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Subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: a systematic review of 63 patients reported in the literature
To review and summarize the clinical features, treatment strategies, and prognosis of subcutaneous panniculitis-like T-cell lymphoma complicated with hemophagocytic lymphohistiocytosis (SPTCL–HLH). We searched the Web of Science, Embase, Cochrane Library, and PubMed databases. The keywords were subc...
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Published in: | Clinical and experimental medicine 2023-12, Vol.23 (8), p.4575-4583 |
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description | To review and summarize the clinical features, treatment strategies, and prognosis of subcutaneous panniculitis-like T-cell lymphoma complicated with hemophagocytic lymphohistiocytosis (SPTCL–HLH). We searched the Web of Science, Embase, Cochrane Library, and PubMed databases. The keywords were subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis or hemophagocytic syndrome. The patients were divided into a mutated group and a wild-type group based on the existence of
HAVCR2
gene mutation. A total of 45 reports, including 63 patients with SPTCL–HLH, were included in the systematic review. Twelve patients detected gene mutations, including 11 with the
HAVCR2
gene mutation and 1 with the
STXBP2
gene mutation. Thirty-one patients were tested for autoantibodies. Compared with the wild-type group, patients in the mutated group were younger (
p
= 0.017), and the autoantibody-positive rate was higher (
p
= 0.006). The main treatment target of 17 patients was to control HLH, yielding an ORR of 88.2%. Two cases relapsed, and both were treated with corticosteroid monotherapy. The corticosteroid monotherapy experienced a higher recurrence rate than the corticosteroids plus other immunoregulatory agents therapy (66.7 vs. 0.0%,
p
= 0.029). Eighteen patients received initial anthracycline-based chemotherapy, and 50.0% reached remission. The ORR of initial chemotherapy aiming at controlling HLH was higher than those of anthracycline-based chemotherapy (
p
= 0.015). The ORR was higher in patients initially controlled for HLH versus chemotherapy without HLH control first (90.5 vs. 61.5%,
p
= 0.024). Interestingly, one patient with juvenile idiopathic arthritis developed SPTCL–HLH during tocilizumab therapy, discontinuing tocilizumab led to a remission of the disease spontaneously. Sixteen patients received stem cell transplantation (SCT). Fifteen patients, including 5 with relapsed/refractory SPTCL–HLH, responded well and survived after receiving SCT. One case who received a sibling-identical SCT relapsed. Further analysis revealed a homozygous
HAVCR2
mutation with the donor. The 2-year overall survival (OS) was 91.0% ± 4.4%. There was a significant difference in the OS among patients of different age groups, and patients aged 40–60 had the lowest 2-year OS (66.7% ± 19.2%). Patients with
HAVCR2
gene mutations are younger and more likely to be misdiagnosed with autoimmune diseases. Initial treatment of corticosteroids plus immunoregulato |
doi_str_mv | 10.1007/s10238-023-01210-1 |
format | article |
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HAVCR2
gene mutation. A total of 45 reports, including 63 patients with SPTCL–HLH, were included in the systematic review. Twelve patients detected gene mutations, including 11 with the
HAVCR2
gene mutation and 1 with the
STXBP2
gene mutation. Thirty-one patients were tested for autoantibodies. Compared with the wild-type group, patients in the mutated group were younger (
p
= 0.017), and the autoantibody-positive rate was higher (
p
= 0.006). The main treatment target of 17 patients was to control HLH, yielding an ORR of 88.2%. Two cases relapsed, and both were treated with corticosteroid monotherapy. The corticosteroid monotherapy experienced a higher recurrence rate than the corticosteroids plus other immunoregulatory agents therapy (66.7 vs. 0.0%,
p
= 0.029). Eighteen patients received initial anthracycline-based chemotherapy, and 50.0% reached remission. The ORR of initial chemotherapy aiming at controlling HLH was higher than those of anthracycline-based chemotherapy (
p
= 0.015). The ORR was higher in patients initially controlled for HLH versus chemotherapy without HLH control first (90.5 vs. 61.5%,
