scholarly journals Epidemiology of Non-Hodgkin’s Lymphoma

2021 ◽  
Vol 9 (1) ◽  
pp. 5
Author(s):  
Krishna C. Thandra ◽  
Adam Barsouk ◽  
Kalyan Saginala ◽  
Sandeep Anand Padala ◽  
Alexander Barsouk ◽  
...  
Keyword(s):  
T Cell ◽  
Radiation Treatment ◽  
Cell Lymphoma ◽  
Breast Implants ◽  
Occupational Exposures ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Human Herpes Virus 8 ◽  
Human T Cell

Non-Hodgins’s lymphoma (NHL) is the most common hematological malignancy worldwide, accounting for nearly 3% of cancer diagnoses and deaths. NHL is the seventh most prevalent cancer and has the sixth highest mortality among cancers in the US. NHL accounts for 4% of US cancer diagnoses, and incidence has increased 168% since 1975 (while survival has improved 158%). NHL is more common among men, those >65 years old, and those with autoimmune disease or a family history of hematological malignancies. NHL is a heterogenous disease, with each subtype associated with different risk factors. Marginal zone lymphoma (MZL) is strongly associated with Sjogren’s syndrome (SS) and Hashimoto’s thyroiditis, while peripheral T-cell lymphoma (PTCL) is most associated with celiac disease. Occupational exposures among farm workers or painters increases the risk of most of the common subtypes. Prior radiation treatment, obesity, and smoking are most highly associated with diffuse large B-cell lymphoma (DLBCL), while breast implants have been rarely associated with anaplastic large cell lymphoma (ALCL). Infection with Epstein–Barr Virus (EBV) is strongly associated with endemic Burkitts lymphoma. HIV and human herpes virus 8 (HHV-8), is predisposed to several subtypes of DLBCL, and human T-cell lymphoma virus (HTLV-1) is a causative agent of T-cell lymphomas. Obesity and vitamin D deficiency worsen NHL survival. Atopic diseases and alcohol consumption seem to be protective against NHL.

Anti-CD45 Mediated Cytolysis of Human T-Cell Lymphoma Cells.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4637-4637
Author(s):  
Gerald G. Wulf ◽  
Anita Boehnke ◽  
Bertram Glass ◽  
Lorenz Truemper
Keyword(s):  
T Cell ◽  
B Cell ◽  
Cell Lines ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Cytolytic Activity ◽  
T Cell Lymphoma ◽  
Lymphoma Cell ◽  
Lymphoma Cells ◽  
Human T Cell

Abstract Anti-CD45 mediated cytoreduction is an effective means for T-cell depletion in rodents and humans. In man, the CD45-specific rat monoclonal antibodies YTH24 and YTH54 are IgG2b subclass, exert a predominantly complement-dependent cytolytic activity against normal T-lymphocytes, and have been safely given to patients as part of conditioning therapies for allogeneic stem cell transplantation. The efficacy of such antibodies against human lymphoma is unknown. Therefore, we evaluated the cytolytic activity of YTH24 and YTH54 by complement-dependent cytotoxicity (CDC), antibody-dependent cell-mediated cytotoxicity (ADCC), as well as by direct apoptotic and antiproliferative effects, against a panel of Hodgkin disease (HD) and non-Hodgkin lymphoma (NHL) cell lines, and against primary specimens. Significant CDC activity (>50% cytolysis) of the antibodies YTH54 and YTH24 was observed against three of five T-cell lymphoma lines, but against only one of nine B-cell lymphoma lines and none of four HD cell lines. The combination of YTH54 and YTH24 induced ADCC in all T-cell lymphoma cell lines and three primary leukemic T-cell lymphoma specimens, but were ineffective in B-cell lymphoma and HD cell lines.There were only minor effects of either antibody or the combination on lymphoma cell apoptosis or cell cycle arrest. In summary, anti-CD45 mediated CDC and ADCC via the antibodies YTH24 and YTH54 are primarily effective against lymphoma cells with T-cell phenotype, and may be an immunotherapeutic tool for the treatment of human T-cell lymphoma.

