{"id":"https://openalex.org/W2093393591","doi":"https://doi.org/10.1002/ajh.22208","title":"Systemic mastocytosis with associated clonal hematological non\u2010mast\u2010cell lineage disease: A case review","display_name":"Systemic mastocytosis with associated clonal hematological non\u2010mast\u2010cell lineage disease: A case review","publication_year":2011,"publication_date":"2011-09-30","ids":{"openalex":"https://openalex.org/W2093393591","doi":"https://doi.org/10.1002/ajh.22208","mag":"2093393591","pmid":"https://pubmed.ncbi.nlm.nih.gov/22081475"},"language":"en","primary_location":{"id":"doi:10.1002/ajh.22208","doi":"https://doi.org/10.1002/ajh.22208","is_oa":false,"landing_page_url":"https://doi.org/10.1002/ajh.22208","pdf_url":null,"source":{"id":"https://openalex.org/S199810014","display_name":"American Journal of Hematology","issn_l":"0361-8609","issn":["0361-8609","1096-8652"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-1","poland-140","russia-white-list-1"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"American Journal of Hematology","raw_type":"journal-article"},"volume":null,"type":"article","indexed_in":["crossref","pubmed"],"open_access":{"is_oa":false,"oa_status":"closed","oa_url":null,"any_repository_has_fulltext":false},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5071407391","display_name":"Celalettin \u00dcst\u00fcn","orcid":"https://orcid.org/0000-0001-6896-6213","observed_orcids":["https://orcid.org/0000-0001-6896-6213"]},"institutions":[{"id":"https://openalex.org/I130238516","display_name":"University of Minnesota","ror":"https://ror.org/017zqws13","country_code":"US","type":"education","lineage":["https://openalex.org/I130238516"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Celalettin Ustun","raw_affiliation_strings":["Division of Hematology Oncology and Transplantation, Department of Medicine, University of Minnesota, Twin Cities, MN, USA","University of\u00a0Minnesota"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Hematology Oncology and Transplantation, Department of Medicine, University of Minnesota, Twin Cities, MN, USA","institution_ids":["https://openalex.org/I130238516"]},{"raw_affiliation_string":"University of\u00a0Minnesota","institution_ids":["https://openalex.org/I130238516"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5034471031","display_name":"Natasha M. Savage","orcid":"https://orcid.org/0000-0003-1474-9568","observed_orcids":["https://orcid.org/0000-0003-1474-9568"]},"institutions":[{"id":"https://openalex.org/I4210137306","display_name":"Stanford Medicine","ror":"https://ror.org/03mtd9a03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210137306","https://openalex.org/I97018004"]},{"id":"https://openalex.org/I97018004","display_name":"Stanford University","ror":"https://ror.org/00f54p054","country_code":"US","type":"education","lineage":["https://openalex.org/I97018004"]}],"countries":["US"],"is_corresponding":true,"raw_author_name":"Natasha M. Savage","raw_affiliation_strings":["Department of Pathology, Stanford University School of Medicine, Stanford, California, USA","Stanford University"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology, Stanford University School of Medicine, Stanford, California, USA","institution_ids":["https://openalex.org/I4210137306","https://openalex.org/I97018004"]},{"raw_affiliation_string":"Stanford University","institution_ids":["https://openalex.org/I97018004"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5065621786","display_name":"Jason R. Gotlib","orcid":"https://orcid.org/0000-0001-8253-6156","observed_orcids":["https://orcid.org/0000-0001-8253-6156"]},"institutions":[{"id":"https://openalex.org/I4210137306","display_name":"Stanford Medicine","ror":"https://ror.org/03mtd9a03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210137306","https://openalex.org/I97018004"]},{"id":"https://openalex.org/I97018004","display_name":"Stanford University","ror":"https://ror.org/00f54p054","country_code":"US","type":"education","lineage":["https://openalex.org/I97018004"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Jason Gotlib","raw_affiliation_strings":["Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA","Stanford University"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA","institution_ids":["https://openalex.org/I4210137306","https://openalex.org/I97018004"]},{"raw_affiliation_string":"Stanford University","institution_ids":["https://openalex.org/I97018004"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5079069209","display_name":"Kapil