Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male
Primary cutaneous gamma–delta T-cell lymphoma (PCGD-TCL) is a very rare subtype of cutaneous T-cell lymphoma. We report the case of a young Polynesian male who presented with fever and an abdominal wall rash and highlight the workup leading to the diagnosis of PCGD-TCL. As PCGD-TCL is rare and mimic...
Saved in:
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2025-01-01
|
| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/crh/8582804 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1849689628485877760 |
|---|---|
| author | Saeed Arabi Victoria Vardell Timothy Hanley Scott Florell Ahmad Halwani Ming Lim |
| author_facet | Saeed Arabi Victoria Vardell Timothy Hanley Scott Florell Ahmad Halwani Ming Lim |
| author_sort | Saeed Arabi |
| collection | DOAJ |
| description | Primary cutaneous gamma–delta T-cell lymphoma (PCGD-TCL) is a very rare subtype of cutaneous T-cell lymphoma. We report the case of a young Polynesian male who presented with fever and an abdominal wall rash and highlight the workup leading to the diagnosis of PCGD-TCL. As PCGD-TCL is rare and mimics other medical conditions, its diagnosis requires a high index of suspicion and can be challenging. Hemophagocytic lymphohistiocytosis (HLH) occurs with PCGD-TCL and can be a marker of more invasive disease. There are no well-defined treatment guidelines, but the most common treatment approach is anthracycline-based multiagent chemotherapy followed by allogeneic stem cell transplant. Targeted therapies are being increasingly used as well. Prognosis remains poor and 5‐year survival is < 20%, particularly in more invasive disease. We highlight how this patient’s demographic varies from the published literature and discuss some unique particulars of the diagnostic evaluation and treatment, especially in the presence of concurrent HLH. |
| format | Article |
| id | doaj-art-904cb835cfb347ecad5cd02101569c7a |
| institution | DOAJ |
| issn | 2090-6579 |
| language | English |
| publishDate | 2025-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-904cb835cfb347ecad5cd02101569c7a2025-08-20T03:21:34ZengWileyCase Reports in Hematology2090-65792025-01-01202510.1155/crh/8582804Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian MaleSaeed Arabi0Victoria Vardell1Timothy Hanley2Scott Florell3Ahmad Halwani4Ming Lim5Department of Internal MedicineDivision of Hematology and Hematologic MalignanciesDivision of HematopathologyDepartment of DermatologyDivision of Hematology and Hematologic MalignanciesDivision of Hematology and Hematologic MalignanciesPrimary cutaneous gamma–delta T-cell lymphoma (PCGD-TCL) is a very rare subtype of cutaneous T-cell lymphoma. We report the case of a young Polynesian male who presented with fever and an abdominal wall rash and highlight the workup leading to the diagnosis of PCGD-TCL. As PCGD-TCL is rare and mimics other medical conditions, its diagnosis requires a high index of suspicion and can be challenging. Hemophagocytic lymphohistiocytosis (HLH) occurs with PCGD-TCL and can be a marker of more invasive disease. There are no well-defined treatment guidelines, but the most common treatment approach is anthracycline-based multiagent chemotherapy followed by allogeneic stem cell transplant. Targeted therapies are being increasingly used as well. Prognosis remains poor and 5‐year survival is < 20%, particularly in more invasive disease. We highlight how this patient’s demographic varies from the published literature and discuss some unique particulars of the diagnostic evaluation and treatment, especially in the presence of concurrent HLH.http://dx.doi.org/10.1155/crh/8582804 |
| spellingShingle | Saeed Arabi Victoria Vardell Timothy Hanley Scott Florell Ahmad Halwani Ming Lim Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male Case Reports in Hematology |
| title | Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male |
| title_full | Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male |
| title_fullStr | Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male |
| title_full_unstemmed | Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male |
| title_short | Primary Cutaneous Gamma–Delta T-Cell Lymphoma Presenting With Hemophagocytic Lymphohistiocytosis in a Young Polynesian Male |
| title_sort | primary cutaneous gamma delta t cell lymphoma presenting with hemophagocytic lymphohistiocytosis in a young polynesian male |
| url | http://dx.doi.org/10.1155/crh/8582804 |
| work_keys_str_mv | AT saeedarabi primarycutaneousgammadeltatcelllymphomapresentingwithhemophagocyticlymphohistiocytosisinayoungpolynesianmale AT victoriavardell primarycutaneousgammadeltatcelllymphomapresentingwithhemophagocyticlymphohistiocytosisinayoungpolynesianmale AT timothyhanley primarycutaneousgammadeltatcelllymphomapresentingwithhemophagocyticlymphohistiocytosisinayoungpolynesianmale AT scottflorell primarycutaneousgammadeltatcelllymphomapresentingwithhemophagocyticlymphohistiocytosisinayoungpolynesianmale AT ahmadhalwani primarycutaneousgammadeltatcelllymphomapresentingwithhemophagocyticlymphohistiocytosisinayoungpolynesianmale AT minglim primarycutaneousgammadeltatcelllymphomapresentingwithhemophagocyticlymphohistiocytosisinayoungpolynesianmale |