{'fraction_non_alphanumeric': 0.04164321890827237, 'fraction_numerical': 0.032639279684862126, 'mean_word_length': 4.7540453074433655, 'pattern_counts': {'":': 0, '<': 0, '<?xml version=': 0, '>': 0, 'https://': 0, 'lorem ipsum': 0, 'www.': 0, 'xml': 0}, 'pii_count': 0, 'substrings_counts': 0, 'word_list_counts': {'cursed_substrings.json': 0, 'profanity_word_list.json': 0, 'sexual_word_list.json': 0, 'zh_pornsignals.json': 0}}
An imbalance of naive and memory/effector subsets and altered expression of CD38 on T lymphocytes in two girls with hyper‐IgM syndrome

In this report we evaluated CD4+ T, CD8+ T and natural killer (NK) cell counts, the levels of naive/memory subsets within the CD4+ T lymphocyte population, expression of CD38 on T lymphocytes, and CD4+ and CD8+ T cell cytokine production in two girls with hyper‐IgM (HIM) syndrome. Both girls developed recurrent infections early in infancy, presenting a wide spectrum of clinical manifestations, with a strikingly different disease severity between them. CD4+ T cell counts were low in both children (patient 1: 214 cells/mm3 and patient 2: 392 cells/mm3), and the CD4/CD8 T cell ratio was 0·4 for patient 1, the patient with the more severe disease, and 1·4 for patient 2. NK cell numbers were low in patient 1 (60 cells/mm3) and borderline (286 cells/mm3) with regard to normal levels in patient 2. An imbalance of naive and memory/effector cell subsets was found in both girls, with the percentage of CD45RA+ 27+ (naive) CD4+ T lymphocytes being 5·8 and 12·4 for patients 1 and 2, respectively. Expression of CD38 on the surface of T lymphocytes was low in patient 1. Detection of intracellular interferon (IFN)‐γ and tumour necrosis factor (TNF)‐α in CD4+ and CD8+ T lymphocytes upon PMA‐Io stimulus was preserved in both children. In conclusion, we found low numbers of CD4+ T lymphocytes and a dramatic redistribution of naive and memory/effector CD4+ T lymphocytes in two girls with non‐X‐linked HIM syndrome. Furthermore, we found low expression of CD38 on T lymphocytes and low numbers of NK cells in the patient with the more severe disease, indicating a possible role for these cells in the pathogenesis of this immunodeficiency.