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Acquired/immune-mediated thrombotic thrombocytopenic purpura (aTTP/iTTP) is a rare, rapidly progressing, life-threatening medical emergency. It has been reported in literature that, when left untreated, death may occur in up to 90% of patients with aTTP/iTTP, making urgent diagnosis and treatment a necessity.1-6

Complex presentation of TTP often causes a delay in diagnosis7

TTP presents with highly variable, multiorgan symptoms that resemble a series of other TMAs and disorders. It is therefore critical to differentiate TTP from other similarly presenting conditions. The table below compares TTP with other resembling conditions.

Clinical picture of TTP, HUS, and DIC8-15





Presenting symptoms

  • Thrombocytopenia (platelet count <150 × 109/L or >25% reduction from baseline)8,9

  • MAHA (presence of schistocytes)8,9

  • Organ ischemia8,9


Congenital and acquired/immune-mediated TTP (cTTP and aTTP/iTTP)10,11

Infection-associated or atypical/complement-mediated HUS (IA-HUS or aHUS/CM-HUS)11,12

Overt and nonovert DIC13


aTTP/iTTP—usually adults11

IA-HUS—common in
aHUS/CM-HUS—any age14



cTTP—inherited mutations of ADAMTS1311
aTTP/iTTP—autoantibodies against ADAMTS1311

IA-HUS—toxins produced by certain bacteria14,15
aHUS/CM-HUS—activation of the complement system14,15

Occurs as a result of underlying diseases such as sepsis, malignancy, trauma, liver diseases, obstetric disorders, envenomation, vascular anomalies, and major transfusion reactions13

Potential laboratory results

Platelet count <30 × 109/L15
Creatinine <2.25 mg/dL15
PT and aPTT—normal8
ADAMTS13 <10%15

Platelet count <30 × 109/L15
Creatinine >2.25 mg/dL15
PT and aPTT—normal8

Platelet count <50 × 109/L14
PT and aPTT—prolonged13,14

Test(s) confirming the diagnosis

cTTP—ADAMTS13 sequencing11,15

aTTP/iTTP—ADAMTS13 testing11,15

IA-HUS—culture test, PCR, ADAMTS13 testing8,14

culture test, ADAMTS13 testing8,14

ISTH scoring system for overt DIC13

Severe thrombocytopenia, MAHA, and organ ischemia indicate aTTP/iTTP is likely—ADAMTS13 activity <10% confirms a diagnosis10

See what Guidelines suggest about treating as soon as you suspect aTTP/iTTP.

    There are 2 types of TTP: aTTP/iTTP and cTTP. aTTP/iTTP is caused by autoantibody inhibition of ADAMTS13 activity, whereas cTTP is caused by mutations in the ADAMTS13 gene. The following chart describes an algorithm to help distinguish aTTP/iTTP from cTTP.

    Flowchart to differentiate aTTP/iTTP from cTTP after TTP diagnosis

    CABLIVI is not indicated for congenital TTP.

    Adapted from: Kremer Hovinga JA et al. Nat Rev Dis Primers. 2017;3:17020.

EHR capabilities such as Rule Messages can support proactive identification of at-risk patients for further evaluation to differentiate aTTP/iTTP from other conditions

Arterial thrombosis icon

CABLIVI presents similarly to other TMAs

Guideline checkmarks

ISTH Guideline recommendations*

*A conditional recommendation defined as desirable effects of the recommendation probably outweighing the undesirable effects. Assumes timely access to ADAMTS13 testing and clinical diagnosis based on high likelihood of aTTP/iTTP. In de novo patients where no reasonable access to ADAMTS13 activity testing is available, the Guidelines do not recommend CABLIVI; however, treatment of a patient previously diagnosed with aTTP/iTTP could be safely undertaken on clinical grounds without the need for a confirmatory ADAMTS13 test.16

ADAMTS13=a disintegrin and metalloproteinase with a thrombospondin type 1 motif, 13; aPTT=activated partial thromboplastin time; DIC=disseminated intravascular coagulation; EHR=electronic health record; HUS=hemolytic uremic syndrome; IgG=immunoglobulin G; ISTH=International Society on Thrombosis and Haemostasis; MAHA=microangiopathic hemolytic anemia; PCR=polymerase chain reaction
 PT=prothrombin time; TMA=thrombotic microangiopathy; TTP=thrombotic thrombocytopenic purpura.