N. Bhalla","orcid":"https://orcid.org/0000-0001-5209-5126","observed_orcids":["https://orcid.org/0000-0001-5209-5126"]},"institutions":[{"id":"https://openalex.org/I146416000","display_name":"University of Kansas","ror":"https://ror.org/001tmjg57","country_code":"US","type":"education","lineage":["https://openalex.org/I146416000"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Kapil Bhalla","raw_affiliation_strings":["Department of Medicine, University of Kansas, Kansas City, Kansas, USA","University of Kansas"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Medicine, University of Kansas, Kansas City, Kansas, USA","institution_ids":["https://openalex.org/I146416000"]},{"raw_affiliation_string":"University of Kansas","institution_ids":["https://openalex.org/I146416000"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5014241188","display_name":"Elizabeth Manaloor","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I25041050","display_name":"Augusta University","ror":"https://ror.org/012mef835","country_code":"US","type":"education","lineage":["https://openalex.org/I25041050"]},{"id":"https://openalex.org/I4210087454","display_name":"Augusta University Health","ror":"https://ror.org/007rawr89","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210087454"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Elizabeth Manaloor","raw_affiliation_strings":["Department of Pathology, Georgia Health Sciences University, Augusta, Georgia, USA","Medical College of Georgia"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology, Georgia Health Sciences University, Augusta, Georgia, USA","institution_ids":["https://openalex.org/I4210087454"]},{"raw_affiliation_string":"Medical College of Georgia","institution_ids":["https://openalex.org/I25041050"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5015788207","display_name":"Tracy I. George","orcid":"https://orcid.org/0000-0001-5478-7847","observed_orcids":["https://orcid.org/0000-0001-5478-7847"]},"institutions":[{"id":"https://openalex.org/I4210137306","display_name":"Stanford Medicine","ror":"https://ror.org/03mtd9a03","country_code":"US","type":"healthcare","lineage":["https://openalex.org/I4210137306","https://openalex.org/I97018004"]},{"id":"https://openalex.org/I97018004","display_name":"Stanford University","ror":"https://ror.org/00f54p054","country_code":"US","type":"education","lineage":["https://openalex.org/I97018004"]}],"countries":["US"],"is_corresponding":false,"raw_author_name":"Tracy I. George","raw_affiliation_strings":["Department of Pathology, Stanford University School of Medicine, Stanford, California, USA","Stanford University"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Pathology, Stanford University School of Medicine, Stanford, California, USA","institution_ids":["https://openalex.org/I4210137306","https://openalex.org/I97018004"]},{"raw_affiliation_string":"Stanford University","institution_ids":["https://openalex.org/I97018004"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":6,"corresponding_author_ids":["https://openalex.org/A5034471031"],"corresponding_institution_ids":["https://openalex.org/I4210137306","https://openalex.org/I97018004"],"apc_list":{"value":5450,"currency":"USD","value_usd":5450},"apc_paid":null,"fwci":0.2648,"has_fulltext":false,"cited_by_count":4,"citation_normalized_percentile":{"value":0.56236821,"is_in_top_1_percent":false,"is_in_top_10_percent":false},"cited_by_percentile_year":{"min":88,"max":94},"biblio":{"volume":"87","issue":"2","first_page":"191","last_page":"193"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11346","display_name":"Mast cells and histamine","score":0.9984999895095825,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11346","display_name":"Mast cells and histamine","score":0.9984999895095825,"subfield":{"id":"https://openalex.org/subfields/2403","display_name":"Immunology"},"field":{"id":"https://openalex.org/fields/24","display_name":"Immunology and Microbiology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T11909","display_name":"Histiocytic Disorders and Treatments","score":0.00019999999494757503,"subfield":{"id":"https://openalex.org/subfields/2737","display_name":"Physiology (medical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T12600","display_name":"Eosinophilic Disorders and Syndromes","score":9.999999747378752e-05,"subfield":{"id":"https://openalex.org/subfields/2745","display_name":"Rheumatology"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/systemic-mastocytosis","display_name":"systemic