p
= 0.024). Interestingly, one patient with juvenile idiopathic arthritis developed SPTCL–HLH during tocilizumab therapy, discontinuing tocilizumab led to a remission of the disease spontaneously. Sixteen patients received stem cell transplantation (SCT). Fifteen patients, including 5 with relapsed/refractory SPTCL–HLH, responded well and survived after receiving SCT. One case who received a sibling-identical SCT relapsed. Further analysis revealed a homozygous
HAVCR2
mutation with the donor. The 2-year overall survival (OS) was 91.0% ± 4.4%. There was a significant difference in the OS among patients of different age groups, and patients aged 40–60 had the lowest 2-year OS (66.7% ± 19.2%). Patients with
HAVCR2
gene mutations are younger and more likely to be misdiagnosed with autoimmune diseases. Initial treatment of corticosteroids plus immunoregulatory agents attaches great significance to avoiding too aggressive therapies. Intensive anthracycline-based chemotherapy such as CHOP or CHOP-like regimens can also induce long-term remission for aggressive disease. SCT is still a reliable strategy currently. In addition, a watch and wait approach is recommended in patients with mild SPTCL–HLH caused by drugs. The occurrence of HLH does not necessarily mean a more rapidly progressive disease and worse prognosis in patients with SPTCL, but older patients with SPTCL–HLH may be associated with a lower survival rate.</description><identifier>ISSN: 1591-9528</identifier><identifier>ISSN: 1591-8890</identifier><identifier>EISSN: 1591-9528</identifier><identifier>DOI: 10.1007/s10238-023-01210-1</identifier><identifier>PMID: 37840116</identifier><language>eng</language><publisher>Cham: Springer International Publishing</publisher><subject>Adrenal Cortex Hormones ; Anthracycline ; Anthracyclines ; Arthritis ; Autoantibodies ; Autoimmune diseases ; Chemotherapy ; Clinical Practice ; Corticosteroids ; Disease ; Hematology ; Hemophagocytic syndrome ; Histiocytosis ; Humans ; Immunoregulation ; Immunosuppressive agents ; Internal Medicine ; Lymphatic diseases ; Lymphocytes T ; Lymphocytosis ; Lymphohistiocytosis, Hemophagocytic - diagnosis ; Lymphohistiocytosis, Hemophagocytic - drug therapy ; Lymphohistiocytosis, Hemophagocytic - genetics ; Lymphoma ; Medical prognosis ; Medicine ; Medicine & Public Health ; Mutation ; Oncology ; Panniculitis - complications ; Panniculitis - diagnosis ; Panniculitis - drug therapy ; Patients ; Point mutation ; Prognosis ; Remission ; Remission (Medicine) ; Stem cell transplantation ; Steroids ; Systematic review ; T-cell lymphoma</subject><ispartof>Clinical and experimental medicine, 2023-12, Vol.23 (8), p.4575-4583</ispartof><rights>The Author(s), under exclusive licence to Springer Nature Switzerland AG 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.</rights><rights>2023. The Author(s), under exclusive licence to Springer Nature Switzerland AG.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c375t-37d6e9e46152fba322c6ff44e0d6049dbbd2fab727297b090a88148d5a734da93</citedby><cites>FETCH-LOGICAL-c375t-37d6e9e46152fba322c6ff44e0d6049dbbd2fab727297b090a88148d5a734da93</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>315,786,790,27957,27958</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37840116$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ou, Wenxin</creatorcontrib><creatorcontrib>Zhao, Yunze</creatorcontrib><creatorcontrib>Wei, Ang</creatorcontrib><creatorcontrib>Ma, Honghao</creatorcontrib><creatorcontrib>Zhang, Liping</creatorcontrib><creatorcontrib>Lian, Hongyun</creatorcontrib><creatorcontrib>Zhang, Qing</creatorcontrib><creatorcontrib>Wang, Dong</creatorcontrib><creatorcontrib>Li, Zhigang</creatorcontrib><creatorcontrib>Wang, Tianyou</creatorcontrib><creatorcontrib>Zhang, Rui</creatorcontrib><title>Subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: a systematic review of 63 patients reported in the literature</title><title>Clinical and experimental medicine</title><addtitle>Clin Exp Med</addtitle><addtitle>Clin Exp Med</addtitle><description>To review and summarize the clinical features, treatment strategies, and prognosis of subcutaneous panniculitis-like T-cell lymphoma complicated with hemophagocytic lymphohistiocytosis (SPTCL–HLH). We searched the Web of Science, Embase, Cochrane Library, and PubMed databases. The keywords were subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis or hemophagocytic syndrome. The patients were divided into a mutated group and a wild-type group based on the existence of