Favorable Outcome In ALK-Negative Anaplastic Large-Cell Lymphoma Following Intensive Induction Chemotherapy and Autologous Stem Cell Transplantation (ASCT): a Prospective Study by the Nordic Lymphoma Group (NLG-T-01)

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 3566-3566 ◽  
Author(s):  
Thomas Relander ◽  
Grete Fossum Lauritzsen ◽  
Esa Jantunen ◽  
Hans Hagberg ◽  
Harald Anderson ◽  
...  
Keyword(s):  
T Cell ◽  
Induction Chemotherapy ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Serum Lactate Dehydrogenase ◽  
Term Survival

Abstract Abstract 3566 Primary systemic T-cell lymphoma of anaplastic large cell type (ALCL) is an aggressive and predominantly nodal subtype of lymphoma, further subdivided based on expression of the ALK-protein, with ALK-pos ALCL occurring predominantly in younger patients and associated with a favourable prognosis. ALK-neg ALCL is believed to carry a prognosis similar to that of other nodal peripheral T-cell lymphomas and previous studies have shown long-term survival rates below 50%. There is retrospective data suggesting a benefit from ASCT in first-line treatment of this lymphoma subtype. We analyzed the outcome of ALK-neg ALCL patients included in a prospective phase II trial, NLG-T-01, conducted by the Nordic Lymphoma Group. The NLG T-01 trial enrolled 160 patients aged 18–67 years from the Nordic countries with systemic ALK-neg peripheral T-cell lymphoma within the period 2002–2007. The treatment schedule consisted of 6 courses of CHOEP-14 followed by ASCT (BEAM or BEAC) in responding patients. Patients >60 years received CHOP-14 as induction. Altogether, the trial included 31 patients with ALK-neg ALCL (19% of the study population). Median age was 56 years (22-65) with a male:female ratio of 2.4. Stage III-IV was found in 18 patients (58%), B-symptoms in 19 patients (61%) and 6 patients (19%) had a bulky lesion (>10cm). Pre-therapeutic serum lactate dehydrogenase was elevated in 18 patients (58%) and performance score was 2–4 in 10 patients (32%). After 3 and 6 courses of chemotherapy, response status was CR or CRu in 29% and 58% of the patients, respectively. In total, 24 out of 31 patients (77%) underwent BEAM/BEAC therapy followed by ASCT. Four patients did not respond or had disease progression during induction chemotherapy. The remaining 3 patients did not undergo ASCT for other reasons (mobilization failure, lung insufficiency, patient decision, respectively). Overall response rate after ASCT was 74% for the entire initial population and 96% for those undergoing ASCT. Median follow-up was 45 months. Six patients relapsed after ASCT. There was a total of nine deaths (29%): six due to lymphoma, two due to toxicity and one from second malignancy (colon cancer). With a median follow-up of 45 months, 3-year overall and progression-free survival values were 73% and 64%, respectively. Intensive chemotherapy followed by ASCT was feasible in the majority of the patients included in this prospective trial. Long-term outcome appears promising when compared to previously published data, with the survival curve suggesting a plateau. In ASCT eligible patients, intensive induction chemotherapy consolidated by upfront ASCT is an effective treatment that yields outcome results at least as good as those obtained in age-comparable patients with diffuse large B-cell lymphoma. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Primary cutaneous CD4+ pleomorphic small to medium cell T cell lymphoma: a clinical case

2021 ◽  
Author(s):  
Н.В. Зильберберг ◽  
М.М. Кохан ◽  
Г.Д. Сафонова ◽  
О.Г. Римар ◽  
И.А. Куклин ◽  
...  
Keyword(s):  
T Cell ◽  
Clinical Case ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Tumor Stage ◽  
T Cell Lymphoma ◽  
Rare Occurrence ◽  
Laboratory Examination ◽  
Immunohistochemical Studies

