Hematopoietic stem cell transplantation rescues the immunologic phenotype and prevents vasculopathy in patients with adenosine deaminase 2 deficiency

Vasculopathology and immunopathology in patients with ADA2 deficiency. A, Sagittal T1-weighted MRI of P1 showing pineal gland hemorrhage (arrow). B, Hematoxylin and eosin staining of jejunal ulceration in P2 showing chronic ulcer with predominant eosinophils (arrows), some neutrophils and lymphocytes, and very few plasma cells. Plasma cells stained by means of CD138 staining are indicated by arrows in the inset.