Clinical Features and Genetic Analysis of 20 Chinese Patients with X-Linked Hyper-IgM Syndrome

Table 5

Transplantation strategies for the six XHIGM patients.

Patients	Age at diagnosis (y)	Age at HSCT (y)	Pretransplantation status	Donors	Complications	Outcomes
P3	1	2	Oral ulcer, elevated liver enzyme, neutropenia	Haploidentical sibling	VOD, paralytic ileus, pulmonary infection, DIC	Died
P6	5	6.5	Gingivitis, neutropenia	HLA-identical unrelated donor	GVHD Grade I,; ADH abnormal secretion.	9 y, clinical remission.; Oral ulcer.; Free of IVIG.
P8	10.25	21	Oral ulcer	HLA-identical unrelated donor	GVHD Grade III,; sepsis (Brucella),; CMV infection.	27 y, clinical remission. Pneumonia once a year within the first two years after HSCT. Free of IVIG.
P10	5	6	Oral and perianal ulcer, abdominal pain, diarrhea, fever, prolonged APTT, anemia	HLA-identical unrelated donor	GVHD Grade I,; Hemorrhagic shock, VOD, CMV infection, diarrhea.	9 y, clinical remission.; Free of IVIG.
P13	3	4.5	Oral ulcer, diarrhea, neutropenia	Haploidentical sibling	Sepsis	Died
P20	3.4	4.6	Oral ulcer, neutropenia	HLA-identical unrelated donor	GVHD Grade II,; pulmonary infection.	4 y 10 m, clinical remission.

Note: HSCT: hematopoietic stem cell transplantation; VOD: hepatic venoocclusive disease; DIC: disseminated intravascular coagulation;
GVHD: graft-versus-host disease; ADH: antidiuretic hormone; IVIG: intravenous immunoglobulin.