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Differentiating aTTP/iTTP

Is it TTP or aHUS?

The clinical presentation of acquired/immune-mediated thrombotic thrombocytopenic purpura (aTTP/iTTP) can be strikingly similar to that of atypical hemolytic uremic syndrome (aHUS), which is why diagnosis can often be complicated. The creatinine levels, platelet count, and ADAMTS13 levels may guide toward distinguishing one from the other.1,2

Less than symbol

Indicators of aTTP/iTTP3,4

  • Creatinine <2.25 mg/dL
  • Platelet count <30 × 109/L
  • ADAMTS13 activity <10%
Greater than symbol

Indicators of aHUS/CM-HUS3,4

  • Creatinine ≥2.25 mg/dL
  • Platelet count >30 ×109/L
  • ADAMTS13 activity ≥10%

Differentiating TTP from HUS is crucial in order to start an appropriate therapy2

The following chart can help guide diagnosis to quickly differentiate patients with TTP in need of emergency care.

Flowchart for differentiating TTP from HUS and other TMAs

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

Biopsies can be useful in differentiating aTTP/iTTP from aHUS in difficult cases5

Biopsies can be useful in difficult diagnostic situations. Sampling of any accessible, highly vascular site may help inform the differential diagnosis of a TMA, based on pathologic distinctions between aTTP/iTTP and aHUS.

Icon of platelets with vWF

aTTP/iTTP

Biopsy sample of any accessible, highly vascular site shows the following:

 

  • Microthrombi appear as “white clots” composed of platelets and vWF, with only small amounts of fibrin

 

  • Vascular or perivascular inflammatory cell infiltrations are minimal or absent
Icon of platelets with fibrin

aHUS/CM-HUS

Biopsy sample of any accessible, highly vascular site shows the following:

 

  • Microthrombi appear as “red clots,” predominated by fibrin

 

  • An inflammatory infiltrate may be seen together with deposits of C5b-9

The ISTH TTP Guidelines provide support for diagnosing and treating aTTP/iTTP quickly

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Guidelines checkmarks

ISTH Guideline recommendations*

SEE THE RECOMMENDATIONS
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Choose CABLIVI® (caplacizumab-yhdp) on day 1 in combination with PEX and immunosuppressive therapy

DISCOVER HOW

Who should not start CABLIVI?

  • CABLIVI is contraindicated in patients with a previous severe hypersensitivity reaction to caplacizumab-yhdp or to any of its excipients
  • Withhold CABLIVI treatment 7 days prior to elective surgery, dental procedures, or other invasive interventions

*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.6

ADAMTS13=a disintegrin and metalloproteinase with a thrombospondin type 1 motif, 13; CM-HUS=complement-mediated hemolytic uremic syndrome; HUS=hemolytic uremic syndrome; ISTH=International Society on Thrombosis and Haemostasis; PEX=plasma exchange; TMA=thrombotic microangiopathy; TTP=thrombotic thrombocytopenic purpura; vWF=von Willebrand factor.

INDICATIONS

CABLIVI (caplacizumab-yhdp) is indicated for the treatment of adult patients with acquired thrombotic thrombocytopenic purpura (aTTP), in combination with plasma exchange and immunosuppressive therapy.

IMPORTANT SAFETY INFORMATION

CONTRAINDICATIONS:

CABLIVI is contraindicated in patients with a previous severe hypersensitivity reaction to caplacizumab-yhdp or to any of its excipients. Hypersensitivity reactions have included urticaria.

WARNINGS AND PRECAUTIONS:

Hemorrhage:

  • CABLIVI increases the risk of bleeding. In clinical studies, severe bleeding adverse reactions of epistaxis, gingival bleeding, upper gastrointestinal hemorrhage, and metrorrhagia were each reported in 1% of subjects. Overall, bleeding events occurred in approximately 58% of patients on CABLIVI versus 43% of patients on placebo.
  • In the postmarketing setting cases of life-threatening and fatal bleeding were reported in patients receiving CABLIVI.
  • The risk of bleeding is increased in patients with underlying coagulopathies (e.g. hemophilia, other coagulation factor deficiencies). It is also increased with concomitant use of CABLIVI with drugs affecting hemostasis and coagulation.
  • Avoid concomitant use of CABLIVI with antiplatelet agents or anticoagulants. If clinically significant bleeding occurs, interrupt use of CABLIVI. Von Willebrand factor concentrate may be administered to rapidly correct hemostasis. If CABLIVI is restarted, monitor closely for signs of bleeding.
  • Withhold CABLIVI for 7 days prior to elective surgery, dental procedures or other invasive interventions. If emergency surgery is needed, the use of von Willebrand factor concentrate may be considered to correct hemostasis. After the risk of surgical bleeding has resolved, and CABLIVI is resumed, monitor closely for signs of bleeding. 

ADVERSE REACTIONS:

The most common adverse reactions (>15% of patients) were epistaxis (29%), headache (21%) and gingival bleeding (16%).

CONCOMITANT USE OF ANTICOAGULANTS OR ANTIPLATELET AGENTS:

Concomitant use of CABLIVI with any anticoagulant or antiplatelet agent may increase the risk of bleeding. Avoid concomitant use when possible. Assess and monitor closely for bleeding with concomitant use.

PREGNANCY:

There are no available data on CABLIVI use in pregnant women to inform a drug associated risk of major birth defects and miscarriage.

  • Fetal/neonatal adverse reactions: CABLIVI may increase the risk of bleeding in the fetus and neonate. Monitor neonates for bleeding.
  • Maternal adverse reactions: All patients receiving CABLIVI, including pregnant women, are at risk for bleeding. Pregnant women receiving CABLIVI should be carefully monitored for evidence of excessive bleeding.

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INDICATIONS

IMPORTANT SAFETY INFORMATION

References:  1. Joly BS, Coppo P, Veyradier A. Thrombotic thrombocytopenic purpura. Blood. 2017;129(21):2836-2846. doi:10.1182/blood-2016-10-709857 2. Chiasakul T, Cuker A. Clinical and laboratory diagnosis of TTP: an integrated approach. Hematology Am Soc Hematol Educ Program. 2018;2018(1):530-538. doi:10.1182/asheducation-2018.1.530 3. Kremer Hovinga JA, Coppo P, Lämmle B, Moake JL, Miyata T, Vanhoorelbeke K. Thrombotic thrombocytopenic purpura. Nat Rev Dis Primers. 2017;3:17020. doi:10.1038/nrdp.2017.20 4. Vincent J-L, Castro P, Hunt BJ, et al. Thrombocytopenia in the ICU: disseminated intravascular coagulation and thrombotic microangiopathies—what intensivists need to know. Crit Care. 2018;22(1):158. doi:10.1186/s13054-018-2073-2 5. Laurence J, Haller H, Mannucci PM, Nangaku M, Praga M, de Cordoba SR. Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis. Clin Adv Hematol Oncol. 2016;14(11)(suppl 11):2-15. 6. Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for the diagnosis of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):2486-2495. doi:10.1111/jth.15006

CABLIVI and Sanofi are registered trademarks of Sanofi or an affiliate. MAT-US-2108055-v5.0-05/2025 Last Updated: May 2025.

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