Idiopathic CD4+ T cell lymphocytopenia evolving to monoclonal immunoglobulins and progressive renal damage responsive to IL-2 therapy.
Idiopathic CD4+ T cell lymphocytopenia was unexpectedly detected in a 33-year-old, otherwise healthy young woman with no HIV or other viral infection, autoimmune, or neoplastic disease or increased susceptibility to infection. CD4+ T cell levels were 60-140/microl over a 3.5-year period. Following an uneventful pregnancy, the patient developed anemia and interstitial nephritis associated with a plasma cell dyscrasia with a monoclonal IgA gammopathy and a shifting immunoglobulin pattern that included IgG and IgA monoclonal proteins and increased urinary light chains. Osteolytic lesions were never detected and bone marrow aspirations revealed up to 10% atypical plasma cells. Various therapies often used in treating multiple myeloma only temporarily controlled the increasing renal damage. IL-2 therapy of 600,000 to 1 million units subcutaneously daily resulted in increased CD4+ T cells to normal levels, a decrease in the gammopathy, a return of renal function, energy, and weight gain, and apparently normal health status sustained for 2 years. The findings are compatible with a potentially fatal but nonmalignant immunoregulatory disorder that can be controlled by IL-2 administration.
Wilhelm
M
M
Medizinische Poliklinik, Julius-Maximilian-University, Wuerzburg, Germany.
Weissinger
F
F
Kunzmann
V
V
Muller
J G
JG
Fahey
J L
JL
D000328:Adult; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007073:Immunoglobulin E / Q000097:blood; D007376:Interleukin-2 / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000276:immunology / Q000473:pathology / Q000628:therapy*; D010265:Paraproteinemias / Q000209:etiology* / Q000276:immunology*; D018344:T-Lymphocytopenia, Idiopathic CD4-Positive / Q000150:complications* / Q000276:immunology*
D007070:Immunoglobulin A; D007376:Interleukin-2; D007073:Immunoglobulin E