Document Type: Original Article
Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran
Introduction: Hyper IgM (HIGM) syndrome is a rare kind of primary Immunodeficiency disease (PID) characterized by normal to the increased serum IgM and very low or undetectable IgG, IgA, and IgE. Broad spectrum of clinical manifestations and laboratory findings are observed in the HIGM patients including hematologic problem and malignancy. This study was conducted to assess demographic data, clinical manifestation, and immunological findings in the HIGM patients.
Methods: Lab findings and clinical presentations data of 79 Iranian patients diagnosed with HIgM syndrome were collected. All the patients were classified into two different groups including the patients with hematological problems and those without hematological problems.
Results: Hematologic problems were observed in 34 patients (43%, 23 males and 11 females). The most common hematologic problems types were anemia and leukemia (33 and 25%, respectively). Also, 19 patients (24.1%) had a family history of PID. Significant data that were higher in the patients with hematologic problems, were the oral ulcer (p=0.037), failure to thrive (p=0.022), recurrent diarrhoea (p=0.021), chronic diarrhoea (p=0.022), urinary tract infections (p=0.037), anemia (p=0.000), neutropenia (p=0.000), thrombocytopenia (p=0.001), gastrointestinal problem (p=0.011), neurologic problem (p=0.000), multiple site problem (p=0.000), platelet count (p=0.005), and IgG level (p=0.048).
Conclusion: The association between HIgM syndrome and hematologic problems could lead to severe clinical disorders. Therefore, it is necessary for immunologists to be aware of these situations.