SPECTRA OPTIA
Report
- Report Number
- 1722028-2024-00320
- Event Type
- Injury
- Date Received
- August 6, 2024
- Date of Event
- November 5, 2020
- Report Date
- August 6, 2024
- Manufacturer
- TERUMO BCT
- Product Code
- LKN
- UDI-DI
- 05020583122208
- PMA / PMN Number
- K183081
- Adverse Event
- Yes
- Report Source
- Manufacturer report
- Reporter Location
- SP
- Reporter Occupation
- OTHER HEALTH CARE PROFESSIONAL
- Health Professional
- Yes
Narratives
INVESTIGATION IS IN PROCESS, A FOLLOW-UP REPORT WILL BE PROVIDED. CITATION: CID, J., PREZ-VALENCIA, A. I., TORRENTE, M. VAREZ-LARRN, A., DAZ-RICART, M., ESTEVE, J., & LOZANO, M. (2021). SUCCESSFUL MANAGEMENT OF THREE PATIENTS WITH AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA WITH PARADIGM-CHANGING THERAPY: CAPLACIZUMAB, STEROIDS, PLASMA EXCHANGE, RITUXIMAB, AND INTRAVENOUS IMMUNOGLOBULINS (CASPERI). TRANSFUSION AND APHERESIS SCIENCE, 60(1), 103011. HTTPS://DOI.ORG/10.1016/J.TRANSCI.2020.103011.
THIS REPORT IS BEING FILED TO PROVIDE ADDITIONAL INFORMATION IN H.6 AND H.11. INVESTIGATION: SINCE THIS WAS A JOURNAL PUBLICATION INTO THE SUCCESSFUL MANAGEMENT OF THREE PATIENTS WITH AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA WITH PARADIGM-CHANGING THERAPY INVOLVING PLASMA EXCHANGE (PE) PERFORMED WITH SPECTRA OPTIA IN 2020, THE LOT NUMBERS WERE NOT REQUESTED; THEREFORE, A DISPOSABLE LOT HISTORY SEARCH COULD NOT BE CONDUCTED. SINCE THIS WAS A JOURNAL PUBLICATION INTO THE SUCCESSFUL MANAGEMENT OF THREE PATIENTS WITH AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA WITH PARADIGM-CHANGING THERAPY INVOLVING PLASMA EXCHANGE (PE) PERFORMED WITH SPECTRA OPTIA IN 2020, THE LOT NUMBERS WERE NOT REQUESTED; THEREFORE, A DHR SEARCH COULD NOT BE CONDUCTED FOR THIS SPECIFIC INCIDENT. ALL LOTS MUST MEET ACCEPTANCE CRITERIA FOR RELEASE. WE REVIEWED THREE PATIENTS WHO WERE DIAGNOSED AT HOSPITAL CLÍNIC DURING 2020 WITH A FIRST EPISODE OF ATTP. THE DIAGNOSIS WAS MADE BECAUSE PATIENTS HAD MICROANGIOPATHIC HEMOLYTIC ANEMIA, THROMBOCYTOPENIA, ORGAN FAILURE, ADAMTS13 ACTIVITY LESS THAN 5%, AND ADAMTS13 AUTOANTIBODIES WERE DETECTED. THE STUDY PROTOCOL WAS REVIEWED AND APPROVED BY THE ETHICS COMMITTEE OF THE HOSPITAL CLÍNIC. ALL PATIENTS SIGNED AN INFORMED CONSENT TO RECEIVE TREATMENT AND CONSENTED TO DATA RELEASE. THERAPEUTIC PLAN: CAPLACIZUMAB (CABLIVI, SANOFI-AVENTIS, MADRID, SPAIN) WAS ADMINISTERED FOLLOWING MANUFACTURER¿S INSTRUCTIONS. TWO DOSES OF CAPLACIZUMAB WERE ADMINISTERED THE FIRST DAY: ONE DOSE OF CAPLACIZUMAB (10 MG INTRAVENOUS) WAS ADMINISTERED BEFORE PERFORMING THE FIRST PE AND A SECOND DOSE (10 MG SUBCUTANEOUS) WAS ADMINISTERED AFTER FINISHING THE FIRST PE. SUBSEQUENT DOSES (10 MG SUBCUTANEOUS) WERE