Acquired Angioedema Associated with Lymphoproliferative Disorders.

Autor: Ng JY; Department of Hematology, Canberra Hospital, Canberra, ACT, Australia., Ooi M; Department of Hematology, Canberra Hospital, Canberra, ACT, Australia., Bennett SK; Department of Hematology, Canberra Hospital, Canberra, ACT, Australia., Rady K; Department of Hematology, Canberra Hospital, Canberra, ACT, Australia., Choi P; Department of Hematology, Canberra Hospital, Canberra, ACT, Australia., Lee WI; Department of Immunology, Canberra Hospital, Canberra, ACT, Australia., Cook MC; Department of Immunology, Canberra Hospital, Canberra, ACT, Australia.; Centre for Personalised Immunology, John Curtin School of Medical Research, Australian National University, Canberra, ACT, Australia.; Department of Medicine and Cambridge Institute for Therapeutic Immunology and Infectious Disease, Jeffrey Cheah Biomedical Centre, University of Cambridge, Cambridge, UK., Randall KL; Department of Immunology, Canberra Hospital, Canberra, ACT, Australia.; School of Medicine and Psychology, Australian National University, Canberra, ACT, Australia., Pati NK; Department of Hematology, Canberra Hospital, Canberra, ACT, Australia.
Jazyk: angličtina
Zdroj: Case reports in oncology [Case Rep Oncol] 2024 Feb 23; Vol. 17 (1), pp. 329-336. Date of Electronic Publication: 2024 Feb 23 (Print Publication: 2024).
DOI: 10.1159/000536458
Abstrakt: Introduction: Acquired angioedema due to C1 esterase inhibitor deficiency (C1INH-AAE) is most associated with lymphoproliferative disorders (LPDs), particularly low-grade B-cell subtypes. The condition remains under-recognized with long diagnostic delays due to various challenges including a lack of awareness of the condition.
Case Presentation: We discuss 4 cases of C1INH-AAE associated with low-grade B-cell LPDs, including various diagnostic and management challenges. As our cases illustrate, constitutional symptoms or overt manifestations of LPD at diagnosis are often absent. Hence, a comprehensive multimodal approach to screening for an underlying B-LPD is important when a diagnosis of acquired angioedema is made. Levels of complement C4, C1q, and C1INH are useful for diagnosing C1INH-AAE and for monitoring disease activity. Changes in these parameters may also indicate relapse of the underlying hematological malignancy. Treating the underlying disorder is important as this commonly leads to clinical improvement with decreased episodes of angioedema and normalization of complement studies.
Conclusion: Awareness of C1INH-AAE can lead to an early diagnosis of hematological malignancies. The absence of constitutional symptoms emphasizes the need for a comprehensive multimodal approach to screening for LPD in C1INH-AAE. C4, C1INH level, and function are useful for monitoring disease activity.
Competing Interests: The authors have no conflicts of interest to declare.
(© 2024 The Author(s). Published by S. Karger AG, Basel.)
Databáze: MEDLINE
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