Investigations

1st investigations to order

platelet count

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Degree of thrombocytopenia varies, but decreased platelets are required for the diagnosis of TTP. Platelet count is <20 x 10⁹/L in approximately 95% of patients.[44]

Result

decreased

haemoglobin

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Approximate prevalence 80%.[44]

Result

<8 g/L

haptoglobin

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Haptoglobin is significantly decreased during haemolysis.[45]

Result

decreased

peripheral smear

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Schistocytes might be absent from the blood film in the first 24 to 48 hours, but they are usually found on evaluation of the blood film at presentation.[Figure caption and citation for the preceding image starts]: Peripheral smear of a patient with TTP showing many fragmented, partly rounded cells. Also note the lack of plateletsFrom the collection of Dr R.F. Connor, Harvard Medical School, Boston [Citation ends].com.bmj.content.model.Caption@a63ee73

Result

microangiopathic blood film with schistocytes

reticulocyte count

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Typically raised in TTP.

Result

raised

urinalysis

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Result
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Mild renal abnormalities of proteinuria and renal insufficiency occur in approximately 40% of patients.[44]

Result

proteinuria

urea and creatinine

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Severe renal failure occurs in approximately 5% of patients.[44]

Result

increased

direct Coombs' test

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To rule out autoimmune haemolytic anaemia.

Result

negative

Investigations to consider

ADAMTS-13 activity assay and inhibitor titres

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There is debate over whether the von Willebrand factor cleaving enzyme (ADAMTS-13) activity assay can help in the management of patients with TTP. It does not appear to predict who will respond to plasma exchange. [46] For the diagnosis of TTP, ADAMTS-13 activity levels of <5% to 10% are diagnostic.[1][3][4]

Result

decreased activity

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