Selective IgM deficiency: functional assessment of peripheral blood lymphocytes in vitro.
The functional aspects of the peripheral blood lymphocytes from 6 patients with primary selective IgM deficiency (sIgMD) were analyzed to elucidate its pathogenesis. The surface IgM positive B cells were present at almost the same percentage as in controls, but the percentage of cluster of differentiation (CD)4+ T cells was higher and that of CD8+ T cells was low. The patient B cells showed a significantly lower proliferative response to Staphylococcus aureus Cowan strain I (SAC) than control B cells and did not produce a significant amount of IgM when co-cultured with control T cells. Interestingly, mitomycin C (MMC)-treated patient T cells induced a greater amount of IgM production by control B cells. In addition, patient B cells treated with SAC and B cell differentiation factors (BCDF) failed to secrete IgM. These results suggest that the pathogenesis of sIgMD may be mainly due to an intrinsic defect in B cell maturation.
Yamasaki
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Second Department of Internal Medicine, Hyogo College of Medicine, Japan.
D000276:Adjuvants, Immunologic / Q000627:therapeutic use; D000328:Adult; D000361:Agammaglobulinemia / Q000097:blood* / Q000628:therapy; D000943:Antigens, Differentiation / Q000032:analysis; D001402:B-Lymphocytes / Q000473:pathology; D002454:Cell Differentiation; D002455:Cell Division; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000172:deficiency*; D015850:Interleukin-6 / Q000032:analysis / Q000627:therapeutic use; D008213:Lymphocyte Activation; D008214:Lymphocytes / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D016685:Mitomycin / Q000627:therapeutic use; D011947:Receptors, Antigen, B-Cell / Q000032:analysis; D013211:Staphylococcus aureus; D050378:T-Lymphocytes, Regulatory / Q000276:immunology
D000276:Adjuvants, Immunologic; D000943:Antigens, Differentiation; D007075:Immunoglobulin M; D015850:Interleukin-6; D011947:Receptors, Antigen, B-Cell; D016685:Mitomycin