scholarly journals NCCN Guidelines Insights: T-Cell Lymphomas, Version 1.2021

2020 ◽  
Vol 18 (11) ◽  
pp. 1460-1467
Author(s):  
Steven M. Horwitz ◽  
Stephen Ansell ◽  
Weiyun Z. Ai ◽  
Jeffrey Barnes ◽  
Stefan K. Barta ◽  
...  
Keyword(s):  
T Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Bone Marrow Involvement ◽  
T Cell Lymphoma ◽  
Nccn Guidelines ◽  
T Cell Lymphomas ◽  
Hepatosplenic T Cell Lymphoma ◽  
Aggressive Clinical Course ◽  
Worse Prognosis

Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of T-cell lymphoma associated with an aggressive clinical course and a worse prognosis. HSTCL develops in the setting of chronic immune suppression or immune dysregulation in up to 20% of cases and is most often characterized by spleen, liver, and bone marrow involvement. Diagnosis and management of HSTCL pose significant challenges given the rarity of the disease along with the absence of lymphadenopathy and poor outcome with conventional chemotherapy regimens. These Guidelines Insights focus on the diagnosis and treatment of HSTCL as outlined in the NCCN Guidelines for T-Cell Lymphomas.

Cytologic transformation in cutaneous T cell lymphoma: a clinicopathologic entity associated with poor prognosis.

1987 ◽  
Vol 5 (2) ◽  
pp. 208-215 ◽  
Author(s):  
E Dmitrovsky ◽  
M J Matthews ◽  
P A Bunn ◽  
G P Schechter ◽  
R W Makuch ◽  
...  
Keyword(s):  
T Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Cutaneous T Cell Lymphoma ◽  
Blood Smears ◽  
Large Cell Lymphoma ◽  
Aggressive Clinical Course ◽  
Peripheral Blood Smears

The clinical course of cutaneous T cell lymphoma (mycosis fungoides and Sezary syndrome) is generally indolent, but in occasional patients becomes fulminant. We found that biopsies from patients with accelerating disease can reveal cytologic transformation from previously observed small, convoluted lymphocytes to large cells that are similar to cells seen in large-cell lymphoma. The cerebriform nuclei characteristic of malignant T cells can only rarely be identified. Of 150 cutaneous T cell lymphoma patients we treated from 1976 to 1984, cytologic transformation was identified in 12 after review of peripheral blood smears and biopsies from skin, lymph nodes, and visceral sites. Patients who developed cytologic transformation were initially characterized by advanced stage (11 of 12), with lymph node effacement (seven of 11) and erythroderma (five of 12). The tumor cell DNA content after transformation was aneuploid (four of four), and the ability to form rosettes with sheep erythrocytes was retained in transformed cells (three of three). The median time from diagnosis of cutaneous T cell lymphoma to cytologic transformation was 21.5 months (range, 4 to 64), and the median survival from transformation was only 2 months (range, 0 to 19+). We conclude that cytologic transformation in cutaneous T cell lymphoma represents a distinct clinicopathologic entity, characterized by an aggressive clinical course.

Retrospective Study on T Cell Malignent Lymphoma: Classification, Manifestation, Laboratory Finding and Survival.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 4652-4652
Author(s):  
Hongyan Tong ◽  
Feng Xiao ◽  
Tieying Dai ◽  
Jie Jin ◽  
Haitao Meng ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Cell Lymphoma ◽  
Bone Marrow Involvement ◽  
Autologous Bone Marrow Transplantation ◽  
T Cell Lymphoma ◽  
World Health ◽  
Clinical Staging ◽  
T Cell Lymphomas

