Our experience in treatment of multicentric plasma-cell Castleman disease associated with vasculitis manifestations – case report and literature review
Authors:
P. Szturz 1; Z. Adam 1; M. Moulis 2; L. Šmardová 1; M. Klincová 3; R. Šlaisová 4; R. Koukalová 5; Z. Řehák 5; P. Volfová 1; J. Chovancová 3; O. Stehlíková 1; J. Mayer 1
Authors‘ workplace:
Interní hematologická a onkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Mayer, CSc.
1; Ústav patologie Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta doc. MUDr. Josef Feit, CSc.
2; Lékařská fakulta Masarykovy univerzity, Brno, děkan prof. MUDr. Jiří Mayer, CSc.
3; Radiologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Vlastimil Válek, CSc.
4; Oddělení nukleární medicíny PET Centra Masarykova onkologického ústavu Brno, přednosta prim. MUDr. Karol Bolčák
5
Published in:
Vnitř Lék 2012; 58(9): 679-690
Category:
Case Reports
Overview
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 enrollement in the Celgene’s 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 patient’s 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.
Key words:
Castleman disease – glucocorticoids – chemotherapy – rituximab – thalidomide – monoclonal antibody – tocilizumab – lenalidomide – positron emission tomography – computed tomography
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