Lymphocytic panniculitis: an algorithmic approach to lymphocytes in subcutaneous tissue

2015 ◽  
Vol 68 (12) ◽  
pp. 954-962 ◽  
Author(s):  
Carolyn J Shiau ◽  
Marie S Abi Daoud ◽  
Se Mang Wong ◽  
Richard I Crawford
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Subcutaneous Tissue ◽  
Treatment Options ◽  
Immunosuppressive Agents ◽  
T Cell Lymphoma ◽  
Algorithmic Approach ◽  
Specific Diagnosis ◽  
Lupus Profundus

The diagnosis of panniculitis is a relatively rare occurrence for many practising pathologists. The smaller subset of lymphocyte-predominant panniculitis is further complicated by the diagnostic consideration of T cell lymphoma involving the subcutaneous tissue, mimicking inflammatory causes of panniculitis. Accurate classification of the panniculitis is crucial to direct clinical management as treatment options may vary from non-medical therapy to immunosuppressive agents to aggressive chemotherapy. Many diseases show significant overlap in clinical and histological features, making the process of determining a specific diagnosis very challenging. However, with an adequate biopsy including skin and deep subcutaneous tissue, a collaborative effort between clinician and pathologist can often lead to a specific diagnosis. This review provides an algorithmic approach to the diagnosis of lymphocyte-predominant panniculitis, including entities of septal-predominant pattern panniculitis (erythema nodosum, deep necrobiosis lipoidica, morphea profunda and sclerosing panniculitis) and lobular-predominant pattern panniculitis (lupus erythematous panniculitis/lupus profundus, subcutaneous panniculitis-like T cell lymphoma, cutaneous γ-δ T cell lymphoma, Borrelia infection and cold panniculitis).

scholarly journals Controversies and Considerations in the Diagnosis of Primary Cutaneous CD4+ Small/Medium T-Cell Lymphoma

2014 ◽  
Vol 138 (10) ◽  
pp. 1307-1318 ◽  
Author(s):  
Thanh T. Ha Lan ◽  
Noah A. Brown ◽  
Alexandra C. Hristov
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Cell Lymphoma ◽  
Treatment Options ◽  
Lymphoid Hyperplasia ◽  
T Cell Lymphoma ◽  
Reactive Lymphoid Hyperplasia ◽  
Genetic Features ◽  
Patient Prognosis

Context.—Primary cutaneous CD4+ small/medium T-cell lymphoma is a provisional and controversial entity with a broad differential diagnosis. Despite being an uncommon lymphoma, it is a frequent diagnostic consideration in cutaneous biopsies with a dense lymphoid infiltrate because it shows overlapping features with reactive lymphoid hyperplasia (pseudolymphoma) and a variety of other primary cutaneous and systemic lymphomas. However, proper classification of this process is important for determining patient prognosis and treatment options. Objective.—To review the clinical, morphologic, immunophenotypic, and genetic features of primary cutaneous CD4+ small/medium T-cell lymphoma and contrast those features with entities in the differential diagnosis. Data Sources.—Applicable literature will be reviewed with emphasis on current controversies and distinguishing characteristics. Conclusions.—Although many consider primary cutaneous CD4+ small/medium T-cell lymphoma to be indistinguishable from reactive lymphoid hyperplasia/pseudolymphoma, it can be differentiated from other primary cutaneous and systemic lymphomas. Patients with solitary lesions of primary cutaneous CD4+ small/medium T-cell lymphoma generally have an excellent prognosis. Nevertheless, a subset of patients who have been reported to meet criteria for this lymphoma have followed a more-aggressive course; however, those patients show some differing clinical, morphologic, and immunophenotypic features.

