Medication Summary

Platelet inhibition is helpful in patients with thrombocytosis. Aspirin is most often used, but anagrelide may also be effective.^[52]The optimal pharmacologic therapy in primary erythromelalgia is still unknown.

Class Summary

Aspirin is preferred because it provides relief lasting longer than that of indomethacin or other nonsteroidal anti-inflammatory drugs (NSAIDs), presumably because of irreversible platelet inhibition.

Aspirin

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Aspirin inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2.

Anagrelide (Agrylin)

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Anagrelide may inhibit cyclic nucleotide phosphodiesterase and the release of arachidonic acid from phospholipase possibly by inhibiting phospholipase A2. A decrease in megakaryocyte hypermaturation may result in a reduction in platelet production.

Questions

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Media Gallery

Cardinal symptoms of erythromelalgia.

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Author

Drew H Barnes, MD Fellow Physician, Hospice and Palliative Medicine, The Ohio State University Wexner Medical Center

Drew H Barnes, MD is a member of the following medical societies: American Academy of Hospice and Palliative Medicine, American College of Physicians, American Medical Association, American Society of Clinical Oncology, American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Claudia M da Costa Dourado, MD Clinical Assistant Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Program Director, Hematology and Medical Oncology Fellowship Program, Attending Physician, Division of Hematology and Medical Oncology, Department of Medicine, Einstein Medical Center Philadelphia

Claudia M da Costa Dourado, MD is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Ali T Taher, MD, PhD, FRCP Professor of Medicine, Associate Chair of Research, Department of Internal Medicine, Division of Hematology/Oncology, Director of Research, NK Basile Cancer Center, American University of Beirut Medical Center, Lebanon

Disclosure: Nothing to disclose.

Antoine N Saliba, MD Post-Doctoral Research Fellow, Division of Hematology/Oncology, Department of Internal Medicine, American University of Beirut Medical Center

Disclosure: Nothing to disclose.

Acknowledgements

Robert J Nardino, MD Director, Internal Medicine Residency Program, Assistant Clinical Professor, Department of Internal Medicine, Hospital of Saint Raphael, Yale University School of Medicine

Robert J Nardino, MD is a member of the following medical societies: American College of Physicians, Association of Program Directors in Internal Medicine, and Society of General Internal Medicine

Disclosure: Nothing to disclose.

Andrea LM Silber, MD Medical Oncologist, Father Michael J McGivney Center for Cancer Care, Saint Raphael Hospital

Andrea LM Silber, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine and American Society of Preventive Oncology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment