Jodele et al reported criteria for the diagnosis of hematopoietic stem cell transplant (HSCT) associated thrombotic microangiopathy (TMA). The authors are from Cincinnati Children’s Hospital and Children’s Hospital of Philadelphia.
Patient selection: hematopoietic stem cell transplant (HSCT)
Criteria for thrombotic microangiopathy:
(1) serum LDH above the upper limit of normal for age
(2) schistocytes on blood smear
(3) de novo thrombocytopenia
(4) de novo anemia and/or transfusion required
(5) hypertension
(6) proteinuria with >= 30 mg/dL
(7) terminal complement activation (elevated soluble C5b-9)
(8) thrombotic microangiopathy on biopsy
Differentiation from TTP:
(1) ADAMTS13 > 10%
Prognostic findings:
(1) proteinuria with >= 30 mg/dL
(2) terminal complement activation (elevated soluble C5b-9)
A patient with both prognostic findings should be considered for therapy with eculizumab.