Abstract T-cell lymphoma is the special malignant type of non-Hodgkin’s lymphoma. The diagnosis and the treatment were usually troublesome for physician in clinical practice. We retrospectively reviewed 63 cases of T-cell lymphomas from 360 cases of lymphomas in our hospital during the period from January 2000 to July 2006. This study is to determine the clinicopathological characteristics of T cell lymphomas. The patients were reclassified according to the World Health Organization classification system. Clinical data, including age, gender, clinical staging, and follow-up, were scrutinized. The median follow-up duration was 5 months (range 21days to 36 months). There were slightly more males than females (36 versus 27), and the median age at the onset were 40 years (range 13 to 77 years). The major subtype was peripheral T-cell lymphoma, which accounted for 78% (49/63). Besides, there were 5 cases of anaplastic T large cell lymphoma, 3 lymphoblastic lymphoma, 2 T/NK-cell lymphoma, 2 angioimmunoblastic lymphoma, 1 mycosis fungoides and 1 pre-T cell lymphoma. The most common manifestation was fever, which accounted for 60% (38/63). 27% (17/63) patients presented with obvious enlargement of lymphonodes. Other manifestation included skin rash or phymata, pruritus, jaundice, abdominal pain, rhinorrhagia, puffiness, diarrhea, hoarseness and ulcus. Interestingly, we found that only 32% obvious enlarged lymphonodes could be confirmed by physical examination, hepatomegaly 33% and Splenomegaly 44% respectively. Besides, there were several significant laboratory findings: 40% cases had cytopenia of at least 2 cell lines, 68% had high level of LDH, 70% had elevated β2-microglobulin and 68% were detected T-cell receptor (TCR) and immunoglobulin heavy chain (IgH) gene rearrangement. Furthermore, 53% (33/63) patients had bone marrow involvement at the onset and 27% were diagnosed only by bone marrow biopsy. We also observed 20 cases of lymphoma associated hemophagocytic syndromes (LAHS). The median age for this disease was 37 year. The median life span was 39 days (range 21days to 10 months). The initial manifestations included fever (19/20), splenohepatomegaly (18/20), and cytopenias in all patients. Only 15% patients had enlargement of lymphonodes, which was suggested to be infrequent in LAHS. Immatural T-cell infiltration in bone marrow was detected in 75% (15/20) cases. Chromosome disorder of [der(21)(p11), −22] was detected in 3 cases. We also found that 2 cases which underwent plasmapheresis got much better after chemotherapy. 19 cases were under our follow-up. 17 patients could not survival longer than 6 months. The 6-month overall survival (OS) for LAHS was merely 2 of all 20. Furthermore, nobody survived more than 1 year, which indicated the poor prognosis of LAHS. There were 11 out of 63 cases had received trial chemotherapy including liposomal Doxorubicin, L-asparaginase, velcade, autologous bone marrow transplantation, or plasmapheresis before chemotherapy. The median survival time prolonged obviously from 2 months up to 8 months, which suggested the encouraging efficiency of these methods.

Clinicopathological Characteristics and Clinical Course of CD8 Expressing Primary Cutaneous Peripheral T-Cell Lymphomas (CTCL) - Retrospective Case Study

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5213-5213
Author(s):  
Deniz Peker ◽  
Yizhou Zhang ◽  
Young Yu ◽  
Zhigang Zhao ◽  
Yafei Wang ◽  
...  
Keyword(s):  
Overall Survival ◽  
T Cell ◽  
Clinical Outcome ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Clinicopathological Characteristics ◽  
Therapeutic Strategies ◽  
T Cell Lymphoma ◽  
T Cell Lymphomas

Abstract Abstract 5213 Background: CD8-positive primary cutaneous T cell lymphomas (CTCL) are rare disorders and mainly include primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (AECTL) and CD8+ variant mycosis fungoides (MF). In contrast to primary cutaneous CD8+ AECTL, which frequently exhibits strikingly aggressive and unfavorable clinical behavior, CD8+ MF shows debatable clinical course, from an indolent to aggressive behavior. As previously reported, the indolent subtype CD8+ MF occur more frequently in pediatric group, while both clinical subtypes have been observed in adults. Albeit single case studies or small case series have been reported in the literature, it still lacks a large scale of study to enlighten the clinicopathological aspects of CD8+ primary CTCLs, in order to develop the appropriate therapeutic strategies. This study aims to retrospectively review these two entities to demonstrate their clinicopathologic characteristics and to correlate them with the clinical outcome. Design: The hematopathology files from H. Lee Moffitt Cancer Center & Research Institute (PATHNET) and Tianjian Cancer Research Institute were retrieved. The patients with a primary diagnosis of CD8 expressing primary CTCLs, diagnosed and treated between January 2004 and June 2011, were included. Cutaneous involvement by systemic peripheral T-cell lymphoma, primary cutanous gamma delta T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma were excluded. The corresponding patient demographics, laboratory datas, therapeutic strategies and the clinical outcomes were reviewed. All available histology slides, along with all of the ancillary study results were reviewed and correlated with the clinical outcome. Results: Total of 10 cases were included based on the confirmed histomorphological diagnosis. Cases were divided into two groups: 1) CD8+ MF (n=5) and 2) CD8+ non-MF (n=5) including 2 cases with definitive diagnosis of AECTL and 3 cases diagnosed as CD8-positive primary cutaneous T cell lymphoma, not further classifiable. Clinicopathological characteristics including patients' demographic data, diagnosis, site of involvement, treatment, duration of follow up and clinical outcomes are summarized in table 1. The overall survival time for CD8+CTCLs, non-MF type (excluding 1 patient with lost follow up) varied from 5 to 90 months (averaging 20.5 months) while it was shorter in CD8+ MF, 12.6 months (5 to 23 months). Of note, 1 patient with AECTL expired shortly after diagnosis, within 3 months, however; the other one received allogeneic hematopoietic stem cell transplant (allo-HSCT) and has been alive up to date. Conclusion: CD8-positive CTCLs remain a diagnostic challenge. CD8+ MF in adults exhibit dual growth patterns: localized or systemically disseminated disease. The latter could have a very short median overall survival regardless of the aggressive therapies. Allo-HSCT might be beneficial to those with AECTL. Larger series of CD8+ MF should be investigated for molecular gene profiling in order to establish genetic, molecular and phenotypic parameters not only to separate the indolent form from the aggressive subtype, but also to distinguish it from primary cutaneous CD8-positive AECTL. Disclosures: No relevant conflicts of interest to declare.

