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This case series presents three patients with atypical extranodal presentations who show remarkable and timely responses to Zanubrutinib, alongside a limited side effect profile. The report frames LPL within the spectrum including Waldenström’s macroglobulinemia, discusses typical treatment options and their limitations, and motivates targeted BTK inhibition as an effective strategy.",{"@graph":69,"@context":123},[70,84,106],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/extranodal-lymphoplasmacytic-lymphoma-responding-rapidly-to-zanubrutinib-a-case-series/419724/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/extranodal-lymphoplasmacytic-lymphoma-responding-rapidly-to-zanubrutinib-a-case-series/419724.png","ImageObject",300,407,{"name":92,"@type":93},"Ben ","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-11-05","2026-09-28",true,{"@type":102,"interactionType":103,"userInteractionCount":105},"InteractionCounter",{"@type":104},"ViewAction",11,{"@type":107,"mainEntity":108},"FAQPage",[109,115,119],{"name":110,"@type":111,"acceptedAnswer":112},"What condition is studied in this case series?","Question",{"text":113,"@type":114},"The case series focuses on extranodal lymphoplasmacytic lymphoma (LPL), including atypical extranodal manifestations.","Answer",{"name":116,"@type":111,"acceptedAnswer":117},"What treatment is used for the patients?",{"text":118,"@type":114},"All three patients are assessed for response to the BTK inhibitor Zanubrutinib, with treatment history described for each case.",{"name":120,"@type":111,"acceptedAnswer":121},"What genetic or molecular feature is emphasized?",{"text":122,"@type":114},"The report highlights the MYD88 L265P mutation, detected in the patients and linked to downstream pro-survival signaling.","https://schema.org",{"og:url":83,"og:type":125,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":127,"canonical":83},"index,follow",{"doc_id":129,"site_id":62},419724,1791169495,{"code":4,"msg":5,"data":132},{"doc_id":129,"user_id":133,"nickname":92,"user_avatar":134,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":135,"file_id":136,"file_url":137,"file_type":138,"file_size":139,"view_count":105,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":34,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145,"preview_page_count":4},2336478951081,"https://ap-avatar.wpscdn.com/davatar_276721f389ce27ea32af1340a28f341c","Clinical Case Reports  \nCASE SERIES  OPEN ACCESS   \nExtranodal Lymphoplasmacytic Lymphoma Responding Rapidly to Zanubrutinib: A Case Series  \nSamuel Brown1  | Jillian Rusbridge1  | George Follows2 | Anna Santarsieri3   \n1Clinical School of Medicine, University of Cambridge, Cambridge, UK | 2Department of Haematology, Addenbrooke's Hospital, Cambridge,  \nUK | 3Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK Correspondence: Samuel Brown ([sb2382@cam.ac.uk](sb2382@cam.ac.uk))  \nReceived: 19 March 2025 | Revised: 9 November 2025 | Accepted: 21 December 2025  \nKeywords: chronic diseases | hematology | oncology | pharmacology and pharmacy  \n\n| ABSTRACT\u003Cbr>Management of recurrent or atypical lymphoplasmacytic lymphoma poses a significant therapeutic challenge. Here we demonstrate a case series of three patients with atypical presentations of extranodal lymphoplasmacytic lymphoma that each show remarkable and timely responses to Zanubrutinib with a limited side effect profile. |  |\n| --- | --- |\n| 1 | Introduction | also lead to blood hyperviscosity, which increases the risk of headaches and strokes and may induce an inflammatory re- |\n| Lymphoplasmacytic lymphomas (LPL) are a rare, incurable | sponse and cause neuropathies [6] . |\n| group of non-Hodgkin lymphoma (0.27 per 100,000 people in the USA [1]) . They arise from post-germinal centre B-cell | Management of symptomatic patients with LPL gener- |\n| lymphoproliferation with, usually, IgM monoclonal gammop- | ally includes Rituximab as monotherapy, or in combination |\n| athy. When IgM paraprotein is produced, this disease can be | with chemotherapy [7] . These therapies confer good 2-year |\n| termed Waldenström's Macroglobulinemia (WM) [2] . The me- | progression-free survival (PFS) of 89% with Rituximab and |\n| dian survival is 5.3 years for individuals > 75 years [3] . More | Bendamustine and 81% with Bortezomib, Dexamethasone, |\n| than 95% of WM demonstrate an L265P mutation in the adap- | Rituximab, and Cyclophosphamide in a recent meta-analysis |\n| tor protein MYD88 (Myeloid Differentiation Primary Response 88) involved in IRAK1 and NF-κB signaling, promoting cell | [8]; but chemotherapy is associated with side effects including gastrointestinal symptoms, cytopenias, risk of infections, and |\n| survival [4] . This mutation leads to the constitutive homodime- | neutropenic sepsis, and the longer-term risk of secondary can- |\n| risation of MYD88 and subsequent recruitment and activation | cers [9]. Also, there is a limited number of lines of chemotherapy |\n| of several intracellular molecules, including Bruton Tyrosine Kinase (BTK) [5] . | that can be used due to cumulative toxicity. |\n|  | More recently, BTK inhibitors (Ibrutinib, Zanubrutinib) have |\n| The symptoms of LPL are largely due to bone marrow infiltra- | been licensed for LPL and Zanubrutinib has been approved |\n| tion and associated cytopaenias, commonly with constitutional | by NICE for treatment of patients with relapsed LPL [10] . BTK |\n| symptoms such as weight loss and fatigue [2], as well as mod- | inhibitors reduce the pro-survival downstream signaling from |\n| erate hepatosplenomegaly, lymphadenopathy, and anemia. | dysregulated MYD88 (L265P) homodimers [4] . These targeted |\n| Occasionally, platelet dysfunction or coagulopathy may be ob- | therapies have revolutionized LPL treatment, with a high re- |\n| served due to paraproteinaemia [2] . The IgM paraprotein can | sponse rate and favorable safety profile [11] . |\n| This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.\u003Cbr>© 2026 The Author(s). Clinical Case Reports published by John Wiley & Sons Ltd. |  |\n\nClinical Case Reports, 2026; 14:e71756 1 of 7  \n[https://doi.org/10.1002/ccr3.71756](https://doi.org/10.1002/ccr3.71756)  \nHere we present three patients with rare extranodal manifestations","cbCaioD8xCrjxwdp","https://ap.wps.com/l/cbCaioD8xCrjxwdp","pdf",563378,"English","# Abstract\n# Introduction\n# Case History and Examination\n## Patient A\n## Patient B","[{\"question\":\"What condition is studied in this case series?\",\"answer\":\"The case series focuses on extranodal lymphoplasmacytic lymphoma (LPL), including atypical extranodal manifestations.\"},{\"question\":\"What treatment is used for the patients?\",\"answer\":\"All three patients are assessed for response to the BTK inhibitor Zanubrutinib, with treatment history described for each case.\"},{\"question\":\"What genetic or molecular feature is emphasized?\",\"answer\":\"The report highlights the MYD88 L265P mutation, detected in the patients and linked to downstream pro-survival signaling.\"}]","Extranodal Lymphoplasmacytic Lymphoma Responding Rapidly to Zanubrutinib - A Case Series | PDF",1790618170,18]