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

2021 ◽  
Vol 35 ◽  
pp. 205873842110093
Author(s):  
Liangliang Li ◽  
Chongyang Wu ◽  
Ye Chai ◽  
Chi Dong ◽  
Li Zhao
Keyword(s):  
T Cell ◽  
Unusual Case ◽  
Cell Lymphoma ◽  
Subcutaneous Tissue ◽  
Immunosuppressive Agents ◽  
T Cell Lymphoma ◽  
Cytotoxic T Cell ◽  
Hematopoietic Stem ◽  
Initial Symptoms

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare primary cutaneous lymphoma composed of CD8+ cytotoxic T-cell that is primarily localized in the subcutaneous tissue. No standard treatments are currently available for SPTCL due to its rarity. Chemotherapy, radiotherapy, immunosuppressive agents, and hematopoietic stem cell transplantation (HSCT) have been used frequently, however, the effects of these treatment approaches remain controversial. In this report, we present an unusual case of SPTCL in a 47-year-old woman whose initial symptoms were atypical. The patient was started on etoposide, vincristine, cyclophosphamide, doxorubicin, and prednisone (EPOCH) chemotherapy once diagnosed. After two cycles of chemotherapy, her clinical symptoms were not significantly improved. Subsequently, histone deacetylase (HDAC) inhibitor chidamide was added to the chemotherapy from the third cycle. She recovered gradually and achieved complete remission (CR) after four cycles of chemotherapy combined with chidamide, followed by chidamide monotherapy for maintenance. More than 1 year after the therapy, she remained in CR. Our case illustrates, for the first time, chidamide can be an effective agent to induce long-term remission for rare SPTCL.

Long-term survival of the nasal NK/T cell lymphoma

2008 ◽  
Vol 149 (17) ◽  
pp. 801-805
Author(s):  
Péter Rajnics ◽  
László Krenács ◽  
András Kenéz ◽  
Zoltán Járay ◽  
Enikő Bagdi ◽  
...  
Keyword(s):  
T Cell ◽  
Treatment Guidelines ◽  
Cell Lymphoma ◽  
Treatment Options ◽  
Diagnostic Procedures ◽  
T Cell Lymphoma ◽  
Term Survival ◽  
Barr Virus ◽  
Significant Difference ◽  
Nk T Cell

The nasal NK/T cell lymphoma is a rare, extranodal non-Hodgkin lymphoma in western civilizations, which has poor prognosis. The Epstein–Barr virus can be detected in tumor cells in nearly all cases. There are no definite treatment guidelines in our days. There is no significant difference in survival between radiotherapy and chemotherapy according to Asian studies. In this case study we show our diagnostic procedures, our treatment options and we present the summary of this illness based on the data found in the literature.

scholarly journals Update on Gastrointestinal Lymphomas

2018 ◽  
Vol 142 (11) ◽  
pp. 1347-1351 ◽  
Author(s):  
Steven C. Weindorf ◽  
Lauren B. Smith ◽  
Scott R. Owens
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
World Health ◽  
Barr Virus ◽  
Not Otherwise Specified ◽  
Gastrointestinal Lymphomas ◽  
Epstein Barr ◽  
Health Organization

Herein we review the following selection of gastrointestinal lymphomas: monomorphic epitheliotropic intestinal T-cell lymphoma; indolent T-cell lymphoproliferative disorder of the gastrointestinal tract; intestinal T-cell lymphoma, not otherwise specified; duodenal-type follicular lymphoma; and Epstein-Barr virus–positive mucocutaneous ulcer. Definitions reflect the 2016 revision of the World Health Organization classification of lymphoid neoplasms. Clinical, morphologic, and immunophenotypic characteristics of each entity are emphasized.

scholarly journals Frequent Mutations in Natural Killer/T Cell Lymphoma

2018 ◽  
Vol 49 (1) ◽  
pp. 1-16 ◽  
Author(s):  
Yanjie Zhang ◽  
Chaoping Li ◽  
Weili Xue ◽  
Mingzhi Zhang ◽  
Zhaoming Li
Keyword(s):  
T Cell ◽  
Natural Killer ◽  
Cell Lymphoma ◽  
Treatment Options ◽  
Gene Mutations ◽  
T Cell Lymphoma ◽  
Somatic Gene ◽  
Frequent Mutations ◽  
New Biomarkers ◽  
Next Generation Sequencing Ngs

