Raras
Buscar doenças, sintomas, genes...
Linfoma de células T, paniculite subcutânea-like
ORPHA:86884CID-10 · C86.3CID-11 · 2B00OMIM 618398DOENÇA RARA

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.

Mantido por Agente Raras·Colaborar como especialista →

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.

Pesquisas ativas
4 ensaios
12 total registrados no ClinicalTrials.gov
Publicações científicas
513 artigos
Último publicado: 2026 Apr 9

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Worldwide
Início
All ages
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C86.3
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🩸
Sangue
3 sintomas
🫃
Digestivo
2 sintomas
🧬
Pele e cabelo
2 sintomas
📏
Crescimento
1 sintomas
🛡️
Imunológico
1 sintomas
😀
Face
1 sintomas

+ 16 sintomas em outras categorias

Características mais comuns

100%prev.
Paniculite
Muito frequente (99-80%)
100%prev.
Linfoma de células T tipo paniculite subcutânea
Frequência: 16/16
90%prev.
Pápula eritematosa
Muito frequente (99-80%)
55%prev.
Placa eritematosa
Frequente (79-30%)
55%prev.
Hepatoesplenomegalia
Frequente (79-30%)
55%prev.
Febre
Frequente (79-30%)
26sintomas
Muito frequente (3)
Frequente (9)
Ocasional (4)
Muito raro (6)
Sem dados (4)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 26 características clínicas mais associadas, ordenadas por frequência.

PaniculitePanniculitis
Muito frequente (99-80%)100%
Linfoma de células T tipo paniculite subcutâneaSubcutaneous panniculitis-like T-cell lymphoma
Frequência: 16/16100%
Pápula eritematosaErythematous papule
Muito frequente (99-80%)90%
Placa eritematosaErythematous plaque
Frequente (79-30%)55%
HepatoesplenomegaliaHepatosplenomegaly
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico513PubMed
Últimos 10 anos200publicações
Pico202537 papers
Linha do tempo
2026Hoje · 2026🧪 2006Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

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.

HAVCR2Hepatitis A virus cellular receptor 2Disease-causing germline mutation(s) inTolerante
FUNÇÃO

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

LOCALIZAÇÃO

Cell membraneCell junction

VIAS BIOLÓGICAS (1)
Interleukin-2 family signaling
EXPRESSÃO TECIDUAL(Ubíquo)
Pulmão
25.9 TPM
Baço
24.8 TPM
Sangue
18.0 TPM
Brain Spinal cord cervical c-1
14.4 TPM
Rim - Córtex
11.5 TPM
OUTRAS DOENÇAS (1)
subcutaneous panniculitis-like T-cell lymphoma
HGNC:18437UniProt:Q8TDQ0

Variantes genéticas (ClinVar)

17 variantes patogênicas registradas no ClinVar.

🧬 HAVCR2: GRCh37/hg19 5q33.2-34(chr5:154849998-164037131)x1 ()
🧬 HAVCR2: NM_032782.5(HAVCR2):c.833A>G (p.Asn278Ser) ()
🧬 HAVCR2: NM_032782.5(HAVCR2):c.319A>T (p.Ile107Phe) ()
🧬 HAVCR2: NC_000005.9:g.(?_155338082)_(156899968_?)del ()
🧬 HAVCR2: GRCh37/hg19 5q33.1-35.2(chr5:150535183-172906793)x3 ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 7 variantes classificadas pelo ClinVar.

2
3
2
Patogênica (28.6%)
VUS (42.9%)
Benigna (28.6%)
VARIANTES MAIS SIGNIFICATIVAS
HAVCR2: NM_032782.5(HAVCR2):c.291A>G (p.Ile97Met) [Conflicting classifications of pathogenicity]
HAVCR2: NM_032782.5(HAVCR2):c.245A>G (p.Tyr82Cys) [Conflicting classifications of pathogenicity]
HAVCR2: NM_032782.5(HAVCR2):c.395-2A>T [Uncertain significance]
HAVCR2: NM_032782.5(HAVCR2):c.523-1G>A [Uncertain significance]
HAVCR2: NM_032782.5(HAVCR2):c.332G>A (p.Arg111Gln) [Uncertain significance]

Vias biológicas (Reactome)

1 via biológica associada aos genes desta condição.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
Aprovado1
2Fase 26
1Fase 12
·Pré-clínico3
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 12 ensaios
Carregando informações de tratamento...