mastocytosis","score":0.9990000128746033},{"id":"https://openalex.org/keywords/urticaria-pigmentosa","display_name":"urticaria pigmentosa","score":0.871999979019165},{"id":"https://openalex.org/keywords/monocytosis","display_name":"monocytosis","score":0.5960000157356262},{"id":"https://openalex.org/keywords/megakaryocyte-dysplasia","display_name":"megakaryocyte dysplasia","score":0.7379999756813049},{"id":"https://openalex.org/keywords/myelodysplastic-syndrome","display_name":"myelodysplastic syndrome","score":0.6570000052452087},{"id":"https://openalex.org/keywords/myeloproliferative-neoplasms","display_name":"myeloproliferative neoplasms","score":0.8500000238418579},{"id":"https://openalex.org/keywords/mast-cell-infiltration","display_name":"mast cell infiltration","score":0.4309999942779541},{"id":"https://openalex.org/keywords/hepatosplenomegaly","display_name":"hepatosplenomegaly","score":0.765999972820282},{"id":"https://openalex.org/keywords/lymphadenopathy","display_name":"lymphadenopathy","score":0.8769999742507935},{"id":"https://openalex.org/keywords/differential-diagnosis","display_name":"differential diagnosis","score":0.3790000081062317},{"id":"https://openalex.org/keywords/mast-cell-mediators","display_name":"mast cell mediators","score":0.40299999713897705}],"concepts":[{"id":"https://openalex.org/C2909606595","wikidata":"https://www.wikidata.org/wiki/Q112670","display_name":"Cutaneous Mastocytosis","level":4,"score":0.9607976078987122},{"id":"https://openalex.org/C2779970125","wikidata":"https://www.wikidata.org/wiki/Q3886247","display_name":"Urticaria pigmentosa","level":2,"score":0.9596023559570312},{"id":"https://openalex.org/C2780581156","wikidata":"https://www.wikidata.org/wiki/Q112670","display_name":"Systemic mastocytosis","level":3,"score":0.8646876811981201},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.8267205953598022},{"id":"https://openalex.org/C16005928","wikidata":"https://www.wikidata.org/wiki/Q171171","display_name":"Dermatology","level":1,"score":0.6305676102638245},{"id":"https://openalex.org/C2778930412","wikidata":"https://www.wikidata.org/wiki/Q6785569","display_name":"Mastocytoma","level":3,"score":0.4975116550922394},{"id":"https://openalex.org/C2780439664","wikidata":"https://www.wikidata.org/wiki/Q7535376","display_name":"Skin biopsy","level":3,"score":0.4832347333431244},{"id":"https://openalex.org/C142724271","wikidata":"https://www.wikidata.org/wiki/Q7208","display_name":"Pathology","level":1,"score":0.4773305058479309},{"id":"https://openalex.org/C2779440204","wikidata":"https://www.wikidata.org/wiki/Q1260186","display_name":"Hepatosplenomegaly","level":3,"score":0.44481441378593445},{"id":"https://openalex.org/C2779726688","wikidata":"https://www.wikidata.org/wiki/Q191989","display_name":"Mast cell","level":2,"score":0.42104101181030273},{"id":"https://openalex.org/C2775934546","wikidata":"https://www.wikidata.org/wiki/Q179991","display_name":"Biopsy","level":2,"score":0.3639102280139923},{"id":"https://openalex.org/C2779134260","wikidata":"https://www.wikidata.org/wiki/Q12136","display_name":"Disease","level":2,"score":0.32623350620269775},{"id":"https://openalex.org/C203014093","wikidata":"https://www.wikidata.org/wiki/Q101929","display_name":"Immunology","level":1,"score":0.3055906891822815},{"id":"https://openalex.org/C502942594","wikidata":"https://www.wikidata.org/wiki/Q3421914","display_name":"Cancer research","level":1,"score":0.0},{"id":"https://openalex.org/C3020616263","wikidata":"https://www.wikidata.org/wiki/Q1216998","display_name":"Tumor cells","level":2,"score":0.0}],"mesh":[{"descriptor_ui":"D000970","descriptor_name":"Antineoplastic Agents","qualifier_ui":"Q000494","qualifier_name":"pharmacology","is_major_topic":false},{"descriptor_ui":"D000970","descriptor_name":"Antineoplastic Agents","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":false},{"descriptor_ui":"D006529","descriptor_name":"Hepatomegaly","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D006529","descriptor_name":"Hepatomegaly","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D007621","descriptor_name":"Karyotyping","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008206","descriptor_name":"Lymphatic Diseases","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D008206","descriptor_name":"Lymphatic Diseases","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D008214","descriptor_name":"Lymphocytes","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008214","descriptor_name":"Lymphocytes","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008407","descriptor_name":"Mast