ADMINISTERED EVERY DAY AFTER PERFORMING PE. PE WAS PERFORMED WITH SPECTRA OPTIA (TERUMO BCT, LEUVEN, BELGIUM) AS PREVIOUSLY REPORTED [8]. BRIEFLY, TRAINED NURSES EVALUATED VASCULAR ACCESS AND, IF NECESSARY, A CENTRAL LINE WAS PLACED BY TRAINED PERSONNEL IN THE ANGIORADIOLOGY UNIT UNDER ULTRASOUND AND X-RAY CONTROL. WE EXCHANGED 1.0¿1.5 PLASMA VOLUMES PER SESSION. FLUID REPLACEMENT USED WAS QUARANTINED OR METHYLENE BLUE-INACTIVATED FRESH FROZEN PLASMA (FFP), 5% ALBUMIN SOLUTION (ALBUTEIN 5 %, GRIFOLS, BARCELONA, SPAIN), OR BOTH. CITRATE (ACD-A, GRIFOLS) WAS USED AS ANTICOAGULANT AND PROPHYLACTIC CA-MG SOLUTION WAS ADMINISTERED THROUGHOUT THE PE. STEROIDS (METHYLPREDNISOLONE) WERE ADMINISTERED AT A DOSE OF 1 MG/ KG/DAY WHILE PATIENT WAS RECEIVING PE PROCEDURES. THEN, DOSE WAS REDUCED TO 0.5 MG/KG/DAY AND FURTHER PROGRESSIVE REDUCTION WAS PERFORMED. RITUXIMAB WAS ADMINISTERED WEEKLY AT A DOSE OF 375 MG/M2 /WEEK FOR 4 WEEKS. FIRST DOSE WAS ADMINISTERED WHEN ADAMTS13 AUTOANTIBODIES WERE DETECTED. AFTER PERFORMING PE PROCEDURES USING ALBUMIN AS REPLACEMENT SOLUTION, INTRAVENOUS IMMUNOGLOBULINS (IVIG; FLEBOGAMMA, GRIFOLS) WERE ADMINISTERED AT A DOSE OF 100 MG/KG PER PE PROCEDURE. RESULTS: PATIENT 2 WAS A 61-YEAR-OLD WOMAN WITH AN UNEVENTFUL PAST MEDICAL HISTORY WHO WAS ATTENDED IN THE EMERGENCY ROOM (DAY 1) BECAUSE OF AN ACUTE EPISODE OF TRANSIENT APHASIA. LABORATORY TESTS ARE SHOWN ON TABLE 1. A CEREBRAL CT SCAN WAS PERFORMED WITH NO RELEVANT FINDINGS. TMA WAS DIAGNOSED AND ATTP WAS HIGHLY SUSPECTED. CAPLACIZUMAB, PE, AND STEROIDS WERE STARTED ON DAY 1 IN THE MORNING, AND RITUXIMAB WAS ADDED ON DAY 4, WHEN THE RESULTS OF ADAMTS13 ACTIVITY AND AUTOANTI BODIES WERE KNOWN. FIG. 1B SHOWS THE EVOLUTION OF PLATELET COUNT, ADAMTS13 ACTIVITY AND ADAMTS13 AUTOANTIBODIES WHILE THE PATIENT WAS RECEIVING TREATMENT. BECAUSE OF OUR PREVIOUS EXPERIENCE, WITH THE AIM OF REDUCING THE QUANTITY OF AUTOANTIBODIES AS FAST AS POSSIBLE, WE DECIDED TO PERFORM DAILY PES UNTIL WE OBSERVED THAT ADAMTS13 AUTO ANTIBODIES WERE NEGATIVE. HOWEVER, THE PATIENT EXPERIENCED A SEVERE ALLERGIC REACTION TO FFP USED AS REPLACEMENT SOLUTION DURING THE 6TH PE PROCEDURE AND WE DECIDED TO CONTINUE PERFORMING PE PROCEDURES EVERY OTHER DAY USING ALBUMIN AS REPLACEMENT SOLUTION. AFTER 4 ADDITIONAL PE PROCEDURES USING ALBUMIN AS REPLACEMENT SOLUTION, ADAMTS13 AUTO ANTIBODIES WERE NEGATIVE, AND IVIG AT A DOSE OF 400 MG/KG WAS ADMINISTERED. RITUXIMAB WAS ALSO ADMINISTERED ON DAYS 10, 17, AND 24. CAPLACIZUMAB WAS ADMINISTERED DAILY UNTIL DAY 21. AT LAST FOLLOW-UP, AFTER 3 MONTHS FROM DIAGNOSIS, THE PATIENT WAS WELL WITH PLATELET COUNT 281 × 109 /L, ADAMTS 13 ACTIVITY 19 %, AND ADAMTS13 AUTO ANTIBODIES WERE NEGATIVE. IN SUMMARY, THE