The clinical spectrum of Fabry disease includes a "renal variant" phenotype in patients without classic symptoms who develop ESRD. Affected males undergoing hemodialysis or renal transplantation can be readily diagnosed by plasma alpha-Gal A assays. These patients and their family members may benefit from enzyme replacement therapy for the later, life-threatening cardiovascular and cerebrovascular complications of Fabry disease.

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A 64-year-old Japanese male patient with lupus nephritis associated with adult T cell leukemia (ATL) is described. Percutaneous renal biopsy demonstrated findings consistent with membranous lupus nephritis. To our knowledge, this is the first case of lupus nephritis complicated by ATL, suggesting that human T cell leukemia virus type I may be correlated not only to outbreaks of ATL but also to lupus nephritis/systemic lupus erythematosus.

IgD multiple myelomais a unique type of multiple myelomawhich is characterized by increased serum IgD and IgD type M-component in immunoelectrophoresis. It frequently shows renal involvement but it is a rare form of myeloma. The distinctive features of IgD myelomaare the dominance in males, high frequency in younger persons, and the uncertain appearance of M-component in serum electrophoresis. Weexperienced 3 cases of IgD multiple myelomawith renal failure which required hemodialysis before IgD myelomawas diagnosed. It is important to consider IgD myeloma when treating the patients with renal involvement of unknown origin.

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