Larynx Angioedema as a Signal in Chronic Lymphocytic Leukemia: A Case-based Guide for Acquired Angioedema in the Setting of Lymphoproliferative Disorders

IF 0.3 Q4 ALLERGY

Astim Allerji Immunoloji Pub Date : 2021-08-23 DOI:10.21911/aai.614

Z. Kisakurek, Sadi Can Sönmez, E. Osmanbaşoğlu, E. Öztürk, A. Öztürk

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Abstract

ABSTRACT Patients with angioedema can present to the internal medicine, emergency medicine, dermatology, or ear nose throat clinics. Physicians may need to assess the patients whose angioedema is unresponsive to antihistamines systematically in collaboration with other subspecialties including hematology, rheumatology, allergy, and immunology. We aimed to provide a concise review of the diagnosis and multi-disciplinary management of acquired angioedema through a case presentation. A 61-year-old woman presented with recurrent angioedema of 4 episodes within one year. She was evaluated by various disciplines such as dermatology and emergency medicine. Antihistamines and steroids were not effective. The complete blood count (CBC) results indicated lymphocytosis (lymphocyte count=9100 k/μL) and further evaluation of the lymphocytosis with flow cytometry immunophenotyping confirmed a diagnosis of chronic lymphocytic leukemia. Since the acquired angioedema diagnosis was confirmed with low C4, C1q, and C1 esterase inhibitor levels, Rituximab 375 mg/m2 was administered once a week for 4 weeks. The frequency of attacks decreased after rituximab therapy and none of them were life-threatening. In conclusion, when the effective treatment is initiated for the primary diagnosis in acquired angioedema, the numerous emergency department visits, hospitalizations, and the mortality due to life-threatening angioedema episodes can be avoided. Keywords: Angioedema, larynx angioedema, emergency, chronic lymphocytic leukemia, life-threatening angioedema

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慢性淋巴细胞白血病的喉头血管水肿信号：淋巴增生性疾病背景下获得性血管水肿的病例指南

摘要血管性水肿患者可到内科、急诊、皮肤科或耳鼻咽喉科就诊。医生可能需要与血液学、风湿病、过敏和免疫学等其他亚专业合作，系统评估血管性水肿对抗组胺药无反应的患者。我们旨在通过一个病例介绍，对获得性血管性水肿的诊断和多学科治疗进行简要回顾。一位61岁的女性在一年内出现4次复发性血管性水肿。她接受了皮肤科和急诊医学等多个学科的评估。抗组胺药和类固醇无效。全血细胞计数（CBC）结果显示淋巴细胞增多（淋巴细胞计数=9100 k/μL），流式细胞术免疫表型对淋巴细胞增多的进一步评估证实了慢性淋巴细胞白血病的诊断。由于获得性血管性水肿诊断为C4、C1q和C1酯酶抑制剂水平低，因此利妥昔单抗每周给药一次，持续4周。利妥昔单抗治疗后，发作频率降低，没有一例危及生命。总之，当对获得性血管性水肿的初步诊断开始有效治疗时，可以避免因危及生命的血管性水肿发作而导致的大量急诊就诊、住院和死亡。关键词：血管性水肿，喉血管性水肿；急诊；慢性淋巴细胞白血病；危及生命的血管性水肿

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来源期刊

Astim Allerji Immunoloji ALLERGY-

CiteScore

0.80

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0.00%

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期刊介绍： Asthma Allergy Immunology has been published three times a year in April, August and December as the official and periodical journal of the Turkish National Society of Allergy and Clinical Immunology since 2003. All articles published in the journal have been available online since 2003. A peer reviewed system is used in evaluation of the manuscripts submitted to Asthma Allergy Immunology. The official language of the journal is English. The aim of the journal is to present advances in the field of allergic diseases and clinical immunology to the readers. In accordance with this goal, manuscripts in the format of original research, review, case report, articles about clinical and practical applications and editorials, short report and letters to the editor about allergic diseases and clinical immunology are published in the journal. The target reader population of the Asthma Allergy Immunology includes specialists and residents of allergy and clinical immunology, pulmonology, internal medicine, pediatrics, dermatology and otolaryngology as well as physicians working in other fields of medicine interested in allergy and immunological diseases.