Severe graft versus host disease in a patient with globoid cell leukodystrophy following umbilical cord blood transplantation: resemblance to the twitcher mouse model.

Abstract

Bone marrow transplantation (BMT) is currently the treatment of choice for patients with globoid cell leukodystrophy (GLD), particularly in the early phases of the disease. Elevated interleukin (IL)-6 levels in the central nervous system of the twitcher mouse, an animal model for GLD, have been held responsible for severe graft versus host disease (GVHD), and IL-6 knock-out mice have shown lower incidence of GVHD after BMT. Here we report an eight-year-old girl with late-onset advanced stage of GLD who developed severe GVHD and died following unrelated 5/6 matched cord blood transplantation. Serum IL-6 levels pre-BMT and at day +38 were elevated (20 pg/ml and 15 pg/ml, respectively). This observation may support the findings in twitcher mice suggesting a possible role for IL-6 in the pathogenesis of GVHD in transplanted patients with GLD.

• Authors: Maide Ozen, Duygu Uçkan, Mualla Cetin, Ilhan Tezcan, Murat Tuncer, Banu Anlar
• Journal: The Turkish journal of pediatrics (Turk J Pediatr) 2007 Jul-Sep Vol. 49 Issue 3 Pg. 304-6 ISSN: 0041-4301 [Print] Turkey
• PMID: 17990586 (Publication Type: Case Reports, Journal Article)

Chemical References

• Interleukin-6

Topics

• Blood Grouping and Crossmatching
• Child
• Fatal Outcome
• Female
• Fetal Blood (transplantation)
• Graft vs Host Disease (etiology, physiopathology)
• Humans
• Interleukin-6 (blood)
• Leukodystrophy, Globoid Cell (physiopathology, therapy)