Symptomatic Porphyria in a Case of Felty’s Syndrome. I. Clinical and Routine Biochemical Studies

[摘要] This report deals with the case of a 64-year-old white woman with long-standing Felty’s syndrome who developed symptomatic porphyria during the last three years of her life. Clinical and biochemical findings were typical of those for symptomatic porphyria, except for lack of exposure to alcohol and the absence of hepatic siderosis. The course of this patient was dominated by repeated infections, which were undoubtedly related to the persistent neutropenia and the multiple portals of infection provided by the porphyric skin lesions. Splenectomy and steroid therapy led to a brief increase in the leukocyte count. Despite the apparent effectiveness of methenolone therapy in returning the leukocyte count toward normal, she died as a result of a severe pulmonary infection, and at autopsy a lung abscess was demonstrated. Daily porphyrin excretion progressively declined during the two periods of hospitalization, but cutaneous porphyric lesions continued to appear, although with less intensity. The large amount of porphyrin excreted in the early phases provided abundant material for the intensive investigations reported in Part II of this report.

[学科分类] 过敏症与临床免疫学