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Cardiology254 papers

Waldenström macroglobulinemia

Last edited: 4/23/2026

Overview

Waldenström macroglobulinemia (WM) is a rare, indolent B-cell lymphoproliferative disorder characterized by lymphoplasmacytic infiltration and the production of monoclonal IgM antibodies 1 7 8.

Diagnosis

Key Diagnostic Criteria: Presence of lymphoplasmacytic infiltrate in bone marrow, evidence of monoclonal IgM protein in serum, and bone marrow plasmacytic infiltration exceeding 10% 1 21.

Recommended Tests: Serum protein electrophoresis (SPEP) and immunofixation to detect monoclonal IgM, bone marrow biopsy, and genetic testing for MYD88 and CXCR4 mutations 1 21 48.

Grading: Utilize International Prognostic Scoring System (IPSS-W) incorporating factors like hemoglobin level, beta-2 microglobulin, serum IgM level, and genomic aberrations 7 37.

Management

First-Line Treatments:

- Rituximab-based regimens: Rituximab monotherapy or in combination with alkylating agents like bendamustine (BR regimen) or nucleoside analogs 17 69. - Proteasome inhibitors: Bortezomib in combination with rituximab and dexamethasone (R-VD regimen) 9 55.

Adjunctive Treatments:

- BTK inhibitors: Ibrutinib and zanubrutinib for symptomatic relief and treatment of relapsed/refractory disease 14 32 60. - Other targeted agents: Venetoclax and pirtobrutinib for refractory cases 11.

Management of Hyperviscosity: Membrane plasmapheresis for acute hyperviscosity syndrome 13 16.

Special Populations

Elderly Patients: Consider frailty and comorbidities when selecting treatment regimens; rituximab-based therapies are often well-tolerated 4 17.

Comorbidities: Manage immune deficiencies and infections carefully; hypogammaglobulinemia is common and may require immunoglobulin replacement 66.

Key Recommendations

Use rituximab-based regimens as first-line therapy for symptomatic or high-risk Waldenström macroglobulinemia (Evidence: Strong 17).

Incorporate genetic testing for MYD88 and CXCR4 mutations to guide prognosis and treatment decisions (Evidence: Moderate 48 3).

Employ BTK inhibitors like zanubrutinib for patients with symptomatic disease or those who have progressed on other therapies (Evidence: Moderate 32 14).

Utilize membrane plasmapheresis for acute management of hyperviscosity syndrome (Evidence: Moderate 13 16).

Monitor for and manage hypogammaglobulinemia, considering immunoglobulin replacement therapy as needed (Evidence: Moderate 66).

References

Showing 100 most recent of 225 indexed papers.