HAVCR2
gene mutation. A total of 45 reports, including 63 patients with SPTCL–HLH, were included in the systematic review. Twelve patients detected gene mutations, including 11 with the
HAVCR2
gene mutation and 1 with the
STXBP2
gene mutation. Thirty-one patients were tested for autoantibodies. Compared with the wild-type group, patients in the mutated group were younger (
p
= 0.017), and the autoantibody-positive rate was higher (
p
= 0.006). The main treatment target of 17 patients was to control HLH, yielding an ORR of 88.2%. Two cases relapsed, and both were treated with corticosteroid monotherapy. The corticosteroid monotherapy experienced a higher recurrence rate than the corticosteroids plus other immunoregulatory agents therapy (66.7 vs. 0.0%,
p
= 0.029). Eighteen patients received initial anthracycline-based chemotherapy, and 50.0% reached remission. The ORR of initial chemotherapy aiming at controlling HLH was higher than those of anthracycline-based chemotherapy (
p
= 0.015). The ORR was higher in patients initially controlled for HLH versus chemotherapy without HLH control first (90.5 vs. 61.5%,
p
= 0.024). Interestingly, one patient with juvenile idiopathic arthritis developed SPTCL–HLH during tocilizumab therapy, discontinuing tocilizumab led to a remission of the disease spontaneously. Sixteen patients received stem cell transplantation (SCT). Fifteen patients, including 5 with relapsed/refractory SPTCL–HLH, responded well and survived after receiving SCT. One case who received a sibling-identical SCT relapsed. Further analysis revealed a homozygous
HAVCR2
mutation with the donor. The 2-year overall survival (OS) was 91.0% ± 4.4%. There was a significant difference in the OS among patients of different age groups, and patients aged 40–60 had the lowest 2-year OS (66.7% ± 19.2%). Patients with
HAVCR2
gene mutations are younger and more likely to be misdiagnosed with autoimmune diseases. Initial treatment of corticosteroids plus immunoregulatory agents attaches great significance to avoiding too aggressive therapies. Intensive anthracycline-based chemotherapy such as CHOP or CHOP-like regimens can also induce long-term remission for aggressive disease. SCT is still a reliable strategy currently. In addition, a watch and wait approach is recommended in patients with mild SPTCL–HLH caused by drugs. The occurrence of HLH does not necessarily mean a more rapidly progressive disease and worse prognosis in patients with SPTCL, but older patients with SPTCL–HLH may be associated with a lower survival rate.</description><subject>Adrenal Cortex Hormones</subject><subject>Anthracycline</subject><subject>Anthracyclines</subject><subject>Arthritis</subject><subject>Autoantibodies</subject><subject>Autoimmune diseases</subject><subject>Chemotherapy</subject><subject>Clinical Practice</subject><subject>Corticosteroids</subject><subject>Disease</subject><subject>Hematology</subject><subject>Hemophagocytic syndrome</subject><subject>Histiocytosis</subject><subject>Humans</subject><subject>Immunoregulation</subject><subject>Immunosuppressive agents</subject><subject>Internal Medicine</subject><subject>Lymphatic diseases</subject><subject>Lymphocytes T</subject><subject>Lymphocytosis</subject><subject>Lymphohistiocytosis, Hemophagocytic - diagnosis</subject><subject>Lymphohistiocytosis, Hemophagocytic - drug therapy</subject><subject>Lymphohistiocytosis, Hemophagocytic - genetics</subject><subject>Lymphoma</subject><subject>Medical prognosis</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Mutation</subject><subject>Oncology</subject><subject>Panniculitis - complications</subject><subject>Panniculitis - diagnosis</subject><subject>Panniculitis - drug therapy</subject><subject>Patients</subject><subject>Point mutation</subject><subject>Prognosis</subject><subject>Remission</subject><subject>Remission (Medicine)</subject><subject>Stem cell transplantation</subject><subject>Steroids</subject><subject>Systematic review</subject><subject>T-cell