Table 3.

Modified Jodele criteria and Transplant-Associated Thrombotic Microangiopathy Working Group definition of high-risk thrombotic microangiopathy

TA-TMA Criteria
Biopsy-proven disease (kidney or gastrointestinal) or
Clinical criteria: must meet ≥4 of the following seven criteria within 14 d at two consecutive time points
Anemiaa	Defined as one of the following
	1. Failure to achieve transfusion independence for pRBCs despite evidence of neutrophil engraftment
	2. Hemoglobin decline from patient's baseline by 1 g/dl
	3. New onset of transfusion dependence
Thrombocytopenia	Defined as one of the following
	1. Failure to achieve platelet engraftment
	2. Higher than expected platelet transfusion needs
	3. Refractoriness to platelet transfusions
	4. 50% reduction or greater in baseline platelet count after full platelet engraftment
Elevated LDH	>ULN for age
Schistocytes	Present
Hypertension	>99th percentile for age (<18 yr), or systolic BP ≥140 mm Hg or diastolic BP ≥90 mm Hg (≥18 yr)
Elevated sC5b-9	≥ULN
Proteinuria	≥1 mg/mg rUPCR

hrTMA Definition
Defined by any one of the following
• Elevated sC5b-9 (≥ULN)
• rUPCR ≥1 mg/mg
• Elevated LDH (≥2 times ULN)
• Concurrent grade 2–4 aGVHD
• Concurrent infection (bacterial or viral)
• Organ dysfunction

Ref. 82. aGVHD, acute graft-versus-host disease; hrTMA, high-risk transplant-associated thrombotic microangiopathy; LDH, lactate dehydrogenase; pRBCs, packed red blood cells; PRCA, pure red cell aplasia; rUPCR, random urine protein to creatinine ratio; sC5b-9, soluble C5b-9; TA-TMA, transplant-associated thrombotic microangiopathy; ULN, upper limit of normal.

^aRule out other causes of anemia, such as autoimmune hemolytic anemia and pure red cell aplasia.