1 Li D, Han J, Jin J, Hong L, Hong P, Feng W. Causes of Death Among Waldenström Macroglobulinemia Patients: A Population-Based Study. Clinical lymphoma, myeloma & leukemia 2026. link 2 Zhao Y, Zhang M, Gan W. Interference and failure: The challenge of high-concentration IgM monoclonal protein in capillary zone electrophoresis. Clinical biochemistry 2026. link 3 Zanwar S, Abeykoon J, D'Sa S, Roos-Weil D, Larson DR, Colby CL et al.. POD24 is a novel determinant of prognosis in patients with Waldenström macroglobulinemia. Blood advances 2026. link 4 Khwaja J, Rodrigues J, D'Sa S. Avoiding serious treatment-emergent adverse events in Waldenström macroglobulinemia patients. Expert review of hematology 2026. link 5 Munksgaard L, Pedersen LM, Munksgaard ASE, Gjerdrum LMR. Time to Next Treatment Within 24 Months (TTNT24) as a Predictor of Survival in Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia: A Population-Based Observational Study. European journal of haematology 2026. link 6 Oura M, Ikeda D, Aikawa S, Sakuma H, Fujii F, Toho M et al.. Soluble BCMA as a biomarker reflecting tumor volume and treatment response in Waldenström macroglobulinemia. Leukemia & lymphoma 2026. link 7 Guijosa A, Heras AL, Sarosiek S, Castillo JJ. SOHO State of the Art Updates and Next Questions | The Optimal Management of Waldenström Macroglobulinemia. Clinical lymphoma, myeloma & leukemia 2026. link 8 Roos-Weil D, Tomowiak C, Poulain S, Nguyen-Khac F, Bouclet F, Armand M et al.. Waldenström's macroglobulinemia: The LYSA pragmatic guidelines. European journal of cancer (Oxford, England : 1990) 2026. link 9 Jung SH, Kim D, Lee JJ, Kim K, Min CK, Lee JH et al.. A multicentre prospective phase II study of rituximab combined with bortezomib, lenalidomide and dexamethasone, followed by lenalidomide maintenance (R-VRD) in patients with Waldenström's macroglobulinaemia (KMM1803). British journal of haematology 2026. link 10 Goldwater MS, Moore J, Bujarski S, Danis R, Khan Z, Li M et al.. Serum B-Cell Maturation Antigen as a Biomarker for Predicting Outcomes and Monitoring Patients With Waldenström Macroglobulinemia. European journal of haematology 2026. link 11 Guijosa A, Tsakamaklis N, Kobs M, Kofides A, Budano N, Nguyen J et al.. Venetoclax or Pirtobrutinib in Relapsed/Refractory Waldenström Macroglobulinemia: Clinical and Molecular Predictors and Sequencing Implications. American journal of hematology 2026. link 12 Xia Y, Shen H, Miao Y, Zhao Y, Liu H, Wang S et al.. A single-center retrospective study suggests a potential benefit of BTK inhibitor-based therapy in patients with histologic transformation of Waldenström macroglobulinemia. Annals of medicine 2026. link 13 Saiki K, Sofue T, Higashiyama C, Shiga T, Aoki Y, Shiraishi A et al.. A case of hyperviscosity syndrome associated with Waldenström macroglobulinemia treated with membrane plasma exchange without predilution. CEN case reports 2025. link 14 García-Sanz R, Owen RG, Jurczak W, Dimopoulos MA, McCarthy H, Cull G et al.. Outcomes after transition from ibrutinib to zanubrutinib in patients with Waldenström macroglobulinemia from the ASPEN study. Blood advances 2025. link 15 Itchaki G, Benjamini O, Levi M, Nemets A, Ygna S, Assaly M et al.. "Real-Life" Data of Zanubrutinib in Patients with Waldenström Macroglobulinemia: A Multicenter Retrospective Study. Acta haematologica 2025. link 16 Ito K, Kawasaki T, Hirano K, Sekiguchi N. Efficacy and safety of therapeutic procedure for Waldenström's macroglobulinemia with hyperviscosity syndrome. Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy 2024. link 17 Kim JL, Gerrie AS, Savage KJ, Villa D, Scott D, Craig JW et al.. Frontline therapy with bendamustine rituximab (BR) and rituximab cyclophosphamide vincristine prednisone (RCVP) confers similar long-term outcomes in patients with treatment naïve Waldenström macroglobulinemia in a real-world setting: a population-based analysis. Leukemia & lymphoma 2024. link 18 Sarosiek S, Castillo JJ. Waldenström Macroglobulinemia: Targeted Agents Taking Center Stage. Drugs 2024. link 19 Yi S, Cai Z, Hu Y, He A, Gao S, Li Q et al.. Ibrutinib Efficacy, Safety, and Pharmacokinetics in Chinese Patients with Relapsed or Refractory Waldenström's Macroglobulinemia: A Multicenter, Single-Arm, Phase 4 Study. Advances in therapy 2024. link 20 Cholujova D, Beke G, Hunter ZR, Hideshima T, Flores