lymphoma</subject><issn>1591-9528</issn><issn>1591-8890</issn><issn>1591-9528</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2023</creationdate><recordtype>article</recordtype><recordid>eNp9kc1u1TAQhSMEoqXwAiyQJTZsAmM7iR12qKIFqRILytpykknjksTB47S6b8Oj4pDLj1iwGVvjb87M-GTZcw6vOYB6QxyE1HkKOXDBIecPslNe1jyvS6Ef_nU_yZ4Q3QLwUkt4nJ1IpQvgvDrNvn9em3aNdka_ElvsPLt2HV10lI_uK7LrvMVxZONhWgY_WWaJfOtsxI7duziwASe_DPbGt4fo2iM3OIpuy3hy9JZZRgeKONmNCHjn8J75nlUy9YsO50gpu_iwibqZxQFZmgCDjWvAp9mj3o6Ez47nWfbl4v31-Yf86tPlx_N3V3krVRlzqboKaywqXoq-sVKItur7okDoKijqrmk60dtGCSVq1UANVmte6K60ShadreVZ9mrXXYL_tiJFMznadt-_xgitNHCVxBL68h_01q9hTtMZUYNQALLiiRI71QZPFLA3S3CTDQfDwWz-md0_k4L56Z_Zil4cpddmwu53yS_DEiB3gNLTfIPhT-__yP4ATlqpkA</recordid><startdate>20231201</startdate><enddate>20231201</enddate><creator>Ou, Wenxin</creator><creator>Zhao, Yunze</creator><creator>Wei, Ang</creator><creator>Ma, Honghao</creator><creator>Zhang, Liping</creator><creator>Lian, Hongyun</creator><creator>Zhang, Qing</creator><creator>Wang, Dong</creator><creator>Li, Zhigang</creator><creator>Wang, Tianyou</creator><creator>Zhang, Rui</creator><general>Springer International Publishing</general><general>Springer Nature B.V</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>7TK</scope><scope>H94</scope><scope>K9.</scope><scope>7X8</scope></search><sort><creationdate>20231201</creationdate><title>Subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: a systematic review of 63 patients reported in the literature</title><author>Ou, Wenxin ; Zhao, Yunze ; Wei, Ang ; Ma, Honghao ; Zhang, Liping ; Lian, Hongyun ; Zhang, Qing ; Wang, Dong ; Li, Zhigang ; Wang, Tianyou ; Zhang, Rui</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c375t-37d6e9e46152fba322c6ff44e0d6049dbbd2fab727297b090a88148d5a734da93</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2023</creationdate><topic>Adrenal Cortex Hormones</topic><topic>Anthracycline</topic><topic>Anthracyclines</topic><topic>Arthritis</topic><topic>Autoantibodies</topic><topic>Autoimmune diseases</topic><topic>Chemotherapy</topic><topic>Clinical Practice</topic><topic>Corticosteroids</topic><topic>Disease</topic><topic>Hematology</topic><topic>Hemophagocytic syndrome</topic><topic>Histiocytosis</topic><topic>Humans</topic><topic>Immunoregulation</topic><topic>Immunosuppressive agents</topic><topic>Internal Medicine</topic><topic>Lymphatic diseases</topic><topic>Lymphocytes T</topic><topic>Lymphocytosis</topic><topic>Lymphohistiocytosis, Hemophagocytic - diagnosis</topic><topic>Lymphohistiocytosis, Hemophagocytic - drug therapy</topic><topic>Lymphohistiocytosis, Hemophagocytic - genetics</topic><topic>Lymphoma</topic><topic>Medical prognosis</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Mutation</topic><topic>Oncology</topic><topic>Panniculitis - complications</topic><topic>Panniculitis - diagnosis</topic><topic>Panniculitis - drug therapy</topic><topic>Patients</topic><topic>Point mutation</topic><topic>Prognosis</topic><topic>Remission</topic><topic>Remission (Medicine)</topic><topic>Stem cell transplantation</topic><topic>Steroids</topic><topic>Systematic review</topic><topic>T-cell lymphoma</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ou, Wenxin</creatorcontrib><creatorcontrib>Zhao, Yunze</creatorcontrib><creatorcontrib>Wei, Ang</creatorcontrib><creatorcontrib>Ma, Honghao</creatorcontrib><creatorcontrib>Zhang, Liping</creatorcontrib><creatorcontrib>Lian, Hongyun</creatorcontrib><creatorcontrib>Zhang, Qing</creatorcontrib><creatorcontrib>Wang, Dong</creatorcontrib><creatorcontrib>Li, Zhigang</creatorcontrib><creatorcontrib>Wang, Tianyou</creatorcontrib><creatorcontrib>Zhang, Rui</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>Neurosciences Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>Clinical and experimental medicine</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ou, Wenxin</au><au>Zhao, Yunze</au><au>Wei, Ang</au><au>Ma, Honghao</au><au>Zhang, Liping</au><au>Lian, Hongyun</au><au>Zhang, Qing</au><au>Wang, Dong</au><au>Li, Zhigang</au><au>Wang, Tianyou</au><au>Zhang, Rui</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: a systematic review of 63 patients reported in the literature</atitle><jtitle>Clinical and experimental medicine</jtitle><stitle>Clin Exp Med</stitle><addtitle>Clin Exp Med</addtitle><date>2023-12-01</date><risdate>2023</risdate><volume>23</volume><issue>8</issue><spage>4575</spage><epage>4583</epage><pages>4575-4583</pages><issn>1591-9528</issn><issn>1591-8890</issn><eissn>1591-9528</eissn><notes>ObjectType-Article-2</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Feature-3</notes><notes>content type line 23</notes><notes>ObjectType-Review-1</notes><abstract>To review and summarize the clinical features, treatment strategies, and prognosis of subcutaneous panniculitis-like T-cell lymphoma complicated with hemophagocytic lymphohistiocytosis (SPTCL–HLH). We searched the Web of Science, Embase, Cochrane Library, and PubMed databases. The keywords were subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis or hemophagocytic syndrome. The patients were divided into a mutated group and a wild-type group based on the existence of
