Successful treatment of steroid-refractory autoimmune thrombocytopenia associated with Castleman disease with anti-CD-20 antibody (rituximab)

Abstract

Multicentric Castleman disease (MCD) is a lymphoproliferative disorder of incompletely understood etiology and with various clinical presentations. The best therapeutic option for this disease is not well established. MCD is known to be associated with autoimmune phenomena. A 70-year-old female patient of MCD with progressive nodal disease associated with autoimmune thrombocytopenia failed steroid treatment and showed a transient response to intravenous immunoglobulin. The patient achieved complete recovery of her platelet count and a very good response in nodal disease after 3 weekly doses of anti-CD-20 antibody (rituximab). Anti-CD20 antibody treatment could be a good therapeutic option for MCD, mainly when associated with immune-related disorders.

Sjögren's and plasma cell variant Castleman disease: a case report

“…Most MCD cases treated to date with rituximab have been HIV and HHV-8 positive patients. [33][34][35][36] Rituximab was chosen in our patient as the first-line treatment since she also had a history of Sjögren's syndrome.…”

Incomplete POEMS syndrome with multicentric Castleman’s disease

“…En la EC localizada, varios autores han mostrado la mejoría de la amiloidosis después de la resección de la masa linfoide 6 . Así, en la EC multicéntrica, parece que el control del estado inflamatorio mejora el curso de la amiloidosis 7 , si bien se han utilizado fármacos como toziluzumab que bloquen la acción del receptor soluble de la IL6 8 , el rituximab con su efecto anti CD20 9 y la colchicina con un efecto antiinflamatorio 10 . Para confirmar el diagnóstico de sín-drome POEMS se requiere la conjunción de dos criterios mayores mandatorios (polineuropatía desmielinizante y gammapatía monoclonal), un criterio mayor (EC y/o lesiones esclerosas óseas) y un criterio menor (elevación del factor de crecimiento endotelial vascular (VEGF), organomegalia, aumento del volumen extravascular, endocrinopatía, cambios en la piel, papiledema, trombocitosis/policitemia).…”

“…La EC es uno de los criterios que ayudan al diagnóstico del síndrome de POEMS 3 . Las asociaciones de estas dos entidades han sido ampliamente descritas en la literatura actual, aunque su asociación con amiloidosis, es menos frecuente y descrita como casos clínicos aislados [4][5][6][7][8][9][10] . Presentamos un caso clínico con esta triple asociación, EC, POEMS y amiloidosis.…”

Multicentric Castleman’s Disease, Associated with Idiopathic Thrombocytopenic Purpura

“…The association of CD with autoimmune disorders including myasthenia gravis [15], Evans' syndrome [16], vitiligo [17], coeliac disease [18], Graves' disease [19], and ulcerative colitis [20] suggests a possible autoimmune pathology. Of considerable interest, there are two previous reports of the association between CD and immune thrombocytopenia [21, 22]. However, it is not clear if autoimmunity is the underlying cause or result of CD [23].…”

“…Although infrequent, it is well known that hematological abnormalities, including anemia, leukopenia, or thrombocytopenia, can be encountered during the clinical course of CD [34–36]. However, the presence of CD during the clinical course of immune thrombocytopenia has been rarely described [21, 22]. …”