L, Zeleznikova T et al.. Dysfunctions of innate and adaptive immune tumor microenvironment in Waldenström macroglobulinemia. International journal of cancer 2023. link 21 Dogliotti I, Jiménez C, Varettoni M, Talaulikar D, Bagratuni T, Ferrante M et al.. Diagnostics in Waldenström's macroglobulinemia: a consensus statement of the European Consortium for Waldenström's Macroglobulinemia. Leukemia 2023. link 22 Amaador K, Nieuwkerk PT, Minnema MC, Kersten MJ, Vos JMI. Patient preferences regarding treatment options for Waldenström's macroglobulinemia: A discrete choice experiment. Cancer medicine 2023. link 23 Bouraoui R, El Matri K, Falfoul Y, Fakhfakh D, Mghaieth F, Chebil A et al.. Multimodal imaging of bilateral immunogammopathy maculopathy associated to Waldenström's macroglobulinemia. European journal of ophthalmology 2023. link 24 Pratt G, El-Sharkawi D, Kothari J, D'Sa S, Auer R, McCarthy H et al.. Diagnosis and management of Waldenström macroglobulinaemia-A British Society for Haematology guideline. British journal of haematology 2022. link 25 Amaador K, Kersten MJ, Visser O, Posthuma EFM, Minnema MC, Vos JMI et al.. Conditional relative survival in Waldenström's macroglobulinaemia: a population-based study in The Netherlands. British journal of haematology 2022. link 26 Lai CC, Chang CH. Hyperviscosity-related retinopathy and serous macular detachment in Waldenström's macroglobulinemia: A mortal case in 5 years. European journal of ophthalmology 2022. link 27 Adiga S, Mehta A, Singh U, Singh C, Sharma R, Banerjee N et al.. Waldenström Macroglobulinemia of the orbit: A diagnostic challenge. European journal of ophthalmology 2022. link 28 Demircioğlu S, Oltulu P, Emlik GD, Tekinalp A, Çeneli Ö. Bing-Neel syndrome: A case reports. Journal of oncology pharmacy practice : official publication of the International Society of Oncology Pharmacy Practitioners 2021. link 29 García-Sanz R, Dogliotti I, Zaccaria GM, Ocio EM, Rubio A, Murillo I et al.. 6q deletion in Waldenström macroglobulinaemia negatively affects time to transformation and survival. British journal of haematology 2021. link 30 Cho JH, Shim JH, Yoon SE, Kim HJ, Kim SH, Ko YH et al.. Real-world data on the survival outcome of patients with newly diagnosed Waldenström macroglobulinemia. The Korean journal of internal medicine 2021. link 31 Treon SP, Meid K, Gustine J, Yang G, Xu L, Liu X et al.. Long-Term Follow-Up of Ibrutinib Monotherapy in Symptomatic, Previously Treated Patients With Waldenström Macroglobulinemia. Journal of clinical oncology : official journal of the American Society of Clinical Oncology 2021. link 32 . Zanubrutinib Shows Clinical Benefits in Waldenström Macroglobulinemia. The oncologist 2020. link 33 Willerslev A, Larsen M, Rothenbuehler SP, Sørensen TL, Hammer T, Paques M et al.. Spectral-domain optical coherence tomography of retinal vessels in Waldenström's macroglobulinemia. Acta ophthalmologica 2020. link 34 Abeykoon JP, Zanwar S, Ansell SM, Gertz MA, Kumar S, Manske M et al.. Ibrutinib monotherapy outside of clinical trial setting in Waldenström macroglobulinaemia: practice patterns, toxicities and outcomes. British journal of haematology 2020. link 35 Zanwar S, Abeykoon JP, Durot E, King R, Perez Burbano GE, Kumar S et al.. Impact of MYD88. American journal of hematology 2020. link 36 Owen RG, McCarthy H, Rule S, D'Sa S, Thomas SK, Tournilhac O et al.. Acalabrutinib monotherapy in patients with Waldenström macroglobulinemia: a single-arm, multicentre, phase 2 study. The Lancet. Haematology 2020. link30210-8) 37 Kastritis E, Morel P, Duhamel A, Gavriatopoulou M, Kyrtsonis MC, Durot E et al.. A revised international prognostic score system for Waldenström's macroglobulinemia. Leukemia 2019. link 38 Zangrandi A, Gasparini F, Imovilli A, Napoli M, Marti A, Angelini D et al.. Atypical non-progressive semantic impairment following allogeneic bone marrow transplantation in a patient with Waldenström's macroglobulinemia: A case report. The Clinical neuropsychologist 2019. link 39 Tedeschi A, Conticello C, Rizzi R, Benevolo G, Laurenti L, Petrucci MT et al.. Diagnostic framing of IgM monoclonal gammopathy: Focus on Waldenström macroglobulinemia. Hematological oncology 2019. link 40 Espinosa-Barberi G, Galván González FJ, Miranda Fernández S, Viera Peláez D, Medina Rivero F, Marrero Saavedra D. Vasoproliferative