PP - PRIMARY CUTANEOUS CD8+ T-CELL LYMPHOMA: A CASE REPORT WITH UNUSUAL FACIAL INVOLVEMENT AND AN AGGRESSIVE CLINICAL COURSE

2017 ◽  
Vol 123 (2) ◽  
pp. e56
Author(s):  
DAPHINE CAXIAS TRAVASSOS ◽  
DARCY FERNANDES ◽  
ELAINE MARIA SGAVIOLI MASSUCATO ◽  
CLAUDIA MARIA NAVARRO ◽  
MIRIAN APARECIDA ONOFRE ◽  
...  
Keyword(s):  
Case Report ◽  
T Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Cd8 T Cell ◽  
T Cell Lymphoma ◽  
Aggressive Clinical Course

scholarly journals An Unusual Case of Hepatosplenic αβ T-Cell Lymphoma Presenting with Coombs'-Negative Hemolytic Anemia

2015 ◽  
Vol 9 ◽  
pp. CMO.S35120 ◽  
Author(s):  
Feryal A. Ibrahim ◽  
Vignesh Shanmugam ◽  
Aliaa Amer ◽  
Halima El-Omri ◽  
Ahmad Al-Sabbagh ◽  
...  
Keyword(s):  
T Cell ◽  
Hemolytic Anemia ◽  
Cell Lymphoma ◽  
Treatment Options ◽  
Cell Receptor ◽  
T Cell Lymphoma ◽  
Γδt Cell ◽  
T Cell Lymphomas ◽  
Dismal Prognosis ◽  
Hepatosplenic T Cell Lymphoma

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive extranodal T-cell lymphoma that comprises <5% of peripheral T-cell lymphomas. The majority of cases harbor the γδT-cell receptor (TCR), but recently, a few cases have been shown to express the αß TCR. Comparison of these two subtypes (αβ and γδ) shows similar clinicopathologic and cytogenetic features; however, due to the paucity of reported cases, it is not clear whether they are prognostically distinct entities. We report a case of αβ HSTCL with a rather unusual presentation of Coombs'-negative hemolytic anemia. Diagnosis proved challenging due to an unusual blastoid morphology with the absence of typical intrasinusoidal distribution of tumor cells in the bone marrow. This unique case adds to the growing list of this rare subtype of T-cell lymphomas, which warrant urgent attention due to the lack of effective treatment options and dismal prognosis.

scholarly journals Peripheral Blood Involvement By Flow Cytometry As a Prognostic Factor in Aggressive T Cell Lymphomas Following Autologous Stem Cell Transplantation

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4055-4055
Author(s):  
Namrata S Chandhok ◽  
Scott F. Huntington ◽  
Iris Isufi ◽  
Lohith Gowda ◽  
Mina L Xu ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Flow Cytometry ◽  
T Cell ◽  
Peripheral Blood ◽  
Cell Lymphoma ◽  
Bone Marrow Involvement ◽  
T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
First Remission ◽  
Peripheral Blood Involvement

Introduction: Aggressive T cell lymphomas (TCL) are a heterogenous group of lymphomas that are frequently associated with poor outcomes. Autologous stem cell transplantation (ASCT) is recommended according to the NCCN guidelines and by practice standards for most subtypes as a consolidation for patients in first remission. A large prospective study of up-front ASCT by the Nordic Lymphoma group identified age, ECOG performance status <2, and bone marrow involvement as important prognostic factors. We have identified peripheral blood involvement by flow cytometry at diagnosis in up to one third of patients with aggressive TCL and analyzed whether this was a prognostic factor for outcomes after ASCT. Methods: We retrospectively analyzed data from consecutively treated patients (pts) with aggressive T-cell lymphomas who underwent ASCT at our institution from July 2009 to February 2019. Patient and disease characteristics were summarized using descriptive statistics. Kaplan-Meier analysis was used to estimate progression free survival (PFS) that was defined as the time from SCT to the first evidence of recurrence, and overall survival (OS) that was defined as the time from SCT to death or last institutional follow up with a hematologist. We collected data on age, co-morbidities, disease subtype, stage, response to therapy and treatment both pre and post SCT. Flow cytometry was obtained at diagnosis and phenotype of atypical circulating cells was compared with immunophenotype from tumor biopsy specimens. Results: 50 pts with TCL who received ASCT were identified for this analysis. Of this population, 41 (80%) of pts had peripheral blood flow available at the time of initial diagnosis. T-cell lymphoma types included peripheral T cell lymphoma not otherwise specified (PTCL NOS, 17 pts), angioimmunoblastic T cell lymphoma (AITCL, 15pts), ALK negative anaplastic large cell lymphoma (ALCL, 1pt), enteropathy-type T-cell lymphoma (EATL, 2pts), extranodal natural killer T-cell lymphoma (NKTCL, 2pts) and panniculitis like T cell lymphoma (2 pts) (Table 1). Median age of the cohort was 62 years (range 20-75 years) and all patients included had an ECOG PS 0-1 at the time of diagnosis. The majority had stage 4 disease (36/41, 87.8%), but analysis included a small number of patients with stage 2 (1/41, 2.4%) and stage 3 (4/41,9.7%) disease. Bone marrow involvement by morphologic criteria was noted on bone marrow biopsy in 8/41 (19.5%) pts; bone marrow was negative in 28/41 or 61% pts and not evaluated in 8/41 or 19.5% pts. Flow cytometry of peripheral blood performed as part of initial staging was positive for circulating malignant cells in 13/41 pts (31.7%) at the time of diagnosis. All patients underwent ASCT in first remission. The median PFS and OS were 15.2 and 29.9 months respectively in the flow positive group, while neither median PFS nor OS were reached in the flow negative group (Figures 1 and 2). Flow cytometry results from time of diagnosis was not strongly associated with PFS (log rank, p = 0.39), however, it was associated with overall survival (log rank, p = 0.012). There were 11 deaths in the cohort- 4 in the flow negative group and 7 in the flow positive group. Further, when bone marrow involvement was evaluated, 7 of 13 pts with positive flow cytometry (53.8%) and 5 of 28 (17.8%) pts with negative flow cytometry had BM involvement, suggesting a correlation between positive bone marrow and detection of lymphoma cells in the peripheral blood at the time of diagnosis. Conclusions: We demonstrate in our cohort of patients that detection of circulating lymphoma cells at diagnosis by flow cytometry was associated with a worse outcome in patients with aggressive T cell lymphomas undergoing ASCT as a consolidation in first remission. Larger cohorts will be needed to validate these findings, but these results suggest peripheral blood involvement by sensitive flow cytometry may identify patients with worse outcomes who might benefit from a more aggressive strategy such as allogeneic stem cell transplantation or alternative consolidation strategies. Disclosures Huntington: Bayer: Consultancy, Honoraria; Pharmacyclics: Honoraria; Celgene: Consultancy, Research Funding; DTRM Biopharm: Research Funding; Genentech: Consultancy; AbbVie: Consultancy. Isufi:Celgene: Consultancy; Novartis: Consultancy; Astra Zeneca: Consultancy. Foss:Seattle Genetics: Consultancy, Other: fees for non-CME/CE services ; Mallinckrodt: Consultancy; miRagen: Consultancy; Spectrum: Other: fees for non-CME/CE services ; Eisai: Consultancy; Acrotech: Consultancy.

Primary cutaneous CD4+ small/medium T-cell lymphoma with aggressive clinical course

2014 ◽  
Vol 55 (4) ◽  
pp. 304-306 ◽  
Author(s):  
Masahito Yasuda ◽  
Naoya Igarashi ◽  
Yayoi Nagai ◽  
Atsushi Tamura ◽  
Osamu Ishikawa
Keyword(s):  
T Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Aggressive Clinical Course

scholarly journals THERAPY OF THE RESISTANT FORMS OF SUBCUTANEOUS PANNICULITIS-LIKE T-CELL LYMPHOMA

2019 ◽  
Vol 64 (3) ◽  
pp. 353-361
Author(s):  
L. G. Gorenkova ◽  
S. K. Kravchenko ◽  
M. A. Silaev ◽  
N. V. Ryzhikova
Keyword(s):  
T Cell ◽  
Clinical Course ◽  
Cell Lymphoma ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Lymphoproliferative Disorders ◽  
T Cell Lymphoma ◽  
Aggressive Clinical Course ◽  
Rare Group

Introduction. Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) belongs to a rare group of skin lymphoproliferative disorders, which is characterised by panniculitis-like clinical manifestations, the α/β cytotoxic immunophenotype of tumour cells, as well as a multidirectional course from indolent to aggressive forms.Aim. To examine patients with SPTCL having an aggressive clinical course and characterised by refractoriness to several lines of chemotherapy.Results. We present two case reports of patients with a generalised lesions and unfavourable prognostic factors, who achieved complete long-term remission of the disease as a result of gemcitabine chemotherapy.Conclusion. Despite the fact that both observed SPTCL patients demonstrated refractoriness to at least three types of treatment, the use of gemcitabine allowed long-term complete remissions of the disease to be achieved. 

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