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

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

2 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

Outros ensaios clínicos

12 ensaios clínicos encontrados, 4 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
297 papers (10 anos)
#1

Clinical characteristics, treatment options, and prognosis of subcutaneous panniculitis-like T-cell lymphoma from the SEER database.

Hematology (Amsterdam, Netherlands)2026 Dec 31

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.

#2

Histopathologic Spectrum of Primary Cutaneous T-Cell Lymphomas: Insights From a Vietnamese Case Series.

The American Journal of dermatopathology2026 Jan 01

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.

#3

Subcutaneous Panniculitis-like T-cell Lymphoma with Secondary Haemophagocytic Lymphohistiocytosis Arising at Widespread Tattoo Sites: A Case Report.

Acta dermato-venereologica2026 Feb 03
#4

Persistent fever and scalp rash in a 2.5-year-old male: A rare case of pediatric subcutaneous panniculitis-like T-Cell lymphoma.

JAAD case reports2026 Feb
#5

Ciclosporin 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 international2026

Publicações recentes

Ver todas no PubMed

📚 EuropePMC336 artigos no totalmostrando 198

2026

Clinical characteristics, treatment options, and prognosis of subcutaneous panniculitis-like T-cell lymphoma from the SEER database.

Hematology (Amsterdam, Netherlands)
2026

Subcutaneous Panniculitis-like T-cell Lymphoma with Secondary Haemophagocytic Lymphohistiocytosis Arising at Widespread Tattoo Sites: A Case Report.

Acta dermato-venereologica
2026

Persistent fever and scalp rash in a 2.5-year-old male: A rare case of pediatric subcutaneous panniculitis-like T-Cell lymphoma.

JAAD case reports
2026

Ciclosporin 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 international
2025

Real-World Insights Into the Clinical Characteristics and Survival Outcomes of Extranodal Peripheral T-Cell Lymphomas: A Multiethnic Asian Cohort Study.

EJHaem
2025

P13 The cellulitis that wasn't: a case of subcutaneous panniculitis-like T-cell lymphoma in a toddler.

The British journal of dermatology
2025

Unusual Presentation of Subcutaneous Panniculitis-Like T-Cell Lymphoma.

Clinical case reports
2025

C-MYC expression analysis by immunohistochemistry in cases of subcutaneous panniculitis T-cell lymphoma and lupus panniculitis.

Indian journal of dermatology, venereology and leprology
2025

Subcutaneous panniculitis-like T-cell lymphoma in a patient with dermatomyositis and rheumatoid arthritis.

Scandinavian journal of rheumatology
2025

Subcutaneous panniculitis-like T-cell lymphoma complicated by hemophagocytic syndrome with significant response to pralatrexate: A case report.

JAAD case reports
2026

Histopathologic Spectrum of Primary Cutaneous T-Cell Lymphomas: Insights From a Vietnamese Case Series.

The American Journal of dermatopathology
2025

Subcutaneous Panniculitis-like T-Cell Lymphoma.

The New England journal of medicine
2025

Subcutaneous Panniculitis-Like T-Cell Lymphoma in Children: Two Case Reports.

Clinical, cosmetic and investigational dermatology
2025

A Case of Pediatric Subcutaneous Panniculitis-like T-Cell Lymphoma Successfully Treated with Immunosuppressive Therapy.

Children (Basel, Switzerland)
2025

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 research
2025

Orbital atypical lymphocytic panniculitis preceding conjunctival marginal zone lymphoma in the same patient with undifferentiated connective tissue disease: a case-based review.

Rheumatology international
2025

Subcutaneous panniculitis-like T-cell lymphoma in pregnancy.

BMJ case reports
2025

Subcutaneous 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 Adriatica
2025

Subcutaneous 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)
2025

CHOP is insufficient for newly-diagnosed subcutaneous panniculitis-like T-cell lymphoma patients: a retrospective study of 32 patients in China.

BMC cancer
2025

Subcutaneous Panniculitis-Like T-Cell Lymphoma in a 2-Year-Old: Case Report and Literature Review.

The American Journal of dermatopathology
2025

Primary cutaneous lymphoma is a microsatellite stable tumor: An analysis of microsatellite instability.

Drug discoveries &amp; therapeutics
2025

Solitary Subcutaneous Nodular Lymphoid Lesions in Dogs: Histopathologic and Immunophenotypic Comparison of B-Cell Pseudolymphoma and Subcutaneous Panniculitis-like T-Cell Lymphoma.

