{"id":5541,"date":"2021-05-19T17:22:34","date_gmt":"2021-05-19T15:22:34","guid":{"rendered":"https:\/\/meddists.com\/learn\/clinical\/hematology\/hematological-oncology\/plasma-cell-disorders-dyscrasias\/waldenstroms-macroglobulinemia\/"},"modified":"2021-05-19T19:02:47","modified_gmt":"2021-05-19T17:02:47","slug":"waldenstroms-macroglobulinemia","status":"publish","type":"page","link":"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/plasma-cell-disorders-dyscrasias\/waldenstroms-macroglobulinemia\/","title":{"rendered":"Waldenstr\u00f6m\u2019s macroglobulinemia (lymphoplasmacytic lymphoma)"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\"><div class=\"intro\">Waldenstr\u00f6m\u2019s macroglobulinemia, also known as lymphoplasmacytic lymphoma, is a type of non-Hodgkin lymphoma in which there&#8217;s a clonic proliferation of malignant B-cells, producing excessive amounts of monoclonal IgM antibodies.<\/div><\/p>\n\n\n<span class=\"block-heading\" id=\"header_1\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Background<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_1\">\n\n\n<ul class=\"wp-block-list\"><li>These plasma cells are usually monoclonal and therefore produce monoclonal immunoglobulin referred to as <strong>protein M\u00a0<\/strong>(M\u00a0as in\u00a0myeloma) or <strong>paraprotein<\/strong>.<\/li><li>Levels of protein M can be measured:<ul><li>From the blood (electrophoresis)<ul><li>&#8220;M spike&#8221; is seen indicating a high level of immunoglobulins<\/li><\/ul><\/li><\/ul><ul><li>From the urine (in glomerular damage)<\/li><\/ul><\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_2\">\n<h3 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title1\">Waldenstr\u00f6m\u2019s macroglobulinemia<\/h3>\n<\/span><span class=\"block-content\" id=\"contents_2\">\n\n\n<p class=\"wp-block-paragraph\">Waldenstr\u00f6m\u2019s macroglobulinemia is a clonic proliferation of B-cells in the <strong>bone marrow<\/strong>. The cells are producing monoclonal IgM antibodies.<\/p>\n\n\n\n<ul class=\"wp-block-list\"><li><strong>Waldenstr\u00f6m\u2019s <\/strong>= author name; <strong>macro <\/strong>= increased; <strong>globulin <\/strong>= antibody; <strong>emia <\/strong>= in the blood.<\/li><li>Waldenstrom macroglobulinemia is considered a type of non-Hodgkin&#8217;s lymphoma, although it involves plasma cells.<\/li><li>Clinically and genetically resembles CLL.<\/li><li>MYD88\u00a0mutation is seen in 90% of cases.<\/li><li>Presents with hyperviscosity and cold AIHA (<strong>cryoglobulinemia<\/strong>).<\/li><li><strong>Amyloidosis<\/strong> may develop (AL-amyloidosis) presenting with macroglossia.<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_3\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Clinical manifestation<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_3\">\n\n\n<ul class=\"wp-block-list\"><li>Bleeding<\/li><li>Recurrent infections<\/li><li>Lymphadenopathy<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_4\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Diagnosis<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_4\">\n\n\n<ul class=\"wp-block-list\" id=\"block-bf391c5a-e4cb-4fe6-87b2-3c47d8f15ff1\"><li>CBC (demonstrating anemia)<\/li><li>ESR (elevated)<\/li><li>Plasma (<strong>SPEP<\/strong>) and urine (<strong>UPEP<\/strong>) electrophoresis (demonstrating M-protein; in the urine, it is referred to as <strong>Bence-Jones protein<\/strong>)<\/li><li>Serum beta2-microglobulin (elevated; negative prognostic sign)<\/li><li>Blood smear (<strong>Rouleaux formation<\/strong> &#8211; clumping of RBCs)<\/li><li>Free light-chain essay<\/li><li>Bone marrow sample (demonstrating increased clonal plasma cell population)<\/li><li>Imaging (x-ray, MRI)<\/li><li>Kidney function tests<\/li><\/ul>\n\n\n<\/span><span class=\"block-heading\" id=\"header_5\">\n<h4 class=\"wp-block-heading\" class=\"wp-block-heading\" class=\"title_collection title2\">Treatment<\/h4>\n<\/span><span class=\"block-content\" id=\"contents_5\">\n\n\n<ul class=\"wp-block-list\"><li>Plasma exchange (to deal with the hyperviscosity and cryoglobulinemia)<\/li><li>Chemotherapy (cyclophosphamide, fludarabine)<\/li><li>Lenalidomide<\/li><li>Bortezomib (protease inhibitor)<\/li><li>Ibrutinib (BTK inhibitor)<\/li><li>Rituximab<\/li><\/ul>\n<\/span><div id=\"the_titles\" style=\"display:none;\"><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Background<\/h3><h3 class=\"wp-block-heading\" class=\"wp-block-heading\">Waldenstr\u00f6m\u2019s macroglobulinemia<\/h3><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Clinical manifestation<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Diagnosis<\/h4><h4 class=\"wp-block-heading\" class=\"wp-block-heading\">Treatment<\/h4><\/div>","protected":false},"excerpt":{"rendered":"<p>Background These plasma cells are usually monoclonal and therefore produce monoclonal immunoglobulin referred to as protein M\u00a0(M\u00a0as in\u00a0myeloma) or paraprotein. Levels of protein M can be measured: From the blood (electrophoresis) &#8220;M spike&#8221; is seen indicating a high level of immunoglobulins From the urine (in glomerular damage) Waldenstr\u00f6m\u2019s macroglobulinemia Waldenstr\u00f6m\u2019s macroglobulinemia is a clonic proliferation [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":5539,"menu_order":1,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-5541","page","type-page","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.9 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Waldenstr\u00f6m\u2019s macroglobulinemia (lymphoplasmacytic lymphoma) &#8211; Meddists<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/meddists.com\/learn\/clinical\/internal-medicine\/hematology\/oncological-disorders\/plasma-cell-disorders-dyscrasias\/waldenstroms-macroglobulinemia\/\" \/>\n<meta name=\"twitter:label1\" content=\"Est. reading time\" \/>\n\t<meta name=\"twitter:data1\" content=\"2 minutes\" \/>\n<script type=\"application\/ld+json\" class=\"yoast-schema-graph\">{\"@context\":\"https:\\\/\\\/schema.org\",\"@graph\":[{\"@type\":\"WebPage\",\"@id\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/oncological-disorders\\\/plasma-cell-disorders-dyscrasias\\\/waldenstroms-macroglobulinemia\\\/\",\"url\":\"https:\\\/\\\/meddists.com\\\/learn\\\/clinical\\\/internal-medicine\\\/hematology\\\/oncological-disorders\\\/plasma-cell-disorders-dyscrasias\\\/waldenstroms-macroglobulinemia\\\/\",\"name\":\"Waldenstr\u00f6m\u2019s macroglobulinemia (lymphoplasmacytic lymphoma) &#8211; 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