Cells","qualifier_ui":"Q000378","qualifier_name":"metabolism","is_major_topic":false},{"descriptor_ui":"D008407","descriptor_name":"Mast Cells","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true},{"descriptor_ui":"D008875","descriptor_name":"Middle Aged","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D013163","descriptor_name":"Splenomegaly","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":false},{"descriptor_ui":"D013163","descriptor_name":"Splenomegaly","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D017354","descriptor_name":"Point Mutation","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D019009","descriptor_name":"Proto-Oncogene Proteins c-kit","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D019070","descriptor_name":"Cell Lineage","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D034721","descriptor_name":"Mastocytosis, Systemic","qualifier_ui":"Q000175","qualifier_name":"diagnosis","is_major_topic":true},{"descriptor_ui":"D034721","descriptor_name":"Mastocytosis, Systemic","qualifier_ui":"Q000188","qualifier_name":"drug therapy","is_major_topic":false},{"descriptor_ui":"D034721","descriptor_name":"Mastocytosis, Systemic","qualifier_ui":"Q000235","qualifier_name":"genetics","is_major_topic":true},{"descriptor_ui":"D034721","descriptor_name":"Mastocytosis, Systemic","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":true}],"locations_count":2,"locations":[{"id":"doi:10.1002/ajh.22208","doi":"https://doi.org/10.1002/ajh.22208","is_oa":false,"landing_page_url":"https://doi.org/10.1002/ajh.22208","pdf_url":null,"source":{"id":"https://openalex.org/S199810014","display_name":"American Journal of Hematology","issn_l":"0361-8609","issn":["0361-8609","1096-8652"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-b","doyens","jufo-2","ki-jl-2","medline","norway-1","poland-140","russia-white-list-1"],"host_organization":"https://openalex.org/P4310320595","host_organization_name":"Wiley","host_organization_lineage":["https://openalex.org/P4310320595"],"host_organization_lineage_names":["Wiley"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"American Journal of Hematology","raw_type":"journal-article"},{"id":"pmid:22081475","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/22081475","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"American journal of hematology","raw_type":"Case Reports"}],"best_oa_location":null,"sustainable_development_goals":[{"id":"https://metadata.un.org/sdg/3","score":0.98580002784729,"display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/case-report","display_name":"Case Report"}],"awards":[],"funders":[],"has_content":{"pdf":false,"grobid_xml":false},"content_urls":null,"referenced_works_count":30,"referenced_works":["https://openalex.org/W1510071644","https://openalex.org/W1969530308","https://openalex.org/W1973053230","https://openalex.org/W1981135441","https://openalex.org/W1985830858","https://openalex.org/W1986712923","https://openalex.org/W1993468893","https://openalex.org/W1993937549","https://openalex.org/W2003055734","https://openalex.org/W2011838221","https://openalex.org/W2019598915","https://openalex.org/W2024510824","https://openalex.org/W2028737964","https://openalex.org/W2030028624","https://openalex.org/W2048860492","https://openalex.org/W2054635920","https://openalex.org/W2061597003","https://openalex.org/W2061637822","https://openalex.org/W2072594494","https://openalex.org/W2082707906","https://openalex.org/W2085785612","https://openalex.org/W2095807579","https://openalex.org/W2111914345","https://openalex.org/W2115823813","https://openalex.org/W2117754708","https://openalex.org/W2121216306","https://openalex.org/W2157298304","https://openalex.org/W2164833398","https://openalex.org/W2557604960","https://openalex.org/W2980118725"],"related_works":["https://openalex.org/W1987946408","https://openalex.org/W2405153450","https://openalex.org/W44581811","https://openalex.org/W1995158497","https://openalex.org/W4283460669","https://openalex.org/W2413881959","https://openalex.org/W4205129495","https://openalex.org/W2763312208","https://openalex.org/W2056378658","https://openalex.org/W4249942469"],"abstract_inverted_index":{"A":[0,316,397,584,619,1058],"54-year-old":[1],"male":[2],"was":[3,22,28,298,368,372,621,678,711,833,840,850,857,1160,1166,1175,1658,1676],"originally":[4],"referred":[5],"to":[6,63,157,178,184,722,774,787,797,810,820,903,911,1130,1152,1169,1277,1280,1285,1378,1412,1422,1471,1494,1505,1519,1535,1714,1727,1801,1831],"our":[7,582,967,1772,1819],"