HAVCR2
gene mutation. A total of 45 reports, including 63 patients with SPTCL–HLH, were included in the systematic review. Twelve patients detected gene mutations, including 11 with the
HAVCR2
gene mutation and 1 with the
STXBP2
gene mutation. Thirty-one patients were tested for autoantibodies. Compared with the wild-type group, patients in the mutated group were younger (
p
= 0.017), and the autoantibody-positive rate was higher (
p
= 0.006). The main treatment target of 17 patients was to control HLH, yielding an ORR of 88.2%. Two cases relapsed, and both were treated with corticosteroid monotherapy. The corticosteroid monotherapy experienced a higher recurrence rate than the corticosteroids plus other immunoregulatory agents therapy (66.7 vs. 0.0%,
p
= 0.029). Eighteen patients received initial anthracycline-based chemotherapy, and 50.0% reached remission. The ORR of initial chemotherapy aiming at controlling HLH was higher than those of anthracycline-based chemotherapy (
p
= 0.015). The ORR was higher in patients initially controlled for HLH versus chemotherapy without HLH control first (90.5 vs. 61.5%,
p
= 0.024). Interestingly, one patient with juvenile idiopathic arthritis developed SPTCL–HLH during tocilizumab therapy, discontinuing tocilizumab led to a remission of the disease spontaneously. Sixteen patients received stem cell transplantation (SCT). Fifteen patients, including 5 with relapsed/refractory SPTCL–HLH, responded well and survived after receiving SCT. One case who received a sibling-identical SCT relapsed. Further analysis revealed a homozygous
HAVCR2
mutation with the donor. The 2-year overall survival (OS) was 91.0% ± 4.4%. There was a significant difference in the OS among patients of different age groups, and patients aged 40–60 had the lowest 2-year OS (66.7% ± 19.2%). Patients with
HAVCR2
gene mutations are younger and more likely to be misdiagnosed with autoimmune diseases. Initial treatment of corticosteroids plus immunoregulatory agents attaches great significance to avoiding too aggressive therapies. Intensive anthracycline-based chemotherapy such as CHOP or CHOP-like regimens can also induce long-term remission for aggressive disease. SCT is still a reliable strategy currently. In addition, a watch and wait approach is recommended in patients with mild SPTCL–HLH caused by drugs. The occurrence of HLH does not necessarily mean a more rapidly progressive disease and worse prognosis in patients with SPTCL, but older patients with SPTCL–HLH may be associated with a lower survival rate.</abstract><cop>Cham</cop><pub>Springer International Publishing</pub><pmid>37840116</pmid><doi>10.1007/s10238-023-01210-1</doi><tpages>9</tpages></addata></record> |
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subjects | Adrenal Cortex Hormones Anthracycline Anthracyclines Arthritis Autoantibodies Autoimmune diseases Chemotherapy Clinical Practice Corticosteroids Disease Hematology Hemophagocytic syndrome Histiocytosis Humans Immunoregulation Immunosuppressive agents Internal Medicine Lymphatic diseases Lymphocytes T Lymphocytosis Lymphohistiocytosis, Hemophagocytic - diagnosis Lymphohistiocytosis, Hemophagocytic - drug therapy Lymphohistiocytosis, Hemophagocytic - genetics Lymphoma Medical prognosis Medicine Medicine & Public Health Mutation Oncology Panniculitis - complications Panniculitis - diagnosis Panniculitis - drug therapy Patients Point mutation Prognosis Remission Remission (Medicine) Stem cell transplantation Steroids Systematic review T-cell lymphoma |
title | Subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: a systematic review of 63 patients reported in the literature |
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