O linfoma subcutâneo de células T semelhante à paniculite (SPTCL) é um linfoma cutâneo citotóxico raro que foi reconhecido como um subconjunto distinto de linfomas periféricos de células T originários e apresentando-se principalmente no tecido adiposo subcutâneo.
Introdução
O que você precisa saber de cara
O linfoma subcutâneo de células T semelhante à paniculite (SPTCL) é um linfoma cutâneo citotóxico raro que foi reconhecido como um subconjunto distinto de linfomas periféricos de células T originários e apresentando-se principalmente no tecido adiposo subcutâneo.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 16 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 26 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição. Padrão de herança: Not applicable.
Cell surface receptor implicated in modulating innate and adaptive immune responses. Generally accepted to have an inhibiting function. Reports on stimulating functions suggest that the activity may be influenced by the cellular context and/or the respective ligand (PubMed:24825777). Regulates macrophage activation (PubMed:11823861). Inhibits T-helper type 1 lymphocyte (Th1)-mediated auto- and alloimmune responses and promotes immunological tolerance (PubMed:14556005). In CD8+ cells attenuates T
Cell membraneCell junction
Variantes genéticas (ClinVar)
17 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 7 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
1 via biológica associada aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Linfoma de células T, paniculite subcutânea-like
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Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
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Outros ensaios clínicos
12 ensaios clínicos encontrados, 4 ativos.
Publicações mais relevantes
Clinical characteristics, treatment options, and prognosis of subcutaneous panniculitis-like T-cell lymphoma from the SEER database.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of cutaneous lymphoma lacking standardized treatments. Consequently, patient outcomes vary significantly. This study explored the clinical characteristics and prognostic factors of 205 SPTCL patients from 2000 to 2021 in the Surveillance, Epidemiology, and End Results (SEER) database. Overall survival (OS) at 1, 3, and 5 years were 78.3%, 75.7%, and 66.4%, respectively. Patients diagnosed after 2008 (possibly excluding the γ-δ subtype) (HR = 0.197, 95% CI = 0.106-0.364, p = 0.000) and Asian or Pacific Islanders (HR = 0.210, 95% CI = 0.049-0.902, p = 0.036) were independent predictors of favorable survival, whereas age 50-60 years (HR = 3.213, 95% CI = 1.357-7.607, p = 0.008) and age > 60 years (HR = 5.039, 95% CI = 2.327-10.911, p = 0.000) were independently associated with poor survival. Patients who received radiotherapy alone exhibited a significantly lower hazard risk compared to those receiving no chemotherapy or radiation (HR = 0.216, 95% CI = 0.048-0.983, p = 0.048). No statistically significant differences in prognosis were observed between patients who received no chemotherapy or radiation and those who received either chemotherapy alone (HR = 1.276, 95% CI = 0.644-2.529, p = 0.485) or radiochemotherapy (HR = 1.283, 95% CI = 0.463-3.558, p = 0.632). These associations persisted after IPTW adjustment, with age, race, year of diagnosis, and treatment remaining independent predictors of OS in SPTCL. The Ann Arbor staging was not suitable for SPTCL. Radiotherapy represents an appropriate therapeutic option for patients with single or localized skin lesions. No statistically significant differences in prognosis were observed between patients who received no chemotherapy or radiation and those who received either chemotherapy alone or radiochemotherapy. This finding suggests that immunomodulatory agents may be preferable to cytotoxic therapy as initial treatment for SPTCL, an inflammatory lymphoma.
Histopathologic Spectrum of Primary Cutaneous T-Cell Lymphomas: Insights From a Vietnamese Case Series.
Primary cutaneous T-cell lymphomas (PCTCLs) are a rare and heterogeneous group of extranodal non-Hodgkin lymphomas originating from skin-homing T cells. Most current knowledge about PCTCLs is derived from Western populations, with limited data available from Southeast Asia, including Vietnam. To characterize the clinical, histopathologic, and immunohistochemical features of PCTCLs in a cohort of Vietnamese patients. A retrospective case series was conducted involving 39 PCTCL cases. Demographic and clinical data were collected from medical records and archived clinical images. Histopathologic and immunohistochemical evaluations were independently performed by 3 pathologists, following the fifth edition of the WHO Classification of Haematolymphoid Tumours. Subcutaneous panniculitis-like T-cell lymphoma was most frequent (36%), followed by mycosis fungoides (MF, 28%) and primary cutaneous anaplastic large cell lymphoma (pcALCL, 15%). Subcutaneous panniculitis-like T-cell lymphoma predominantly affected young women, whereas MF and pcALCL occurred in older adults (median age, 64 years). Beyond canonical clinicopathologic features, notable findings included high rates of Pautrier microabscesses and eosinophilic infiltration in MF (each 73%), 1 ulcerated MF case, and 1 pcALCL with a CD4-/CD8- phenotype. Although clinicopathologic features largely aligned with established diagnostic criteria, several differences from international series were observed. These disparities likely reflect delayed diagnosis, referral bias toward more severe disease, and broader systemic and cultural factors in Vietnam, and constrained availability of advanced diagnostic assays, underscoring the need to strengthen clinicopathologic integration and expand access to specialized care.