Диагностика первичной кожной CD4+ плеоморфной Т-клеточной лимфомы кожи из клеток малого и среднего размера сложна ввиду редкой встречаемости данной нозологии. Заболевание необходимо дифференцировать от первичной В-клеточной лимфомы кожи, анапластической крупноклеточной CD30+ лимфомы кожи, опухолевой стадии грибовидного микоза, псевдолимфомы. Показаны важность клинической онконастороженности врачей и необходимость проведения клинико-лабораторного обследования больного с использованием современных гистологических и иммуногистохимических методов исследования. В статье приведен случай диагностики редкого варианта первичной лимфомы кожи – первичной кожной CD4+ плеоморфной Т-клеточной лимфомы кожи из клеток малого и среднего размера у пациента 70 лет, обратившегося на консультацию к дерматовенерологу. Диагноз верифицирован патоморфологическими и иммуногистохимическими исследованиями биоптата пораженной кожи. The diagnosis of primary cutaneous CD4+ pleomorphic T-cell lymphoma of the skin from small and medium-sized cells is difficult due to the rare occurrence of this nosology. The disease must be differentiated from primary B-cell lymphoma of the skin, anaplastic large cell CD30+ skin lymphoma, tumor stage of fungal mycosis, pseudolymphoma. The importance of clinical oncological alertness of doctors and the need for clinical and laboratory examination of a patient using modern histological and immunohistochemical research methods are shown. The article presents a case of diagnostics of a rare variant of primary skin lymphoma of cellular skin lymphoma – primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma in a 70-year-old patient. The diagnosis was verified by pathomorphological and immunohistochemical studies of the biopsy of the affected skin.

scholarly journals Violet - Colored Inguinal Located Cutaneous Tumour?

2018 ◽  
Vol 6 (1) ◽  
pp. 137-138
Author(s):  
Georgi Tchernev ◽  
Anastasiya Atanasova Chokoeva ◽  
Torello Lotti ◽  
Uwe Wollina ◽  
Irina Yungareva ◽  
...  
Keyword(s):  
T Cell ◽  
Clinical Manifestation ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Surgical Excision ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Disease Stage ◽  
High Recurrence Rate ◽  
Systemic Involvement

Anaplastic large cell lymphoma (ALCL) represents an aggressive CD30 – positive T cell lymphoma, as it is the second most common T cell lymphoma and 2% to 5% of all non - Hodgkin lymphomas. The cutaneous involvement can be primary or secondary within systemic ALCL, resembling inflammatory and other neoplastic lesions both clinically and cytologically. Various pigmented cutaneous tumours with a different origin, cutaneous metastasis and B-cell lymphoma must be carefully considered in the differential diagnostic plan. While simple surgical excision is usually curative, with good prognosis, systemic involvement must also be excluded. We present a case of a patient, with clinically unspecific single violet nodular lesion, as the only clinical manifestation of ALCL. The diagnosis was confirmed histologically, as the surgical excision was enough therapeutic management, regarding the early disease stage. Further following up with the patient is mandatory, because of the high recurrence rate. We want to emphasise the diversity of clinical manifestation of ALCL, regarding the importance of its early diagnosis and treatment.

Response to brentuximab vedotin by CD30 expression: Results from five trials in PTCL, CTCL, and B-cell lymphomas.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 7543-7543
Author(s):  
Deepa Jagadeesh ◽  
Steven M. Horwitz ◽  
Nancy L. Bartlett ◽  
Ranjana H. Advani ◽  
Eric D. Jacobsen ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Cell Lymphoma ◽  
Objective Response ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Brentuximab Vedotin ◽  
T Cell Lymphoma ◽  
Antibody Drug Conjugate ◽  
Non Hodgkin Lymphoma