Veterinary sciences
2025

Subcutaneous Panniculitis-Like T-Cell Lymphoma With Increased γδ T Cells: A Potential Diagnostic Pitfall.

The American Journal of dermatopathology
2025

Indurated Plaques in an Otherwise Healthy Child.

Pediatric dermatology
2025

Pralatrexate is effective in cytotoxic cutaneous T-cell lymphomas.

Blood advances
2025

Pediatric subcutaneous panniculitis-like T-cell lymphoma: a case report and review of the literature.

Pediatric radiology
2025

Clinical characteristics and outcomes among patients with subcutaneous panniculitis-like T-cell lymphoma.

Expert review of anticancer therapy
2025

Immune checkpoint inhibitors induced subcutaneous panniculitis-like T-cell lymphoma.

Annales de dermatologie et de venereologie
2025

Clinical Images: Subcutaneous panniculitis-like T cell lymphoma.

Arthritis &amp; rheumatology (Hoboken, N.J.)
2025

Drug- and Vaccine-Induced Cutaneous T-Cell Lymphoma: A Systematic Review of the Literature.

Journal of skin cancer
2025

Central Nervous System Manifestations of Cutaneous Lymphomas.

Current neurology and neuroscience reports
2025

Refractory Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome Treated with Romidepsin and Allogeneic Hematopoietic Cell Transplantation.

Case reports in oncology
2025

Overall and Cause-Specific Mortality Among Patients With Cutaneous T-Cell Lymphoma in the United States.

EJHaem
2025

Immunosuppressive therapy as first-line and relapse treatment in paediatric subcutaneous panniculitis-like T-cell lymphoma.

British journal of haematology
2024

Subcutaneous panniculitis-like T-cell lymphoma and follicular lymphoma emerging during immune checkpoint inhibitor therapy.

European journal of dermatology : EJD
2025

Primary 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 dermatopathology
2025

Subcutaneous Infection With Snow Leopard Sign on 18 F-FDG PET/CT Mimicking Subcutaneous Panniculitis-Like T-Cell Lymphoma.

Clinical nuclear medicine
2025

Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting as a Targetoid Plaque in a Pediatric Patient: A Case Report and Review of Literature.

Pediatric dermatology
2025

SNMMI PIC case competition finalist: Subcutaneous Panniculitis-Like T-Cell Lymphoma on 18 F-FDG PET/CT.

Clinical nuclear medicine
2024

Chemo-free salvage treatment outperforms traditional chemotherapy in advanced lines of relapsed/refractory subcutaneous panniculitis-like T-cell lymphoma.

Frontiers in immunology
2024

Characteristics 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 diseases
2024

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) in a 15-Year-old male presenting with orofacial Swelling.

BMC oral health
2024

Genetic profiles and clinical features in subcutaneous panniculitis-like T-cell lymphomas.

Cancer science
2024

Development of Subcutaneous Panniculitis-Like T-cell Lymphoma After Immune Checkpoint Inhibitor Treatment.

Cureus
2024

Clinical and Histologic Variants of CD8+ Cutaneous T-Cell Lymphomas.

Cancers
2024

Prevalence, 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 reports
2024

Toward Inclusive Oncology: Challenges in the Therapy of Pediatric Non-B Non-Hodgkin Lymphomas.

In vivo (Athens, Greece)
2024

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 hematology
2024

Hemophagocytic Lymphohistiocytosis in the Context of Hematological Malignancies and Solid Tumors.

Advances in experimental medicine and biology
2024

Clinical 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)
2024

A Difficult-to-Diagnose Case of Subcutaneous Panniculitis-Like T-Cell Lymphoma with a Fatal Outcome.

Indian dermatology online journal
2025

Different Mutational Profiles of Subcutaneous Panniculitis-like T-cell Lymphoma and Lupus Panniculitis: An Additional Case Series.

Actas dermo-sifiliograficas
2024

Subcutaneous panniculitis-like T-cell lymphoma and lupus erythematosus profundus: a diagnostic dilemma.

BMJ case reports
2024

Subcutaneous Panniculitis-like T Cell Lymphoma Diagnosed with a Slowly Progressive Course: A Case Report.

The Tokai journal of experimental and clinical medicine
2024

An investigation of germline variants of HAVCR2 in subcutaneous panniculitis-like T-cell lymphoma and related lesions in a North American population.