Subcutaneous Panniculitis-like T-cell Lymphoma with Secondary Haemophagocytic Lymphohistiocytosis Arising at Widespread Tattoo Sites: A Case Report.
Persistent fever and scalp rash in a 2.5-year-old male: A rare case of pediatric subcutaneous panniculitis-like T-Cell lymphoma.
Ciclosporin as upfront therapy in Subcutaneous Panniculitis-like T-cell Lymphoma with aggressive features and in uncomplicated disease: Two case reports and literature review.
Publicações recentes
HAVCR2 in subcutaneous panniculitis like T cell lymphoma.
Clinicopathological Features and Survival Prognosis of Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Population-Based Study from the SEER Database with Supplementary Single-Center Case Validation.
Clinical characteristics, treatment options, and prognosis of subcutaneous panniculitis-like T-cell lymphoma from the SEER database.
Subcutaneous Panniculitis-like T-cell Lymphoma with Secondary Haemophagocytic Lymphohistiocytosis Arising at Widespread Tattoo Sites: A Case Report.
Persistent fever and scalp rash in a 2.5-year-old male: A rare case of pediatric subcutaneous panniculitis-like T-Cell lymphoma.
📚 EuropePMC336 artigos no totalmostrando 198
Clinical characteristics, treatment options, and prognosis of subcutaneous panniculitis-like T-cell lymphoma from the SEER database.
Hematology (Amsterdam, Netherlands)Subcutaneous Panniculitis-like T-cell Lymphoma with Secondary Haemophagocytic Lymphohistiocytosis Arising at Widespread Tattoo Sites: A Case Report.
Acta dermato-venereologicaPersistent fever and scalp rash in a 2.5-year-old male: A rare case of pediatric subcutaneous panniculitis-like T-Cell lymphoma.
JAAD case reportsCiclosporin as upfront therapy in Subcutaneous Panniculitis-like T-cell Lymphoma with aggressive features and in uncomplicated disease: Two case reports and literature review.
Clinical hematology internationalReal-World Insights Into the Clinical Characteristics and Survival Outcomes of Extranodal Peripheral T-Cell Lymphomas: A Multiethnic Asian Cohort Study.
EJHaemP13 The cellulitis that wasn't: a case of subcutaneous panniculitis-like T-cell lymphoma in a toddler.
The British journal of dermatologyUnusual Presentation of Subcutaneous Panniculitis-Like T-Cell Lymphoma.
Clinical case reportsC-MYC expression analysis by immunohistochemistry in cases of subcutaneous panniculitis T-cell lymphoma and lupus panniculitis.
Indian journal of dermatology, venereology and leprologySubcutaneous panniculitis-like T-cell lymphoma in a patient with dermatomyositis and rheumatoid arthritis.
Scandinavian journal of rheumatologySubcutaneous panniculitis-like T-cell lymphoma complicated by hemophagocytic syndrome with significant response to pralatrexate: A case report.
JAAD case reportsHistopathologic Spectrum of Primary Cutaneous T-Cell Lymphomas: Insights From a Vietnamese Case Series.
The American Journal of dermatopathologySubcutaneous Panniculitis-like T-Cell Lymphoma.
The New England journal of medicineSubcutaneous Panniculitis-Like T-Cell Lymphoma in Children: Two Case Reports.
Clinical, cosmetic and investigational dermatologyA Case of Pediatric Subcutaneous Panniculitis-like T-Cell Lymphoma Successfully Treated with Immunosuppressive Therapy.
Children (Basel, Switzerland)Autologous fat grafting to correct severe facial lipoatrophy secondary to subcutaneous panniculitis-like T-cell lymphoma: A case report with 2-year follow-up.
The Journal of international medical researchOrbital atypical lymphocytic panniculitis preceding conjunctival marginal zone lymphoma in the same patient with undifferentiated connective tissue disease: a case-based review.
Rheumatology internationalSubcutaneous panniculitis-like T-cell lymphoma in pregnancy.
BMJ case reportsSubcutaneous panniculitis-like T-cell lymphoma with hemophagocytic lymphohistiocytosis in a psoriasis patient after sequential biologics including extended brodalumab: a case report.
Acta dermatovenerologica Alpina, Pannonica, et AdriaticaSubcutaneous Panniculitis-like T-Cell Lymphoma: Diagnostic Challenge and Successful Multimodal Management with Integra® Dermal Matrix-Case Report and Review of the Literature.
Diseases (Basel, Switzerland)CHOP is insufficient for newly-diagnosed subcutaneous panniculitis-like T-cell lymphoma patients: a retrospective study of 32 patients in China.
BMC cancerSubcutaneous Panniculitis-Like T-Cell Lymphoma in a 2-Year-Old: Case Report and Literature Review.
The American Journal of dermatopathologyPrimary cutaneous lymphoma is a microsatellite stable tumor: An analysis of microsatellite instability.
Drug discoveries & therapeuticsSolitary Subcutaneous Nodular Lymphoid Lesions in Dogs: Histopathologic and Immunophenotypic Comparison of B-Cell Pseudolymphoma and Subcutaneous Panniculitis-like T-Cell Lymphoma.
Veterinary sciencesSubcutaneous Panniculitis-Like T-Cell Lymphoma With Increased γδ T Cells: A Potential Diagnostic Pitfall.
The American Journal of dermatopathologyIndurated Plaques in an Otherwise Healthy Child.
Pediatric dermatologyPralatrexate is effective in cytotoxic cutaneous T-cell lymphomas.
Blood advancesPediatric subcutaneous panniculitis-like T-cell lymphoma: a case report and review of the literature.
Pediatric radiologyClinical characteristics and outcomes among patients with subcutaneous panniculitis-like T-cell lymphoma.
Expert review of anticancer therapyImmune checkpoint inhibitors induced subcutaneous panniculitis-like T-cell lymphoma.
Annales de dermatologie et de venereologieClinical Images: Subcutaneous panniculitis-like T cell lymphoma.
Arthritis & rheumatology (Hoboken, N.J.)Drug- and Vaccine-Induced Cutaneous T-Cell Lymphoma: A Systematic Review of the Literature.
Journal of skin cancerCentral Nervous System Manifestations of Cutaneous Lymphomas.
Current neurology and neuroscience reportsRefractory Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome Treated with Romidepsin and Allogeneic Hematopoietic Cell Transplantation.
Case reports in oncologyOverall and Cause-Specific Mortality Among Patients With Cutaneous T-Cell Lymphoma in the United States.
EJHaemImmunosuppressive therapy as first-line and relapse treatment in paediatric subcutaneous panniculitis-like T-cell lymphoma.
British journal of haematologySubcutaneous panniculitis-like T-cell lymphoma and follicular lymphoma emerging during immune checkpoint inhibitor therapy.
European journal of dermatology : EJDPrimary Cutaneous Methotrexate-Associated T-Cell Lymphoproliferative Disorder in the Setting of Autoimmune Disease: A Case Series and Review of the Literature.
The American Journal of dermatopathologySubcutaneous Infection With Snow Leopard Sign on 18 F-FDG PET/CT Mimicking Subcutaneous Panniculitis-Like T-Cell Lymphoma.
Clinical nuclear medicineSubcutaneous Panniculitis-Like T-Cell Lymphoma Presenting as a Targetoid Plaque in a Pediatric Patient: A Case Report and Review of Literature.
Pediatric dermatologySNMMI PIC case competition finalist: Subcutaneous Panniculitis-Like T-Cell Lymphoma on 18 F-FDG PET/CT.
Clinical nuclear medicineChemo-free salvage treatment outperforms traditional chemotherapy in advanced lines of relapsed/refractory subcutaneous panniculitis-like T-cell lymphoma.
Frontiers in immunologyCharacteristics and therapeutic outcomes of subcutaneous panniculitis-like T-cell lymphoma with and without germline HAVCR2 mutations in Thai children and adolescents.
Orphanet journal of rare diseasesSubcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a 15-Year-old male presenting with orofacial Swelling.
BMC oral healthGenetic profiles and clinical features in subcutaneous panniculitis-like T-cell lymphomas.
Cancer scienceDevelopment of Subcutaneous Panniculitis-Like T-cell Lymphoma After Immune Checkpoint Inhibitor Treatment.
CureusClinical and Histologic Variants of CD8+ Cutaneous T-Cell Lymphomas.
CancersPrevalence, clinical features, and survival outcome trends of 627 patients with primary cutaneous lymphoma over 29 years: a retrospective review from single tertiary center in Korea.
Scientific reportsToward Inclusive Oncology: Challenges in the Therapy of Pediatric Non-B Non-Hodgkin Lymphomas.
In vivo (Athens, Greece)Treatment outcomes between cyclosporin and chemotherapy in adult subcutaneous panniculitis-like T-cell lymphoma: a report from nation-wide Thai lymphoma study group registry.
Annals of hematologyHemophagocytic Lymphohistiocytosis in the Context of Hematological Malignancies and Solid Tumors.
Advances in experimental medicine and biologyClinical analysis of 10 cases with subcutaneous panniculitis-like T-cell lymphoma and tissue AURKA expression.
Skin research and technology : official journal of International Society for Bioengineering and the Skin (ISBS) [and] International Society for Digital Imaging of Skin (ISDIS) [and] International Society for Skin Imaging (ISSI)A Difficult-to-Diagnose Case of Subcutaneous Panniculitis-Like T-Cell Lymphoma with a Fatal Outcome.
Indian dermatology online journalDifferent Mutational Profiles of Subcutaneous Panniculitis-like T-cell Lymphoma and Lupus Panniculitis: An Additional Case Series.
Actas dermo-sifiliograficasSubcutaneous panniculitis-like T-cell lymphoma and lupus erythematosus profundus: a diagnostic dilemma.
BMJ case reportsSubcutaneous Panniculitis-like T Cell Lymphoma Diagnosed with a Slowly Progressive Course: A Case Report.
The Tokai journal of experimental and clinical medicineAn investigation of germline variants of HAVCR2 in subcutaneous panniculitis-like T-cell lymphoma and related lesions in a North American population.
HaematologicaClinicopathological Characteristics and Outcome of Cytophagic Histiocytic Panniculitis: A Single-Center, Retrospective Study.
Clinical, cosmetic and investigational dermatologyRuxolitinib Monotherapy for a Child With HAVCR2 Gene Mutation Associated Subcutaneous Panniculitis-like T-cell Lymphoma: A Case Report.
Journal of pediatric hematology/oncologySubcutaneous Panniculitis-Like T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis.
Journal of investigative medicine high impact case reportsA Novel Subtype of Acquired Generalized Lipodystrophy Associated With Subcutaneous Panniculitis-Like T-cell Lymphoma.
JCEM case reportsCurrent and upcoming treatment approaches to uncommon subtypes of PTCL (EATL, MEITL, SPTCL, and HSTCL).
BloodPaediatric cutaneous lymphomas including rare subtypes: A 40-year experience at a tertiary referral centre.
Journal of the European Academy of Dermatology and Venereology : JEADVCD39 is expressed by a wide range of cutaneous T-cell lymphomas.
Skin health and diseaseSubcutaneous panniculitis-like T-cell lymphoma: fever and facial swelling with hemophagocytic syndrome.
BMJ case reportsMorphea-like subcutaneous panniculitis-like T-cell lymphoma.
JAAD case reportsJAK Inhibitors in Cutaneous T-Cell Lymphoma: Friend or Foe? A Systematic Review of the Published Literature.
CancersSubcutaneous panniculitis-like T-cell lymphoma: a case report.
European journal of dermatology : EJDAn unusual presentation of subcutaneous panniculitis-like T-cell lymphoma: Extensive necrosis and hemophagocytic lymphohistiocytosis: A case report.
Clinical case reportsTherapeutic efficacy of allogenic haematopoietic stem cell transplantation from an appropriate sibling donor to a patient with subcutaneous panniculitis-like T-cell lymphoma with a germline homozygous HAVCR2 mutation.
Journal of the European Academy of Dermatology and Venereology : JEADVSubcutaneous panniculitic-like T-cell lymphoma localized to a site of peginterferon alfa-2a administration.
Leukemia & lymphomaA Case of Subcutaneous Panniculitis-Like T-cell Lymphoma With Hemophagocytic Lymphohistiocytosis in an HIV Patient.
CureusCytophagic Histiocytic Panniculitis Presenting as Subcutaneous Nodules and Generalized Edema - A Case Report.
Clinical, cosmetic and investigational dermatologyCase Report: HAVCR2 mutation-associated Hemophagocytic lymphohistiocytosis.
Frontiers in immunologyBiology and genetics of extranodal mature T-cell and NKcell lymphomas and lymphoproliferative disorders.
HaematologicaFew and far between: clinical management of rare extranodal subtypes of mature T-cell and NK-cell lymphomas.
HaematologicaSubcutaneous panniculitis-like T-cell lymphoma: Morphological, immunophenotypical and clonality assessment in six cats.
Veterinary dermatologyCoexistence of Subcutaneous Panniculitis-Like T-Cell Lymphoma and Dermatomyositis in a 12-Year-Old Boy.
Annals of dermatologySubcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: a systematic review of 63 patients reported in the literature.
Clinical and experimental medicineCase report: Successful treatment of Chidamide in a refractory/recurrent SPTCL with ARID1A mutation on the basis of CHOP plus auto-HSCT.
MedicineIRF8 in Conjunction With CD123 and CD20 to Distinguish Lupus Erythematosus Panniculitis From Subcutaneous Panniculitis-like T-Cell Lymphoma.
The American journal of surgical pathologyPANNICULITIC GAMMA DELTA T-CELL LYMPHOMA WITH CONCOMITANT FEATURES OF AUTOIMMUNE DISEASE EMPHASIZING A PATHOPHYSIOLOGIC CONTINUUM WITH THE PANNICULITIC T CELL LYMPHOMAS.
Clinics in dermatologyPanniculitic primary cutaneous gamma delta T-cell lymphoma with concomitant features of autoimmune disease emphasizing a pathophysiologic continuum of lupus profundus with the panniculitic T cell lymphomas.
Clinics in dermatologyDescriptive Analysis and Factors Influencing Survival in Patients With Primary Cutaneous Gamma-Delta T Cell Lymphoma. A Retrospective National Cancer Database Study.
Clinical lymphoma, myeloma & leukemiaImages in Vascular Medicine: Subcutaneous panniculitis-like T-cell lymphoma mimicking lipodermatosclerosis.
Vascular medicine (London, England)Subcutaneous panniculitis-like T-cell lymphoma in two unrelated individuals with BENTA disease.
Clinical immunology (Orlando, Fla.)Blistering and aggressive paniculite cutaneous T-cell lymphoma.
Boletin medico del Hospital Infantil de MexicoGood prognosis of adult hemophagocytic lymphohistiocytosis associated with the germline HAVCR2 mutation.
Experimental hematologySubcutaneous panniculitis-like T-cell lymphoma presenting as facial infiltration with long-term response to methotrexate: Two cases.
Annales de dermatologie et de venereologieSOHO State of the Art Updates and Next Questions | Challenging Cases in Rare T-Cell Lymphomas.
Clinical lymphoma, myeloma & leukemiaPediatric Primary Cutaneous Lymphoma in China: A Retrospective Study.
Dermatology (Basel, Switzerland)Subcutaneous Panniculitic T-cell Lymphoma Presenting With Anasarca in a Patient With Known Chronic Lymphocytic Leukemia.
Cutis18F-Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Scan Findings in a Rare Case of Subcutaneous Panniculitis-Like T-Cell Lymphoma.
Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, IndiaEfficacy of ruxolitinib for HAVCR2 mutation-associated hemophagocytic lymphohistiocytosis and panniculitis manifestations in children.
British journal of haematologyGermline HAVCR2 mutations and their relation to the clinical spectrum of subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis: results from a multicenter study and meta-analysis.
HaematologicaSubcutaneous panniculitis-like T-cell lymphoma post-mRNA-1273 COVID-19 vaccination.
Clinical case reportsSubcutaneous Panniculitis-like T-cell Lymphoma Lacking Subcutaneous Tumor Mimicking Adult-onset Still's Disease.
Internal medicine (Tokyo, Japan)Diagnosis and management of cutaneous lymphomas and lymphoid proliferations in children, adolescents and young adults (CAYA).
Best practice & research. Clinical haematologyLévy R, Fusaro M, Guerin F, et al. Efficacy of ruxolitinib in subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis. Blood Adv. 2020;4(7):1383-1387.
Blood advancesF-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography in a Case of Subcutaneous T-cell Lymphoma Presenting as Breast Mass and Panniculitis-Like Features: A Rare Entity.
Indian journal of nuclear medicine : IJNM : the official journal of the Society of Nuclear Medicine, IndiaNon-Hodgkin Lymphoma Developed Shortly after mRNA COVID-19 Vaccination: Report of a Case and Review of the Literature.
Medicina (Kaunas, Lithuania)A case report and literature review: subcutaneous panniculitis-like T-cell lymphoma with liver failure as the main manifestation.
Annals of translational medicineSubcutaneous panniculitis-like T-cell lymphoma of the breast in an adolescent female: An uncommon presentation.
Pediatric blood & cancerInverted FDG Uptake Pattern in Subcutaneous Panniculitis-Like T-Cell Lymphoma.
Clinical nuclear medicineRecalcitrant Subcutaneous Panniculitis-Like T-Cell Lymphoma Responsive to Histone Deacetylase Inhibitor.
Case reports in oncologyA retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?
Orphanet journal of rare diseasesSubcutaneous Panniculitis-like T-Cell Lymphoma with a Transformation to Lupus Erythematosus Panniculitis: A Case Report.
Case reports in dermatologySubcutaneous panniculitis-like T-cell lymphoma: an unusual cause of right thigh hypertrophy.
BMJ case reportsA unique case of subcutaneous panniculitis-like T-cell lymphoma presenting as an abscess following an arthropod bite.
Dermatology online journalA novel germline HAVCR2 (TIM-3) compound heterozygous mutation is related to hemophagocytic lymphohistiocytic syndrome in EBV-positive peripheral T-cell lymphoma (NOS) with down-regulated TIM-3 signaling.
Frontiers in oncologyRelapsing subcutaneous panniculitis-like T-cell lymphoma with B symptoms, remitting on low dose methotrexate.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGSubcutaneous Panniculitis-like T-cell Lymphoma with a HAVCR2 Mutation Diagnosed after 10 Years of Treatment with Glucocorticoids and Cyclosporine as Lupus Panniculitis.
Internal medicine (Tokyo, Japan)CD20-positive subcutaneous panniculitis-like T-cell lymphoma presenting as polycranial neuropathy: A CARE-compliant case report and literature review.
