Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
Oncology and Therapy
Published in: Oncology and Therapy 1/2024

Open Access 02-02-2024 | Multiple Myeloma | Case Report

Long Term Remission of Capillary Leak Syndrome Associated with Monoclonal Gammopathy with Progression to Multiple Myeloma After Autologous Stem Cell Transplantation: a Case Report and Review of the Literature

Authors: Gerardo Hermida, Rodolfo Alvarez-Nuño, Jesús San Miguel-Izquierdo, Santiago González-Quijada, Tomás José González-López

Published in: Oncology and Therapy | Issue 1/2024

Login to get access

Abstract

Background

Clarkson’s disease is a very rare entity characterised by acute episodes of systemic oedema and severe hypotension associated with paraproteinaemia. Its classical treatment relies on methylxanthine combined with terbutaline. Although this prophylactic therapy reduces the mortality rate, relapses are frequent. Eighty percent of patients with Clarkson’s disease present with monoclonal gammopathy of unknown significance (MGUS). The risk of progression to multiple myeloma is 1% per year.

Case Description

Here, we present a 49-year-old woman who suffered multiple such episodes requiring treatment in the intensive care unit. Treatment with terbutaline and theophylline was ineffective. She was diagnosed with multiple myeloma (MM) 8 years after the first of these acute episodes. Antimyeloma treatment with bortezomib and dexamethasone was started, followed by autologous haemopoietic transplantation, with no further acute episodes since then.

Conclusion

Our case is, to our knowledge, unique because eradication of MM was followed by complete disappearance of acute episodes of capillary leakage. Our case report is also the first to support the use of bortezomib and dexamethasone in this setting. Furthermore, autologous peripheral blood progenitor cell transplantation consolidated the MM stringent complete remission achieving a very long progression-free survival (> 11 years) of both MM and Clarkson’s disease.
Literature
1.
Clarkson B, Thompson D, Horwith M, Luckey EH. Cyclical edema and shock due to increased capillary permeability. Am J Med. 1960;29:193–216.CrossRefPubMed
2.
3.
Atkinson JP, Waldmann TA, Stein SF, Gelfand JA, Macdonald WJ, Heck LW, et al. Systemic capillary leak syndrome and monoclonal IgG gammopathy; studies in a sixth patient and a review of the literature. Medicine (Baltimore). 1977;56(3):225–39.CrossRefPubMed
4.
Barnadas MA, Cisteró A, Sitjas D, Pascual E, Puig X, de Moragas JM. Systemic capillary leak syndrome. J Am Acad Dermatol. 1995;32(2 Pt 2):364–6.CrossRefPubMed
5.
Sala-Mateus C, de Febrer G, Sotorra O, Bertrán N, Castro A. Systemic capillary leak syndrome. An Med Interna. 1997;14(5):247–9.PubMed
6.
Dolberg-Stolik OC, Putterman C, Rubinow A, Rivkind AI, Sprung CL. Idiopathic capillary leak syndrome complicated by massive rhabdomyolysis. Chest. 1993;104(1):123–6.CrossRefPubMed
7.
8.
Rauzy O, Adoue D, Arlet P. Chronic systemic capillary leak syndrome not requiring treatment? Am J Med. 1998;105(4):360.PubMed
9.
Amoura Z, Papo T, Ninet J, Hatron PY, Guillaumie J, Piette AM, et al. Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment. Am J Med. 1997;103(6):514–9.CrossRefPubMed
10.
Xie Z, Chan E, Yin Y, Ghosh CC, Wisch L, Nelson C, et al. Inflammatory markers of the systemic capillary leak syndrome (Clarkson Disease). J Clin Cell Immunol. 2014;5:1000213.PubMedPubMedCentral
11.
Xie Z, Ghosh CC, Patel R, Iwaki S, Gaskins D, Nelson C, et al. Vascular endothelial hyperpermeability induces the clinical symptoms of Clarkson disease (the systemic capillary leak syndrome). Blood. 2012;119(18):4321–32.CrossRefPubMedPubMedCentral
12.
Corada M, Mariotti M, Thurston G, Smith K, Kunkel R, Brockhaus M, et al. Vascular endothelial-cadherin is an important determinant of microvascular integrity in vivo. Proc Natl Acad Sci U S A. 1999;96(17):9815–20.ADSCrossRefPubMedPubMedCentral
13.
Hiraoka E, Matsushima Y, Inomoto-Naribayashi Y, Nakata H, Nakamura A, Kawanami C, et al. Systemic capillary leak syndrome associated with multiple myeloma of IgG kappa type. Intern Med. 1995;34(12):1220–4.CrossRefPubMed
14.
Gousseff M, Arnaud L, Lambert M, Hot A, Hamidou M, Duhaut P, et al. The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Ann Intern Med. 2011;154(7):464–71.CrossRefPubMed
15.
Druey KM, Parikh SM. Idiopathic systemic capillary leak syndrome (Clarkson disease). J Allergy Clin Immunol. 2017;140(3):663–70.CrossRefPubMed
16.
de Chambrun MP, Gousseff M, Mauhin W, Lega JC, Lambert M, Rivière S, et al. Intravenous immunoglobulins improve survival in monoclonal gammopathy-associated systemic capillary-leak syndrome. Am J Med. 2017;130(10):1219.e19-e27.CrossRef
17.
Pecker MS, Hammudi M, Melchio R, Eisch AR, Verlicchi F, Druey KM. Management of acute episodes of clarkson disease (monoclonal gammopathy-associated systemic capillary leak Syndrome) with intravenous immunoglobulins. Ann Intern Med Clin Cases. 2022;1(6):e220496.CrossRef
18.
19.
Winter O, Dame C, Jundt F, Hiepe F. Pathogenic long-lived plasma cells and their survival niches in autoimmunity, malignancy, and allergy. J Immunol. 2012;189(11):5105–11.CrossRefPubMed
20.
García-Ortiz A, Rodríguez-García Y, Encinas J, Maroto-Martín E, Castellano E, Teixidó J, et al. The role of tumor microenvironment in multiple myeloma development and progression. Cancers (Basel). 2021;13(2):217.CrossRefPubMedPubMedCentral
21.
22.
Bøe OW, Sveen K, Børset M, Druey KM. Raised Serum levels of syndecan-1 (CD138), in a case of acute idiopathic systemic capillary leak syndrome (SCLS) (Clarkson’s disease). Am J Case Rep. 2018;19:176–82.CrossRefPubMedPubMedCentral
23.
Metadata
Title
Long Term Remission of Capillary Leak Syndrome Associated with Monoclonal Gammopathy with Progression to Multiple Myeloma After Autologous Stem Cell Transplantation: a Case Report and Review of the Literature
Authors
Gerardo Hermida
Rodolfo Alvarez-Nuño
Jesús San Miguel-Izquierdo
Santiago González-Quijada
Tomás José González-López
Publication date
02-02-2024
Publisher
Springer Healthcare
Published in
Oncology and Therapy / Issue 1/2024
Print ISSN: 2366-1070
Electronic ISSN: 2366-1089
DOI
https://doi.org/10.1007/s40487-024-00263-w

Other articles of this Issue 1/2024

Oncology and Therapy 1/2024 Go to the issue

Original Research

Experiences and Support Needs of Caregivers of Patients with Higher-Risk Myelodysplastic Syndrome via Online Bulletin Board in the USA, Canada and UK

Original Research

Patient Versus Physician Perspective in the Management of Chronic Myeloid Leukemia During Treatment with Tyrosine Kinase Inhibitors

Review

Intratumoral Injection of Large Surface Area Microparticle Taxanes in Carcinomas Increases Immune Effector Cell Concentrations, Checkpoint Expression, and Synergy with Checkpoint Inhibitors: A Review of Preclinical and Clinical Studies

Original Research

A Retrospective Study of Cemiplimab Effectiveness in Elderly Patients with Squamous Cell Carcinoma of the Skin: Insights from a Real-Life Scenario

Editorial

Is HER2-Low a New Clinical Entity or Merely a Biomarker for an Antibody Drug Conjugate?

Case Report

Repotrectinib’s Clinical Benefit and Its Brain Penetration in a Patient with Meningeal Carcinomatosis from G2032R-Mutated ROS-1 Positive Non-Small Cell Lung Cancer
Webinar | 19-02-2024 | 17:30 (CET)

Keynote webinar | Spotlight on antibody–drug conjugates in cancer

Watch now

Antibody–drug conjugates (ADCs) are novel agents that have shown promise across multiple tumor types. Explore the current landscape of ADCs in breast and lung cancer with our experts, and gain insights into the mechanism of action, key clinical trials data, existing challenges, and future directions.

Dr. Véronique Diéras
Prof. Fabrice Barlesi
Developed by: Springer Medicine
Image Credits