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LETTER

Letters: CD4+ Lymphocytopenia in Systemic Lupus Erythematosus

right arrow Michael Guma, DO, and Randal Krakauer, MD

15 January 1994 | Volume 120 Issue 2 | Pages 168-169


TO THE EDITOR:

We read with great interest Laurence's review [1] on CD4+ T-lymphocytopenia. We report a case of CD4+ lymphocytopenia in a young woman with systemic lupus erythematosus.

A 35-year-old white woman was seen in 1987 reporting recurrent arthralgias, rash, increased hair loss, and the Raynaud phenomena. Her physical examination showed a malar flush, the presence of herpes zoster along the lateral aspect of her abdomen, and synovitis of her wrists and ankles. Laboratory results included: a positive antinuclear antibody count (1:640 homogeneous), an erythrocyte sedimentation rate of 20 mm/h, a moderately positive double-stranded DNA, a normal C3, and a slightly low C4.

She was given hydroxychloroquine and a gradually tapering dose of prednisone, which she tolerated well. She reported frequent episodes of candidiasis and recurrent herpes zoster attacks with subsequent postherpetic neuralgia. Along with a positive double-stranded DNA, T-cell studies showed a total lymphocyte count of 396/mm3 (6.4%) (normal, 1049/mm3 to 3500/mm3 or 10% to 47%). Her CD3 count was 111/mm3 or 28% (normal, 870/mm3 to 2147/mm3 or 50% to 84%). Her CD4 counts were 20/mm3 or 5% (normal, 536/mm3 to 1571/mm3 or 31% to 59%) and her CD8 counts were 83/mm3 or 21% (normal, 235/mm3 to 753/mm3 or 12% to 3%). Her T4:T8 ratio was.24 (normal, 1.2 to 4.8). Several human immunodeficiency virus (HIV) test results were negative. Recently she presented with a cavitary lung lesion that was shown to be caused by Cryptococcus neoformans. Her CD4+ level (6 years after the initial study) is 40/mm3 and her CD8+ level is 135/mm3. Test results for HIV-1, HIV-2, and human T-cell lymphotropic virus types 1 and 2 remain negative. She continues to deny any risk factors for HIV.

Although many researchers have speculated about the cause or causes of idiopathic CD4+ T-lymphocytopenia [2-4], few have considered autoimmune diseases as a possible cause. Depletion of CD4+ cells by antilymphocytic antibodies has been described [5]. Differences in antilymphocytic antibodies could account for some of the clinical variation found in some patients with this disease.


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Seton Hall University, School of Graduate Medical Education; South Orange, NJ 07079-2689


References
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1. Laurence J. T-cell subsets in health, infectious disease, and idiopathic CD4+ T Lymphocytopenia. Ann Intern Med. 1993; 119:55-62.

2. Duncan RA, Von Reyn CF, Alliegro GM, Toossi Z, Sugar AM, Levitz SM. Idiopathic CD4+ T-lympopenia—four patients with opportunistic infections and no evidence of HIV infection. N Engl J Med. 1993; 328:393-8.

3. Spira TJ, Jones BM, Nicholson JK, Lal RB, Rowe T, Mawle AC, et al. Idiopathic CD4+ T-lymphocytopenia—an analysis of five patients with unexplained opportunistic infections. N Engl J Med. 1993; 386-92.

4. Smith DK, Neal JJ, Holmberg SD. Unexplained opportunistic infections and CD4+ T-lymphopenia without HIV infection. N Engl J Med. 1993; 328:372-9.

5. Winfield JB, Shaw M, Yamada A, Minota S. Subset specificity of antilymphocyte antibodies in systemic lupus erythematosus. II. Preferential reactivity with T4+ cells is associated with relative depletion of autologous T4+ cells. Arthritis Rheum. 1987; 30:162-8.

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Rheumatology (Oxford)Home page
G. Henriksson, R. Manthorpe, and A. Bredberg
Antibodies to CD4 in primary Sjogren's syndrome
Rheumatology, February 1, 2000; 39(2): 142 - 147.
[Abstract] [Full Text] [PDF]


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