7543 Background: Brentuximab vedotin (BV), an antibody-drug conjugate targeting CD30, has been evaluated in multiple trials in patients (pts) with peripheral T-cell lymphoma (PTCL), cutaneous T-cell lymphoma (CTCL), or B-cell lymphoma. We examined the ability of CD30 expression level to predict response to BV across these patient populations. Methods: Data were integrated from 275 pts with PTCL, CTCL, and B-cell lymphoma treated with BV from 5 prospective clinical trials. Study SGN35-012 evaluated BV plus rituximab or BV monotherapy in pts with relapsed/refractory non-Hodgkin lymphoma. The ALCANZA study compared BV to physician’s choice of methotrexate or bexarotene in pts with mycosis fungoides (MF) or primary cutaneous anaplastic large cell lymphoma (pcALCL). Three investigator-sponsored trials evaluated BV monotherapy in pts with relapsed PTCL, MF, and pcALCL (35-IST-030, 35-IST-001, 35-IST-002). Exploratory analyses were conducted to examine the relationship between CD30 expression and objective response rate (ORR) for pts with CD30 expression ≥10%, <10%, or undetectable (0%) by IHC (malignant cells or lymphoid infiltrate; local review). Results: 143 pts had tumors with CD30 <10%, including 58/143 with undetectable CD30. Activity with BV was observed at all levels of CD30 expression, including CD30=0 (Table). Analysis of the interaction between CD30 and duration of response is ongoing and will be presented in the final poster. ORR by CD30 expression, n/N (%). Clinical trial information: NCT01421667, NCT02588651, NCT01578499, NCT01352520, NCT01396070. Conclusions: CD30 expression levels ≥10%, <10%, or undetectable did not predict response to BV in a range of CD30-expressing lymphomas: Clinical responses occurred in pts with CD30 low and CD30 undetectable lymphomas. Limitations of IHC, the dynamic nature and heterogeneity of cell-surface CD30 expression, and multiple mechanisms of action of BV may all contribute to this observation.[Table: see text]

Clinical Applications of the Genomic Landscape of Aggressive Non-Hodgkin Lymphoma

2017 ◽  
Vol 35 (9) ◽  
pp. 955-962 ◽  
Author(s):  
Andrea B. Moffitt ◽  
Sandeep S. Dave
Keyword(s):  
T Cell ◽  
B Cell ◽  
Hodgkin Lymphoma ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Adult T Cell Leukemia ◽  
Non Hodgkin Lymphoma ◽  
T Cell Lymphomas

In this review, we examine the genomic landscapes of lymphomas that arise from B, T, and natural killer cells. Lymphomas represent a striking spectrum of clinical behaviors. Although some lymphomas are curable with standard therapy, the majority of the affected patients succumb to their disease. Here, the genetic underpinnings of these heterogeneous entities are reviewed. We consider B-cell lymphomas, including Burkitt lymphoma, diffuse large B-cell lymphoma, Hodgkin lymphoma, and primary mediastinal B-cell lymphoma. We also examine T-cell lymphomas, including anaplastic large-cell lymphoma, angioimmunoblastic T-cell lymphoma, cutaneous T-cell lymphoma, adult T-cell leukemia/lymphoma, and other peripheral T-cell lymphomas. Together, these malignancies make up most lymphomas diagnosed around the world. Genomic technologies, including microarrays and next-generation sequencing, have enabled a better understanding of the molecular underpinnings of these cancers. We describe the broad genomics findings that characterize these lymphoma types and discuss new therapeutic opportunities that arise from these findings.

scholarly journals Increased Expression of Phosphorylated FADD in Anaplastic Large Cell and Other T-Cell Lymphomas

2014 ◽  
Vol 9 ◽  
pp. BMI.S16553 ◽  
Author(s):  
Suketu Patel ◽  
Derek Murphy ◽  
Eugenia Haralambieva ◽  
Zainalabideen A. Abdulla ◽  
Kah Keng Wong ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Death Receptor ◽  
Adaptor Protein ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Death Domain ◽  
Normal Tissues ◽  
T Cell Lymphomas ◽  
Human T Cell