MedicineRuxolitinib bridging therapy to allogeneic SCT for high-risk refractory subcutaneous panniculitis-like T-cell lymphoma.
Leukemia & lymphomaClinical and Pathological Characteristics and Outcomes Among Patients With Subcutaneous Panniculitis-like T-Cell Lymphoma and Related Adipotropic Lymphoproliferative Disorders.
JAMA dermatologySubcutaneous panniculitis-like T-cell lymphoma after COVID-19 vaccination.
JAAD case reportsNonhealing Ulcers with Rimming of the Adipose Tissues.
Case reports in dermatologySubcutaneous panniculitis-like T-cell lymphoma associated with methotrexate treatment.
The Journal of dermatologyRole of PET imaging in peritoneal involvement of subcutaneous panniculitis-like T-cell lymphoma.
Journal of radiology case reportsExpression pattern and diagnostic utility of BCL11B in mature T- and NK-cell neoplasms.
PathologyPrimary cutaneous gamma-delta T-cell lymphoma masquerading as leukemia cutis in a patient recently diagnosed with small lymphocytic lymphoma: Clues to the diagnosis.
Journal of cutaneous pathologyKoh J, Jang I, Mun S, et al. Genetic profiles of subcutaneous panniculitis-like T-cell lymphoma and clinicopathological impact of HAVCR2 mutations. Blood Adv. 2021;5(20):3919-3930.
Blood advancesPrimary Cutaneous Gamma-Delta T-Cell Lymphoma Initially Diagnosed as Subcutaneous Panniculitis-like T-Cell Lymphoma with Dermatomyositis.
Dermatopathology (Basel, Switzerland)The pathophysiology and current treatments for the subcutaneous panniculitis-like T cell lymphoma: An updated review.
Asia-Pacific journal of clinical oncologyPrimary cutaneous diffuse large B-Cell lymphoma, leg type mimicking subcutaneous panniculitis-like T-cell lymphoma in a COVID-19 setting: case report and review of literature.
Dermatology online journalSubcutaneous Panniculitis-Like T-Cell Lymphoma Revealed By Immunophenotyping of Necrosis.
The American Journal of dermatopathologyB-cell lymphoma-2 downregulation is a useful feature supporting a neoplastic phenotype in mature T-cell lymphomas.
Human pathologyOrbital Mass as the Only Presenting Sign with Overlapping Features of Lupus Erythematosus Panniculitis and Subcutaneous Panniculitis-Like T-Cell Lymphoma.
Ocular immunology and inflammationCritical Role of Flow Cytometric Immunophenotyping in the Diagnosis, Subtyping, and Staging of T-Cell/NK-Cell Non-Hodgkin's Lymphoma in Real-World Practice: A Study of 232 Cases From a Tertiary Cancer Center in India.
Frontiers in oncologySubcutaneous panniculitis-like T-cell lymphoma in a young girl presenting with periorbital edema and fever: A case report.
Clinical case reportsSubcutaneous panniculitis-like T-cell lymphoma: brief review and report of successful treatment with mycophenolate mofetil.
Clinical and experimental dermatologyLack of PRAME Expression in Cutaneous T-Cell Lymphomas.
Dermatopathology (Basel, Switzerland)[PD-1 monoclonal antibody combined with chemotherapy in the treatment of subcutaneous panniculitis-like T-cell lymphoma: a case report and literature review].
Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhiPrimary cutaneous CD8+ cytotoxic T-cell lymphoma of the face with intraoral involvement, resulting in facial nerve palsy after chemotherapy.
Journal of cutaneous pathologyEyelid erythema as the first manifestation of subcutaneous panniculitis-like T-cell lymphoma mimicking dermatomyositis: a case-based review.
Clinical rheumatologySubcutaneous Panniculitis-Like T-Cell Lymphoma: Clinical Features and Outcomes from a Single Tertiary Center.
Indian journal of hematology & blood transfusion : an official journal of Indian Society of Hematology and Blood TransfusionPediatric Subcutaneous Panniculitis-like T-cell Lymphoma of the Orbit.
Ophthalmic plastic and reconstructive surgeryDiffuse painful erythema on right thigh.
JAAD case reportsSubcutaneous panniculitis-like T cell lymphoma arising in association with chronic lymphocytic leukaemia.
BMJ case reportsGenetic profiles of subcutaneous panniculitis-like T-cell lymphoma and clinicopathological impact of HAVCR2 mutations.
Blood advancesSubcutaneous panniculitis-like T-cell lymphomas with homozygous inheritance of HAVCR2 mutations in Vietnamese pedigrees.
Pediatric blood & cancerSubcutaneous panniculitis-like T-cell lymphoma in a 14-year-old female homozygous for HAVCR2 mutation.
The Australasian journal of dermatologyMolecular features of non-anaplastic peripheral T-cell lymphoma in children and adolescents.