institution":[8],"2":[9,1406,1521,1530],"years":[10,31,1522,1531,1590],"prior":[11,1411],"with":[12,160,211,309,327,359,611,643,655,680,689,743,860,862,874,907,937,1074,1079,1098,1111,1143,1181,1195,1203,1233,1292,1362,1414,1500,1550,1641,1666,1744,1757,1783,1792,1796],"a":[13,48,68,161,169,194,288,322,409,517,594,606,640,728,768,828,881,1182,1215,1220,1237,1241,1245,1275,1281,1323,1492,1513,1572,1633,1688,1705,1721,1742,1754,1803],"diagnosis":[14,1593],"of":[15,72,130,144,172,229,241,251,307,325,331,340,356,469,494,542,551,591,674,783,791,801,813,824,905,959,1000,1053,1101,1132,1201,1229,1260,1374,1392,1509,1541,1571,1582,1601,1697,1702,1734,1806],"systemic":[16,213,1553],"mastocytosis":[17,191],"(SM).":[18],"Past":[19],"medical":[20],"history":[21],"significant":[23],"for":[24,175,433,462,699,746,765,888,926,971,1047,1219,1262,1538,1616,1650,1669,1685,1695],"urticaria":[25,105,232],"pigmentosa,":[26],"which":[27,40,430,684,839,1085,1126,1165,1217,1328,1375],"diagnosed":[29,441,859],"15":[30],"prior,":[32],"and":[33,36,56,97,120,138,140,181,183,198,215,239,256,277,286,303,336,351,364,370,380,408,446,548,573,599,605,624,653,663,704,737,749,794,805,816,847,955,1011,1014,1050,1116,1150,1244,1332,1401,1416,1429,1445,1456,1463,1614,1623,1639,1652,1699,1711,1717,1720,1767],"fatigue,":[34,96],"diarrhea,":[35],"weight":[37,98,1473],"loss":[38],"(\u223c10%),":[39],"were":[41,284,431,579,665,685,697,756,767,1409,1611,1648,1687],"more":[42,629,1021,1198,1288,1552,1558],"recent":[43,49,1514],"in":[44,76,164,204,260,300,375,480,485,497,556,581,687,739,761,1009,1018,1193,1290,1360,1385,1441,1498,1544,1548,1578,1672,1681,1741,1780,1790],"nature.":[45,416],"In":[46,150,188,295,354,560,671,706,734,1119,1294,1465],"addition,":[47,296,672,707,735,1466],"computed":[50],"tomography":[51],"(CT)":[52],"scan":[53],"revealed":[54,253,275,425,639,1069,1595,1632],"lymphadenopathy":[55,572,1122,1442],"hepatosplenomegaly.":[57],"Mastocytosis":[58],"is":[59,155,168,208,440,464,521,529,534,568,880,962,974,1028,1040,1045,1127,1214,1287,1298,1322,1502,1527,1746,1775],"currently":[60],"defined,":[61],"according":[62],"the":[64,128,147,158,173,176,249,261,301,341,357,376,389,403,467,481,495,498,543,589,675,843,884,898,963,977,1043,1095,1102,1153,1173,1301,1309,1318,1333,1349,1417,1477,1495,1506,1539,1565,1569,1579,1608,1629,1656,1776,1781,1813],"2008":[65],"WHO,":[66],"as":[67,104,134,890,892,932,979,1346,1348,1483,1491],"clonal,":[69],"neoplastic":[70,148,415,427,557,1282,1325,1580],"proliferation":[71],"mast":[73,279,282,328,342,365,393,404,682,693,700,1072,1330,1383],"cells":[74,283,329,343,1073,1147,1307,1331,1384,1581,1610,1647],"(MCs)":[75],"one":[77,443,447,1019],"or":[78,450,484,502,510,554,993,1002,1020,1036,1042,1443,1555],"multiple":[79,426,656,1596,1768],"organs":[80],"[1-3].":[81],"Clinical":[82],"symptoms":[83,93,109],"are":[84,125,456,500,508,575,909,945,1086,1148,1420,1799],"divided":[85],"into":[86,1094],"four":[87],"separate":[88,1736],"categories,":[89],"including":[90,263,646,727,947],"(1)":[91,492,980,1390],"constitutional":[92],"(i.e.,":[94],"fever,":[95],"loss),":[99],"(2)":[100,516,989,1395],"skin":[101,159,216],"manifestations":[102],"(such":[103],"pigmentosa),":[106],"(3)":[107,540,997,1397],"mediator-related":[108],"(to":[110],"include":[111],"abdominal":[112],"pain,":[113],"flushing,":[114],"headache,":[115],"hypotension,":[116],"tachycardia":[117],"among":[118],"others),":[119],"(4)":[121,549,1004,1399],"musculoskeletal":[122],"complaints.":[123],"These":[124,692,1646],"caused":[126],"by":[127,142,146,362,392,466,695,713,835,897,976,1081,1089,1357,1381],"release":[129],"chemical":[131],"mediators":[132],"such":[133],"histamine,":[135],"eicosanoids,":[136],"proteases,":[137],"heparin":[139],"also":[141,401,1594],"infiltration":[143,154,1093],"tissues":[145],"MCs.":[149],"cutaneous":[151,190,206,230,234,237],"mastocytosis,":[152,231,235,238],"MC":[153,472],"limited":[156],"typical":[162,1099],"presentation":[163],"childhood.":[165],"Urticaria":[166],"pigmentosa":[167],"macroscopic":[170],"description":[171],"tendency":[174],"lesions":[177,217],"urticate":[179],"(sting":[180],"blister)":[182],"accumulate":[185],"intraepidermal":[186],"pigment.":[187],"children,":[189],"typically":[192,220],"has":[193,1137,1367,1468],"benign":[195],"clinical":[196],"course":[197],"may":[199,564,1107,1123,1355,1827],"regress":[200],"spontaneously":[201],"[4].":[202],"However,":[203,571,1025,1794],"adults,":[205],"disease":[207,870,919,1438],"often":[209,569],"associated":[210,536,864,934,943,1142,1302,1350,1510],"indolent":[212],"involvement":[214,361,391,1080,1380],"do":[218],"not":[219,530,622,1029],"regress.":[221],"The":[222,270,458,633,758,855,940,1158,1251,1404,1678,1732],"WHO":[223,899,978],"now":[224],"recognizes":[225],"three":[226,453],"major":[227,444,459],"variants":[228],"pigmentosa/maculopapular":[233],"diffuse":[236],"mastocytoma":[240],"skin.":[242],"(a)":[243],"Peripheral":[244],"blood":[245,586,983,1010,1486],"smear,":[246],"performed":[247,841,1062,1410,1588,1627,1662],"at":[248,451,588,667,852,914,1264,1730],"time":[250,590],"referral,":[252,592],"absolute":[254],"monocytosis":[255,626,984,1044,1054,1567],"dysplastic":[257],"changes":[258],"noted":[259,369,580,834,910,1017,1109],"neutrophils":[262],"hypogranular":[264,647],"forms.":[265],"(1,000X,":[266],"Wright":[267,292,313],"Giemsa).":[268,293,314],"(b)":[269],"initial":[271,1592],"bone":[272,318,421,482,634,844,1012],"marrow":[273,319,422,483,635,642,845],"aspirate":[274,514,846,1631,1657],"eosinophilia":[276,367],"increased":[278,612,709,1071,1112],"cells.":[280,394,1135],"Numerous":[281],"atypical":[285,305,346,504,511,681,1382],"had":[287,627],"spindled":[289,347,686],"morphology.":[290],"(\u00d7400,":[291,312,435],"(c)":[294],"dysplasia":[297,371,645,1016],"pronounced":[299],"megakaryocytes":[302,654],"included":[304,1368],"clusters":[306],"micromegakaryocytes":[308],"hypolobated":[310,651],"nuclei.":[311,691],"(d)":[315],"concurrent":[317,1759],"biopsy":[320,358,423],"showed":[321,344,593,1516],"marked":[323,390],"infiltrate":[324,406,499],"fibrosis":[326,363,710,1117],"(>60%":[330],"overall":[332,676,1393,1523],"cellularity).":[333],"(\u00d7100,":[334],"Hematoxylin":[335,350,379],"Eosin).":[337,352,381],"(e)":[338],"Many":[339],"an":[345,535,724,863,933,1032,1064,1206,1230],"appearance.":[348],"(\u00d7200,":[349,378],"(f)":[353],"areas":[355,1097],"less":[360,668,1461],"cells,":[366,683,694,1114],"pronounced,":[373],"especially":[374],"megakaryocytes.":[377],"(g)":[382],"Mast":[383],"cell":[384,405,701,868,1304,1335,1352,1487,1738,1761,1786,1810],"tryptase":[385,519,849,1451],"immunohistochemical":[386,437],"stain":[387,400,411],"highlighted":[388,402,712],"(\u00d7100).":[395],"(h)":[396],"CD117":[398],"immuohistochemical":[399],"(\u00d7100),":[407],"CD25":[410],"helped":[412],"confirm":[413],"their":[414,918],"(insert,":[417],"\u00d7200).":[418],"(i)":[419],"Follow-up":[420,1366,1585,1660],"5":[424,793,1027],"lymphoid":[428,1598,1609],"aggregates,":[429],"positive":[432,698,1613],"CD20.":[434],"CD20":[436,1612,1640],"stain).":[438],"SM":[439,463,861,873,889,908,938,1196,1204,1345,1745,1755],"when":[442,1141],"criterion":[445,449,461,1026],"minor":[448,454],"least":[452],"criteria":[455,490,887,970],"present.":[457],"diagnostic":[460],"fulfilled":[465],"presence":[468],"multifocal":[470],"dense":[471],"infiltrates":[473],"(\u226515":[474],"MCs":[475,496,558],"per":[476],"aggregate)":[477],"detected":[478],"histologically":[479],"another":[486,893],"extracutaneous":[487],"organ(s).":[488],"Minor":[489],"include:":[491],"\u226525%":[493,507],"spindle-shaped":[501],"show":[503,1091,1379],"morphology":[505],"or,":[506],"immature":[509],"on":[512,842,1063,1163,1177,1628,1655,1663],"BM":[513,1371,1389,1407,1586,1630],"smears;":[515],"serum":[518,848,1450],"level":[520],"persistently":[522],"greater":[523],"than":[524,630,669,1006,1199,1812],"20":[525],"ng/mL":[526],"(this":[527],"parameter":[528],"valid":[531],"if":[532,1031,1557],"there":[533,1297,1321],"clonal":[537,865,1034,1149],"myeloid":[538,1022,1154],"disorder);":[539],"detection":[541,1696],"characteristic":[544],"D816V":[545],"KIT":[546,829,1211,1213,1268,1311],"mutation":[547,832,1209,1273,1312],"expression":[550],"CD2,":[552],"CD25,":[553],"both":[555],"[1-5].":[559],"these":[561,762,1146,1545,1682],"patients,":[562,1546],"splenomegaly":[563],"be":[565,1108,1124,1342,1520,1562],"appreciated,":[566],"but":[567,578,1512],"minimal.":[570],"hepatomegaly":[574],"much":[576],"rarer":[577],"patient.":[583],"complete":[585],"count,":[587],"normal":[595],"hemoglobin":[596],"(14.6":[597],"g/dL)":[598],"platelet":[600,750],"count":[601],"(326":[602],"\u00d7":[603,609,617,986],"109/L),":[604],"leukocytosis":[607],"(17.1":[608],"109/L)":[610],"monocytes":[613],"(absolute":[614],"monocytes:":[615],"1.4":[616],"109/L).":[618],"lymphocytosis":[620],"present,":[623],"his":[625,1423,1449,1480,1583,1833],"lasted":[628],"3":[631,1048,1419],"months.":[632],"(BM)":[636],"aspiration/biopsy":[637],"slides":[638],"normocellular":[641],"moderate":[644],"neutrophils,":[648],"micromegakaryocytes,":