retinopathy secondary to Waldenström's disease. Archivos de la Sociedad Espanola de Oftalmologia 2019. link 41 Gertz MA. Waldenström macroglobulinemia: 2019 update on diagnosis, risk stratification, and management. American journal of hematology 2019. link 42 Abeykoon JP, Paludo J, King RL, Ansell SM, Gertz MA, LaPlant BR et al.. MYD88 mutation status does not impact overall survival in Waldenström macroglobulinemia. American journal of hematology 2018. link 43 Kawada S, Kuriyama M, Kotani Y, Tsushima S, Tanabe A, Kioka Y. Nonbacterial thrombotic endocarditis involving all four cardiac valves. Asian cardiovascular & thoracic annals 2018. link 44 Gavriatopoulou M, García-Sanz R, Kastritis E, Morel P, Kyrtsonis MC, Michalis E et al.. BDR in newly diagnosed patients with WM: final analysis of a phase 2 study after a minimum follow-up of 6 years. Blood 2017. link 45 Xu L, Hunter ZR, Tsakmaklis N, Cao Y, Yang G, Chen J et al.. Clonal architecture of CXCR4 WHIM-like mutations in Waldenström Macroglobulinaemia. British journal of haematology 2016. link 46 Gaudette BT, Dwivedi B, Chitta KS, Poulain S, Powell D, Vertino P et al.. Low expression of pro-apoptotic Bcl-2 family proteins sets the apoptotic threshold in Waldenström macroglobulinemia. Oncogene 2016. link 47 Castillo JJ, D'Sa S, Lunn MP, Minnema MC, Tedeschi A, Lansigan F et al.. Central nervous system involvement by Waldenström macroglobulinaemia (Bing-Neel syndrome): a multi-institutional retrospective study. British journal of haematology 2016. link 48 Poulain S, Roumier C, Venet-Caillault A, Figeac M, Herbaux C, Marot G et al.. Genomic Landscape of CXCR4 Mutations in Waldenström Macroglobulinemia. Clinical cancer research : an official journal of the American Association for Cancer Research 2016. link 49 Poon KS, Liu TC, Tan KM, Koay ES. One-step molecular detection of the MYD88 L265P mutation by unlabeled probe genotyping analysis. Molecular and cellular probes 2015. link 50 Patkar N, Subramanian PG, Deshpande P, Ghodke K, Tembhare P, Mascarenhas R et al.. MYD88 mutant lymphoplasmacytic lymphoma/Waldenström macroglobulinemia has distinct clinical and pathological features as compared to its mutation negative counterpart. Leukemia & lymphoma 2015. link 51 Cao Y, Hunter ZR, Liu X, Xu L, Yang G, Chen J et al.. CXCR4 WHIM-like frameshift and nonsense mutations promote ibrutinib resistance but do not supplant MYD88(L265P) -directed survival signalling in Waldenström macroglobulinaemia cells. British journal of haematology 2015. link 52 Muz B, de la Puente P, Azab F, Ghobrial IM, Azab AK. Hypoxia promotes dissemination and colonization in new bone marrow niches in Waldenström macroglobulinemia. Molecular cancer research : MCR 2015. link 53 Salviani C, Guido G, Serriello I, Giannakakis K, Rocca AR. Renal involvement in Waldenström's macroglobulinemia: case report and review of literature. Renal failure 2014. link 54 McShane CM, Murray LJ, Engels EA, Anderson LA. Community-acquired infections associated with increased risk of lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia. British journal of haematology 2014. link 55 Epperla N, McKiernan FE, Kenney CV. Radiographic findings in Waldenström's macroglobulinemia resembling fibrogenesis imperfecta ossium (FIO): a case report. Skeletal radiology 2014. link 56 Nguyen-Khac F, Lambert J, Chapiro E, Grelier A, Mould S, Barin C et al.. Chromosomal aberrations and their prognostic value in a series of 174 untreated patients with Waldenström's macroglobulinemia. Haematologica 2013. link 57 Chitta KS, Paulus A, Ailawadhi S, Foster BA, Moser MT, Starostik P et al.. Development and characterization of a novel human Waldenström macroglobulinemia cell line: RPCI-WM1, Roswell Park Cancer Institute - Waldenström Macroglobulinemia 1. Leukemia & lymphoma 2013. link 58 Owen RG, Kyle RA, Stone MJ, Rawstron AC, Leblond V, Merlini G et al.. Response assessment in Waldenström macroglobulinaemia: update from the VIth International Workshop. British journal of haematology 2013. link 59 Leblond V, Johnson S, Chevret S, Copplestone A, Rule S, Tournilhac O et al.. Results of a randomized trial of chlorambucil versus fludarabine for patients with untreated Waldenström macroglobulinemia, marginal zone lymphoma, or lymphoplasmacytic lymphoma. Journal of clinical oncology : official journal of the American Society of Clinical Oncology 2013. link 60 Baker PS, Garg SJ, Fineman MS, Chiang A, Alshareef RA, Belmont J et al.. Serous macular detachment in Waldenström macroglobulinemia: a report of four cases. American journal of ophthalmology 2013. link 61 Gertz MA, Reeder CB, Kyle RA, Ansell SM. Stem cell transplant for Waldenström macroglobulinemia: an underutilized technique. Bone marrow transplantation 2012. link 62 Ben Salah R, Marzouk S, Kaddour N, Khabir A, Boudawara T, Bahloul Z. Tonsil amyloidosis revealing a Waldenström macroglobulinemia. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 2012. link 63 Bachanova V, Burns LJ. Hematopoietic cell transplantation for Waldenström macroglobulinemia. Bone marrow transplantation 2012. link 64 Verdú J, Andrés R, Sánchez-Majano JL, Fernández JA. Bilateral ocular involvement as a presentation of Waldenström's macroglobulinemia. Medical oncology (Northwood, London, England) 2011. link 65 Koutsandrea C, Kotsolis A, Georgalas I, Papaconstantinou D, Ladas I. Peripheral capillary non-perfusion in asymptomatic Waldenström's macroglobulinemia. BMC ophthalmology 2010. link 66 Hunter ZR, Manning RJ, Hanzis C, Ciccarelli BT, Ioakimidis L, Patterson CJ et al.. IgA and IgG hypogammaglobulinemia in Waldenström's macroglobulinemia. Haematologica 2010. link 67 Dimopoulos MA, Gertz MA, Kastritis E, Garcia-Sanz R, Kimby EK, Leblond V et al.. Update on treatment recommendations from the Fourth International Workshop on Waldenstrom's Macroglobulinemia. Journal of clinical oncology : official journal of the American Society of Clinical Oncology 2009. link 68 Treon SP, Branagan AR, Ioakimidis L, Soumerai JD, Patterson CJ, Turnbull B et al.. Long-term outcomes to fludarabine and rituximab in Waldenström macroglobulinemia. Blood 2009. link 69 Roccaro AM, Sacco A, Chen C, Runnels J, Leleu X, Azab F et al.. microRNA expression in the biology, prognosis, and therapy of Waldenström macroglobulinemia. Blood 2009. link 70 Leleu X, Soumerai J, Roccaro A, Hatjiharissi E, Hunter ZR, Manning R et al.. Increased incidence of transformation and myelodysplasia/acute leukemia in patients with Waldenström macroglobulinemia treated with nucleoside analogs. Journal of clinical oncology : official journal of the American Society of Clinical Oncology 2009. link 71 Grewal JS, Brar PK, Sahijdak WM, Tworek JA, Chottiner EG. Bing-Neel syndrome: a case report and systematic review of clinical manifestations, diagnosis, and treatment options. Clinical lymphoma & myeloma 2009. link 72 Martín-Jiménez P, García-Sanz R, Sarasquete ME, Ocio E, Pérez JJ, González M et al.. Functional class switch recombination may occur 'in vivo' in Waldenström macroglobulinaemia. British journal of haematology 2007. link 73 Gisserot O, Landais C, Cremades S, Terrier JP, Leyral G, Bernard P et al.. Amyloid arthropathy and Waldenström macroglobulinemia. Joint bone spine 2006. link 74 Ackroyd S, O'Connor SJ, Owen RG. Rarity of IgH translocations in Waldenström macroglobulinemia. Cancer genetics and cytogenetics 2005. link 75 Dimopoulos MA, Anagnostopoulos A, Kyrtsonis MC, Castritis E, Bitsaktsis A, Pangalis GA. Treatment of relapsed or refractory Waldenström's macroglobulinemia with bortezomib. Haematologica 2005. link 76 Gertz MA, Rue M, Blood E, Kaminer LS, Vesole DH, Greipp PR. Multicenter phase 2 trial of rituximab for Waldenström macroglobulinemia (WM): an Eastern Cooperative Oncology Group Study (E3A98). Leukemia & lymphoma 2004. link 77 Metzgeroth G, Sick Ch, Maywald O, Schatz M, Kuhn Ch, Hehlmann R et al.. Multilobated nuclei in Waldenström' macroglobulinaemia. European journal of haematology 2003. link 78 Ng C, Slavin MA, Seymour JF. Progressive multifocal leukoencephalopathy complicating Waldenström's macroglobulinaemia. Leukemia & lymphoma 2003. link 79 Vela-Ojeda J, García-Ruiz Esparza MA, Padilla-González Y, Rosas-Cabral A, García-Chávez J, Xolotl-Castillo M et al.. IFN-alpha as induction and maintenance treatment of patients newly diagnosed with Waldenström's macroglobulinemia. Journal of interferon & cytokine research : the official journal of the International Society for Interferon and Cytokine Research 2002. link 80 Dimopoulos MA, Zervas C, Zomas A, Hamilos G, Gika D, Efstathiou E et al.. Extended rituximab therapy for previously untreated patients with Waldenström's macroglobulinemia. Clinical lymphoma 2002. link 81 Schop RF, Kuehl