Table 2.

Clinical manifestations of TA-TMA by organ system with differential diagnoses and suggested evaluations.

Organ system	Clinical manifestations	Differential diagnoses	Diagnostic considerations (excluding tissue biopsy)
Kidney	Proteinuria, hypertension, acute, or chronic kidney injury	CNI, steroids (causing hypertension), nephrotoxic medications, acute cystitis, BK nephritis, and engraftment syndrome	—Urinalysis with urine culture
			—Urine protein/creatinine
			—Urine and blood PCR for BK virus
GI Tract	Severe abdominal pain, intestinal bleeding, diarrhea, vomiting, and ascites	GVHD, hepatic SOS, infectious colitis, drug-induced colitis, and engraftment syndrome	—Liver ultrasound
			—Coagulation studies
			—Liver blood tests (total and direct bilirubin, albumin, alkaline phosphatase, AST, ALT)
			—Amylase, lipase
			—Viral serologies if not already performed (including hepatitis, CMV, HSV)
			—Consider stool culture
			—Consider skin biopsy if question of GVHD
CNS	Headache, seizures, confusion, and hallucinations	Encephalitis, fludarabine toxicity, delirium, psychiatric disorder, and engraftment syndrome	—Brain MRI
			—Viral serologies
			—Nutritional evaluation including thiamine
Cardio-pulmonary/polyserositis	Pulmonary hypertension, pleural effusion, and refractory pericardial effusion	Infection, pulmonary edema, engraftment syndrome, GVHD, PVOD, drug toxicity, radiation pneumonitis, connective tissue disease, idiopathic pneumonia syndrome, and ACS	—Chest imaging
			—Echocardiogram
			—Bronchoscopy
			—Pulmonary function testing
			—ECG, troponin
			—Skin biopsy

ALT alanine transaminase, AST aspartate transaminase, ACS acute coronary syndrome, CMV cytomegalovirus, CNI calcineurin inhibitor, ECG electrocardiogram, GI gastrointestinal, GVHD graft versus host disease, HSV herpes simplex virus, PCR polymerase chain reaction, PVOD pulmonary veno-occlusive disease, MRI magnetic resonance imaging, SOS sinusoidal obstruction syndrome, TA-TMA transplant-associated thrombotic microangiopathy.