Haematologica
2024

Clinicopathological Characteristics and Outcome of Cytophagic Histiocytic Panniculitis: A Single-Center, Retrospective Study.

Clinical, cosmetic and investigational dermatology
2024

Ruxolitinib Monotherapy for a Child With HAVCR2 Gene Mutation Associated Subcutaneous Panniculitis-like T-cell Lymphoma: A Case Report.

Journal of pediatric hematology/oncology
2024

Subcutaneous Panniculitis-Like T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis.

Journal of investigative medicine high impact case reports
2024

A Novel Subtype of Acquired Generalized Lipodystrophy Associated With Subcutaneous Panniculitis-Like T-cell Lymphoma.

JCEM case reports
2024

Current and upcoming treatment approaches to uncommon subtypes of PTCL (EATL, MEITL, SPTCL, and HSTCL).

Blood
2025

Paediatric cutaneous lymphomas including rare subtypes: A 40-year experience at a tertiary referral centre.

Journal of the European Academy of Dermatology and Venereology : JEADV
2024

CD39 is expressed by a wide range of cutaneous T-cell lymphomas.

Skin health and disease
2024

Subcutaneous panniculitis-like T-cell lymphoma: fever and facial swelling with hemophagocytic syndrome.

BMJ case reports
2024

Morphea-like subcutaneous panniculitis-like T-cell lymphoma.

JAAD case reports
2024

JAK Inhibitors in Cutaneous T-Cell Lymphoma: Friend or Foe? A Systematic Review of the Published Literature.

Cancers
2023

Subcutaneous panniculitis-like T-cell lymphoma: a case report.

European journal of dermatology : EJD
2024

An unusual presentation of subcutaneous panniculitis-like T-cell lymphoma: Extensive necrosis and hemophagocytic lymphohistiocytosis: A case report.

Clinical case reports
2024

Therapeutic 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 : JEADV
2024

Subcutaneous panniculitic-like T-cell lymphoma localized to a site of peginterferon alfa-2a administration.

Leukemia &amp; lymphoma
2023

A Case of Subcutaneous Panniculitis-Like T-cell Lymphoma With Hemophagocytic Lymphohistiocytosis in an HIV Patient.

Cureus
2023

Cytophagic Histiocytic Panniculitis Presenting as Subcutaneous Nodules and Generalized Edema - A Case Report.

Clinical, cosmetic and investigational dermatology
2023

Case Report: HAVCR2 mutation-associated Hemophagocytic lymphohistiocytosis.

Frontiers in immunology
2023

Biology and genetics of extranodal mature T-cell and NKcell lymphomas and lymphoproliferative disorders.

Haematologica
2023

Few and far between: clinical management of rare extranodal subtypes of mature T-cell and NK-cell lymphomas.

Haematologica
2024

Subcutaneous panniculitis-like T-cell lymphoma: Morphological, immunophenotypical and clonality assessment in six cats.

Veterinary dermatology
2023

Coexistence of Subcutaneous Panniculitis-Like T-Cell Lymphoma and Dermatomyositis in a 12-Year-Old Boy.

Annals of dermatology
2023

Subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic lymphohistiocytosis: a systematic review of 63 patients reported in the literature.

Clinical and experimental medicine
2023

Case report: Successful treatment of Chidamide in a refractory/recurrent SPTCL with ARID1A mutation on the basis of CHOP plus auto-HSCT.

Medicine
2023

IRF8 in Conjunction With CD123 and CD20 to Distinguish Lupus Erythematosus Panniculitis From Subcutaneous Panniculitis-like T-Cell Lymphoma.

The American journal of surgical pathology
2023

PANNICULITIC GAMMA DELTA T-CELL LYMPHOMA WITH CONCOMITANT FEATURES OF AUTOIMMUNE DISEASE EMPHASIZING A PATHOPHYSIOLOGIC CONTINUUM WITH THE PANNICULITIC T CELL LYMPHOMAS.

Clinics in dermatology
2023

Panniculitic 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 dermatology
2023

Descriptive Analysis and Factors Influencing Survival in Patients With Primary Cutaneous Gamma-Delta T Cell Lymphoma. A Retrospective National Cancer Database Study.

Clinical lymphoma, myeloma &amp; leukemia
2023

Images in Vascular Medicine: Subcutaneous panniculitis-like T-cell lymphoma mimicking lipodermatosclerosis.

