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SEE aTTP/iTTP† |
START CABLIVI* |
SUPPORT WITH ADAMTS13 |
| Diagnosis through clinical assessment | Consider early administration of CABLIVI in combination with PEX and immunosuppressive therapy | ADAMTS13 test results inform treatment decisions |
*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.2
†The ISTH TTP Guidelines refer to aTTP as iTTP.
ISTH TTP Guidelines are the first evidence-based, international guidelines on the diagnosis, treatment, and management of acquired/immune-mediated thrombotic thrombocytopenic purpura (aTTP/iTTP)1,2
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Treatment recommendations have evolved to include PEX, immunosuppressive therapy, and CABLIVI
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Starting CABLIVI early is believed to have the greatest benefit in the early phase of acute aTTP/iTTP events (initial or relapsing)
Identifying aTTP/iTTP is crucial for initiation of an appropriate therapeutic strategy2
Clinical assessment‡ | OR |
Risk assessment tools¶ |
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Thrombocytopenia Evidence of MAHA§ Relatively preserved renal function |
Available risk assessment tools include:
The higher the risk assessment score, the more likely patients have severe ADAMTS13 deficiency and aTTP/iTTP |
†The ISTH TTP Guidelines refer to aTTP as iTTP.
‡List includes laboratory tests and results only; exclusive of physical symptoms, such as petechiae.
§Hb and hematocrit below reference range, low haptoglobin, elevated LDH, presence of schistocytes in peripheral blood smear.
¶ISTH did not appraise the evidence for these 2 tools.
Recommended diagnostic and management strategy for initial, acute events with access to ADAMTS13 results within 7 days
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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.2
‡List includes laboratory tests and results only; exclusive of physical symptoms, such as petechiae.
Adapted from ISTH Guidelines for Diagnosis of TTP.
<10%
Continue CABLIVI or consider STARTING CABLIVI*
10%–20%
Use clinical judgment to guide treatment and consider other diagnoses
>20%
Stop CABLIVI and consider other diagnoses
According to the ISTH TTP Guidelines, treatment of relapses for a patient previously diagnosed with aTTP/iTTP could be started safely based on clinical grounds without the need for a confirmatory ADAMTS13 test2
Timely access to ADAMTS13 results is key to providing optimal care for patients with aTTP/iTTP
Select US labs testing ADAMTS13 activity, inhibitors, and antibodies:
These listings do not constitute an endorsement by Sanofi and are not included in the ISTH Guidelines. The above is a selection of national laboratories offering ADAMTS13 tests for activity, inhibitor, and antibody testing. This is not an exhaustive list of labs that offer one or more of these tests or an endorsement of any lab. Other testing options may be available, including at local or regional laboratories. Content is current as of July 2020, and tests may not be available in all states. Please call laboratory to confirm test availability, sample shipping information, and all other logistics.
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
ADAMTS13=a disintegrin and metalloproteinase with a thrombospondin type 1 motif, 13; Hb=hemoglobin; ISTH=International Society on Thrombosis and Haemostasis; LDH=lactate dehydrogenase; MAHA=microangiopathic hemolytic anemia; PEX=plasma exchange; TTP=thrombotic thrombocytopenic purpura.
INDICATIONS
References: 1. Zheng XL, Vesely SK, Cataland SR, et al. ISTH guidelines for treatment of thrombotic thrombocytopenic purpura. J Thromb Haemost. 2020;18(10):2496-2502. doi:10.1111/jth.15010 2. 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 3. Oliveira DS, Lima TG, Neri Benevides FL, et al. PLASMIC score applicability for the diagnosis of thrombotic microangiopathy associated with ADAMTS13-acquired deficiency in a developing country. Hematol Transfus Cell Ther. 2019;41(2):119-124. doi:10.1016/j.htct.2018.10.002
