Vnitr Lek 2016, 62(4):287-298

Treatment of 14 cases of Castleman's disease: the experience of one centre and an overview of literature

Zdeněk Adam^1,*, Petr Szturz¹, Marta Krejčí¹, Renata Koukalová², Eva Michalková¹, Zdeněk Řehák², Eva Pourová³, Luděk Pour¹, Pavlína Volfová¹, Viera Sandecká¹, Zdeňka Čermáková^4,9, Leoš Křen⁵, Filip Sokol⁵, Ivo Hanke⁶, Igor Penka⁶, Hana Petrášová⁷, Sabina Ševčíková⁸, Zdeněk Král¹, Jiří Mayer¹

¹ Interní hematologická a onkologická klinika LF MU a FN Brno, pracoviště Bohunice

² Oddělení nukleární medicíny, centrum PET, RECAMO, Masarykův onkologický ústav, Brno

³ Ordinace praktického lékaře pro dospělé, Pustiměř

⁴ Oddělení klinické biochemie FN Brno, pracoviště Bohunice

⁵ Ústav patologie LF MU a FN Brno, pracoviště Bohunice

⁶ Chirurgická klinika LF MU a FN Brno, pracoviště Bohunice

⁷ Radiologická klinika LF MU a FN Brno, pracoviště Bohunice

⁸ Ústav patologické fyziologie LF MU Brno

⁹ Katedra laboratorních metod LF MU, Brno

Castleman's disease is the term for reactive lymphocytary and plasmocytary proliferation which occurs in the unicentric (localized) form, usually without systemic symptoms, or in the generalized/multicentric form, typically with systemic symptoms (www.vzacne-diagnozy.cz). Over the past 25 years we diagnosed, treated and followed 14 histologically proven cases of Castleman's diseases. Seven patients had the localised form of the disease. In 5 of 7 cases the pathological lesion was located intrathoracically or intraabdominally and in only 2 cases it was on the surface of the body. No clinical symptoms were present in any of the patients with the unicentric form of the disease and surgical treatment led to the total removing of the disease in all of them. As opposed to that, all 7 patients with the multicentric form of Castleman's disease experienced febrile or subfebrile temperatures. Three of the 7 patients complained of severe troubling night sweats. Clinical expressions of vasculitis which was the cause of stroke, were present in 1 of 7 patients. Osteosclerotic changes on the skeleton were detected in 1 patient, who also suffered from fluid retention likely associated with this disease. Polyclonal propagation of immunoglobulins, predominantly immunoglobulin IgG type, was present in 5 of 7 patients with the multicentric form. In one case there was one complete molecule of monoclonal imunoglobuline present and in one case loose light chains κ were increased More than 1 sampling of material for histological examination of enlarged lymph nodes were needed in 6 of 7 patients for diagnosing the multicentric form of the disease. It has turned out beneficial with respect to diagnosing the disease to carry out surgical removal and histological examination of the nodes which accumulated the most fluorodeoxyglucose within PET-CT examination. The text describes experience of the treatment. In recent years the basis for the treatment has been the monoclonal antibody antiCD20 rituximab, or thalidomide and lenalidomide, or possibly their combination. The new medicine for these patients is interleukin-6 antibody called siltuximab (Sylvant), of which we have no own experience so far. Five of our seven patients with the multicentric form received treatment, 1 patient refused treatment and in one patient the signs of the disease activity are not expressed to such extent that would require treatment. The therapy containing rituximab reached complete remission in 2 patients and the therapy containing thalidomide and lenalidomide achieved the complete remission of the disease in 3 patients. In one of the above described cases the disease did not respond to the initial treatment with rituximab and remission was reached by thalidomide and lenalidomide and in one case the disease did not respond to the initial treatment with thalidomide and complete remission was reached with rituximab. Following the treatment, no patient with the multicentric form of Castleman's disease has had a relapse until now.

Keywords: anemia of chronic diseases; Castleman's disease; hyperproteinemia; lenalidomide; polyclonal hypergammaglobulinemia; rituximab; siltuximab; thalidomide

Received: August 4, 2015; Accepted: October 1, 2015; Published: April 1, 2016  Show citation