Vascular medicine (London, England)
2023

Subcutaneous panniculitis-like T-cell lymphoma in two unrelated individuals with BENTA disease.

Clinical immunology (Orlando, Fla.)
2023

Blistering and aggressive paniculite cutaneous T-cell lymphoma.

Boletin medico del Hospital Infantil de Mexico
2023

Good prognosis of adult hemophagocytic lymphohistiocytosis associated with the germline HAVCR2 mutation.

Experimental hematology
2023

Subcutaneous panniculitis-like T-cell lymphoma presenting as facial infiltration with long-term response to methotrexate: Two cases.

Annales de dermatologie et de venereologie
2023

SOHO State of the Art Updates and Next Questions | Challenging Cases in Rare T-Cell Lymphomas.

Clinical lymphoma, myeloma &amp; leukemia
2023

Pediatric Primary Cutaneous Lymphoma in China: A Retrospective Study.

Dermatology (Basel, Switzerland)
2023

Subcutaneous Panniculitic T-cell Lymphoma Presenting With Anasarca in a Patient With Known Chronic Lymphocytic Leukemia.

Cutis
2023

18F-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, India
2023

Efficacy of ruxolitinib for HAVCR2 mutation-associated hemophagocytic lymphohistiocytosis and panniculitis manifestations in children.

British journal of haematology
2023

Germline 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.

Haematologica
2023

Subcutaneous panniculitis-like T-cell lymphoma post-mRNA-1273 COVID-19 vaccination.

Clinical case reports
2023

Subcutaneous Panniculitis-like T-cell Lymphoma Lacking Subcutaneous Tumor Mimicking Adult-onset Still's Disease.

Internal medicine (Tokyo, Japan)
2023

Diagnosis and management of cutaneous lymphomas and lymphoid proliferations in children, adolescents and young adults (CAYA).

Best practice &amp; research. Clinical haematology
2023

Lévy 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 advances
2022

F-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, India
2023

Non-Hodgkin Lymphoma Developed Shortly after mRNA COVID-19 Vaccination: Report of a Case and Review of the Literature.

Medicina (Kaunas, Lithuania)
2022

A case report and literature review: subcutaneous panniculitis-like T-cell lymphoma with liver failure as the main manifestation.

Annals of translational medicine
2023

Subcutaneous panniculitis-like T-cell lymphoma of the breast in an adolescent female: An uncommon presentation.

Pediatric blood &amp; cancer
2023

Inverted FDG Uptake Pattern in Subcutaneous Panniculitis-Like T-Cell Lymphoma.

Clinical nuclear medicine
2022

Recalcitrant Subcutaneous Panniculitis-Like T-Cell Lymphoma Responsive to Histone Deacetylase Inhibitor.

Case reports in oncology
2022

A retrospective study of 18 children with subcutaneous panniculitis-like T-cell lymphoma: multidrug combination chemotherapy or immunomodulatory therapy?

Orphanet journal of rare diseases
2022

Subcutaneous Panniculitis-like T-Cell Lymphoma with a Transformation to Lupus Erythematosus Panniculitis: A Case Report.

Case reports in dermatology
2022

Subcutaneous panniculitis-like T-cell lymphoma: an unusual cause of right thigh hypertrophy.

BMJ case reports
2022

A unique case of subcutaneous panniculitis-like T-cell lymphoma presenting as an abscess following an arthropod bite.

Dermatology online journal
2022

A 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 oncology
2022

Relapsing 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 : JDDG
2023

Subcutaneous 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)
2022

CD20-positive subcutaneous panniculitis-like T-cell lymphoma presenting as polycranial neuropathy: A CARE-compliant case report and literature review.

Medicine
2022

Ruxolitinib bridging therapy to allogeneic SCT for high-risk refractory subcutaneous panniculitis-like T-cell lymphoma.

Leukemia &amp; lymphoma
2022

Clinical and Pathological Characteristics and Outcomes Among Patients With Subcutaneous Panniculitis-like T-Cell Lymphoma and Related Adipotropic Lymphoproliferative Disorders.

JAMA dermatology
2022

Subcutaneous panniculitis-like T-cell lymphoma after COVID-19 vaccination.

JAAD case reports
2022

Nonhealing Ulcers with Rimming of the Adipose Tissues.

Case reports in dermatology
2022

Subcutaneous panniculitis-like T-cell lymphoma associated with methotrexate treatment.

