Castleman disease is a rare idiopathic non-neoplastic
lymphoproliferative disorder with 2 clinical (unicentric and multicentric) and 3 histomorphological (hyaline-vascular, plasma-cell and mixed) forms identified. The case report given here describes the 3-year experience with
therapy in a patient, male born 1961, diagnosed with multicentric plasma-cell
Castleman disease (HIV and HHV-8 negative) with the finding of generalized
lymphadenopathy and
splenomegaly. During first line treatment (R-CHOP:
rituximab,
cyclophosphamide,
doxorubicin,
vincristine,
prednisone, 3 cycles in total, 12/2008-2/2009) the development of bilateral upper and lower limb edemas with clinical manifestation of
vasculitis occurred and a restaging computed tomography (CT) examination revealed a stable finding of the lymphadenomegaly. Greater success was achieved with
thalidomide regimen (CTD:
cyclophosphamide,
thalidomide,
dexamethasone, 10 cycles, 3/2009-1/2010) leading to reduction in the size of the hypervascularized lymph nodes (almost by 50%) as well as their
radiopharmaceutical (fluorodeoxyglucose) uptake as seen on a combined positron emission tomography and computed tomography (PET/CT) scan imaging.
Thalidomide was given daily at doses between 100 and 200 mg. We returned to the CTD regimen again in April 2010 after a short period of
monoclonal antibody tocilizumab treatment (400 mg intravenous in 2-week intervals with 50%
dose reduction due to a limited supply of the
drug, 5 doses in total) during which edemas reoccurred with a CT scan finding of stable lymphadenomegaly. However, the renewed regimen with
thalidomide was stopped after 2.5 cycles due to adverse effects of
thalidomide (neuropathy) and
corticoids (
Cushing syndrome). In September 2010, after enrollment in the Celgenes Compassionate Use Program we were able to start treating the patient with the derivative of
thalidomide,
lenalidomide, at a dosage of 25 mg on days 1-21 in a 28-day cycle, 15 cycles in total (10/2010-12/2011). The monotherapy with
lenalidomide was very well tolerated by the patient without any effects of myelotoxicity,
thromboembolism or relapses of edemas and
vasculitis, additionally now with apparent improvement of fatic disorder and the patients motor abilities. Thus,
lenalidomide represents an attractive alternative agent for patients with
Castleman disease after
rituximab and
cytostatics failures. It has a favourable safety profile and could be therefore considered for administering in first line treatment.