Differentials

Thrombotic thrombocytopenic purpura (TTP)

SIGNS / SYMPTOMS
INVESTIGATIONS
SIGNS / SYMPTOMS

Low levels of the von Willebrand cleaving enzyme, ADAMTS13, in many patients with presentations typical of TTP.[42]

INVESTIGATIONS

ADAMTS13 levels more consistently low in TTP than in HUS.[41]

Malignant hypertension

SIGNS / SYMPTOMS
INVESTIGATIONS
SIGNS / SYMPTOMS

Distinguishing feature is significantly elevated blood pressure.[43]

INVESTIGATIONS

Platelet counts are generally normal in malignant hypertension.[43]

Systemic lupus erythematosus (SLE)

SIGNS / SYMPTOMS
INVESTIGATIONS
SIGNS / SYMPTOMS

There are many overlapping features between SLE and TTP/HUS, and because the two can co-exist, a distinction between them is generally not possible.[44]

INVESTIGATIONS

Serological tests of underlying condition may be positive, but there are no tests to differentiate the cause of HUS.

Catastrophic antiphospholipid syndrome

SIGNS / SYMPTOMS
INVESTIGATIONS
SIGNS / SYMPTOMS

Can present in a way that is indistinguishable from TTP/HUS.[45][46]

INVESTIGATIONS

Antiphospholipid antibodies can be helpful in suggesting a diagnosis. However, even if these are positive, plasma exchange is often recommended in patients with manifestations of severe disease.[46]

Disseminated intravascular coagulation (DIC)

SIGNS / SYMPTOMS
INVESTIGATIONS
SIGNS / SYMPTOMS

Patients with DIC have a higher likelihood of bleeding manifestations and often have obvious predisposing causes, such as sepsis.

INVESTIGATIONS

The peripheral smear in a patient with DIC generally has fewer schistocytes. Coagulation parameters are typically abnormal in patients with DIC, whereas they are normal in patients with TTP/HUS.

Pre-eclampsia

SIGNS / SYMPTOMS
INVESTIGATIONS
SIGNS / SYMPTOMS

Distinguishing between the various causes of thrombocytopenia that present in the peri- and postpartum period can be difficult.[34][47] HUS tends to present postpartum, which distinguishes it from pre-eclampsia.

INVESTIGATIONS

The peripheral smear in patients with TTP/HUS generally has more schistocytes than are seen in pre-eclampsia.

HELLP syndrome

SIGNS / SYMPTOMS
INVESTIGATIONS
SIGNS / SYMPTOMS

HUS tends to present postpartum, which distinguishes it from HELLP (haemolysis, elevated liver enzyme levels, and a low platelet count) syndrome.

INVESTIGATIONS

The peripheral smear in patients with TTP/HUS generally has more schistocytes than are seen in HELLP syndrome.

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