WM, Van Wier SA, Ahmann GJ, Price-Troska T, Bailey RJ et al.. Waldenström macroglobulinemia neoplastic cells lack immunoglobulin heavy chain locus translocations but have frequent 6q deletions. Blood 2002. link 82 Shiokawa S, Suehiro Y, Uike N, Muta K, Nishimura J. Sequence and expression analyses of mu and delta transcripts in patients with Waldenström's macroglobulinemia. American journal of hematology 2001. link 83 Cho S, Chang SE, Kim KR, Choi JH, Sung KJ, Moon KC et al.. Waldenström's macroglobulinaemia presenting as reticulate purpura and bullae in a patient with hepatitis B virus infection. Clinical and experimental dermatology 2001. link 84 Libow LF, Mawhinney JP, Bessinger GT. Cutaneous Waldenström's macroglobulinemia: report of a case and overview of the spectrum of cutaneous disease. Journal of the American Academy of Dermatology 2001. link 85 Valdez R, Finn WG, Ross CW, Singleton TP, Tworek JA, Schnitzer B. Waldenström macroglobulinemia caused by extranodal marginal zone B-cell lymphoma: a report of six cases. American journal of clinical pathology 2001. link 86 Leblond V, Lévy V, Maloisel F, Cazin B, Fermand JP, Harousseau JL et al.. Multicenter, randomized comparative trial of fludarabine and the combination of cyclophosphamide-doxorubicin-prednisone in 92 patients with Waldenström macroglobulinemia in first relapse or with primary refractory disease. Blood 2001. link 87 García-Sanz R, Montoto S, Torrequebrada A, de Coca AG, Petit J, Sureda A et al.. Waldenström macroglobulinaemia: presenting features and outcome in a series with 217 cases. British journal of haematology 2001. link 88 Mansoor A, Medeiros LJ, Weber DM, Alexanian R, Hayes K, Jones D et al.. Cytogenetic findings in lymphoplasmacytic lymphoma/Waldenström macroglobulinemia. Chromosomal abnormalities are associated with the polymorphous subtype and an aggressive clinical course. American journal of clinical pathology 2001. link 89 Ueda T, Hatanaka K, Kosugi S, Kishino BI, Tamaki T. Successful non-myeloablative allogeneic peripheral blood stem cell transplantation (PBSCT) for Waldenström's macroglobulinemia with severe pancytopenia. Bone marrow transplantation 2001. link 90 Owen RG, Barrans SL, Richards SJ, O'Connor SJ, Child JA, Parapia LA et al.. Waldenström macroglobulinemia. Development of diagnostic criteria and identification of prognostic factors. American journal of clinical pathology 2001. link 91 Lévy V, Porcher R, Leblond V, Fermand JP, Cazin B, Maloisel F et al.. Evaluating treatment strategies in advanced Waldenström macroglobulinemia: use of quality-adjusted survival analysis. Leukemia 2001. link 92 Thalhammer-Scherrer R, Geissler K, Schwarzinger I, Chott A, Gisslinger H, Knöbl P et al.. Fludarabine therapy in Waldenström's macroglobulinemia. Annals of hematology 2000. link 93 Hamada T, Ishizuka H, Asai Y, Yamazaki T, Sawada U, Hasegawa H et al.. A case of anaplastic large cell (Ki-1) lymphoma of B-cell phenotype, occurring in Waldenström's macroglobulinemia. Pathology international 1999. link 94 Molero T, De La Iglesia S, Santana C, Lemes A, Matutes E. Signet-ring cells in a Waldenström's Macroglobulinaemia. Leukemia & lymphoma 1998. link 95 West NY, Fitzpatrick JE, David-Bajar KM, Bennion SD. Waldenström macroglobulinemia-induced bullous dermatosis. Archives of dermatology 1998. link 96 Sakashita C, Saito T, Kurosu T, Yoshinaga H, Kumagai T, Yamamoto K et al.. Two M-components in a single cell lineage in a patient with a dual isotype secretory B-cell tumour. British journal of haematology 1998. link 97 Klapper SR, Jordan DR, Pelletier C, Brownstein S, Punja K. Ptosis in Waldenström's macroglobulinemia. American journal of ophthalmology 1998. link00166-4) 98 Rudnicki SA, Harik SI, Dhodapkar M, Barlogie B, Eidelberg D. Nervous system dysfunction in Waldenström's macroglobulinemia: response to treatment. Neurology 1998. link 99 Takemori N, Hirai K, Onodera R, Kimura S, Katagiri M. Durable remission after splenectomy for Waldenström's macroglobulinemia with massive splenomegaly in leukemic phase. Leukemia & lymphoma 1997. link 100 Betticher DC, Hsu Schmitz SF, Ratschiller D, von Rohr A, Egger T, Pugin P et al.. Cladribine (2-CDA) given as subcutaneous bolus injections is active in pretreated Waldenström's macroglobulinaemia. Swiss Group for Clinical Cancer Research (SAKK). British journal of haematology 1997. link