AUTHORS PRESENTED THEIR PARADIGM-CHANGING THERAPEUTIC STRATEGY FOR PATIENTS WITH ATTP THAT INCLUDES CAPLACIZUMAB, STEROIDS, PE, RITUXIMAB, AND IVIG (CASPERI). WITH SUCH A COMBINATION, THE AUTHORS SHOWED ITS SAFETY AND EFFICACY AND OBTAINED SUCCESSFUL OUTCOMES IN THREE PATIENTS WITH ATTP. ACCORDING TO THERAPEUTIC APHERESIS: A PHYSICIAN'S HANDBOOK, ADVERSE EVENTS OCCUR DURING THERAPEUTIC PROCEDURES WITH A FREQUENCY OF 4.8%. SYMPTOMS OF THESE ALLERGIC REACTIONS MAY INCLUDE HIVES, DYSPNEA, WHEEZING, BURNING EYES, TACHYCARDIA, HYPOTENSION, AND OR FACIAL SWELLING AND FLUSHING. MILD REACTIONS CAN BE TREATED WITH DIPHENHYDRAMINE ADMINISTERED THROUGH AN IV. ROOT CAUSE: BASED ON THE AVAILABLE INFORMATION WITHIN THE JOURNAL ARTICLE, THE AUTHORS ATTRIBUTED THE SEVERE ALLERGIC REACTION, EXPERIENCED BY PATIENT 2, TO THE FRESH FROZEN PLASMA (FFP) USED AS REPLACEMENT SOLUTION DURING TPE. CITATION: CID, J., PÉREZ-VALENCIA, A. I., TORRENTE, M. Á., ÁVAREZ-LARRÁN, A., DÍAZ-RICART, M., ESTEVE, J., & LOZANO, M. (2021). SUCCESSFUL MANAGEMENT OF THREE PATIENTS WITH AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA WITH PARADIGM-CHANGING THERAPY: CAPLACIZUMAB, STEROIDS, PLASMA EXCHANGE, RITUXIMAB, AND INTRAVENOUS IMMUNOGLOBULINS (CASPERI). TRANSFUSION AND APHERESIS SCIENCE, 60(1), 103011. HTTPS://DOI.ORG/10.1016/J.TRANSCI.2020.103011.
PER JOURNAL ARTICLE "SUCCESSFUL MANAGEMENT OF THREE PATIENTS WITH AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA WITH PARADIGM-CHANGING THERAPY: CAPLACIZUMAB, STEROIDS, PLASMA EXCHANGE, RITUXIMAB, AND INTRAVENOUS IMMUNOGLOBULINS (CASPERI)" BY JOAN CID, AMANDA ISABEL PEREZ-VALENCIA, MIGUEL ANGEL TORRENTE, ALBERTO AVAREZ-LARRAN, MARIBEL DAZ-RICART, JORDI ESTEVE, MIQUEL LOZANO AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IS A SEVERE DISEASE CAUSED BY THE PRODUCTION OF AUTOANTIBODIES AGAINST VON WILLEBRAND FACTOR (VWF)-CLEAVING ADAMTS13 (A DISINTEGRIN AND METALLOPROTEINASE WITH THROMBOSPONDIN-1 MOTIFS; 13TH MEMBER OF THE FAMILY). IN 2018, CAPLACIZUMAB WAS APPROVED FOR THE TREATMENT OF PATIENTS WITH ACUTE ATTP IN CONJUNCTION WITH PLASMA EXCHANGE (PE) AND IMMUNOSUPPRESSIVE THERAPY. IMMUNOSUPPRESSIVE STANDARD OF CARE INCLUDES MAINLY STEROIDS WHEREAS RITUXIMAB IS USUALLY RESERVED FOR REFRACTORY CASES. WE REPORT THREE PATIENTS WITH A FIRST ACUTE EPISODE OF ATTP WHO WERE SUCCESSFULLY TREATED WITH A PARADIGM-CHANGING SCHEME INCLUDING STANDARD OF CARE (CAPLACIZUMAB, STEROIDS AND PE) PLUS UPFRONT THERAPY WITH RITUXIMAB AND INTRAVENOUS IMMUNOGLOBULINS (CASPERI). RITUXIMAB WAS ADDED 14 DAYS AFTER DIAGNOSIS, WHEN ADAMTS13 AUTOANTIBODIES WERE DETECTED AND INTRAVENOUS IMMUNOGLOBULINS WERE ADMINISTERED AFTER PERFORMING PE USING ALBUMIN AS REPLACEMENT SOLUTION. SUCCESSFUL OUTCOME WAS OBSERVED IN ALL THREE PATIENTS: PLATELET RECOVERY (150 109/L) WAS OBSERVED AFTER 3, 4, AND 5 DAYS FROM DIAGNOSIS; ADAMTS13 ACTIVITY 5% AND ADAMTS13 AUTOANTIBODIES WERE NEGATIVE AFTER 14, 15, AND 21 DAYS FROM DIAGNOSIS. IN CONCLUSION, CAPLACIZUMAB, STEROIDS, PE (USING FRESH FROZEN PLASMA OR ALBUMIN AS REPLACEMENT SOLUTION AND ADDING INTRAVENOUS IMMUNOGLOBULINS) PLUS UPFRONT RITUXIMAB THERAPY WAS A SAFE AND EFFICIENT COMBINATION TO INDUCE REMISSION IN CASE OF ACUTE ATTP. PATIENT 2 WAS A 61-YEAR-OLD WOMAN WITH AN UNEVENTFUL PAST MEDICAL HISTORY WHO WAS ATTENDED IN THE EMERGENCY ROOM (DAY 1) BECAUSE OF AN ACUTE EPISODE OF TRANSIENT APHASIA. A CEREBRAL CT SCAN WAS PERFORMED WITH NO RELEVANT FINDINGS. TMA WAS DIAGNOSED AND ATTP WAS HIGHLY SUSPECTED. CAPLACIZUMAB, PE, AND STEROIDS WERE STARTED ON DAY 1 IN THE MORNING, AND RITUXIMAB WAS ADDED ON DAY 4, WHEN THE RESULTS OF ADAMTS13 ACTIVITY AND AUTOANTIBODIES WERE KNOWN. BECAUSE OF OUR PREVIOUS EXPERIENCE, WITH THE AIM OF REDUCING THE QUANTITY OF AUTOANTIBODIES AS FAST AS POSSIBLE, WE DECIDED TO PERFORM DAILY PES UNTIL WE OBSERVED THAT ADAMTS13 AUTOANTIBODIES WERE NEGATIVE.THE PATIENT EXPERIENCED A SEVERE ALLERGIC REACTION TO FFP USED AS REPLACEMENT SOLUTION DURING THE 6TH PE PROCEDURE. THEY DECIDED TO CONTINUE PERFORMING PE PROCEDURES EVERY PROCEDURES USING ALBUMIN AS REPLACEMENT SOLUTION, ADAMTS13 AUTOANTIBODIES WERE NEGATIVE, AND IVIG AT A DOSE OF 400 MG/KG WAS ADMINISTERED. RITUXIMAB WAS ALSO ADMINISTERED ON DAYS 10, 17, AND 24. CAPLACIZUMAB WAS ADMINISTERED DAILY UNTIL DAY 21. AT LAST FOLLOW-UP, AFTER 3 MONTHS FROM DIAGNOSIS, THE PATIENT WAS WELL WITH PLATELET COUNT 281 109/L, ADAMTS 13 ACTIVITY 19 %, AND ADAMTS13 AUTOANTIBODIES WERE NEGATIVE. PATIENT WEIGHT IS NOT AVAILABLE. THE COLLECTION SET IS NOT AVAILABLE FOR RETURN BECAUSE IT WAS DISCARDED BY THE CUSTOMER.
PER JOURNAL ARTICLE "SUCCESSFUL MANAGEMENT OF THREE PATIENTS WITH AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA WITH PARADIGM-CHANGING THERAPY: CAPLACIZUMAB, STEROIDS, PLASMA EXCHANGE, RITUXIMAB, AND INTRAVENOUS IMMUNOGLOBULINS (CASPERI)" BY JOAN CID, AMANDA ISABEL P´EREZ-VALENCIA, MIGUEL ´ANGEL TORRENTE, ALBERTO ´AVAREZ-LARR´AN, MARIBEL DÍAZ-RICART, JORDI ESTEVE, MIQUEL LOZANO AUTOIMMUNE THROMBOTIC THROMBOCYTOPENIC PURPURA (ATTP) IS A SEVERE DISEASE CAUSED BY THE PRODUCTION OF AUTOANTIBODIES AGAINST VON WILLEBRAND FACTOR (VWF)-CLEAVING ADAMTS13 (A DISINTEGRIN AND METALLOPROTEINASE WITH THROMBOSPONDIN-1 MOTIFS; 13TH MEMBER OF THE FAMILY). IN 2018, CAPLACIZUMAB WAS APPROVED FOR THE TREATMENT OF PATIENTS WITH ACUTE ATTP IN CONJUNCTION WITH PLASMA EXCHANGE (PE) AND IMMUNOSUPPRESSIVE THERAPY. IMMUNOSUPPRESSIVE STANDARD OF CARE INCLUDES