Pediatric blood & cancerReport of Eleven Patients of Subcutaneous Panniculitis-Like T-Cell Lymphoma: Clinicopathologic Features, 18F-FDG PET/CT Findings and Outcome.
Frontiers in oncologySubcutaneous panniculitis-like T-cell lymphoma manifested as a giant plaque on the trunk.
International journal of dermatologyPrimary Cutaneous Lymphomas in Thailand: A 10-Year Retrospective Study.
BioMed research internationalIntrapleural infections in patients with subcutaneous panniculitis-like T-cell lymphoma are susceptible to hemophagocytic lymphohistiocytosis.
Dermatologic therapyA hungry Histiocyte, altered immunity and myriad of problems: Diagnostic challenges for Pediatric HLH.
International journal of laboratory hematologyGamma/Delta Phenotype in Primary Cutaneous T-cell Lymphomas and Lymphoid Proliferations: Challenges for Diagnosis and Classification.
Surgical pathology clinicsDiagnosis and Treatment of Subcutaneous Panniculitis-like T-cell Lymphoma: A Systematic Literature Review.
Hematology/oncology and stem cell therapySubcutaneous panniculitis-like T-cell lymphoma, lupus erythematosus profundus, and overlapping cases: molecular characterization through the study of 208 genes.
Leukemia & lymphomaPrimary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part II: Prognosis and management.
Journal of the American Academy of DermatologyPrimary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part I: Clinical and histologic features and diagnosis.
Journal of the American Academy of DermatologyChidamide induces long-term remission in rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review.
International journal of immunopathology and pharmacologySingle-Cell RNA-seq Reveals Characteristics of Malignant Cells and Immune Microenvironment in Subcutaneous Panniculitis-Like T-Cell Lymphoma.
Frontiers in oncologySubcutaneous panniculitis-like T-cell lymphoma invading central nervous system in long-term clinical remission with lenalidomide: A case report.
World journal of clinical casesSubcutaneous Panniculitis-like T-Cell Lymphoma Without Erythema and Subcutaneous Tumors.
Yonago acta medica[Research Progression of Subcutaneous Panniculitis-like T-Cell Lymphoma--Review].
Zhongguo shi yan xue ye xue za zhiRuxolitinib as adjunctive therapy for secondary hemophagocytic lymphohistiocytosis: A case series.
European journal of haematologyA Rare Case of Subcutaneous Panniculitis-Like T Cell Lymphoma with Hemophagocytic Lymphohistiocytosis Mimicking Cellulitis.
The American journal of case reportsTreatment considerations in case of subcutaneous panniculitis like T cell lymphoma with hemophagocytic syndrome.
Dermatologic therapySubcutaneous Panniculitis-Like T-Cell Lymphoma Presenting with Full Facial Edema and Hemophagocytic Syndrome.
SkinmedA Longstanding, Persistent and Recurrent Case of Cryptogenic Panniculitis.
Case reports in dermatologyHemophagocytic Lymphohistiocytosis as the Initial Presentation of Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Rare Case Responding to Cyclosporine A and Steroids.
Journal of investigative medicine high impact case reportsComplete response to romidepsin as monotherapy in treatment-resistant subcutaneous panniculitis-like T-cell lymphoma.
JAAD case reportsSubcutaneous panniculitis-like T cell lymphoma presented as erythema nodosum: A case report.
Dermatologic therapy[Significance of immune checkpoints in lymphoid malignancies].
[Rinsho ketsueki] The Japanese journal of clinical hematologyFamilial aggregation of subcutaneous panniculitis-like T cell lymphoma: A case report of monozygotic twin brothers.
MedicineSubcutaneous Panniculitis-like T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis Syndrome in Children and Its Essential Role of HAVCR2 Gene Mutation Analysis.
Journal of pediatric hematology/oncologySubcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) - how to treat it?
The Journal of dermatological treatmentUnraveling subcutaneous panniculitis-like T-cell lymphoma: An association between subcutaneous panniculitis-like T-cell lymphoma, autoimmune lymphoproliferative syndrome, and familial hemophagocytic lymphohistiocytosis.
Journal of cutaneous pathologyConcurrent Subcutaneous Panniculitis-like T-Cell Lymphoma and B-Cell Acute Lymphoblastic Leukemia in 2 Pediatric Patients.
Journal of pediatric hematology/oncologyRare non-Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric-type follicular lymphoma, marginal zone lymphoma, and nonanaplastic peripheral T-cell lymphoma.
Pediatric blood & cancerT-cell lymphomas in a tertiary care center of Mangalore.
Journal of cancer research and therapeuticsSecond primary malignancies in subcutaneous panniculitis-like T-cell lymphoma: a national database study.
Clinical and experimental dermatologyTIM-3 deficiency presenting with two clonally unrelated episodes of mesenteric and subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis.
Pediatric blood & cancerEfficacy of ruxolitinib in subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis.
