RESUMO
The combination of rituximab, cyclophosphamide, and dexamethasone (RCD) is highly effective in the treatment of warm autoimmune hemolytic anemia (WAIHA) associated with chronic lymphocytic leukemia (CLL). We treated a cohort of patients with relapsed/refractory WAIHA, without CLL, with RCD. The primary objective was to evaluate the overall response (OR) of RCD therapy. Complete response (CR) was defined as a hemoglobin (Hgb) ≥12 g/dL. Partial response (PR) was defined as Hgb 10-11.9 g/dL or ≥2 g/dL increase in Hgb. Sustained response was defined as Hgb ≥10 g/dL with no treatment changes. A total of 16 patients with relapsed/refractory WAIHA received RCD (7 primary WAIHA, 9 secondary WAIHA) for a median of 4 cycles (range: 2-6). The median pretreatment Hgb was 10.0 g/dL (range: 4.3-12.2). The median best Hgb achieved was 12.5 g/dL (range: 10.6-15.1) with a median of 2 cycles until best Hgb response. The OR was 94% (11 CR, 4 PR). Two immunocompromised patients were admitted for infections during RCD treatment. There were no deaths during the treatment or follow-up period. Following a response to RCD, 4 patients received noncorticosteroid immune modulation therapy and 4 patients continued on corticosteroid therapy. Seven patients received no additional treatment.
Assuntos
Anemia Hemolítica Autoimune/tratamento farmacológico , Ciclofosfamida/uso terapêutico , Dexametasona/uso terapêutico , Rituximab/uso terapêutico , Corticosteroides/uso terapêutico , Adulto , Idoso , Anemia Hemolítica Autoimune/sangue , Anemia Hemolítica Autoimune/cirurgia , Terapia Combinada , Ciclofosfamida/administração & dosagem , Dexametasona/administração & dosagem , Quimioterapia Combinada , Feminino , Seguimentos , Hemoglobinas/análise , Humanos , Fatores Imunológicos/uso terapêutico , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Recidiva , Estudos Retrospectivos , Rituximab/administração & dosagem , Esplenectomia , Resultado do TratamentoRESUMO
The behavior of phyllodes tumors is unpredictable and can behave as benign fibroadenomas or malignant neoplasms mimicking the course of aggressive sarcomas, characterized by distant metastases and a high short-term mortality. The malignant forms are treated with surgery and adjuvant chemotherapy, but often with poor outcomes. We examine the first reported case of an aggressive osteosarcoma subtype of phyllodes tumor that recurred in the skull after total mastectomy and adjuvant chemotherapy. The skull lesion was treated with excision, and the patient currently remains disease-free.