FAS-associated protein with death domain (FADD) is a major adaptor protein involved in extrinsic apoptosis, embryogenesis, and lymphocyte homeostasis. Although abnormalities of the FADD/death receptor apoptotic pathways have been established in tumorigenesis, fewer studies have analyzed the expression and role of phosphorylated FADD (pFADD). Our identification of FADD as a lymphoma-associated autoantigen in T-cell lymphoma patients raises the possibility that pFADD, with its correlation with cell cycle, may possess role(s) in human T-cell lymphoma development. This immunohistochemical study investigated pFADD protein expression in a range of normal tissues and lymphomas, particularly T-cell lymphomas that require improved therapies. Whereas pFADD was expressed only in scattered normal T cells, it was detected at high levels in T-cell lymphomas (eg, 84% anaplastic large cell lymphoma and 65% peripheral T cell lymphomas, not otherwise specified). The increased expression of pFADD supports further study of its clinical relevance and role in lymphomagenesis, highlighting phosphorylation of FADD as a potential therapeutic target.

scholarly journals Concordance Study of CD30 Expression Detected By Multiple Immunohistochemistry Assays and Ventana CD30 Assay in Chinese Lymphoma Patients

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4553-4553
Author(s):  
Xiang-Nan Jiang ◽  
Yang Shi ◽  
Xiao-Qiu Li
Keyword(s):  
T Cell ◽  
B Cell ◽  
Malignant Lymphoma ◽  
Expert Panel ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
Ffpe Sample ◽  
T Cell Lymphoma ◽  
Chinese Patients

Abstract Background: CD30 is emerging as an important biomarker guiding the clinical diagnosis, treatment, and evaluating the efficacy and prognosis of lymphoma. However, there is no standard procedure for specification and interpretation of the pathological detection of CD30 in China. This study intends to evaluate the staining and interpretation concordance of CD30 expression detected by the VENTANA CD30 assay and other multiple immunohistochemistry (IHC) assay in Chinese malignant lymphoma patients. Design: This is a multi-center, observational study enrolling 1,000 adult patients with a diagnosis of histologically confirmed malignant lymphoma in China. Patients, aged 18 years or older at diagnosis and who provided available formalin-fixed paraffin-embedded (FFPE) samples stored within 2 years, with diagnosis of classical Hodgkin's lymphoma, anaplastic large-cell lymphoma, large cell transformation of mycosis fungoides, diffuse large B-cell lymphoma, primary mediastinal B-cell lymphoma, extranodal NK/T-cell lymphoma, peripheral T cell lymphoma not otherwise specified, angioimmunoblastic T-cell lymphoma, and pcCD30+ T-cell lymphoproliferative disease were eligible to participate. Exclusion criteria included sample insufficiency for CD30 testing, incomplete sample information, lack of written informed consent. Methods: Each eligible patient provides an available r FFPE sample. Tissue slides obtained from each eligible FFPE sample will be stained by the VENTANA CD30 assay and at least one of the following IHC assays [umAB256 (zhongshanjinqiao) + Ventana BenchMark; Ber-H2 (Maixin) + Ventana BenchMark；Ber-H2 (Maixin) + DAKO; Ber-H2 (DAKO) + DAKO; Ber-H2 (DAKO) + Ventana BenchMark;umAB256 (Zhongshanjinqiao) + Leica; JCM182 (Leica) + Ventana BenchMark;JCM182 (Leica) + Leica; Ber-H2 (DAKO) + Leica]. CD30 expression of all immunostained slides will be independently interpreted as a percentage of CD30 positive cells by trained pathologists. The primary endpoint will be the staining concordance of percentages of positive cells for CD30 expression detected by each of the nine IHC assays and VENTANA CD30 assay (interpreted by expert panel) Secondary endpoints will be the interpretation concordance of pathologists at sites and the expert panel by assessing CD30 expression detected by various IHC assays. Both primary and secondary endpoint will be evaluated by intraclass correlation coefficient ICC, Bland-Altman, and Pearson correlation method. Discussion: This planned study will explore more equivalent testing methods to detect the expression of CD30 in Chinese patients with lymphoma. Disclosures Shi: Takeda Pharmaceuticals: Current Employment. Li: Takeda Pharmaceuticals: Membership on an entity's Board of Directors or advisory committees, Other: Receiving lecture fees.

