Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it?
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare, cutaneous lymphoma involving subcutaneous adipose tissue. SPTL is associated in less than 20% with hemophagocytic syndrome (HPS). A 5-year overall survival rate is inferior in patients with SPTL and HPS (46%) as compared with 91% in pa...
| Main Authors: | , , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Taylor & Francis Group
2022-07-01
|
| Series: | Journal of Dermatological Treatment |
| Subjects: | |
| Online Access: | http://dx.doi.org/10.1080/09546634.2020.1809624 |
| _version_ | 1797683555199877120 |
|---|---|
| author | Agnieszka Giza Krystyna Gałązka Marcin Jońca Małgorzata Raźny Dagmara Zimowska-Curyło Mateusz Wilk Sarah Goldman-Mazur Beata Piątkowska-Jakubas Tomasz Sacha |
| author_facet | Agnieszka Giza Krystyna Gałązka Marcin Jońca Małgorzata Raźny Dagmara Zimowska-Curyło Mateusz Wilk Sarah Goldman-Mazur Beata Piątkowska-Jakubas Tomasz Sacha |
| author_sort | Agnieszka Giza |
| collection | DOAJ |
| description | Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare, cutaneous lymphoma involving subcutaneous adipose tissue. SPTL is associated in less than 20% with hemophagocytic syndrome (HPS). A 5-year overall survival rate is inferior in patients with SPTL and HPS (46%) as compared with 91% in patients without HPS. No standardized therapy for SPTCL has yet been established. This is a case of 35-year-old Caucasian man with a one-month history of B symptoms with the suspicion of Still’s disease, at admission with leucopenia, high LDH, ferritin, sIl-R2, and triglycerides levels, hepatosplenomegaly, small right supraclavicular nodule, and irregular thickening of trunk subcutaneous tissue. The abdomen MRI showed generalized thickening of mesentery and colonic mucosa. In the patient, diagnosis of SPTCL was established with secondary HPS. CHOEP chemotherapy and modified HLH 2014 protocol were applied with subsequent high dose chemotherapy (BEAM) supported by autologous stem cells transplantation. Treatment was complicated by pancytopenia and pneumonia. The outcome of the disease treated by intensive protocol seems to be good. |
| first_indexed | 2024-03-12T00:15:55Z |
| format | Article |
| id | doaj.art-5a83d006cf5b426ea3e72d46ff49ee91 |
| institution | Directory Open Access Journal |
| issn | 0954-6634 1471-1753 |
| language | English |
| last_indexed | 2024-03-12T00:15:55Z |
| publishDate | 2022-07-01 |
| publisher | Taylor & Francis Group |
| record_format | Article |
| series | Journal of Dermatological Treatment |
| spelling | doaj.art-5a83d006cf5b426ea3e72d46ff49ee912023-09-15T14:28:49ZengTaylor & Francis GroupJournal of Dermatological Treatment0954-66341471-17532022-07-013352674267610.1080/09546634.2020.18096241809624Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it?Agnieszka Giza0Krystyna Gałązka1Marcin Jońca2Małgorzata Raźny3Dagmara Zimowska-Curyło4Mateusz Wilk5Sarah Goldman-Mazur6Beata Piątkowska-Jakubas7Tomasz Sacha8Department of Hematology, Jagiellonian University Medical CollegeDepartment of Clinical Pathomorphology, Jagiellonian University Medical CollegeDepartment of Hematology, Rydygier’s Specialistic HospitalDepartment of Hematology, Rydygier’s Specialistic HospitalDepartment of Hematology, Jagiellonian University Medical CollegeDepartment of Hematology, Jagiellonian University Medical CollegeDepartment of Hematology, Jagiellonian University Medical CollegeDepartment of Hematology, Jagiellonian University Medical CollegeDepartment of Hematology, Jagiellonian University Medical CollegeSubcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare, cutaneous lymphoma involving subcutaneous adipose tissue. SPTL is associated in less than 20% with hemophagocytic syndrome (HPS). A 5-year overall survival rate is inferior in patients with SPTL and HPS (46%) as compared with 91% in patients without HPS. No standardized therapy for SPTCL has yet been established. This is a case of 35-year-old Caucasian man with a one-month history of B symptoms with the suspicion of Still’s disease, at admission with leucopenia, high LDH, ferritin, sIl-R2, and triglycerides levels, hepatosplenomegaly, small right supraclavicular nodule, and irregular thickening of trunk subcutaneous tissue. The abdomen MRI showed generalized thickening of mesentery and colonic mucosa. In the patient, diagnosis of SPTCL was established with secondary HPS. CHOEP chemotherapy and modified HLH 2014 protocol were applied with subsequent high dose chemotherapy (BEAM) supported by autologous stem cells transplantation. Treatment was complicated by pancytopenia and pneumonia. The outcome of the disease treated by intensive protocol seems to be good.http://dx.doi.org/10.1080/09546634.2020.1809624cutaneous t cell lymphomasubcutaneous panniculitis-like t-cell lymphomahemophagocytic syndromehigh dose chemotherapy |
| spellingShingle | Agnieszka Giza Krystyna Gałązka Marcin Jońca Małgorzata Raźny Dagmara Zimowska-Curyło Mateusz Wilk Sarah Goldman-Mazur Beata Piątkowska-Jakubas Tomasz Sacha Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it? Journal of Dermatological Treatment cutaneous t cell lymphoma subcutaneous panniculitis-like t-cell lymphoma hemophagocytic syndrome high dose chemotherapy |
| title | Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it? |
| title_full | Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it? |
| title_fullStr | Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it? |
| title_full_unstemmed | Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it? |
| title_short | Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) – how to treat it? |
| title_sort | subcutaneous panniculitis like t cell lymphoma sptcl with probable mesentery involvement with associated hemophagocytic syndrome hps how to treat it |
| topic | cutaneous t cell lymphoma subcutaneous panniculitis-like t-cell lymphoma hemophagocytic syndrome high dose chemotherapy |
| url | http://dx.doi.org/10.1080/09546634.2020.1809624 |
| work_keys_str_mv | AT agnieszkagiza subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit AT krystynagałazka subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit AT marcinjonca subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit AT małgorzatarazny subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit AT dagmarazimowskacuryło subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit AT mateuszwilk subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit AT sarahgoldmanmazur subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit AT beatapiatkowskajakubas subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit AT tomaszsacha subcutaneouspanniculitisliketcelllymphomasptclwithprobablemesenteryinvolvementwithassociatedhemophagocyticsyndromehpshowtotreatit |