RESUMO
We herein report the rare case of a 72-year-old female who presented with paraneoplastic pemphigus (PNP) and bronchiolitis obliterans (BO) associated with follicular lymphoma (FL), who was successfully treated with obinutuzumab (GA101; G) and bendamustine (B). The patient had severe erosive stomatitis and bilateral conjunctival hyperemia that persisted for more than 6 months. A huge mass was found in the abdominal cavity, and a biopsy revealed grade 1 FL (stage IV). Based on a lip biopsy result, the patient was diagnosed with PNP associated FL. The patient received bendamustine and obinutuzumab (BG) chemotherapy and FL and PNP responded very well, but BO was additionally associated during the course of BG. BO progressed without exacerbation as BG therapy progressed to a 2 year maintenance therapy with G, and combination of azithromycin, inhaled bronchodilator therapy, and corticosteroid. She was followed up at the outpatient department with no pulmonary function decline or FL and PNP recurrence. Our case suggests that BG could be a promising treatment option for PNP and BO.
Assuntos
Bronquiolite Obliterante , Linfoma Folicular , Síndromes Paraneoplásicas , Pênfigo , Idoso , Anticorpos Monoclonais Humanizados , Cloridrato de Bendamustina/uso terapêutico , Bronquiolite Obliterante/complicações , Bronquiolite Obliterante/tratamento farmacológico , Feminino , Humanos , Linfoma Folicular/complicações , Linfoma Folicular/diagnóstico , Linfoma Folicular/tratamento farmacológico , Síndromes Paraneoplásicas/complicações , Síndromes Paraneoplásicas/etiologia , Pênfigo/complicações , Pênfigo/tratamento farmacológicoAssuntos
Antirreumáticos/efeitos adversos , Cromonas/efeitos adversos , Imunossupressores/efeitos adversos , Transtornos Linfoproliferativos/induzido quimicamente , Metotrexato/efeitos adversos , Sulfonamidas/efeitos adversos , Idoso , Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Cromonas/uso terapêutico , Feminino , Humanos , Imunossupressores/uso terapêutico , Metotrexato/uso terapêutico , Recidiva , Sulfonamidas/uso terapêuticoRESUMO
This study reports a case of a 49-year-old woman having B-cell acute lymphoblastic leukemia with glycophorin A, a representative erythroid marker, expression. According to the WHO criteria for mixed phenotype acute leukemia (MPAL), erythroid lineage is not defined, and to the best of our knowledge, only one other case with erythroid/B-cell biphenotypic acute leukemia has been reported previously. To establish the disease entity and clarify the pathophysiology of erythroid/lymphoid MPAL, additional cases need to be analyzed.