Histopathological features and immunophenotyping of canine transmural gastrointestinal lymphoma using full-thickness biopsy samples

2021 ◽  
pp. 030098582110305
Author(s):  
Kazuhiro Kojima ◽  
James K. Chambers ◽  
Tatsuhito Ii ◽  
Kazumi Nibe ◽  
Takuya Mizuno ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Who Classification ◽  
Cell Lymphoma ◽  
Granzyme B ◽  
B Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
World Health ◽  
Gastrointestinal Lymphomas

To elucidate the histopathological characteristics and immunophenotypes of canine transmural “mass-forming” gastrointestinal lymphomas and plasmacytomas, 83 surgically resected biopsy samples were examined. All lymphomas and plasmacytomas were located in the small or large intestine except for 1 plasmacytoma which was in the stomach. According to the World Health Organization (WHO) classification, B-cell neoplasms (17 cases) included lymphoplasmacytic lymphoma (6/17), plasmacytoma (5/17), follicular lymphoma (3/17), and diffuse large B-cell lymphoma (3/17). Based on nuclear sizes, T-cell neoplasms (66 cases) were broadly divided into large cell lymphoma (LCL; 48/66) and small cell lymphoma (SCL; 18/66). According to the WHO classification, T-cell neoplasms included anaplastic large T-cell lymphoma (ALCL; 10/66), angiotropic T-cell lymphoma (3/66), mixed inflammatory type peripheral T-cell lymphoma (mixed inflammatory type PTCL; 33/66), and PTCL-not otherwise specified (PTCL-NOS; 20/66). Mixed inflammatory type PTCLs were further divided into histiocyte- (27/33) and eosinophil- (6/33) dominant types. Immunohistochemically, lymphoplasmacytic lymphomas were positive for Pax5 (6/6) and IgM (5/6), while plasmacytomas were positive for IgG (5/6) and negative for Pax5. LCLs were immunopositive for granzyme B in 31/48 cases (65%) and CD8 in 9/48 cases (19%), while SCLs were immunopositive for granzyme B in 3/18 cases (17%) and CD8 in 3/18 cases (17%). Furthermore, 8/10 cases (80%) of ALCL and 19/27 cases (70%) of histiocyte-dominant PTCL were immunopositive for granzyme B, whereas 6/20 cases (30%) of PTCL-NOS, 1/6 cases (17%) of eosinophil-dominant PTCL, and no cases of angiotropic T-cell lymphomas were immunopositive for granzyme B. The present study describes the immunophenotypes in different histological types of transmural gastrointestinal lymphomas in the dog.

Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients

Blood ◽  
2003 ◽  
Vol 102 (6) ◽  
pp. 2213-2219 ◽  
Author(s):  
Marcel W. Bekkenk ◽  
Maarten H. Vermeer ◽  
Patty M. Jansen ◽  
Ariënne M. W. van Marion ◽  
Marijke R. Canninga-van Dijk ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Size ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Skin Lesions ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Nor Phenotype ◽  
Peripheral T Cell Lymphomas

Abstract In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30– peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30– large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small- or medium-sized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30– large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small- or medium-sized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3+CD4+CD8– phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group and in the concurrent group, neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size, and expression of a CD4+ or CD8+ phenotype. The only exception was a group of primary cutaneous small- or medium-sized pleomorphic CTCLs with a CD3+CD4+CD8– phenotype and presenting with localized skin lesions.

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