[649],"small":[650],"megakaryocytes,":[652],"discrete":[657],"nuclei":[658],"(Fig.":[659],"1).":[660],"Myeloblasts,":[661],"monoblasts,":[662],"promonocytes":[664],"enumerated":[666],"5%.":[670],"\u223c50%":[673],"cellularity":[677],"replaced":[679],"shape":[688],"elongated":[690],"immunohistochemistry,":[696,1607],"tryptase,":[702],"CD117,":[703],"CD25.":[705],"diffusely":[708],"reticulin":[714],"staining.":[715],"Per":[716],"outside":[717],"report,":[718],"flow":[719],"cytometry":[720,1626],"failed":[721],"reveal":[723],"abnormal":[725],"immunophenotype,":[726],"light":[729,1643],"chain":[730,837,1644],"restricted":[731],"B-cell":[732,1635],"population.":[733],"karyotype":[736],"florescent":[738],"situ":[740],"hybridization":[741],"(FISH)":[742],"probes":[744,809,1667],"specific":[745,808,1668,1694],"myelodysplastic":[747,956],"syndrome":[748,957],"derived":[751],"growth":[752],"factor":[753],"receptor-alpha":[754],"(PDGFRA)":[755],"normal.":[757,1659],"regions/loci":[759,1679],"represented":[760,1680],"probe":[763,1683,1693,1713,1726],"mixes":[764,1684],"MDS":[766],"dual":[769,771,1690,1706,1708,1722],"color":[770,1707,1723],"fusion":[772,995,1691,1709],"ETO/RUNX1":[773],"detect":[775,788,798,811,821,1715,1728],"t(8;21),":[776],"5p15.2":[777],"(D5S721),":[778],"5q33-34":[779],"(CSFIR),":[780],"centromeric":[781],"region":[782,807],"chromosome":[784,802,814,825,992,1703],"7,":[785,795],"7q31(D7S486)":[786],"copy":[789,799,1700],"number/deletion":[790],"chromosomes":[792],"CEP12":[796],"number":[800,823,1701],"12,":[803],"13q14,":[804],"13q34":[806],"deletion/loss":[812],"13,":[815],"20q13":[817],"(AURKA)":[818],"regions":[819],"deletion/copy":[822],"20.":[826],"Finally,":[827],"(D816V)":[830],"point":[831,916,1208,1254],"polymerase":[836],"reaction,":[838],"elevated":[851],"601":[853],"ng/mL.":[854],"patient":[856,885,968,1159,1174,1526,1743,1756,1779,1815],"then":[858],"hematologic":[866,894,923,935,1305,1336,1353,1739,1762,1788,1835],"non-mast":[867,1303,1334,1351,1737,1760,1785],"lineage":[869,1787],"(SM-AHNMD),":[871],"namely":[872,1824],"chronic":[875,951,1764],"myelomonocytic":[876,952],"leukemia-1":[877],"(SM-CMML-1).":[878],"SM-AHNMD":[879,1363,1501],"disorder":[882],"where":[883],"meets":[886],"well":[891,1347,1484],"neoplasm":[895,1155],"recognized":[896],"[1].":[900],"Surprisingly":[901],"up":[902],"30%":[904],"patients":[906,1194,1202,1291,1361,1499,1795,1820],"have":[912,929,1055,1190,1308,1376,1435,1453,1802,1828],"AHNMD":[913],"some":[915,1295,1536],"during":[917],"course.":[920],"Almost":[921],"all":[922,1373],"malignancies":[924,944,1740,1789],"except":[925],"Hodgkin":[927],"lymphoma":[928,1674],"been":[930,1056,1138,1191,1469,1829],"reported":[931,1753],"malignancy":[936,1337,1354],"[6-10].":[939],"most":[941,964,1252,1457],"frequently":[942],"myelogenous":[946,949],"acute":[948],"leukemia,":[950],"leukemia":[953,1766],"(CMML),":[954],"[6];":[958],"these,":[960],"CMML":[961,973],"common.":[965],"Fittingly,":[966],"met":[969],"CMML.":[972,1584],"defined":[975],"persistent":[981,1046],"peripheral":[982,1664],"(>1":[985],"109":[987],"/L);":[988],"no":[990,998],"Philadelphia":[991],"BCR-ABL1":[994],"gene;":[996],"rearrangement":[999],"PDGFRA":[1001],"PDGFRB;":[1003],"fewer":[1005],"20%":[1007],"blasts":[1008],"marrow;":[1013],"(5)":[1015,1402],"lineages":[1023],"[11].":[1024],"required":[1030],"acquired":[1033],"cytogenetic":[1035],"molecular":[1037],"genetic":[1038],"abnormality":[1039],"present":[1041,1577],"months":[1049],"other":[1051,1551,1778],"causes":[1052,1274],"excluded.":[1057],"fine":[1059],"needle":[1060],"aspiration":[1061],"enlarged":[1065],"cervical":[1066],"lymph":[1067],"node":[1068],"mildly":[1070],"admixed":[1075],"mature":[1076,1604],"lymphocytes,":[1077],"consistent":[1078],"mastocytosis.":[1082],"Lymph":[1083],"nodes,":[1084],"rarely":[1087],"involved":[1088],"SM,":[1090],"preferential":[1092],"paracortical":[1096],"preservation":[1100],"nodal":[1103],"architecture.":[1104],"Follicular":[1105],"hyperplasia":[1106],"along":[1110],"plasma":[1113],"eosinophils,":[1115],"[12].":[1118],"CMML,":[1120,1825],"generalized":[1121],"noted,":[1125],"characteristically":[1128],"secondary":[1129],"proliferations":[1131],"plasmacytoid":[1133],"dendritic":[1134],"It":[1136],"proposed":[1139],"that":[1140,1300,1320,1344],"this":[1144,1774],"disorder,":[1145,1823],"related":[1151],"[13,":[1156],"14].":[1157],"initially":[1161],"started":[1162],"dasatinib,":[1164],"discontinued":[1167],"due":[1168,1504],"gastrointestinal":[1170],"discomfort.":[1171],"Later":[1172],"initiated