Objective To analyze the clinical features, diagnosis and treatment of X-Linked Agarnmaglobulinemia (XLA). Methods Clinical features, cellular and humoral immune functions, treatment and prognosis from 3 patients with XLA were retrospectively reviewed. Results The age of onset were from 11 months to 6 years in these 3 cases, however, the median age of diagnosis was 12 years. All patients showed multiple recurrent bacterial infections, arthritis involved large joints such as knee, ankle, elbow and hip. Laboratory examination revealed the decrease of serum gammmaglohulin and absence of B lymphocytes in the peripheral blood. All 3 patients were identiifed BTK mutations, which were frameshift mutation and nonsense mutation in exon 3, frameshift mutation in exon 10, missense mutation in exon 18. After XLA was diagnosed, the patients were managed by intravenous gammagloulin (IVIG) replacement. The non-steroidal anti-inflammatory drugs (NSAIDs) were administrated in patients combined arthritis. The small dose of hormones had been applied. All patients had a significantly improvement. Conclusions The clinical features of XLA have greater variability, with recurrent bacterial infections. Markedly decreased and absent tosils and lymph nodes, serum immunoglobulin may be one of the warning signs for early diagnosis of XLA. IVIG and NSAIDs can be jointly treatment of XLA with arthritis. The steroid and immunosuppressant agents should be used with caution.

Objective To observe and analyze the curative effect of the leukocyte elevating therapy on treatment of subitum children with agranulocytosis.Methods 60 subitum children with agranulocytosis were randomly divided into the observation group and the control group,30 cases in each group.The control group was treated with the conventional treatments,while the ob-servation group was treated with granulocyte colony stimulating factor (G-CSF)subcutaneous in-jection on the basis of the control group.At 2nd day,4th day,6th day,8th day,14th day of the duration,the classification and the counting of the peripheral blood samples of the children in the two groups were detected and compared.Results The proportion of the children with aplastic anemia in the observation group at 6th day was significantly lower than that of the control group (P <0.05).The leukocyte and neutrophil countings in the peripheral blood of the children in the two groups at 4th day of the duration decreased significantly (P <0.05),the level at 6th day was significantly higher than those at 2nd day or 4th day (P <0.05).The levels restored to normal levels at 14th day of the duration.At 6th day or 8th day of the duration,the leukocyte and neu-trophil countings in the peripheral blood of the children in the observation group were significantly higher than those of the control group (P <0.05).Conclusion Application of G-CSF in eleva-tion of the leukocyte can significantly improve the leukocyte and neutrophil levels in the peripheral blood of the patients with subitum,reduce the probability of aplastic anemia in the children,and help the children pass through the key course safely and smoothly.

Objective To observe and analyze the curative effect of the leukocyte elevating therapy on treatment of subitum children with agranulocytosis.Methods 60 subitum children with agranulocytosis were randomly divided into the observation group and the control group,30 cases in each group.The control group was treated with the conventional treatments,while the ob-servation group was treated with granulocyte colony stimulating factor (G-CSF)subcutaneous in-jection on the basis of the control group.At 2nd day,4th day,6th day,8th day,14th day of the duration,the classification and the counting of the peripheral blood samples of the children in the two groups were detected and compared.Results The proportion of the children with aplastic anemia in the observation group at 6th day was significantly lower than that of the control group (P <0.05).The leukocyte and neutrophil countings in the peripheral blood of the children in the two groups at 4th day of the duration decreased significantly (P <0.05),the level at 6th day was significantly higher than those at 2nd day or 4th day (P <0.05).The levels restored to normal levels at 14th day of the duration.At 6th day or 8th day of the duration,the leukocyte and neu-trophil countings in the peripheral blood of the children in the observation group were significantly higher than those of the control group (P <0.05).Conclusion Application of G-CSF in eleva-tion of the leukocyte can significantly improve the leukocyte and neutrophil levels in the peripheral blood of the patients with subitum,reduce the probability of aplastic anemia in the children,and help the children pass through the key course safely and smoothly.