Extranodal natural killer (NK)/T cell lymphoma (ENKTL-NT or NKTCL), with its aggressive nature and poor prognosis, has been widely studied to discover more effective treatment options. Various somatic gene alterations have been identified by traditional Sanger sequencing. However, recently, novel gene mutations in NKTCL have been revealed by next-generation sequencing (NGS) technology, suggesting the potential for novel targeted therapies. This review discusses recurrent aberrations in NKTCL detected by NGS, which can be categorized into three main groups, specifically, tumor suppressors (TP53, DDX3X, and MGA), the JAK/STAT cascade, and epigenetic modifiers (KMT2D, BCOR, ARID1A, and EP300). Some epigenetic dysregulation and DDX3X mutation, which have been rarely identified by traditional sequencing technology, were recently uncovered with high frequencies by NGS. In this review, we summarize the mutational frequencies of various genes in NKTCL. In general, based on our analysis, BCOR is the most frequently mutated gene (16.9%), followed by TP53 (14.7%), and DDX3X (13.6%). The characterization of such genes provides new insight into the pathogenesis of this disease and indicates new biomarkers or therapeutic targets.

Lobular Panniculitic Infiltrates With Overlapping Histopathologic Features of Lupus Panniculitis (Lupus Profundus) and Subcutaneous T-cell Lymphoma

2015 ◽  
Vol 39 (2) ◽  
pp. 206-211 ◽  
Author(s):  
Francesca Bosisio ◽  
Sebastiana Boi ◽  
Valentina Caputo ◽  
Concetta Chiarelli ◽  
Fergus Oliver ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Lupus Panniculitis ◽  
Lupus Profundus ◽  
Histopathologic Features

Hepatosplenic T-cell lymphoma, immunosuppressive agents and biologicals: what are the risks?

2014 ◽  
Vol 44 (3) ◽  
pp. 287-290 ◽  
Author(s):  
K. Subramaniam ◽  
D. Yeung ◽  
F. Grimpen ◽  
J. Joseph ◽  
K. Fay ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Immunosuppressive Agents ◽  
T Cell Lymphoma ◽  
Hepatosplenic T Cell Lymphoma

Monocytosis As Prognostic Factor in Aggressive, Non-Primary Cutaneous Peripheral T-Cell Lymphoma: A Study of 251 Cases

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1608-1608
Author(s):  
Brady E Beltran ◽  
Erick Cotacallapa ◽  
Jorge J Castillo
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
Who Classification ◽  
Cell Lymphoma ◽  
Statistical Significance ◽  
Prognostic Index ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
Worse Prognosis

Abstract Abstract 1608 Background: Peripheral T-cell lymphoma (PTCL) is a heterogeneous family of entities with a worse prognosis, stage by stage, than their B-cell counterparts. We have previously reported that an absolute lymphocyte count (ALC) <1000/uL is associated with a worse prognosis in Peruvian patients with PTCL (Castillo et al. 2010). The goal of this study is to investigate the prognostic value of absolute monocyte count (AMC) in the survival of patients with PTCL. Methods: A total of 251 cases of aggressive, non-primary cutaneous PTCL diagnosed at our institution between January 1997 and January 2012 were reviewed, reevaluated according to their morphological, immunological and clinical characteristics, and reclassified according to the 2008 WHO classification of lymphoid neoplasms. Characteristics will be presented descriptively. Kaplan-Meier method was used to estimate overall survival (OS) curves, which were compared using the log-rank test. The multivariate analysis was performed using the Cox proportional-hazard regression test. Results: According to the new WHO classification of lymphoid neoplasms, 104 cases (41%) were classified as adult T-cell leukemia/lymphoma (ATLL), 103 cases (41%) as PTCL, unspecified (PTCLU), 27 cases (11%) as analplastic lymphoma kinase (ALK)-negative anaplastic large cell lymphoma (ALCL), 11 cases (4%) as extranodal NK/T-cell lymphoma (NKTCL), nasal type, 4 cases (2%) as angioimmunoblastic lymphoma (AIL), and 2 cases (1%) were diagnosed with ALK+ ALCL. The median age at diagnosis was 57 years (range 14–92 years); 47% of patients were >60 years. The male-to-female ratio was 1:1. ECOG performance status >1 was seen in 51%, LDH was elevated in 67%, advanced stage was seen in 73%, and >1 extranodal sites were seen in 22% of the patients. Bone marrow involvement was reported in 30% and B symptoms in 64% of patients. An International Prognostic Index (IPI) score 3–5 was seen in 55%, and a Prognostic Index for PTCLU (PIT) score of 2–4 in 63%. The median overall survival (OS) for the whole group was 10 months. The IPI score, the PIT score, ALC <1000/uL and AMC >800/uL (Figure) showed statistical significance in the univariate survival analysis (p<0.001, p<0.001, p=0.001 and p=0.001, respectively). In the multivariate analysis, PIT score and AMC >800/uL showed statistical significance (p=0.006, p=0.046, respectively). Conclusions: Monocytosis, defined as AMC >800/uL, and the PIT score were independent prognostic factors for OS in patients with aggressive, non-primary cutaneous PTCL. Disclosures: No relevant conflicts of interest to declare.

