RESUMEN
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.
Asunto(s)
Bronquiolitis Obliterante , Linfoma Folicular , Síndromes Paraneoplásicos , Pénfigo , Anciano , Anticuerpos Monoclonales Humanizados , Clorhidrato de Bendamustina/uso terapéutico , Bronquiolitis Obliterante/complicaciones , Bronquiolitis Obliterante/tratamiento farmacológico , Femenino , Humanos , Linfoma Folicular/complicaciones , Linfoma Folicular/diagnóstico , Linfoma Folicular/tratamiento farmacológico , Síndromes Paraneoplásicos/complicaciones , Síndromes Paraneoplásicos/etiología , Pénfigo/complicaciones , Pénfigo/tratamiento farmacológicoAsunto(s)
Antirreumáticos/efectos adversos , Cromonas/efectos adversos , Inmunosupresores/efectos adversos , Trastornos Linfoproliferativos/inducido químicamente , Metotrexato/efectos adversos , Sulfonamidas/efectos adversos , Anciano , Antirreumáticos/uso terapéutico , Artritis Reumatoide/tratamiento farmacológico , Cromonas/uso terapéutico , Femenino , Humanos , Inmunosupresores/uso terapéutico , Metotrexato/uso terapéutico , Recurrencia , Sulfonamidas/uso terapéuticoRESUMEN
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.