![]() The parents claimed that the boy had BCG lymphadenitis (self-healing) previously and has been suffering from upper respiratory infections four to five times a year when peripheral blood tests indicated a decrease in granulocytes. The patient was admitted for further diagnosis and treatment by Department of Immunology. Peripheral blood test revealed a clear increase of white blood cell count (WBC23.9 × 10 9/L) with eosinophilia (58.91%, AEC14.1 × 10 9/L) and neutropenia (3.32%, absolute neutrophil count 0.8 × 10 9/L). Afterwards, the patient was sent to local hospital to take a two-day intravenous administration of cefotaxime sodium. Oral antitussive medicine was taken at home but did not work. No obvious wheezing, dyspnea or fever was observed. On November 12th, 2020, a 2-year-old boy with one-month chronic cough was admitted to Tianjin Children’s Hospital. Once the diagnosis of HIGMS is confirmed, the earlier the HSCT, the better the prognosis. ![]() Steroid therapy can quickly relieve eosinophilia but is easy to rebound if the reduction is too fast. Considering literature review and the corresponding reaction to steroid, we proposed that eosinophilia in HIGMS might be related to infections. Severe eosinophilia is rarely reported in this kind of PIDs. mNGS has obvious advantages for obtaining etiological diagnosis of children with PIDs. Diffuse interstitial lung disease and hypoglobulinemia in a young child predict the diagnosis of a primary immunodeficiency (PID). ConclusionsĬhildren with HIGMS are prone to opportunistic infections such as Pneumocystis jirovecii pneumonia (PJP). Two months after the diagnosis, the patient underwent allogeneic stem cell transplantation (HSCT) and has recovered well. X-linked hyper IgM syndrome was confirmed by gene test. The absolute eosinophil count (AEC) also returned to normal range. After treatments of caspofungin combined with sulfamethoxazole, intravenous immunoglobulin (IVIG) replacement and anti-inflammatory steroid, the clinical symptoms and pulmonary imaging noticeably improved. Pneumocystis jirovecii were detected from the bronchoalveolar lavage fluid (BALF) of the patient by metagenomic next-generation sequencing (mNGS). The lymphocyte subgroup classification was basically normal. Subsequent tests revealed a notable decrease in serum IgG and IgA. Lung computed tomography (CT) scan showed that diffuse ground-glass changes and eosinophils in peripheral blood increased significantly. In this report, we described a 2-year-old boy with chronic cough and symptoms of hypoxia. There are limited reports about HIGMS combined with severe eosinophilia. Hyper IgM syndromes (HIGMS) are a group of rare primary immunodeficiency disorders.
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