Anti-CD13 Abs in children with extensive chronic GVHD and their relation to soluble CD13 after allogeneic blood and marrow transplantation from a Children's Oncology Groups Study, ASCT0031.

Our group previously demonstrated a strong association between elevated plasma soluble CD13 enzyme activity and newly diagnosed extensive chronic GVHD (cGVHD) in children. As cytotoxic anti-CD13 Abs have been documented after blood and marrow transplant (BMT) in association with CMV infection and cGVHD, we hypothesized that soluble CD13 contributes to cGVHD pathogenesis by induction of CD13 reactive Abs and that anti-CD13 Abs could be additional biomarkers for newly diagnosed pediatric extensive cGVHD. Using prospectively collected plasma samples from pediatric allogeneic BMT (allo-BMT) subjects with cGVHD and controls without cGVHD enrolled in a large multi-institution Children's Oncology Group cGVHD therapeutic trial, we evaluated whether soluble CD13 correlates with induction of anti-CD13 Abs. We found that CD13 reactive Abs are present in a proportion of patients after allo-BMT, but did not seem to correlate with the presence of soluble CD13. Anti-CD13 Abs also did not meet our criteria as a diagnostic biomarker for cGVHD. These data do not confirm that induction of CD13 reactive Abs is a mechanism for cGVHD in children nor are part of the pathogenesis of cGVHD associated with elevated soluble CD13. The exact role of CD13 in cGVHD remains to be determined.

Torticollis as a sign of cervico-thoracic epidural haematoma in an infant with severe haemophilia A.

We describe the case of a spinal epidural haematoma in an infant with severe haemophilia A. Initial signs and symptoms were non-specific resulting in delay of the diagnosis and more definitive therapy. The patient eventually developed torticollis, acute flaccid paralysis of the upper extremities, and respiratory distress, prompting radiological examination of the spinal cord. The patient was treated with recombinant FactorVIII and laminectomy. Neurological recovery was complete 3 months following the event. We hypothesize that infants with haemophilia may be at higher risk for this rare complication because of their increasing mobility, frequent falls while cruising furniture, and lack of prophylactic factor replacement. Non-specific signs such as irritability without a focus should alert the clinician to this diagnostic possibility. Torticollis should prompt rapid radiological evaluation of the cervical spine with magnetic resonance imaging to avoid delay in diagnosis.