Table 2.

Initial diagnostic workup in patients suspected of TA-TMA

TestIndicationRationale
CBC/diff, reticulocytes, BMP, LFT, total/direct bilirubin, LDH, haptoglobin All patients Confirm hemolysis, rule out red cell aplasia or aplastic anemia 
Blood smear (serial) All patients Confirm presence & persistence of schistocytosis 
DAT; IAT (ask for agglutination strength) All patients Rule out autoimmune or alloimmune hemolytic anemia 
DIC panel (PT/PTT/INR, D-dimer, fibrinogen) All patients Rule out DIC 
Infectious workup All patients Rule out infection/sepsis 
Vitamin B12 All patients Rule out cobalamin deficiency-related pseudo-TMA 
Urinalysis; spot rUPCR All patients Seek early renal organ damage (proteinuria) 
sC5b9 assay Suspected TA-TMA Prognostic biomarker for TA-TMA 
ADAMTS13; antinuclear antibody; lupus anticoagulant; anticardiolipin; beta-2-glycoprotein Autoimmune history Rule out TTP & other autoimmune causes (rare to occur first-time post-transplant without prior history) 
Stool culture for Shiga toxin; gut biopsy New diarrhea Rule out ST-HUS 
Bone marrow biopsy Inappropriate hematopoiesis or circulating blasts Rule out relapse of pre-transplant disease or graft failure 
Kidney biopsy New kidney injury Confirm diagnosis of TMA on biopsy 
TestIndicationRationale
CBC/diff, reticulocytes, BMP, LFT, total/direct bilirubin, LDH, haptoglobin All patients Confirm hemolysis, rule out red cell aplasia or aplastic anemia 
Blood smear (serial) All patients Confirm presence & persistence of schistocytosis 
DAT; IAT (ask for agglutination strength) All patients Rule out autoimmune or alloimmune hemolytic anemia 
DIC panel (PT/PTT/INR, D-dimer, fibrinogen) All patients Rule out DIC 
Infectious workup All patients Rule out infection/sepsis 
Vitamin B12 All patients Rule out cobalamin deficiency-related pseudo-TMA 
Urinalysis; spot rUPCR All patients Seek early renal organ damage (proteinuria) 
sC5b9 assay Suspected TA-TMA Prognostic biomarker for TA-TMA 
ADAMTS13; antinuclear antibody; lupus anticoagulant; anticardiolipin; beta-2-glycoprotein Autoimmune history Rule out TTP & other autoimmune causes (rare to occur first-time post-transplant without prior history) 
Stool culture for Shiga toxin; gut biopsy New diarrhea Rule out ST-HUS 
Bone marrow biopsy Inappropriate hematopoiesis or circulating blasts Rule out relapse of pre-transplant disease or graft failure 
Kidney biopsy New kidney injury Confirm diagnosis of TMA on biopsy 

BMP, basic metabolic panel; CBC/diff, complete blood count with differential; DAT, direct antiglobulin test (direct Coombs); DIC, disseminated intravascular coagulation; IAT, indirect antiglobulin test; INR, international normalized ratio; LDH, lactate dehydrogenase; LFT, liver function tests; PT, prothrombin time; PTT, partial thromboplastin time; rUPCR, random urine protein creatinine ratio; ST-HUS, Shiga toxin hemolytic uremic syndrome; TTP, thrombotic thrombocytopenic purpura.

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