DOI: 10.12890/2026_006261
Coexisting Waldenström macroglobulinaemia and alveolar echinococcosis presenting with chylothorax that resolved on treatment: a diagnostic and therapeutic challenge
Authors
francois.perramond@outlook.com
Keywords
Waldenström macroglobulinaemia, chylothorax, alveolar echinococcosis, zanubrutinibAbstract
Introduction: Waldenström macroglobulinaemia is a lymphoplasmacytic lymphoma producing monoclonal immunoglobulin M (IgM), and both the disease and its treatment can cause immunosuppression. Alveolar echinococcosis is a parasitic infection that primarily involves the liver but may present with extrahepatic manifestations.
Case description: We report the case of a patient who presented with chylothorax and non-specific hepatic lesions and was then diagnosed with Waldenström macroglobulinaemia. In addition to this diagnosis, alveolar echinococcosis was subsequently confirmed by liver biopsy and serology.
Discussion: Diagnostic interpretation was challenging, as these features may occur in both conditions. A multidisciplinary approach was needed to establish the treatment plan due to overlapping symptomatology and the risk of treatment-related immunosuppression.
Conclusion: This case highlights the importance of considering a broad differential diagnosis for a chylothorax and hepatic lesions in an oncological context and illustrates the therapeutic challenges of managing an indolent haematologic malignancy in the context of a chronic parasitic infection.
References
- Gertz MA. Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management. Am J Hematol 2023;98:348–358. doi:10.1002/ajh.26796
- Ollagnon M, Bresson-Hadni S, Spahr L, Rubbia-Brandt L, Toso C, Chappuis F. Alveolar echinococcosis in the canton of Geneva between 2010 and 2021: a descriptive analysis. Swiss Med Wkly 2025;155:3863. doi:10.57187/s.3863
- Bhatnagar M, Fisher A, Ramsaroop S, Carter A, Pippard B. Chylothorax: pathophysiology, diagnosis, and management – a comprehensive review. J Thorac Dis 2024;16:1645–1661. doi:10.21037/jtd-23-1636
- Baudinov I, Kadyrova A, Ibraimov K, Prabha KC, Bebezov B, Aitbaev S, et al. Diagnostic imaging modalities in the study of hepatic alveolar echinococcosis: a review of literature. Cardiovasc Dis 2025;9: doi: 10.24969/hvt.2025.558
- Pfister M, Gottstein B, Kretschmer R, Cerny T, Cerny A. Elevated carbohydrate antigen 19-9 (CA 19-9) in patients with Echinococcus infection. Clin Chem Lab Med 2001;39:527–530. doi:10.1515/CCLM.2001.088
- Kumar SK, Callander NS, Adekola K, Anderson LD Jr, Baljevic M, Baz R, et al. Waldenström macroglobulinemia/lymphoplasmacytic lymphoma, version 2.2024, NCCN Clinical Practice Guidelines in Oncology. J Natl Compr Canc Netw 2024;22:e240001. doi:10.6004/jnccn.2024.0001
- Kaiser LM, Hunter ZR, Treon SP, Buske C. CXCR4 in Waldenström’s macroglobulinemia: chances and challenges. Leukemia 2021;35:333–345. doi:10.1038/s41375-020-01102-3
- Castillo JJ, Yang K, Liu R, Wang Y, Cohen A, Zimmerman TM, et al. Efficacy and safety of zanubrutinib versus rituximab-based chemoimmunotherapy in Waldenström macroglobulinemia (WM): Matching-adjusted indirect comparisons: hematologic malignancies – lymphoma and chronic lymphocytic leukemia. J Clin Oncol 2021;39:7559. doi: 10.1200/JCO.2021.39.15_suppl.7559
- Chauchet A, Grenouillet F, Knapp J, Richou C, Delabrousse E, Dentan C, et al. FrancEchino Network. Increased incidence and characteristics of alveolar echinococcosis in patients with immunosuppression-associated conditions. Clin Infect Dis 2014;59:1095–1104. doi:10.1093/cid/ciu520