The Journal of dermatology
2022

Role of PET imaging in peritoneal involvement of subcutaneous panniculitis-like T-cell lymphoma.

Journal of radiology case reports
2022

Expression pattern and diagnostic utility of BCL11B in mature T- and NK-cell neoplasms.

Pathology
2022

Primary 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 pathology
2022

Koh 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 advances
2022

Primary Cutaneous Gamma-Delta T-Cell Lymphoma Initially Diagnosed as Subcutaneous Panniculitis-like T-Cell Lymphoma with Dermatomyositis.

Dermatopathology (Basel, Switzerland)
2023

The pathophysiology and current treatments for the subcutaneous panniculitis-like T cell lymphoma: An updated review.

Asia-Pacific journal of clinical oncology
2021

Primary 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 journal
2022

Subcutaneous Panniculitis-Like T-Cell Lymphoma Revealed By Immunophenotyping of Necrosis.

The American Journal of dermatopathology
2022

B-cell lymphoma-2 downregulation is a useful feature supporting a neoplastic phenotype in mature T-cell lymphomas.

Human pathology
2023

Orbital Mass as the Only Presenting Sign with Overlapping Features of Lupus Erythematosus Panniculitis and Subcutaneous Panniculitis-Like T-Cell Lymphoma.

Ocular immunology and inflammation
2022

Critical 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 oncology
2022

Subcutaneous panniculitis-like T-cell lymphoma in a young girl presenting with periorbital edema and fever: A case report.

Clinical case reports
2022

Subcutaneous panniculitis-like T-cell lymphoma: brief review and report of successful treatment with mycophenolate mofetil.

Clinical and experimental dermatology
2021

Lack of PRAME Expression in Cutaneous T-Cell Lymphomas.

Dermatopathology (Basel, Switzerland)
2021

[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 zazhi
2022

Primary cutaneous CD8+ cytotoxic T-cell lymphoma of the face with intraoral involvement, resulting in facial nerve palsy after chemotherapy.

Journal of cutaneous pathology
2022

Eyelid erythema as the first manifestation of subcutaneous panniculitis-like T-cell lymphoma mimicking dermatomyositis: a case-based review.

Clinical rheumatology
2021

Subcutaneous Panniculitis-Like T-Cell Lymphoma: Clinical Features and Outcomes from a Single Tertiary Center.

Indian journal of hematology &amp; blood transfusion : an official journal of Indian Society of Hematology and Blood Transfusion
2022

Pediatric Subcutaneous Panniculitis-like T-cell Lymphoma of the Orbit.

Ophthalmic plastic and reconstructive surgery
2021

Diffuse painful erythema on right thigh.

JAAD case reports
2021

Subcutaneous panniculitis-like T cell lymphoma arising in association with chronic lymphocytic leukaemia.

BMJ case reports
2021

Genetic profiles of subcutaneous panniculitis-like T-cell lymphoma and clinicopathological impact of HAVCR2 mutations.

Blood advances
2021

Subcutaneous panniculitis-like T-cell lymphomas with homozygous inheritance of HAVCR2 mutations in Vietnamese pedigrees.

Pediatric blood &amp; cancer
2021

Subcutaneous panniculitis-like T-cell lymphoma in a 14-year-old female homozygous for HAVCR2 mutation.

The Australasian journal of dermatology
2021

Molecular features of non-anaplastic peripheral T-cell lymphoma in children and adolescents.

Pediatric blood &amp; cancer
2021

Report of Eleven Patients of Subcutaneous Panniculitis-Like T-Cell Lymphoma: Clinicopathologic Features, 18F-FDG PET/CT Findings and Outcome.

Frontiers in oncology
2021

Subcutaneous panniculitis-like T-cell lymphoma manifested as a giant plaque on the trunk.

International journal of dermatology
2021

Primary Cutaneous Lymphomas in Thailand: A 10-Year Retrospective Study.

BioMed research international
2021

Intrapleural infections in patients with subcutaneous panniculitis-like T-cell lymphoma are susceptible to hemophagocytic lymphohistiocytosis.

Dermatologic therapy
2021

A hungry Histiocyte, altered immunity and myriad of problems: Diagnostic challenges for Pediatric HLH.

International journal of laboratory hematology
2021

Gamma/Delta Phenotype in Primary Cutaneous T-cell Lymphomas and Lymphoid Proliferations: Challenges for Diagnosis and Classification.

