ORIGINAL ARTICLE

Evaluation of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies: Lessons learned from a 14-year retrospective study

Jason Tau

Department of Neurobiology, Physiology, and Behavior, University of California, Davis, California, USA

Leonor P. Fernando

Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, California, USA

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Meilen C. Munoz,

Meilen C. Munoz

Department of Internal Medicine, University of California Davis Medical Center, Sacramento, California, USA

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Christina Poh,

Christina Poh

Division of Hematology-Oncology, University of California Davis Medical Center, Sacramento, California, USA

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Viswanathan V. Krishnan,

Viswanathan V. Krishnan

orcid.org/0000-0002-0726-950X

Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, California, USA

Department of Chemistry and Biochemistry, California State University, Fresno, California, USA

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Denis M. Dwyre,

Corresponding Author

Denis M. Dwyre

[email protected]

orcid.org/0000-0002-4345-2731

Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, California, USA

Correspondence

Denis M. Dwyre, Department of Pathology and Laboratory Medicine, University of California Davis Medical Center Sacramento, Davis, CA, USA.

Email: [email protected]

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Jason Tau,

Jason Tau

Department of Neurobiology, Physiology, and Behavior, University of California, Davis, California, USA

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Leonor P. Fernando,

Leonor P. Fernando

Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, California, USA

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Meilen C. Munoz,

Meilen C. Munoz

Department of Internal Medicine, University of California Davis Medical Center, Sacramento, California, USA

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Christina Poh,

Christina Poh

Division of Hematology-Oncology, University of California Davis Medical Center, Sacramento, California, USA

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Viswanathan V. Krishnan,

Viswanathan V. Krishnan

orcid.org/0000-0002-0726-950X

Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, California, USA

Department of Chemistry and Biochemistry, California State University, Fresno, California, USA

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Denis M. Dwyre,

Corresponding Author

Denis M. Dwyre

[email protected]

orcid.org/0000-0002-4345-2731

Department of Pathology and Laboratory Medicine, University of California Davis Medical Center, Sacramento, California, USA

Correspondence

Denis M. Dwyre, Department of Pathology and Laboratory Medicine, University of California Davis Medical Center Sacramento, Davis, CA, USA.

Email: [email protected]

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First published: 02 May 2022

https://doi.org/10.1111/1744-9987.13864

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Abstract

Introduction

Thrombotic thrombocytopenic purpura (TTP) is a clinical thrombotic microangiopathy (TMA) syndrome defined by the pentad of symptoms. Therapeutic plasma exchange with plasma replacement is an ASFA Category I modality that can reduce morbidity and mortality if initiated early. We describe a 14-year review of patients referred for plasma exchange with a suspected diagnosis of TTP.

Methods

For 70 patients referred for urgent plasma exchange, clinical, therapeutic, and laboratory data were retrospectively analyzed, and the diagnosis was determined.

Results

Fifteen of the patients were diagnosed with TTP based upon ADAMTS-13 activity with the other 51 patients having other non-TTP TMA diagnoses. The mortality rate was significant for both TTP and non-TTP TMAs. PLASMIC scores were also calculated retrospectively and were noted to have limited value. TMA is a diagnostic challenge and encompasses different syndromes with similar presentations.

Conclusion

Determining an accurate diagnosis, including prompt ADAMTS-13 testing, makes it possible to initiate appropriate therapy for the multiple different TMAs that can be seen in clinical practice.

CONFLICT OF INTEREST

The authors declare no conflict of interest.

Open Research

DATA AVAILABILITY STATEMENT

Data are contained within the article.

Supporting Information

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Volume27, Issue1

February 2023

Pages 136-145

Evaluation of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies: Lessons learned from a 14-year retrospective study

Abstract

Introduction

Methods

Results

Conclusion

CONFLICT OF INTEREST

Open Research

DATA AVAILABILITY STATEMENT

Supporting Information

REFERENCES

References

Information

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Evaluation of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies: Lessons learned from a 14-year retrospective study

Abstract

Introduction

Methods

Results

Conclusion

CONFLICT OF INTEREST

Open Research

DATA AVAILABILITY STATEMENT

Supporting Information

REFERENCES

References

Related

Information