Blood advancesA Case of Atypical Lymphocytic Lobular Panniculitis.
CureusPrimary Cutaneous Gamma-Delta T-Cell Lymphoma With Long-Term Indolent Clinical Course Initially Mimicking Lupus Erythematosus Profundus.
Frontiers in oncologyHAVCR2 mutations are associated with severe hemophagocytic syndrome in subcutaneous panniculitis-like T-cell lymphoma.
BloodNon-mycosis fungoides cutaneous lymphomas in a referral center in Taiwan: A retrospective case series and literature review.
PloS onePositron emission tomography-computed tomography in subcutaneous panniculitis-like T-cell lymphoma.
World journal of nuclear medicineNecrotizing Granulomatous Dermatitis and Panniculitis Masquerading as T Cell Lymphoma.
Skinmed[Clinical Comparative Analysis of Lupus Panniculitis and Subcutaneous Panniculitis-like T-cell Lymphoma].
Sichuan da xue xue bao. Yi xue ban = Journal of Sichuan University. Medical science edition18F-FDG PET/CT-Guided Diagnosis and Evaluation of Treatment Response to Cyclosporine A in Maldistributed Subcutaneous Panniculitis-like T-Cell Lymphoma Without Typical Skin Rash.
Clinical nuclear medicineClinical features of infantile subcutaneous panniculitis-like T-cell lymphoma.
Pediatrics international : official journal of the Japan Pediatric SocietyPralatrexate Induces Long-Term Remission in Relapsed Subcutaneous Panniculitis-Like T-Cell Lymphoma.
Annals of the Academy of Medicine, SingaporeHigh-throughput Sequencing of Subcutaneous Panniculitis-like T-Cell Lymphoma Reveals Candidate Pathogenic Mutations.
Applied immunohistochemistry & molecular morphology : AIMMNot all swellings are lymph nodes! A case of subcutaneous panniculitis-like T-cell lymphoma.
The Medical journal of MalaysiaA retrospective review of cutaneous lymphoma in Botswana.
International journal of dermatologyActivated-cytotoxic TCRαβ+CD4+ peripheral T-cell lymphoma with hypodermal localization: Case report of a lymphoproliferative disorder probably evolved from the CD4+ cytotoxic T-cell subpopulation.
Journal of cutaneous pathologySubcutaneous Panniculitis-Like T Cell Lymphoma: Approach to Differential Diagnosis on Cytology.
Journal of adolescent and young adult oncologyIs cyclosporine a good option for the treatment of subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic syndrome?
Indian journal of dermatology, venereology and leprologyC-C chemokine receptor 4 expression in CD8+ cutaneous T-cell lymphomas and lymphoproliferative disorders, and its implications for diagnosis and treatment.
HistopathologyPralatrexate for refractory or recurrent subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome.
Journal of the American Academy of DermatologyPanniculitis-like T-cell-lymphoma in the mesentery associated with hemophagocytic syndrome: autopsy case report.
Diagnostic pathologyFever with Positive EBV IgM Antibody Combined Multiple Subcutaneous Nodules on Lower Extremities and Cervical Lymphadenopathy Masquerading as Infectious Mononucleosis Proven as Subcutaneous Panniculitis-like T-cell Lymphoma by Subcutaneous Nodule Biopsy Consultation: a Case Report and Literature Review.
Clinical laboratory[Concurrent subcutaneous panniculitis-like T-cell lymphoma and myelodysplastic syndrome with fibrosis].
[Rinsho ketsueki] The Japanese journal of clinical hematologyThe Challenge of Identifying Identical Twins: Advances in Differentiating Lupus Erythematosus Panniculitis from Subcutaneous Panniculitis-Like T-Cell Lymphoma.
SkinmedAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Clinical characteristics, treatment options, and prognosis of subcutaneous panniculitis-like T-cell lymphoma from the SEER database.
- Histopathologic Spectrum of Primary Cutaneous T-Cell Lymphomas: Insights From a Vietnamese Case Series.
- Subcutaneous Panniculitis-like T-cell Lymphoma with Secondary Haemophagocytic Lymphohistiocytosis Arising at Widespread Tattoo Sites: A Case Report.
- Persistent fever and scalp rash in a 2.5-year-old male: A rare case of pediatric subcutaneous panniculitis-like T-Cell lymphoma.
- Ciclosporin as upfront therapy in Subcutaneous Panniculitis-like T-cell Lymphoma with aggressive features and in uncomplicated disease: Two case reports and literature review.
- HAVCR2 in subcutaneous panniculitis like T cell lymphoma.
- Clinicopathological Features and Survival Prognosis of Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Population-Based Study from the SEER Database with Supplementary Single-Center Case Validation.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:86884(Orphanet)
- OMIM OMIM:618398(OMIM)
- MONDO:0019475(MONDO)
- GARD:10193(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q122905262(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
