Journal List > Korean J Hematol > v.42(1) > 1032762

Lee, Byun, Choi, and Kim: A Case of Idiopathic CD4+ T-lymphocytopenia Associated with Autoimmune Hemolytic Anemia

Abstract

Idiopathic CD4+ T-lymphocytopenia (ICL) is defined by the CDC as depressed numbers of circulating CD4+ T-lymphocytes (<300 cells/μL or <20% of the total T cells) on more than one determination, with the absence of HIV infection and other known causes of immunodeficiency. The clinical spectrum of ICL ranges from asymptomatic laboratory abnormalities to severe opportunistic infections that mimic the clinical course of human immunodeficiency virus (HIV) infected patients. There are a few reports of ICL associated with different diseases such as Sjogren's syndrome, pulmonary sarcoidosis, Down syndrome or non-Hodgkin's lymphoma. We describe here a 5-year-old male patient with a three-year history of recurrent otitis media and pulmonary infection, and he was without any risk factors for HIV infection; this patient presented with autoimmune hemolytic anemia and was ultimately found to have idiopathic CD4+ T-lymphocytopenia.

REFERENCES

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Fig. 1
The peripheral blood smear showed severe anemia with RBC agglutination, polychromasia, spherocytes, and normoblasts (Wright stain, ×1,000)
kjh-42-53f1.tif
Table 1.
Past medical history for this patient
Age (years/months) Diagnosis
2/9 Acute otitis media, pneumonia
3/6 Acute otitis media, sinusitis, pneumonia
4/0 Acute otitis media, bronchitis
4/1 Bronchitis
4/3 Pneumonia
4/4 Acute otitis media, pneumonia
4/6 Acute otitis media, bronchitis
4/10 Acute otitis media, bronchitis
5/0 Acute otitis media, bronchitis
5/1 Oral thrush
Table 2.
The results of antibody screening test and Direct Coombs’ test
Age (years/months) Antibody screening Direct Coombs’ test
Selectogen I Selectogen II IgG C3d Auto contro
5/3∗ 4+ 4+ 4+ - 4+

∗At the time of admission.

Table 3.
The results of immunoglobulin quantitation test and complement test
Age (years/months) IgG mg/dL IgA mg/dL IgM mg/dL IgD mg/dL IgE IU/mL C3 mg/dL C4 mg/dL CH50 U/mL
3/6 1,681 59 149 NT NT 125 22 18.6
Reference ranges∗ 400~1,250 24~192 41~200 0~14 0.0~29.2 70~150 10~30 23~46
5/3 2,108 63 162 2.1 1.5 106 19 31.7
Reference ranges∗ 560~1,307 26~232 47~200 0~14 0.2~17.6 70~150 10~30 23~46

∗Age-specific reference ranges (Source: Wallach J. In: Interpretation of diagnostic tests. 7th ed. Philadelphia, United States Lippincott Williams & Wilkins, 2000:12-7);

See Table 2. Abbreviation: NT, not tested.

Table 4.
The results of lymphocyte subset test
Age (years/months) WBC /μL Lymphocyte B cell % T cell % CD4 cell % CD8 cell % NK cell C % CD4:CD8 ratio CD4 /μL
% /μL
3/6 5,100 28.9 1,473 3.4 25.8 8.6 11.6 60.7 0.74 126.8
4/1 4,000 38.6 1,544 3.5 33.0 10.1 10.4 63.4 0.97 155.9
4/3 6,000 25.5 1,530 2.6 17.0 3.9 3.7 80.4 1.05 59.7
5/3∗ 9,400 13.2 1,637 25.2 46.9 15.2 15.1 27.8 1.01 248.8

∗See Table 2.

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