Unrelated bone marrow transplantation for leukocyte adhesion deficiency.
The severe form of leukocyte adhesion deficiency type I (LAD-I) usually leads to death early in life. Allogeneic haematopoietic transplantation is the only cure. Unrelated transplantation has been reported only once. We describe three children with LAD-I transplanted with T cell non-depleted bone marrow from unrelated HLA-matched donors. All patients engrafted, one of them at second transplant. One patient developed grade I and one grade II acute GVHD. Two patients are alive, one of them with a decrease in CD11/CD18 expression. Early referral for HLA-matched unrelated BMT is a reasonable option for patients with LAD-I lacking an HLA-matched related donor.
Farinha
N J
NJ
Division of Hematology-Oncology, Hôpital Sainte-Justine, Montreal, Canada.
Duval
M
M
Wagner
E
E
Champagne
J
J
Lapointe
N
N
Barrette
S
S
Tapiero
B
B
Busque
L
L
Champagne
M A
MA
D001424:Bacterial Infections / Q000209:etiology; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D017809:Fatal Outcome; D005260:Female; D006085:Graft Survival; D006086:Graft vs Host Disease / Q000209:etiology; D019698:Hepatitis C, Chronic / Q000209:etiology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D018370:Leukocyte-Adhesion Deficiency Syndrome / Q000628:therapy*; D008297:Male; D011015:Pneumonia, Aspiration / Q000209:etiology; D014138:Tracheoesophageal Fistula / Q000209:etiology; D019172:Transplantation Conditioning / Q000009:adverse effects; D014184:Transplantation, Homologous / Q000009:adverse effects