Table 3.

Summary of investigations to determine the degree of organ involvement and differential diagnosis of an acute TMA

InvestigationTestsRationale
Hemolytic screen Hemoglobin, platelet, reticulocytes, LDH, blood film (DCT, haptoglobin) Diagnosis of MAHAT 
Renal function K+, Ur, creatinine urine dipstick: protein/blood/leukocytes protein creatinine ratio Degree of renal impairment 
Liver function Bilirubin With hemolysis 
AST/ALT Question hepatic ischemia 
ALP Liver obstruction 
Coagulation screen PT, APTT, fibrinogen Normal in TTP/CM-HUS, deranged in DIC 
Pregnancy test Urine or serum HCG In all women of child-bearing age 
Other biochemistry Troponin Degree of cardiac involvement 
Thyroid function Useful with pyrexia of TMA to exclude infection 
CRP May be reduced C3 with CM-HUS, C4 in autoimmune disease, eg, SLE 
C3/C4 Consider if possible cancer trigger in men 
PSA 
Vitamins B12/folate Treat deficiencies, folate may be reduced with haemolysis 
Virology Hepatitis A/B/C Baseline investigations especially pre-PEX 
HIV +/−viral load eg, in HSCT 
Further virology screen relevant to history, eg, adenovirus, CMV 
Autoimmune screen ANA/dsDNA Underlying autoimmune disease 
RF 
ENA 
ACLA/LA 
Vasculitis screen (if relevant from history) Anti-GBM antibodies To exclude Goodpasture’s/underlying vasculitis trigger 
ANCA 
Microbiology BCs STEC serology/PCR from stool With temperature 
Radiology USS abdomen Kidneys’ size, obstruction, lymphadenopathy, organomegaly 
MRI head Ischemic changes, infarcts, PRES 
CT chest/abdomen/pelvis If exclude an underlying cancer 
Cardiac investigations ECG echocardiogram Baseline for cardiac function/conduction defects or ischemia 
InvestigationTestsRationale
Hemolytic screen Hemoglobin, platelet, reticulocytes, LDH, blood film (DCT, haptoglobin) Diagnosis of MAHAT 
Renal function K+, Ur, creatinine urine dipstick: protein/blood/leukocytes protein creatinine ratio Degree of renal impairment 
Liver function Bilirubin With hemolysis 
AST/ALT Question hepatic ischemia 
ALP Liver obstruction 
Coagulation screen PT, APTT, fibrinogen Normal in TTP/CM-HUS, deranged in DIC 
Pregnancy test Urine or serum HCG In all women of child-bearing age 
Other biochemistry Troponin Degree of cardiac involvement 
Thyroid function Useful with pyrexia of TMA to exclude infection 
CRP May be reduced C3 with CM-HUS, C4 in autoimmune disease, eg, SLE 
C3/C4 Consider if possible cancer trigger in men 
PSA 
Vitamins B12/folate Treat deficiencies, folate may be reduced with haemolysis 
Virology Hepatitis A/B/C Baseline investigations especially pre-PEX 
HIV +/−viral load eg, in HSCT 
Further virology screen relevant to history, eg, adenovirus, CMV 
Autoimmune screen ANA/dsDNA Underlying autoimmune disease 
RF 
ENA 
ACLA/LA 
Vasculitis screen (if relevant from history) Anti-GBM antibodies To exclude Goodpasture’s/underlying vasculitis trigger 
ANCA 
Microbiology BCs STEC serology/PCR from stool With temperature 
Radiology USS abdomen Kidneys’ size, obstruction, lymphadenopathy, organomegaly 
MRI head Ischemic changes, infarcts, PRES 
CT chest/abdomen/pelvis If exclude an underlying cancer 
Cardiac investigations ECG echocardiogram Baseline for cardiac function/conduction defects or ischemia 

Samples for ADAMTS13 are taken pre-PEX and for complement mutational analysis, and factor H antibodies will take weeks or months before available.

ALP, alkaline phosphatase; ALT, alanine transaminase; ANA, antinuclear antibody; ANCA, antineutrophil cytoplasmic antibody; anti-GBM, antiglomerular basement membrane; AST, aspartate aminotransferase; BC, blood culture; CMV, cytomegalovirus; CRP, C-reactive protein; CT, computer tomography; DCT, direct coombes test; dsDNA, double-stranded DNA; ECG, electrocardiogram; ENA, extractable nuclear antibody; HCG, human chorionic gonadotropin; K+, potassium; PSA, prostate-specific antigen; RF, rheumatoid antibody; Ur, urea; USS, ultrasound.

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