Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich syndrome) : Frequency, clinical implication, and prognosis
Copyright © 2019. Published by Elsevier Inc..
BACKGROUND: Episodic angioedema with eosinophilia (EAE) (Gleich syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia, and frequent elevated serum IgM level.
METHODS: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France.
RESULTS: A total of 30 patients with a median age at diagnosis of 41 years (range, 5-84) were included. The median duration of each crisis was 5.5 days (range, 1-90), with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%), of whom 5 (17%) showed evidence of clonal T-cell receptor gamma locus gene (TRG) rearrangement. The median duration of follow-up was 53 months (range, 31-99). The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio, 4.15; 95% confidence interval, 1.18-14.66; P = .02). At last follow-up, 3 patients (10%) were able to have all treatments withdrawn and 11 (37%) were in clinical and biologic remission with less than 10 mg of prednisone daily.
CONCLUSION: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare.
| Errataetall: |
CommentIn: J Am Acad Dermatol. 2023 May;88(5):1006-1007. - PMID 36958597 |
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| Medienart: |
E-Artikel |
| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:88 |
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| Enthalten in: |
Journal of the American Academy of Dermatology - 88(2023), 5 vom: 10. Mai, Seite e243-e250 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Abisror, Noémie [VerfasserIn] |
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| Links: |
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| Themen: |
Angioedema |
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| Anmerkungen: |
Date Completed 19.04.2023 Date Revised 20.04.2023 published: Print-Electronic CommentIn: J Am Acad Dermatol. 2023 May;88(5):1006-1007. - PMID 36958597 Citation Status MEDLINE |
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| doi: |
10.1016/j.jaad.2019.02.001 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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NLM293640076 |
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| 245 | 1 | 0 | |a Abnormal T-cell phenotype in episodic angioedema with hypereosinophilia (Gleich syndrome) |b Frequency, clinical implication, and prognosis |
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| 500 | |a Date Completed 19.04.2023 | ||
| 500 | |a Date Revised 20.04.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a CommentIn: J Am Acad Dermatol. 2023 May;88(5):1006-1007. - PMID 36958597 | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a Copyright © 2019. Published by Elsevier Inc. | ||
| 520 | |a BACKGROUND: Episodic angioedema with eosinophilia (EAE) (Gleich syndrome) is a rare disorder consisting of recurrent episodes of angioedema, hypereosinophilia, and frequent elevated serum IgM level | ||
| 520 | |a METHODS: We conducted a retrospective multicenter nationwide study regarding the clinical spectrum and therapeutic management of patients with EAE in France | ||
| 520 | |a RESULTS: A total of 30 patients with a median age at diagnosis of 41 years (range, 5-84) were included. The median duration of each crisis was 5.5 days (range, 1-90), with swelling affecting mainly the face and the upper limbs. Total serum IgM levels were increased in 20 patients (67%). Abnormal T-cell immunophenotypes were detected in 12 patients (40%), of whom 5 (17%) showed evidence of clonal T-cell receptor gamma locus gene (TRG) rearrangement. The median duration of follow-up was 53 months (range, 31-99). The presence of an abnormal T-cell population was the sole factor associated with a shorter time to flare (hazard ratio, 4.15; 95% confidence interval, 1.18-14.66; P = .02). At last follow-up, 3 patients (10%) were able to have all treatments withdrawn and 11 (37%) were in clinical and biologic remission with less than 10 mg of prednisone daily | ||
| 520 | |a CONCLUSION: EAE is a heterogeneous condition that encompasses several disease forms. Although patients usually respond well to glucocorticoids, those with evidence of abnormal T-cell phenotype have a shorter time to flare | ||
| 650 | 4 | |a Multicenter Study | |
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| 650 | 4 | |a lymphoma (T-cell, peripheral) | |
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