Surgical pathology clinics
2023

Diagnosis and Treatment of Subcutaneous Panniculitis-like T-cell Lymphoma: A Systematic Literature Review.

Hematology/oncology and stem cell therapy
2021

Subcutaneous panniculitis-like T-cell lymphoma, lupus erythematosus profundus, and overlapping cases: molecular characterization through the study of 208 genes.

Leukemia &amp; lymphoma
2021

Primary cutaneous T-cell lymphomas other than mycosis fungoides and Sézary syndrome. Part II: Prognosis and management.

Journal of the American Academy of Dermatology
2021

Primary 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 Dermatology
2021

Chidamide induces long-term remission in rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review.

International journal of immunopathology and pharmacology
2021

Single-Cell RNA-seq Reveals Characteristics of Malignant Cells and Immune Microenvironment in Subcutaneous Panniculitis-Like T-Cell Lymphoma.

Frontiers in oncology
2021

Subcutaneous panniculitis-like T-cell lymphoma invading central nervous system in long-term clinical remission with lenalidomide: A case report.

World journal of clinical cases
2021

Subcutaneous Panniculitis-like T-Cell Lymphoma Without Erythema and Subcutaneous Tumors.

Yonago acta medica
2021

[Research Progression of Subcutaneous Panniculitis-like T-Cell Lymphoma--Review].

Zhongguo shi yan xue ye xue za zhi
2021

Ruxolitinib as adjunctive therapy for secondary hemophagocytic lymphohistiocytosis: A case series.

European journal of haematology
2021

A Rare Case of Subcutaneous Panniculitis-Like T Cell Lymphoma with Hemophagocytic Lymphohistiocytosis Mimicking Cellulitis.

The American journal of case reports
2021

Treatment considerations in case of subcutaneous panniculitis like T cell lymphoma with hemophagocytic syndrome.

Dermatologic therapy
2020

Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting with Full Facial Edema and Hemophagocytic Syndrome.

Skinmed
2020

A Longstanding, Persistent and Recurrent Case of Cryptogenic Panniculitis.

Case reports in dermatology
2020

Hemophagocytic 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 reports
2020

Complete response to romidepsin as monotherapy in treatment-resistant subcutaneous panniculitis-like T-cell lymphoma.

JAAD case reports
2021

Subcutaneous panniculitis-like T cell lymphoma presented as erythema nodosum: A case report.

Dermatologic therapy
2020

[Significance of immune checkpoints in lymphoid malignancies].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2020

Familial aggregation of subcutaneous panniculitis-like T cell lymphoma: A case report of monozygotic twin brothers.

Medicine
2021

Subcutaneous Panniculitis-like T-Cell Lymphoma With Hemophagocytic Lymphohistiocytosis Syndrome in Children and Its Essential Role of HAVCR2 Gene Mutation Analysis.

Journal of pediatric hematology/oncology
2022

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with probable mesentery involvement with associated hemophagocytic syndrome (HPS) - how to treat it?

The Journal of dermatological treatment
2021

Unraveling 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 pathology
2021

Concurrent Subcutaneous Panniculitis-like T-Cell Lymphoma and B-Cell Acute Lymphoblastic Leukemia in 2 Pediatric Patients.

Journal of pediatric hematology/oncology
2020

Rare 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 &amp; cancer
2020

T-cell lymphomas in a tertiary care center of Mangalore.

Journal of cancer research and therapeutics
2020

Second primary malignancies in subcutaneous panniculitis-like T-cell lymphoma: a national database study.

Clinical and experimental dermatology
2020

TIM-3 deficiency presenting with two clonally unrelated episodes of mesenteric and subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis.

Pediatric blood &amp; cancer
2020

Efficacy of ruxolitinib in subcutaneous panniculitis-like T-cell lymphoma and hemophagocytic lymphohistiocytosis.

Blood advances
2020

A Case of Atypical Lymphocytic Lobular Panniculitis.

Cureus
2020

Primary Cutaneous Gamma-Delta T-Cell Lymphoma With Long-Term Indolent Clinical Course Initially Mimicking Lupus Erythematosus Profundus.

Frontiers in oncology
2020

HAVCR2 mutations are associated with severe hemophagocytic syndrome in subcutaneous panniculitis-like T-cell lymphoma.

Blood
2020

Non-mycosis fungoides cutaneous lymphomas in a referral center in Taiwan: A retrospective case series and literature review.