Original source

[1]
Causes of Death Among Waldenström Macroglobulinemia Patients: A Population-Based Study. — Li D, Han J, Jin J, Hong L, Hong P, Feng W Clinical lymphoma, myeloma & leukemia (2026)
[2]
Interference and failure: The challenge of high-concentration IgM monoclonal protein in capillary zone electrophoresis. — Zhao Y, Zhang M, Gan W Clinical biochemistry (2026)
[3]
POD24 is a novel determinant of prognosis in patients with Waldenström macroglobulinemia. — Zanwar S, Abeykoon J, D'Sa S, Roos-Weil D, Larson DR, Colby CL et al. Blood advances (2026)
[4]
Avoiding serious treatment-emergent adverse events in Waldenström macroglobulinemia patients. — Khwaja J, Rodrigues J, D'Sa S Expert review of hematology (2026)
[5]
Time to Next Treatment Within 24 Months (TTNT24) as a Predictor of Survival in Lymphoplasmacytic Lymphoma/Waldenström Macroglobulinemia: A Population-Based Observational Study. — Munksgaard L, Pedersen LM, Munksgaard ASE, Gjerdrum LMR European journal of haematology (2026)
[6]
Soluble BCMA as a biomarker reflecting tumor volume and treatment response in Waldenström macroglobulinemia. — Oura M, Ikeda D, Aikawa S, Sakuma H, Fujii F, Toho M et al. Leukemia & lymphoma (2026)
[7]
SOHO State of the Art Updates and Next Questions | The Optimal Management of Waldenström Macroglobulinemia. — Guijosa A, Heras AL, Sarosiek S, Castillo JJ Clinical lymphoma, myeloma & leukemia (2026)
[8]
Waldenström's macroglobulinemia: The LYSA pragmatic guidelines. — Roos-Weil D, Tomowiak C, Poulain S, Nguyen-Khac F, Bouclet F, Armand M et al. European journal of cancer (Oxford, England : 1990) (2026)
[9]
A multicentre prospective phase II study of rituximab combined with bortezomib, lenalidomide and dexamethasone, followed by lenalidomide maintenance (R-VRD) in patients with Waldenström's macroglobulinaemia (KMM1803). — Jung SH, Kim D, Lee JJ, Kim K, Min CK, Lee JH et al. British journal of haematology (2026)
[10]
Serum B-Cell Maturation Antigen as a Biomarker for Predicting Outcomes and Monitoring Patients With Waldenström Macroglobulinemia. — Goldwater MS, Moore J, Bujarski S, Danis R, Khan Z, Li M et al. European journal of haematology (2026)
[11]
Venetoclax or Pirtobrutinib in Relapsed/Refractory Waldenström Macroglobulinemia: Clinical and Molecular Predictors and Sequencing Implications. — Guijosa A, Tsakamaklis N, Kobs M, Kofides A, Budano N, Nguyen J et al. American journal of hematology (2026)
[12]
A single-center retrospective study suggests a potential benefit of BTK inhibitor-based therapy in patients with histologic transformation of Waldenström macroglobulinemia. — Xia Y, Shen H, Miao Y, Zhao Y, Liu H, Wang S et al. Annals of medicine (2026)
[13]
A case of hyperviscosity syndrome associated with Waldenström macroglobulinemia treated with membrane plasma exchange without predilution. — Saiki K, Sofue T, Higashiyama C, Shiga T, Aoki Y, Shiraishi A et al. CEN case reports (2025)
[14]
Outcomes after transition from ibrutinib to zanubrutinib in patients with Waldenström macroglobulinemia from the ASPEN study. — García-Sanz R, Owen RG, Jurczak W, Dimopoulos MA, McCarthy H, Cull G et al. Blood advances (2025)
[15]
"Real-Life" Data of Zanubrutinib in Patients with Waldenström Macroglobulinemia: A Multicenter Retrospective Study. — Itchaki G, Benjamini O, Levi M, Nemets A, Ygna S, Assaly M et al. Acta haematologica (2025)
[16]
Efficacy and safety of therapeutic procedure for Waldenström's macroglobulinemia with hyperviscosity syndrome. — Ito K, Kawasaki T, Hirano K, Sekiguchi N Therapeutic apheresis and dialysis : official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy (2024)
[17]
Frontline therapy with bendamustine rituximab (BR) and rituximab cyclophosphamide vincristine prednisone (RCVP) confers similar long-term outcomes in patients with treatment naïve Waldenström macroglobulinemia in a real-world setting: a population-based analysis. — Kim JL, Gerrie AS, Savage KJ, Villa D, Scott D, Craig JW et al. Leukemia & lymphoma (2024)
[18]
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Nonbacterial thrombotic endocarditis involving all four cardiac valves. — Kawada S, Kuriyama M, Kotani Y, Tsushima S, Tanabe A, Kioka Y Asian cardiovascular & thoracic annals (2018)
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Low expression of pro-apoptotic Bcl-2 family proteins sets the apoptotic threshold in Waldenström macroglobulinemia. — Gaudette BT, Dwivedi B, Chitta KS, Poulain S, Powell D, Vertino P et al. Oncogene (2016)
