Bucaretchi Fábio, Vicente Danielly C, Pereira Ricardo M, Tresoldi Antonia T
Departamento de Pediatria, Faculdade de Ciências Médicas, Universidade Estadual de Campinas (UNICAMP), Campinas, SP, Brazil.
Rev Inst Med Trop Sao Paulo. 2004 Nov-Dec;46(6):331-4. doi: 10.1590/s0036-46652004000600006. Epub 2005 Jan 10.
A 12 y old girl was admitted 24 days after start a WHO multidrug therapy scheme for multibacillary leprosy (dapsone, clofazimine and rifampicin) with intense jaundice, generalized lymphadenopathy, hepatoesplenomegaly, oral erosions, conjunctivitis, morbiliform rash and edema of face, ankles and hands. The main laboratory data on admission included: hemoglobin, 8.4 g/dL; WBC, 15,710 cells/mm3; platelet count, 100,000 cells/mm3; INR = 1.49; increased serum levels of aspartate and alanine aminotransferases, gamma-glutamyl transpeptidase, alkaline phosphatase, direct and indirect bilirubin. Following, the clinical conditions had deteriorated, developing exfoliative dermatitis, shock, generalized edema, acute renal and hepatic failure, pancytopenia, intestinal bleeding, pneumonia, urinary tract infection and bacteremia, needing adrenergic drugs, replacement of fluids and blood product components, and antibiotics. Ten days after admission she started to improve, and was discharged to home at day 39th, after start new supervised treatment for leprosy with clofazimine and rifampicin, without adverse effects. This presentation fulfils the criteria for the diagnosis of dapsone hypersensitivity syndrome (fever, generalized lymphadenopathy, exfoliative rash, anemia and liver involvement with mixed hepatocellular and cholestatic features). Physicians, mainly in geographical areas with high prevalence rates of leprosy, should be aware to this severe, and probably not so rare, hypersensitivity reaction to dapsone.
一名12岁女孩在开始接受世界卫生组织针对多菌型麻风病的多药联合治疗方案(氨苯砜、氯法齐明和利福平)24天后入院,出现严重黄疸、全身淋巴结肿大、肝脾肿大、口腔糜烂、结膜炎、麻疹样皮疹以及面部、脚踝和手部水肿。入院时的主要实验室检查数据包括:血红蛋白8.4 g/dL;白细胞15,710个/mm³;血小板计数100,000个/mm³;国际标准化比值(INR)=1.49;血清天冬氨酸和丙氨酸转氨酶、γ-谷氨酰转肽酶、碱性磷酸酶、直接和间接胆红素水平升高。随后,临床状况恶化,出现剥脱性皮炎、休克、全身水肿、急性肾和肝衰竭、全血细胞减少、肠道出血、肺炎、尿路感染和菌血症,需要使用肾上腺素能药物、补充液体和血液制品成分以及使用抗生素。入院十天后她开始好转,并在第39天出院,之后开始接受氯法齐明和利福平的新的麻风病监督治疗,未出现不良反应。该病例符合氨苯砜超敏综合征的诊断标准(发热、全身淋巴结肿大、剥脱性皮疹、贫血以及伴有肝细胞和胆汁淤积混合特征的肝脏受累)。主要在麻风病高流行地区的医生应意识到这种对氨苯砜的严重且可能并不罕见的超敏反应。