R. Lerza et al., Splenectomy induced complete remission in a patient with multicentric Castleman's disease and autoimmune hemolytic anemia, ANN HEMATOL, 78(4), 1999, pp. 193-196
Castleman's disease (CD) is a rare disorder of the lymphoid tissue in which
the clinical manifestations often mimic a malignant lymphoma. Despite the
absence of monoclonality of the lymphoid proliferation, the multicentric va
riant of the disease (MCD) is characterized by severe symptoms and poor pro
gnosis. Etiologic, pathogenetic, and therapeutic aspects of MCD are still u
ncertain. We report the case of a 57-year-old patient affected by MCD compl
icated by severe immunohemolytic anemia. Whereas the clinical and laborator
y response to steroids and chemotherapeutic agents was only partial, splene
ctomy induced a complete remission of hemolysis and disappearance of the co
nstitutional symptoms and of all generalized lymphadenopathies.