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Hématologie

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Tropical splenomegalies: hyperreactive malarial splenomegaly and tropical splenic lymphoma with villous lymphocytes Volume 11, issue 4, Juillet-Août 2005

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Authors
Centre hospitalier universitaire Saint-Antoine, 184 rue du Faubourg Saint-Antoine, 75571 Paris Cedex 12, Clinique médico-universitaire Édouard-Rist, 15 rue Boileau, 75016 Paris
  • Key words: hyperreactive malarial splenomegaly, splenic lymphoma with villous lymphocytes, bcl-2/JH gene rearrangement, FR3/JH gene rearrangement
  • Page(s) : 259-63
  • Published in: 2005

In malaria-endemic African regions, tropical splenomegaly includes two clinical and biological entities: hyperreactive malaria splenomegaly and tropical splenic lymphoma with villous lymphocytes. The differential diagnosis of these two malaria-related lymphoproliferative disorders is difficult, because their clinical and cytological features are similar. Patients present major homogeneous splenomegaly, pancytopenia, high levels of IgM, and presence of circulating villous lymphocytes. Histopathologic and clonality studies in tropical splenomegaly patients remain sporadical. The presence of both hyperreactive malaria splenomegaly and tropical splenic lymphoma with villous lymphocytes in the same malaria-endemic population suggests an ethiopathogenic link between the two entities. Treatment consisting in sustained malarial prophylaxis induces remission in hyperreactive malaria splenomegaly patients, or significant clinical and biological improvement, in tropical splenic lymphoma with villous lymphocytes cases.