For U.S. Healthcare Professionals Only


Sanofi Genzyme is committed to ensuring all patients with aTTP have access to treatment with CABLIVI. The information below will help guide you in obtaining coverage for your patients.

*This information is subject to change and providers should consult relevant references for the description of each code to determine its appropriateness. Use of the information below does not guarantee that the payer will provide coverage for CABLIVI and is not intended to be a substitute for, or an influence on, the independent medical judgment of the prescriber.


The codes discussed below are provided for informational purposes only, are subject to change, and should not be construed as legal advice. The codes listed herein may not apply to all patients or to all health plans. Conversely, additional codes not listed may apply to some patients.

Like other drugs, CABLIVI is expected to be bundled into inpatient payment rates (ie, MS-DRGs) when used in the hospital setting. The 3 MS-DRGs that represent the greatest number of potential patients who may be eligible for treatment involving CABLIVI are shown in the table below.

Potential MS-DRGs1
545 Connective Tissue Disorders With MCC
546 Connective Tissue Disorders With CC
547 Connective Tissue Disorders Without CC/MCC
This may not be reflective of all MS-DRG codes that may be used for CABLIVI. The DRG code is determined by the payer based on the primary diagnosis.

Other reimbursement considerations

The specifics of coverage may vary by payer. Please refer to the individual patient’s plan to determine any applicable coverage requirements.

For patients covered by Medicare, drug costs for doses administered in the hospital are typically included in the MS-DRG payment and are covered under Medicare Part A. After inpatient discharge, most patients will self-administer CABLIVI at home; these drug costs are expected to be covered under the Medicare Part D (pharmacy) benefit.

New Technology Add-on Payment (NTAP)

CMS has approved CABLIVI’s application for an NTAP for FY2020, effective October 1, 2019. The maximum NTAP for CABLIVI is $33,215 for FY2020. Specifically, if the cost of CABLIVI exceeds the full DRG payment (including payments for IME and DSH, but excluding outlier payments), Medicare will make an add-on payment equal to the lesser of: 65% of the cost of CABLIVI or 65% of the amount by which the costs of the case exceed the standard DRG payment.2,3

The specifics of coverage may vary by payer and can be specific to the patient’s unique plan. Please reference the individual patient’s plan to determine any applicable coverage requirements.

Diagnosis and procedure codes

There is no specific ICD-10-CM code for aTTP. Providers should always review payer-specific materials and use their own clinical judgment when submitting claims for use of CABLIVI. However, there are 3 ICD-10-PCS codes that describe the specific procedure of injecting CABLIVI in the hospital inpatient setting to ensure that NTAP is considered.

ICD-10-CM Code4
M31.1 Thrombotic Microangiopathy
ICD-10-PCS Code5
XW013W5 Introduction of Caplacizumab Into Subcutaneous Tissue, Percutaneous Approach, New Technology Group 5
XW033W5 Introduction of Caplacizumab Into Peripheral Vein, Percutaneous Approach, New Technology Group 5
XW043W5 Introduction of Caplacizumab Into Central Vein, Percutaneous Approach, New Technology Group 5
CABLIVI Billing and Coding Guide

CABLIVI Billing and Coding Guide

Find more information regarding coverage and other reimbursement considerations.

CABLIVI Hospital Guide

CABLIVI Hospital Guide

This guide highlights key information related to the procurement of, and patient access to, CABLIVI.

aTTP=acquired thrombotic thrombocytopenia purpura; CC=complication or comorbidity; CMS=Centers for Medicare and Medicaid Services; DRG=Diagnosis Related Group; DSH=disproportionate share hospital; ICD-10-CM=International Classification of Diseases, Tenth Revision, Clinical Modification; ICD-10-PCS=International Classification of Diseases, Tenth Revision, Procedure Coding System; IME=indirect medical education; MCC=major complication or comorbidity; MS-DRG=Medicare Severity Diagnosis Related Group.

Thrombotic microangiopathy has the inclusion note of thrombotic thrombocytopenic purpura, which would describe aTTP.



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.


Bleeding Risk:

  • 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. The risk of bleeding is increased, in patients with underlying coagulopathies and concomitant use of CABLIVI with drugs affecting hemostasis.
  • 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.


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


Concomitant use of CABLIVI with any anticoagulant may increase the risk of bleeding. Assess and monitor closely for bleeding with concomitant use.


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.


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.