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| (1) To establish the suspicion of TMA |
| (i) Thrombocytopenia (<150 × 109/l or >25% of decrease) |
| (ii) Signs of microangiopathic hemolysis |
| (iii) Anemia (±increase in mean corpuscular volume) |
| (iv) Reticulocyte count raised |
| (v) Lactate dehydrogenase (LDH) increased with haptoglobin decreased |
| (vi) Direct Coomb’s test negative |
| (vii) Blood smear searching schistocytes |
|
| (2) To look for organ involvement |
| (i) Neurological: confusion, headache, seizures, encephalopathy, and focal deficits |
| (ii) Renal: ARF, arterial hypertension, proteinuria, and hematuria |
| (iii) Cardiac: cardiac failure, hypotension, and ischemic cardiopathy |
| (iv) Pulmonary: ARDS and respiratory insufficiency |
| (v) Gastrointestinal: abdominal pain, intestinal angina, diarrhea, and vomiting |
| (vi) Hematological (thrombocytopenia): epistaxis, hemoptysis, menorrhagia, retinal hemorrhage, gastrointestinal bleeding, and petechiae |
|
| (3) To confirm organ involvement |
| (i) Blood analysis including renal function, cellular blood count, LDH, liver and pancreatic enzymes, creatin kinase, and troponin I |
| (ii) Renal biopsy: to confirm glomerular microthrombosis |
| (iii) CT/MRI brain: to determine neurological involvement |
| (iv) Electrocardiogram/echocardiogram: to document or monitor cardiac damage |
| (v) Chest radiograph/CT: to document lung involvement |
| (vi) Echography/CT: to document hepatic/pancreatic/intestinal involvement |
| (vii) Fundoscopic examination: to document retinal vessel involvement |
|
| (4) To investigate the etiology |
| (i) ADAMTS 13 activity: <5–10% (TTP) |
| (ii) If gastroenteritis (bloody diarrhea): Shiga toxin/STEC: positive (HUS) |
| (iii) If ADAMTS13 > 10%: secondary or associated TMA |
| (iv) Fundoscopic examination (malignant hypertension) |
| (v) Immunologic profile: ANA, ANCA, and aPL (autoimmune diseases) |
| (vi) Pregnancy test (pregnancy-related) |
| (vii) CT thoracoabdominal or PET: cancer-associated |
| (viii) Clinical history looking for drugs/heparin and anti-PF4 antibodies (HIT) |
| (ix) Complement studies: FH, FB, FI, anti-FH antibodies, and genetic study (aHUS) |
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