PET-CT documented fast onset of treatment response to cyclophosphamide, thalidomide and dexamethasone in patients with multicentric Castleman‘s disease. Case description and treatment information overview

Authors: Z. Adam 1;  P. Szturz 1;  L. Křen 2;  M. Krejčí 1;  L. Pour 1;  T. Svoboda 3;  I. Hanke 3;  I. Penka 3;  R. Koukalová 4;  Z. Řehák 4;  I. Červinková 5;  T. Štorková 1;  Z. Král 1;  J. Mayer 1
Authors‘ workplace: Interní hematologická a onkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Jiří Mayer, CSc. 1;  Ústav patologie Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta doc. MU Dr. Josef Feit, CSc. 2;  Chirurgická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MU Dr. Zdeněk Kala, CSc. 3;  PET-CT oddělení Masarykova onkologického ústavu Brno, přednosta prim. MU Dr. Karol Bolčák 4;  Klinika dětské radiologie Lékařské fakulty MU a FN Brno, pracoviště FDN, přednostka doc. MU Dr. Jarmila Skotáková, CSc. 5
Published in: Vnitř Lék 2013; 59(4): 301-312
Category: Case Reports


Castleman‘s disease (also called angiofollicular lymph node hyperplasia) can take two forms with different prognosis: the localized form can usually be treated by a surgical intervention and has therefore a favourable prognosis. On the other hand, the multicentric form has an unfavourable prognosis and requires systemic treatment. Classic manifestations of multicentric Castleman‘s disease are multiple sites of lymphadenopathy, sometimes hepatomegaly and also splenomegaly or serous cavity effusions. Typical pathological laboratory levels measured in patients with this disease include an increased CRP level, anaemia of chronic diseases, and many patients have an increased total protein concentration, in some cases exceeding even 100g/ l. It is caused by a high concentration of polyclonal immunoglobulins. Typical clinical symptoms include fluctuating subfebrile or febrile temperatures, increased night sweats and fatigue usually related to anaemia. In some patients, the disease is manifested as vasculitis, frequently also affecting cerebral arteries, i.e. leading to cerebrovascular accidents. The aetiology of this disease is unclear; it is a polyclonal lymphocyte proliferation, often with differentiation into plasma cells. It is not a clonal malign disease; however, it can transform into a clonal lymphoproliferative disease. Even though it is not a malign disease in the histomorphological sense, the disease symptoms are so acute that systemic treatment is required. In the past, the treatment method of this disease used to be based on corticoids and cytostatics; however, such treatment was not always successful in achieving its objective, i.e. complete remission. In the past few years, an improvement of treatment results was accomplished by adding a new drug to the basic medication, i.e. to cytostatics and dexamethasone. Many publications describe the benefi t of adding a third drug from the IMiDs group (immunomodulatory drugs), such as thalidomide or lenalidomide. These drugs affect the formation of cytokines and block the angiogenesis, which in turn positively influences the speed of the treatment response. The second new drug that has helped in combination with classical treatment is the anti-CD20 antibody, rituximab. The third new drug to add this list is the monoclonal antibody against the interleukin-6 receptor, tocilizumab. This paper describes a rapid treatment response after combined treatment with cyclophosphamide 500mg/ m2 i.v. infusion 1st and 15th day in a 28- day cycle, dexamethasone 20mg p.o. cycle day 1– 4 and cycle day 15– 18, and thalidomide 100mg daily. In the course of the two-month treatment, the accumulation of fl uorodeoxyglucose during the PET-CT imaging has normalized; the originally pathologically enlarged nodes have become smaller, the originally elevated CRP level has normalized and the originally signifi cantly lower haemoglobin level has risen. This is the second patient with multicentric Castleman‘s disease in the last three years who showed a rapid response to treatment with thalidomide combined with cyclophosphamide and dexamethasone. Therefore, we consider such treatment suitable for newly diagnosed patients with multicentric Castleman‘s disease.

Key words:
Castleman‘s disease – multicentric form – thalidomide – bortezomib – rituximab – tocilizumab – POEMS syndrome – vasculitis – polyclonal hypergammaglobulinemia – anaemia of chronic disease – fever of unknown origin


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