PloS one
2019

Positron emission tomography-computed tomography in subcutaneous panniculitis-like T-cell lymphoma.

World journal of nuclear medicine
2019

Necrotizing Granulomatous Dermatitis and Panniculitis Masquerading as T Cell Lymphoma.

Skinmed
2019

[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 edition
2020

18F-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 medicine
2019

Clinical features of infantile subcutaneous panniculitis-like T-cell lymphoma.

Pediatrics international : official journal of the Japan Pediatric Society
2019

Pralatrexate Induces Long-Term Remission in Relapsed Subcutaneous Panniculitis-Like T-Cell Lymphoma.

Annals of the Academy of Medicine, Singapore
2019

High-throughput Sequencing of Subcutaneous Panniculitis-like T-Cell Lymphoma Reveals Candidate Pathogenic Mutations.

Applied immunohistochemistry &amp; molecular morphology : AIMM
2019

Not all swellings are lymph nodes! A case of subcutaneous panniculitis-like T-cell lymphoma.

The Medical journal of Malaysia
2020

A retrospective review of cutaneous lymphoma in Botswana.

International journal of dermatology
2020

Activated-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 pathology
2020

Subcutaneous Panniculitis-Like T Cell Lymphoma: Approach to Differential Diagnosis on Cytology.

Journal of adolescent and young adult oncology
2019

Is cyclosporine a good option for the treatment of subcutaneous panniculitis-like T-cell lymphoma associated with hemophagocytic syndrome?

Indian journal of dermatology, venereology and leprology
2020

C-C chemokine receptor 4 expression in CD8+ cutaneous T-cell lymphomas and lymphoproliferative disorders, and its implications for diagnosis and treatment.

Histopathology
2020

Pralatrexate for refractory or recurrent subcutaneous panniculitis-like T-cell lymphoma with hemophagocytic syndrome.

Journal of the American Academy of Dermatology
2019

Panniculitis-like T-cell-lymphoma in the mesentery associated with hemophagocytic syndrome: autopsy case report.

Diagnostic pathology
2019

Fever 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
2019

[Concurrent subcutaneous panniculitis-like T-cell lymphoma and myelodysplastic syndrome with fibrosis].

[Rinsho ketsueki] The Japanese journal of clinical hematology
2019

The Challenge of Identifying Identical Twins: Advances in Differentiating Lupus Erythematosus Panniculitis from Subcutaneous Panniculitis-Like T-Cell Lymphoma.

Skinmed
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Doenças relacionadas

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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.

  1. Clinical characteristics, treatment options, and prognosis of subcutaneous panniculitis-like T-cell lymphoma from the SEER database.
    Hematology (Amsterdam, Netherlands)· 2026· PMID 41876379mais citado
  2. Histopathologic Spectrum of Primary Cutaneous T-Cell Lymphomas: Insights From a Vietnamese Case Series.
    The American Journal of dermatopathology· 2026· PMID 41105046mais citado
  3. Subcutaneous Panniculitis-like T-cell Lymphoma with Secondary Haemophagocytic Lymphohistiocytosis Arising at Widespread Tattoo Sites: A Case&#xa0;Report.
    Acta dermato-venereologica· 2026· PMID 41631858mais citado
  4. Persistent fever and scalp rash in a 2.5-year-old male: A rare case of pediatric subcutaneous panniculitis-like T-Cell lymphoma.
    JAAD case reports· 2026· PMID 41551618mais citado
  5. Ciclosporin 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 international· 2026· PMID 41509192mais citado
  6. HAVCR2 in subcutaneous panniculitis like T cell lymphoma.
    Arthritis Rheumatol· 2026· PMID 41958158recente
  7. 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.
    Int J Gen Med· 2026· PMID 41883463recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:86884(Orphanet)
  2. OMIM OMIM:618398(OMIM)
  3. MONDO:0019475(MONDO)
  4. GARD:10193(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. 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

Linfoma de células T, paniculite subcutânea-like
Compêndio · Raras BR

Linfoma de células T, paniculite subcutânea-like

ORPHA:86884 · MONDO:0019475
Prevalência
Unknown
Herança
Not applicable
CID-10
C86.3
CID-11
Ensaios
4 ativos
Início
All ages
Prevalência
0.0 (Worldwide)
MedGen
UMLS
C0522624
EuropePMC
Wikidata
Papers 10a
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