{"id":2561,"date":"2009-02-28T23:28:23","date_gmt":"2009-02-28T23:28:23","guid":{"rendered":"https:\/\/medicalcriteria.com\/web\/hemmast\/"},"modified":"2025-05-14T19:11:50","modified_gmt":"2025-05-14T19:11:50","slug":"hemmast","status":"publish","type":"post","link":"https:\/\/medicalcriteria.com\/web\/hemmast\/","title":{"rendered":"WHO Classification of Mastocytosis"},"content":{"rendered":"<div class=\"99c380e4b4a7b96c35d7ddf7dcb434e8\" data-index=\"1\" style=\"float: none; margin:0px 0 0px 0; text-align:center;\">\n<script async src=\"https:\/\/pagead2.googlesyndication.com\/pagead\/js\/adsbygoogle.js\"><\/script>\r\n<!-- MC 2019- Horizontal -->\r\n<ins class=\"adsbygoogle\"\r\n     style=\"display:block\"\r\n     data-ad-client=\"ca-pub-0127150553352455\"\r\n     data-ad-slot=\"3806776041\"\r\n     data-ad-format=\"auto\"\r\n     data-full-width-responsive=\"true\"><\/ins>\r\n<script>\r\n     (adsbygoogle = window.adsbygoogle || []).push({});\r\n<\/script>\n<\/div>\n<p>Systemic mastocytosis is a clonal disorder of the mast cell and its progenitor. The symptoms of systemic mastocytosis are due to the pathologic accumulation and activation of mast cells in various tissues such as bone marrow, skin, gastrointestinal tract, liver, and spleen.<\/p>\n<p><!--more--><\/p>\n<table style=\"border-collapse: collapse; width: 100%; height: 370px;\" border=\"1\">\n<tbody>\n<tr style=\"height: 56px;\">\n<td style=\"border: 1pt solid windowtext; padding: 0cm 5.4pt; width: 167.4pt; height: 56px;\" valign=\"top\" width=\"223\">\n<p class=\"MsoNormal\"><strong>Variant \u2013 Term (Abbreviation<\/strong><strong>)<\/strong><\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 56px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\"><strong>Subvariants<\/strong><\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 4.4pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 167.4pt; height: 18px;\" rowspan=\"3\" valign=\"top\" width=\"223\">\n<p class=\"MsoNormal\">\u2022 Cutaneous Mastocytosis (CM)<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 10px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Urticaria Pigmentosa (UP) = Maculopapular CM (MPCM)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 4.4pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 4px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Diffuse CM (DCM)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 4.4pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 4px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Mastocytoma of Skin<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 6.6pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 167.4pt; height: 12px;\" rowspan=\"2\" valign=\"top\" width=\"223\">\n<p class=\"MsoNormal\">\u2022 Indolent Systemic Mastocytosis (ISM)<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 6px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Smouldering SM (SSM)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 6.6pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 6px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Isolated bone marrow mastocytosis (BMM)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 6.6pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 167.4pt; height: 36px;\" rowspan=\"6\" valign=\"top\" width=\"223\">\n<p class=\"MsoNormal\">\u2022 Systemic Mastocytosis with an associated clonal hematologic non-mast cell lineage disease (SM-AHNMD)<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 6px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Systemic Mastocytosis with Acute Myeloid Leukemia\u00a0 (SM-AML)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 6.6pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 6px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Systemic Mastocytosis with Myelodysplastic Syndrome (SM-MDS)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 6.6pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 6px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Systemic Mastocytosis with Myeloproliferative Disorder (SM-MPD)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 6.6pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 6px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Systemic Mastocytosis with Chronic Myelomonocytic Leukemia (SM-CMML)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 6.6pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 6px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Systemic Mastocytosis with Non-Hodgkin\u2019s Lymphoma (SM-NHL)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 6.6pt;\">\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 6px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Systemic Mastocytosis with Hypereosinophilic Syndrome (SM-HES)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 80px;\">\n<td style=\"padding: 0cm 5.4pt; width: 167.4pt; height: 80px;\" valign=\"top\" width=\"223\">\n<p class=\"MsoNormal\">\u2022 Aggressive Systemic Mastocytosis (ASM)<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 80px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Lymphadenopathic SM with eosinophilia (In a few cases, the FIP1L1\/PDGFRA-fusion gene may be detected)<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 56px;\">\n<td style=\"padding: 0cm 5.4pt; width: 167.4pt; height: 56px;\" valign=\"top\" width=\"223\">\n<p class=\"MsoNormal\">\u2022 Mast Cell Leucemia (MCL)<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 56px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\u2013 Aleukemic MCL<\/p>\n<\/td>\n<\/tr>\n<tr style=\"height: 56px;\">\n<td style=\"padding: 0cm 5.4pt; width: 167.4pt; height: 56px;\" valign=\"top\" width=\"223\">\n<p class=\"MsoNormal\">\u2022 Mast Cell Sarcoma (MCS)<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 56px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\n<\/td>\n<\/tr>\n<tr style=\"height: 56px;\">\n<td style=\"padding: 0cm 5.4pt; width: 167.4pt; height: 56px;\" valign=\"top\" width=\"223\">\n<p class=\"MsoNormal\">\u2022 Extracutaneous Mastocytoma<\/p>\n<\/td>\n<td style=\"padding: 0cm 5.4pt; width: 264.8pt; height: 56px;\" valign=\"top\" width=\"353\">\n<p class=\"MsoNormal\">\n<\/td>\n<\/tr>\n<\/tbody>\n<\/table>\n<p class=\"MsoNormal\">WHO, World Health Organization<\/p>\n<p>&nbsp;<\/p>\n<p class=\"MsoNormal\"><strong>References:<\/strong><\/p>\n<ol>\n<li>Akin C, Metcalfe DD. Systemic mastocytosis. Annu Rev Med. 2004;55:419-32. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/14746529\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<li>O&#8217;Brien S, Tefferi A, Valent P. Chronic myelogenous leukemia and myeloproliferative disease. Hematology (Am Soc Hematol Educ Program). 2004;:146-62. <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/15561681\/\" target=\"_blank\" rel=\"noopener noreferrer\">[Medline]<\/a><\/li>\n<\/ol>\n<p>&nbsp;<\/p>\n<p>Created: Jun 21, 2005<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Sorry, this entry is only available in Espa\u00f1ol.<\/p>\n","protected":false},"author":2,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_lmt_disableupdate":"no","_lmt_disable":"no","_exactmetrics_skip_tracking":false,"_exactmetrics_sitenote_active":false,"_exactmetrics_sitenote_note":"","_exactmetrics_sitenote_category":0,"footnotes":""},"categories":[171],"tags":[166,167,1381,1716,1715,307,306],"class_list":["post-2561","post","type-post","status-publish","format-standard","hentry","category-hematology","tag-clasificacion","tag-classification","tag-hematology","tag-mastocitosis","tag-mastocytosis","tag-oms","tag-who"],"modified_by":"Guillermo Firman","_links":{"self":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2561","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/comments?post=2561"}],"version-history":[{"count":4,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2561\/revisions"}],"predecessor-version":[{"id":9376,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/posts\/2561\/revisions\/9376"}],"wp:attachment":[{"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/media?parent=2561"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/categories?post=2561"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medicalcriteria.com\/web\/wp-json\/wp\/v2\/tags?post=2561"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}