":[1176],"phase":[1178],"II":[1179],"trail":[1180],"tyrosine":[1183,1224,1247],"kinase":[1184,1248],"inhibitor":[1185],"(TKI),":[1186],"PKC412":[1187],"(midostaurin).":[1188],"TKIs":[1189,1286,1359],"investigated":[1192],"because":[1197],"90%":[1200],"contain":[1205],"activating":[1207],"within":[1210,1267],"[15-19].":[1212],"proto-oncogene,":[1216],"encodes":[1218],"class":[1221],"III":[1222],"receptor":[1223],"kinase.":[1225],"This":[1226,1272,1315],"protein":[1227],"consists":[1228],"extracellular":[1231],"domain":[1232,1249],"five":[1234,1369],"immunoglobulin-like":[1235],"repeats,":[1236],"single":[1238],"transmembrane":[1239],"domain,":[1240,1243],"juxtamembrane":[1242],"cytoplasmic":[1246],"[15].":[1250],"common":[1253,1324],"mutation,":[1255],"KITD816V,":[1256],"results":[1257],"from":[1258,1327],"substitution":[1259],"valine":[1261],"asparagine":[1263],"codon":[1265],"816":[1266],"exon":[1269],"17":[1270],"[20].":[1271],"resistance":[1276],"apoptosis,":[1278],"leading":[1279,1534],"clone.":[1283],"Response":[1284],"interesting":[1289],"SM-AHNMD.":[1293],"reports,":[1296],"evidence":[1299],"malignant":[1306,1329],"same":[1310],"[18,":[1313],"21-25].":[1314],"finding":[1316,1733],"supports":[1317],"belief":[1319],"progenitor":[1326],"arise.":[1338],"Therefore":[1339],"it":[1340],"can":[1341],"assumed":[1343],"improve":[1356],"using":[1358],"[26,":[1364],"27].":[1365],"additional":[1370],"biopsies;":[1372],"continued":[1377],"varying":[1386],"degrees":[1387],"[follow-up":[1388],"50%":[1391],"cellularity,":[1394],"80%,":[1396],"15%,":[1398],"30%,":[1400],"60%].":[1403],"first":[1405],"biopsies":[1408],"treatment":[1413,1425],"PKC412,":[1415],"last":[1418],"subsequent":[1421],"new":[1424],"after":[1426,1532,1591],"3,":[1427],"6,":[1428],"12":[1430],"cycles.":[1431],"Serial":[1432],"CT":[1433],"scans":[1434],"shown":[1436],"stable":[1437],"without":[1439],"increase":[1440],"liver":[1444],"spleen":[1446],"size.":[1447],"Overall,":[1448],"levels":[1452],"trended":[1454],"down,":[1455],"importantly,":[1458],"he":[1459,1467,1475,1826],"reports":[1460],"diarrhea":[1462],"fatigue.":[1464],"able":[1470],"gain":[1472],"since":[1474],"began":[1476],"trial.":[1478],"Interestingly,":[1479],"monocyte":[1481],"counts":[1482,1488],"white":[1485],"decreased":[1489],"significantly":[1490],"response":[1493],"TKI.":[1496],"Prognosis":[1497],"variable":[1503],"vast":[1507],"variety":[1508],"disorders,":[1511],"study":[1515],"median":[1517],"survival":[1518],"[28].":[1524],"Our":[1525],"alive":[1528],"over":[1529],"diagnosis,":[1533],"support":[1537],"use":[1540],"TKI":[1542],"therapy":[1543],"particularly":[1547],"combination":[1549],"therapies":[1554,1560],"alone":[1556],"intensive":[1559],"cannot":[1561],"tolerated.":[1563],"Furthermore,":[1564],"improving":[1566],"raises":[1568],"possibility":[1570],"similar":[1573],"KITD816V":[1574],"clone":[1575],"being":[1576],"5,":[1587],"\u223c2":[1589],"non-paratrabecular":[1597],"aggregates":[1599],"composed":[1600],"small,":[1602],"round,":[1603],"lymphocytes.":[1605],"By":[1606],"negative":[1615,1649],"CD3,":[1617],"CD43,":[1618],"CD5,":[1619],"CD23,":[1620],"CD10,":[1621],"BCL-2,":[1622],"BCL-1.":[1624],"Flow":[1625],"monoclonal":[1634,1808],"population":[1636,1816],"expressing":[1637],"CD19":[1638],"kappa":[1642],"restriction.":[1645],"CD5":[1651],"CD10.":[1653],"Karyotyping":[1654],"FISH,":[1661],"blood,":[1665],"recurring":[1670],"abnormalities":[1671],"non-Hodgkin":[1673],"(NHL)":[1675],"negative.":[1677],"NHL":[1686],"tri-color":[1689],"CMYC/IGH":[1692],"t(8;14)":[1698],"8,":[1704],"IGH/BCL1":[1710],"IGH/BCL2":[1712],"t(11;14)":[1716],"t(14;18),":[1718],"respectively,":[1719],"break-apart":[1724],"BCL6":[1725],"deletion/rearrangement":[1729],"3q27.":[1731],"two":[1735,1758,1784],"exceedingly":[1747],"rare.":[1748],"Recently,":[1749],"Du":[1750],"et":[1751],"al.":[1752],"malignancies,":[1763],"lymphocytic":[1765],"myeloma":[1769],"[29].":[1770],"To":[1771],"knowledge,":[1773],"only":[1777],"literature":[1782],"association":[1791],"SM.":[1793],"myeloproliferative":[1797],"neoplasms":[1798],"believed":[1800],"higher":[1804],"incidence":[1805],"developing":[1807,1832],"B":[1809],"populations":[1811],"general":[1814],"[30].":[1817],"Given":[1818],"known":[1821],"myelodysplastic/myeloproliferative":[1822],"predisposed":[1830],"third":[1834],"neoplasm.":[1836]},"counts_by_year":[{"year":2021,"cited_by_count":1},{"year":2020,"cited_by_count":1},{"year":2014,"cited_by_count":1},{"year":2012,"cited_by_count":1}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}