Brentuximab Vedotin Followed By Allogeneic Stem-Cell Transplantation for Patients with CD30 Anaplastic or T Cell Non Hodgkin Lymphomas: A Study on Behalf of the SFGM-TC.

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4377-4377
Author(s):  
Sylvain Garciaz ◽  
Michael Loschi ◽  
Adele de Masson ◽  
Sylvie François ◽  
Cecile Tomowiak ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
T Cell ◽  
Cell Lymphoma ◽  
Chronic Gvhd ◽  
Immunosuppressive Agents ◽  
Brentuximab Vedotin ◽  
Salvage Treatment ◽  
T Cell Lymphoma ◽  
Control Group ◽  
Peripheral T Cell Lymphoma

Abstract Brentuximab vedotin (BV), an anti-CD30 antibody-drug conjugate, has demonstrated efficacy alone or in combination with chemotherapy in CD30 refractory Non-Hodgkin Lymphoma (NHL). It has been approved in anaplastic large cell lymphoma (ALCL) and promising results have also been published in other CD30 positive T-cell lymphomas such as cutaneous T-cell lymphoma (CTCL) or peripheral T-cell lymphoma (PTCL). In patients with relapsing or refractory NHL, BV has mainly been proposed as a bridge for autologous or allogeneic transplantation (Allo-HSCT). Very few data are available about patients with T-cell NHL receiving Allo-HSCT after BV. The aim of our study was to study safety and efficacy of this procedure in a retrospective series of patients treated on behalf of SFGM-TC. Inclusion criteria were: - CD30 positive T cell NHL including ALCL, CTCL and PTCL, - Partial or Complete response after BV treatment, - Allogeneic HSCT performed after BV as last salvage treatment. BV was administered at 1,8mg/kg dose every 3 weeks in outpatient department. Allo-HSCT was performed according to institutional guidelines. Twenty-six patients receiving Allo-HSCT in France after salvage therapy including BV were identified. Patients characteristic are summarized in Table 1. With a median follow-up of 13 months (1.5-40), 8 patients relapsed and 7 patients died. Two-year OS and PFS were respectively 76% and 47%. Among patients with ALCL (n=15) 2 patients relapsed and 2 patients died. Whereas in the CTCL group (n=5), 5 patients relapsed and 1 patient died and in the PTCL group (n=6), 1 patient relapsed and 4 patients died. Two-year OS were 93%, 80% and 21% (p<0.001) and two-year PFS were 86%, 20% and 0% (p<0.001) for ALCL, CTCL and PTCL respectively. Two-years PFS for patients in CR before Allo-HSCT (n=16) was 67% whereas patients in PR (n=6) or who progressed before transplantation (n=3) have a 25% and 0% 2-years PFS respectively (p=0,08). In multivariate analysis, only anaplastic histology had a positive impact on OS or PFS. We compared data with a control group of patients with T-cell NHL (n=52) transplanted in the same centers during the same period, not receiving BV as a salvage treatment. Day-100 cumulative incidence of acute Grade 2-4 GVHD and Grade 3-4 GVHD were 39% [20-59] and 16% [1-30] in the BV group and 46% [27-66] and 19% [8-30] in the control group (p=NS). Two-years overall chronic GVHD and extensive chronic GVHD were 33% [9-56] and 22% [0-46] in the BV group and 39% [24-53] and 15% [0-26] in the control group (p=NS). Day-100 and 1-year NRM were 12% [0-25] and 16% [1-30] in the BV group and 4% [0-9] and 8% [1-15] in