May 26, 2008 - progressive multifocal leukoencephalopathy, Sjogren syndrome etc.2,3,4 Few reports have also been published describing ICL in children5,6,7 ...
Clinical Brief
Idiopathic CD4+ T-cell Lymphocytopenia Aparna Mukherjee, Rakesh Lodha and SK Kabra Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India
ABSTRACT Idiopathic CD4+ T lymphocytopenia (ICL) is an unusual immune defect in which there is an unexplained deficit of CD4+ T cells, leading to serious opportunistic infections. We report two cases of ICL who presented with low CD4+ count or percentage and various opportunistic infections like candida, cytomegalovirus and Mycobacterium tuberculosis. [Indian J Pediatr 2009; 76 (4) : 430-432] E-mail: rakesh_lodha@ hotmail.com
Key words: Idiopathic CD4+ T-cell lymphocytopenia; Immune deficiency
Idiopathic CD4+ T-cell lymphocytopenia (ICL) is a rare disorder of immune system with heterogeneous clinical manifestations and immunologic profile. It was first described by the CDC in 1992 in individuals who suffered from serious opportunistic infections in conjunction with low CD4 count in whom no evidence of HIV infection could be detected.1 Since then there has been a number of case reports of ICL in adults associated with a varied clinical presentation like cryptococcal meningitis, recalcitrant warts, progressive multifocal leukoencephalopathy, Sjogren syndrome etc.2,3,4 Few reports have also been published describing ICL in children5,6,7 but none from India. The definition of idiopathic CD4+ T-lymphocytopenia among children as given by the CDC includes the following criteria4 1. A CD4+ T-cell count of less than 1000 cells per cubic millimeter in children 0 to 23 months of age and of less than 300 cells per cubic millimeter in children 2 to 12 years of age, or a CD4+ T-lymphocyte count that is less than 20 % of total lymphocytes, on at least two separate measurements; 2. No serologic evidence of infection on HIV testing (even if the child’s mother is HIV-seropositive); 3. Absence of any defined immunodeficiency or therapy associated with T-cell depletion. Keeping in mind the rarity of this clinical entity, we here report 2 children who presented with features compatible with ICL. Correspondence and Reprint requests : Rakesh Lodha, Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India. [DOI–10.1007/s12098–009–0002–8] [Received May 26, 2008; Accepted June 30, 2008]
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CASE REPORTS Case 1 A 15-month-old boy, symptomatic since one month of age, presented with the complaints of recurrent fever, lower respiratory tract infections, recurrent loose stool, multiple skin infections and oral thrush (Table 1). He was hospitalized once for pneumonia and thrush. There was no history of intake of any immunosuppressant or immunomodifying drugs. Examination revealed pallor, onychomycosis of finger nails, and hepatomegaly. Anthropometric measurements showed weight of 8.5 kg (less than 3 rd centile) and height 77 cm (25th centile for age). Investigations revealed a low hemoglobin of 7.7g/ dL, leucopenia - 2100/mm 3 (N: 6000-17500), lymphopenia – 1155/mm3 (N: 4000-10500) and normal platelets. The immunological workup showed a normal immunoglobulin profile (IgG = 1490 mg/dL, IgA = 242 mg/dL, IgM = 89 mg/dL), normal NBT test, negative HIV serology, low CD4 count and altered CD4:CD8 ratio (Table 1). Candida spp. could be isolated repeatedly from skin biopsy and scrapings from oral ulcers. The child was treated with oral antifungals. Thereafter, in view of low leucocytes and low CD4 count, patient was advised co-trimoxazole and itraconazole prophylaxis. In spite of prophylaxis, the child has developed recurrent oral thrush requiring hospitalization and iv amphotericin on one occasion. Case 2 A 4.5-year-old boy was symptomatic since day 5 of life with maculo-papular itchy eruptions all over the body. Since then there had been recurrent episodes of skin Indian Journal of Pediatrics, Volume 76—April, 2009
Idiopathic CD4+T-cell Lymphocytopenia TABLE 1. Clinical Features and Investigations
Age at onset of symptoms Recurrent fever Recurrent pneumonia Recurrent loose stool Recurrent skin infections Oral thrush Recurrent ear discharge Weight for age
