Abstract: To summarize the nursing experience of a child with monocytopenia and mycobacterial infection（MonoMAC） syndrome. In response to characteristics such as the rare disease,high-risk of transplantation,and unknown prognosis,measures were taken to reduce the risk level of invasive diagnosis and treatment,to assist in diagnosis and clarify the needs for transplantation. And we implemented home lung therapy combined with remote rehabilitation to find the best time for transplantation,multidisciplinary collaboration for long-term follow-up to optimize the prognosis of patients. After repeated biopsy and pathological consultation,the patient was diagnosed with Mycobacterium avium infection,PAS（+）,DPAS（+）,GATA2 gene exon 2 insertion mutation,and was diagnosed with MonoMAC syndrome with pulmonary alveolar proteinosis. In October 2022,a hematopoietic stem cell transplantation was performed. Treatment for MAC lung disease continued after the surgery. Follow-up to the present showed that the interstitial lesions have significantly improved compared to that before the transplantation. The patient continued anti-rejection treatment and is currently studying at home.

Key words: MonoMAC Syndrome, MAC Lung Disease, Pulmonary Alveolar Proteinosis, Child, Critical Care Nursing