the control group (p=0,07). One-year relapse incidence was 37% [14-60] and 22% [10-34] in the BV and control groups respectively (p=0,27). BV was not associated with higher GVH, NRM or relapse incidence in multivariate analysis. In conclusion, BV followed by Allo-HSCT is an option for patients with advanced CD 30 positive T cell NHL. Immunotherapy targeting CD30 before Allo-HSCT is not associated with a higher rate of GVHD, NRM or relapse incidence. Patients with ALCL have a better survival than patients with PTCL or CTCL. Table 1. Patients characteristics All patients ALCL CTCL PTCL (n=26) (n=15) (n=5) (n=6) Sexe male 15 (58%) 7 (47%) 4 (80%) 4 (67%) Age (at Allo-HSCT) median (range) 47 (21-62) 40 (21-59) 50 (31-62) 50 (46-59) Stade (at diagnosis) I-II 4 (15%) 3 (20%) - 1 (17%) III-IV 16 (62%) 11 (73%) - 5 (83%) unknown 1 (4%) 1 (7%) - - NA 5 (19%) - 5 (100%) - Auto-HSCT (before BV) patients 8 (31%) 5 (33%) - 3 (50%) Lines of chemotherapy (before BV) median (ranges) 2 (1-7) 2 (1-4) 4 (3-7) 2 (1-3) Status (before BV) refractory 18 (69%) 9 (60%) 5 (100%) 4 (66%) relapsing 8 (31%) 6 (40%) - 2 (34%) cycles of BV median (ranges) 4 (1-8) 4 (1-8) 4 (4-6) 4 (1-4) Treatment associated with BV no 20 (77%) 11 (73%) 5 (100%) 4 (66%) GVD 2 (8%) 2 (13%) - - CHP 2 (8%) - - 2 (33%) DHAP 1 (4%) 1 (7%) - - Radiotherapy 1 (4%) 1 (7%) - - Time between diagnosis and Allo-HSCT Months (range) 21 (6-93) 13 (6-93) 23 (21-43) 23 (12-76) Status before Allo-HSCT CR 16 (62%) 13 (87%) - 3 (50%) PR 6 (23%) 2 (13%) 4 (80%) - SD/PD 3 (12%) - 1 (20%) 2 (33%) Donor type MRD 15 (57%) 5 (33%) 4 (80%) 6 (100%) MUD 7 (27%) 6 (40%) 1 (20%) - CBU 2 (8%) 2 (13%) - - Haploidentical 2 (8%) 2 (13%) - - Conditioning 3 (50%) RIC 17 (65%) 9 (60%) 5 (100%) MAC 9 (35%) 6 (40%) - 3 (50%) Immunosuppressive agents CyA 5 (19%) 4 (27%) - 1 (17%) CyA/MMF 10 (38%) 4 (27%) 5 (100%) 1 (17%) CyA/MTX 11 (42%) 7 (47%) - 4 (66%) Anti-thymocyte globulin 11 (42%) 8 (53%) 1 (20%) 2 (33%) Disclosures Off Label Use: Brentuximab used for CD30+ cutaneous and peripheral T cell lymphoma.

scholarly journals Primary Cutaneous Acral CD8+ T-Cell Lymphoma

2017 ◽  
Vol 141 (11) ◽  
pp. 1469-1475 ◽  
Author(s):  
Vivian M. Hathuc ◽  
Alexandra C. Hristov ◽  
Lauren B. Smith
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Correct Diagnosis ◽  
Cd8 T Cell ◽  
T Cell Lymphoma ◽  
World Health ◽  
T Cell Lymphomas ◽  
Pathologic Features ◽  
Health Organization

Primary cutaneous acral CD8+ T-cell lymphoma is a new provisional entity in the 2016 revision of the World Health Organization classification of lymphoid neoplasms. This is a challenging diagnosis because of its rarity, as well as its morphologic and immunophenotypic overlap with other CD8+ cytotoxic lymphoid proliferations. Appropriate classification of this entity is crucial because of its indolent clinical behavior compared with other CD8+ T-cell lymphomas. Knowledge of the clinical setting, sites of involvement, and morphologic features can aid in correct diagnosis. Here, we review the clinical and pathologic features of primary cutaneous acral CD8+ T-cell lymphoma with an emphasis on the differential diagnosis among other C8+ T-cell lymphomas.

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