MAINLY STEROIDS WHEREAS RITUXIMAB IS USUALLY RESERVED FOR REFRACTORY CASES. WE REPORT THREE PATIENTS WITH A FIRST ACUTE EPISODE OF ATTP WHO WERE SUCCESSFULLY TREATED WITH A PARADIGM-CHANGING SCHEME INCLUDING STANDARD OF CARE (CAPLACIZUMAB, STEROIDS AND PE) PLUS UPFRONT THERAPY WITH RITUXIMAB AND INTRAVENOUS IMMUNOGLOBULINS (CASPERI). RITUXIMAB WAS ADDED 1¿4 DAYS AFTER DIAGNOSIS, WHEN ADAMTS13 AUTOANTIBODIES WERE DETECTED AND INTRAVENOUS IMMUNOGLOBULINS WERE ADMINISTERED AFTER PERFORMING PE USING ALBUMIN AS REPLACEMENT SOLUTION. SUCCESSFUL OUTCOME WAS OBSERVED IN ALL THREE PATIENTS: PLATELET RECOVERY (>150 × 109/L) WAS OBSERVED AFTER 3, 4, AND 5 DAYS FROM DIAGNOSIS; ADAMTS13 ACTIVITY >5% AND ADAMTS13 AUTOANTIBODIES WERE NEGATIVE AFTER 14, 15, AND 21 DAYS FROM DIAGNOSIS. IN CONCLUSION, CAPLACIZUMAB, STEROIDS, PE (USING FRESH FROZEN PLASMA OR ALBUMIN AS REPLACEMENT SOLUTION AND ADDING INTRAVENOUS IMMUNOGLOBULINS) PLUS UPFRONT RITUXIMAB THERAPY WAS A SAFE AND EFFICIENT COMBINATION TO INDUCE REMISSION IN CASE OF ACUTE ATTP. PATIENT 2 WAS A 61-YEAR-OLD WOMAN WITH AN UNEVENTFUL PAST MEDICAL HISTORY WHO WAS ATTENDED IN THE EMERGENCY ROOM (DAY 1) BECAUSE OF AN ACUTE EPISODE OF TRANSIENT APHASIA. A CEREBRAL CT SCAN WAS PERFORMED WITH NO RELEVANT FINDINGS. TMA WAS DIAGNOSED AND ATTP WAS HIGHLY SUSPECTED. CAPLACIZUMAB, PE, AND STEROIDS WERE STARTED ON DAY 1 IN THE MORNING, AND RITUXIMAB WAS ADDED ON DAY 4, WHEN THE RESULTS OF ADAMTS13 ACTIVITY AND AUTOANTIBODIES WERE KNOWN. BECAUSE OF OUR PREVIOUS EXPERIENCE, WITH THE AIM OF REDUCING THE QUANTITY OF AUTOANTIBODIES AS FAST AS POSSIBLE, WE DECIDED TO PERFORM DAILY PES UNTIL WE OBSERVED THAT ADAMTS13 AUTOANTIBODIES WERE NEGATIVE.THE PATIENT EXPERIENCED A SEVERE ALLERGIC REACTION TO FFP USED AS REPLACEMENT SOLUTION DURING THE 6TH PE PROCEDURE. THEY DECIDED TO CONTINUE PERFORMING PE PROCEDURES EVERY PROCEDURES USING ALBUMIN AS REPLACEMENT SOLUTION, ADAMTS13 AUTOANTIBODIES WERE NEGATIVE, AND IVIG AT A DOSE OF 400 MG/KG WAS ADMINISTERED. RITUXIMAB WAS ALSO ADMINISTERED ON DAYS 10, 17, AND 24. CAPLACIZUMAB WAS ADMINISTERED DAILY UNTIL DAY 21. AT LAST FOLLOW-UP, AFTER 3 MONTHS FROM DIAGNOSIS, THE PATIENT WAS WELL WITH PLATELET COUNT 281 × 109/L, ADAMTS 13 ACTIVITY 19 %, AND ADAMTS13 AUTOANTIBODIES WERE NEGATIVE. PATIENT WEIGHT IS NOT AVAILABLE. THE COLLECTION SET IS NOT AVAILABLE FOR RETURN BECAUSE IT WAS DISCARDED BY THE CUSTOMER.
Devices
| Seq | Brand | Generic | Product Code | Manufacturer | Model | Lot | UDI-DI |
|---|---|---|---|---|---|---|---|
| 1702416 | SPECTRA OPTIA | SPECTRA OPTIA EXCHANGE SET | LKN | TERUMO BCT | 05020583122208 |
Patients
| Seq | Age | Sex | Outcome | Treatment |
|---|---|---|---|---|
| 1 | 61 YR | Female | Other |