Immunodeficiency with recurrent panlymphocytopenia, impaired maturation of B lymphocytes, impaired interaction of T and B lymphocytes, and impaired integrity of epithelial tissue: a variant of idiopathic CD4+ T lymphocytopenia?
Idiopathic CD4+ T lymphocytopenia (ICL) has been defined as a cause of immunodeficiency with a variable clinical course and an unknown etiology. Here we describe a now 18-year-old boy with ICL, chronic mucocutaneous candidiasis (CMC), recurrent abscesses, and relapsing aphthous and ulcerous lesions. In addition to ICL the patient frequently showed a panlymphocytopenia. An increased percentage of gamma+delta+ T lymphocytes and IgD+ IgM+ B lymphocytes, and a decreased percentage of CD21+ B lymphocytes, were observed. In vitro assays showed normal T-cell responses to candidin and T-cell mitogens, but impaired B-cell responses to pokeweed mitogen (PWM). B-cell maturation after stimulation with Staphylococcus aureus Cowan I (SAC) and interleukin 2 (IL-2) was nearly normal. The clinical course of the patient improved substantially on administration of constant low-dose therapy with fluconazole.
von Bernuth
Horst
H
Department of Pediatrics, University Hospital Carl Gustav Carus Dresden, Germany. horst.bernuth@gmx.de
Knöchel
Birgit
B
Winkler
Ulf
U
Roesler
Jochen
J
Schlesier
Michael
M
Gahr
Manfred
M
D000038:Abscess / Q000276:immunology / Q000378:metabolism / Q000503:physiopathology; D000293:Adolescent; D001402:B-Lymphocytes / Q000276:immunology / Q000502:physiology*; D002178:Candidiasis, Chronic Mucocutaneous / Q000276:immunology / Q000378:metabolism / Q000503:physiopathology; D006801:Humans; D007072:Immunoglobulin D / Q000276:immunology / Q000378:metabolism; D007073:Immunoglobulin E / Q000276:immunology / Q000378:metabolism; D007074:Immunoglobulin G / Q000276:immunology / Q000378:metabolism; D007075:Immunoglobulin M / Q000276:immunology / Q000378:metabolism; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000378:metabolism*; D018655:Lymphocyte Count; D008297:Male; D012008:Recurrence; D013601:T-Lymphocytes / Q000276:immunology / Q000502:physiology*; D018344:T-Lymphocytopenia, Idiopathic CD4-Positive / Q000276:immunology / Q000378:metabolism / Q000503:physiopathology
D007072:Immunoglobulin D; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007073:Immunoglobulin E