+ and CD5- B-LAHS.
Methods The expression of CDS on lymphoma cells was examined using flow cytometry and immunohistochemical analysis of paraffin sections. The clinical records were reviewed to characterize clinical features.
Patients Twelve patients with B-LAHS; ten men and two women, age ranging from 41 to 82 years (median, 63.5 years) were included in this study.
Results B-LAHS is characterized by fever and hepatosplenomegaly without lymphadenopathy at the initial presentation. Histological examination showed hemophagocytosis and infiltration of lymphoma cells in the bone marrow, and in some cases intravascular proliferation of lymphoid cells characteristic of IVL. All patients showed increased levels of lactate dehydrogenase, C-reactive protein, ferritin and soluble interleukin-2 receptor. In eight of the twelve patients, lymphoma cells were positive for CDS. But no differences were observed in the clinical or laboratory findings between CD5+ B-LAHS and CDS- B-LAHS.
Conclusion No clinical differences were observed between CDS* B-LAHS and CDS- B-LAHS. Further studies are required to elucidate the differences in pathogenesis between these two subgroups of B-LAHS.
(Internal Medicine 40: 878-882, 2001)
