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Erythromelalgia and myeloproliferative disorders
1Department of Clinical Immunology and Biological Therapy, University of Texas M.D. Anderson Hospital and Tumor Institute, Houston 77030.
Archives of Internal Medicine
|January 1, 1989
Summary
Erythromelalgia, a condition causing burning pain and redness, can signal underlying myeloproliferative disorders. Early blood monitoring in erythromelalgia patients is crucial for detecting these serious conditions.
Area of Science:
- Hematology
- Dermatology
- Oncology
Background:
- Erythromelalgia (erythermalgia) presents as episodic burning pain, erythema, and warmth in extremities, triggered by heat and exercise, relieved by cold.
- It exists in primary and secondary forms, with secondary erythromelalgia associated with myeloproliferative disorders like polycythemia vera and essential thrombocythemia.
Observation:
- This study details the first reported case of erythromelalgia associated with chronic myelogenous leukemia.
- A review of 60 global cases of erythromelalgia in myeloproliferative syndromes is presented, comparing primary and secondary forms.
Findings:
- Crucially, erythromelalgia symptoms preceded myeloproliferative disease onset by a median of 2.5 years.
- Abnormal blood counts (hemoglobin, WBC, platelets) or immature cells in patients with erythromelalgia suggest an underlying myeloproliferative disorder, distinguishing it from idiopathic cases.
Implications:
- Periodic blood cell count monitoring is recommended for all erythromelalgia patients to detect potential myeloproliferative diseases early.
- Treatment of the myeloproliferative syndrome can alleviate erythromelalgia symptoms; aspirin also shows significant efficacy in managing both primary and secondary erythromelalgia.