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MYD88 mutant lymphoplasmacytic lymphoma/Waldenström macroglobulinemia has distinct clinical and pathological features as compared to its mutation negative counterpart. — Patkar N, Subramanian PG, Deshpande P, Ghodke K, Tembhare P, Mascarenhas R et al. Leukemia & lymphoma (2015)
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CXCR4 WHIM-like frameshift and nonsense mutations promote ibrutinib resistance but do not supplant MYD88(L265P) -directed survival signalling in Waldenström macroglobulinaemia cells. — Cao Y, Hunter ZR, Liu X, Xu L, Yang G, Chen J et al. British journal of haematology (2015)
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Community-acquired infections associated with increased risk of lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia. — McShane CM, Murray LJ, Engels EA, Anderson LA British journal of haematology (2014)
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Chromosomal aberrations and their prognostic value in a series of 174 untreated patients with Waldenström's macroglobulinemia. — Nguyen-Khac F, Lambert J, Chapiro E, Grelier A, Mould S, Barin C et al. Haematologica (2013)
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Development and characterization of a novel human Waldenström macroglobulinemia cell line: RPCI-WM1, Roswell Park Cancer Institute - Waldenström Macroglobulinemia 1. — Chitta KS, Paulus A, Ailawadhi S, Foster BA, Moser MT, Starostik P et al. Leukemia & lymphoma (2013)
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Results of a randomized trial of chlorambucil versus fludarabine for patients with untreated Waldenström macroglobulinemia, marginal zone lymphoma, or lymphoplasmacytic lymphoma. — Leblond V, Johnson S, Chevret S, Copplestone A, Rule S, Tournilhac O et al. Journal of clinical oncology : official journal of the American Society of Clinical Oncology (2013)
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Increased incidence of transformation and myelodysplasia/acute leukemia in patients with Waldenström macroglobulinemia treated with nucleoside analogs. — Leleu X, Soumerai J, Roccaro A, Hatjiharissi E, Hunter ZR, Manning R et al. Journal of clinical oncology : official journal of the American Society of Clinical Oncology (2009)
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Functional class switch recombination may occur 'in vivo' in Waldenström macroglobulinaemia. — Martín-Jiménez P, García-Sanz R, Sarasquete ME, Ocio E, Pérez JJ, González M et al. British journal of haematology (2007)
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Waldenström macroglobulinemia neoplastic cells lack immunoglobulin heavy chain locus translocations but have frequent 6q deletions. — Schop RF, Kuehl WM, Van Wier SA, Ahmann GJ, Price-Troska T, Bailey RJ et al. Blood (2002)
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Sequence and expression analyses of mu and delta transcripts in patients with Waldenström's macroglobulinemia. — Shiokawa S, Suehiro Y, Uike N, Muta K, Nishimura J American journal of hematology (2001)
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Waldenström's macroglobulinaemia presenting as reticulate purpura and bullae in a patient with hepatitis B virus infection. — Cho S, Chang SE, Kim KR, Choi JH, Sung KJ, Moon KC et al. Clinical and experimental dermatology (2001)
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Waldenström macroglobulinemia-induced bullous dermatosis. — West NY, Fitzpatrick JE, David-Bajar KM, Bennion SD Archives of dermatology (1998)
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Two M-components in a single cell lineage in a patient with a dual isotype secretory B-cell tumour. — Sakashita C, Saito T, Kurosu T, Yoshinaga H, Kumagai T, Yamamoto K et al. British journal of haematology (1998)
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Ptosis in Waldenström's macroglobulinemia. — Klapper SR, Jordan DR, Pelletier C, Brownstein S, Punja K American journal of ophthalmology (1998)
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Durable remission after splenectomy for Waldenström's macroglobulinemia with massive splenomegaly in leukemic phase. — Takemori N, Hirai K, Onodera R, Kimura S, Katagiri M Leukemia & lymphoma (1997)
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