We describe a case of multicentric
Castleman disease with generalized
lymphadenopathy and
splenomegaly, accompanied by typical B symptoms - loss of 15 kg,
fever of non-infectious origin, night sweats, symptoms of
anemia. Histological examination of the nodes with the highest accumulation of fluorodeoxyglucose, taken from mediastinum by thoracoscopy, revealed plasmocellular type of
Castleman disease. Tests for HIV and human herpesvirus 8 (HHV-8) were negative. Three recurrences of
herpes zoster indicating an alteration of immunity preceded the
dia-gnosis of disease. Treatment was initiated with combination of
thalidomide,
dexamethasone, and
cyclophosphamide. The response after 2 months
therapy was not clear and patient doesn't tolerated the
therapy well. Therefore, this treatment was terminated and R-CHOP (
Mabthera -
rituximab,
cyclophosphamide,
adriamycin,
vincristine, and
prednisone) was selected as a second-line
therapy.
Lymphadenopathy and
splenomegaly were reduced during the 2 cycles of treatment, however, serious infectious complications accompanied the
therapy. Therefore, only use of
Mabthera monotherapy 375 mg /m2 was administered in 28-day intervals. This treatment has shown efficacy and tolerability. PET-CT scan has demonstrated disappearance of
lymphadenopathy and
splenomegaly, in addition, normalized accumulation of fluorodeoxyglucose. Monotherapy with
Mabthera has proved to be effective and well tolerated
drug in this case. Currently, there are more effective therapeutic alternatives in multicentric
Castleman disease: treatment with monotherapy of
rituximab or in combination
therapy with
immunomodulatory drugs (
thalidomide or
lenalidomide, treatment with anti-IL-6 (
siltuximab) or against its receptor (
tocilizumab). In the case of ineffectiveness of one treatment option must be tested other alternative. In this case the
therapy based on
thalidomide wasn't successful, whereas the treatment with
Mabthera has achieved disappearance of disease symptoms.