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Types of Lymphoma > T-Cell Lymphoma

Last updated: 03/19/2018

TOPICS
Types of T-cell Lymphomas  | Introduction | Cooperative Research Centers
 Background Articles Clinical TrialsOutcome Abstracts  | In the News

TOPIC SEARCH: PubMed: Diagnosis | Review | Therapies | Prognosis  

See also

Lymphatic System

and Lymphoma Simplified

Introduction

Lymphoma is a blood cell cancer affecting lymphocytes - immune cells that normally protect our health by fighting infection.  Sometimes these specialized blood cells develop defects (mutations) that cause them to divide or persist longer than they should - forming tumors. The malignant lymphocytes may be t-cells (uncommonly) or b-cells.  One important role of normal b-cell lymphocytes is to make antibodies to fight infection.

See also Lymphoma Simplified

Incidence: "Non-Hodgkin's Lymphomas caused by malignant T-Cell lymphocytes represent a smaller subset (about 15% in the US) of the known types of non--Hodgkin's lymphoma - approximately 6,885 new cases diagnosed annually.

There are many types of t-cell lymphomas, based on the cell of origin and areas of presentation.  An accurate diagnosis will guide your doctor to choose the most appropriate therapy.  For example, for an aggressive lymphoma aggressive therapy with curative intent can be most appropriate approach to treatment. Otherwise, a management approach might be chosen - treating as needed with less toxic protocols.

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Types of T-cell Lymphomas

The different types of lymphoma are determined according to what type of lymphocyte has become cancerous and the maturation stage of the abnormal cell. 

Most Common

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Mycosis fungoides (MF) (indolent)
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Transformed MF (aggressive)
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Sézary's syndrome (indolent)
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Peripheral T-cell Lymphoma, unspecified (aggressive)
The most common t-cell lymphoma - 57%

NK and Blastic (aggressive)

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Blastic NK-Cell  lymphoma

Lymphoblastic  Lymphoma
 

NK = natural killer cell 
Blastic = presenting in blood

Precursor  (immature cell -  aggressive)

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Precursor T/NK cell leukemia/lymphoma

Cutaneous Presenting in skin

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Mycosis fungoides (MF) (indolent)
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Transformed MF (aggressive)
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Sézary's syndrome (indolent)
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Subcutaneous panniculitis-like T-cell lymphoma
onlinelibrary.wiley.com
 
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Primary cutaneous (skin) CD30+ disorders: 
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Anaplastic large-cell lymphoma
(ALCL) aggressive
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Lymphomatoid papulosis

Extranodal   Presenting outside lymph system

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Enteropathy type
Intestinal (aggressive)
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Hepatosplenic T-cell Lymphoma  PubMed
Liver/spleen: (aggressive)
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Peripheral T/NK-cell lymphoma
Unspecified (aggressive) 
 (see also Nodal) 
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Nasal: T/NK cell lymphoma  (aggressive)
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Subcutaneous:  panniculitis T-cell Lymphoma (aggressive)

Subcutaneous = under skin
Panniculitis = layer of subcutaneous fat under epidermis of the skin.


About  Doctorsdoctor.com

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Nodal  Presenting in lymph nodes

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Anaplastic large-cell lymphoma,
primary systemic type (aggressive)
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Angioimmunoblastic T-cell lymphoma
(aggressive)
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Peripheral T-cell Lymphoma, unspecified (aggressive) (57%)

Leukemic  Presenting in blood

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Adult T-cell lymphoma/leukemia
(HTLV1+) (aggressive)

HTLV = human t-cell leukemia virus
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T/NK cell leukemia/lymphoma (aggressive)
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T-cell large granular lymphocytic leukemia 
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T-cell prolymphocytic leukemia (T-PLL)

Blood, 2012: How I treat prolymphocytic leukemia by Claire Dearden bloodjournal.hematology

This is a rare type of lymphoma with an aggressive clinical course - however it sometimes behaves indolently and can be observed instead of treated initially sometimes for as long as 2 years. "

Topic Search: PubMed
Trial Search: ClinicalTrials.gov

Other

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CLL/prolymphocytic lymphoma
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Also see NK cell Lymphoma
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Gamma-delta T-cell phenotype
(provisional subtype)
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Lymphomatoid T-cell lymphoma
(uncertain malignant status)

 

Image comparing outcomes based on subtype

 

 

 

Abbreviations

 ALCL = anaplastic large cell lymphoma 
AITL Angioimmunoblastic T-cell lymphoma
Blastic = presenting in blood 
Cutaneous = presenting in skin 

Extranodal = other than lymph nodes
 HTLV = human t-cell leukemia virus
 Leukemic = malignant leukocyte, presenting in blood
Lymphoma = malignant lymphocyte, belonging to lymphatic system 

T-PLL = T-cell prolymphocytic leukemia | 
MF = mycosis fungoides
Neoplasms = abnormal cell growth and survival (malignant)
Nodal = involving lymph nodes
NK = natural killer cell

Precursor =  cell in early phase of maturity
PTCL = peripheral t-cell lymphoma NOS = Not otherwise specified


Background Articles

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New concept:
Off-the-shelf anti-CD7 CAR T-cells for T-cell malignancies -  www.youtube.com
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10/16: Dr. Horwitz video interview on
Combination CHOP Regimens in Peripheral T-cell Lymphoma http://bit.ly/2dLKXdJ
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Cheah: Novel Treatments for T-Cell Lymphoma | 2015 Educational Book | Meeting Library 2015 http://bit.ly/1Q6ulJK

Table of Novel agents for t-cell lymphoma From meetinglibrary.asco.org 2015: http://bit.ly/1Q6tFUQ

Table of doublet studies for t-cell lymphoma From meetinglibrary.asco.org 2015: http://bit.ly/1ePpY57
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Blood 2014: Alison J. Moskowitz, Matthew A. Lunning and Steven M. Horwitz
How I treat the peripheral T-cell lymphomas http://bit.ly/1k8AW1Q

Technical -- but for anyone diagnosed with PTCL ... a very important paper to look over and share with our oncologists - underlining the potential importance of consulting specialists in this area.

One of the more challenging aspects in managing PTCL is confidently arriving at the diagnosis. T-cell lymphomas are rare, and we try to approach new diagnoses with at least a little skepticism. For many B-cell lymphomas, reproducible immunophenotypic patterns and cytogenetic features allow for an algorithmic approach to diagnosis. On the contrary, T-cell lymphomas are often characterized by antigen aberrancy that may vary within a subtype or even over the course of disease.7,8

The most powerful predictor of outcome in our series was interim PET; 53% of patients normalized their PET after 4 cycles of chemotherapy and in those who achieved interim PET-negative status, 59% were progression free at 5 years, including 53% of those with IPI of ≥3.

Our preference for fit patients is for alloSCT in the relapsed/refractory setting. Our institutional data and others have shown that the use of autologous stem cell transplantation for relapsed PTCL, with a possible exception of ALCL, has rarely resulted in long-term disease control.40,41 This is somewhat controversial and a recent analysis from the Center for International Blood and Marrow Transplant Research registry points to better results with ASCT at relapse, although the series is overrepresented by ALCL including many with ALK-positive ALCL.42
 
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CCR, 2010:
New Strategies in Peripheral T-Cell Lymphoma: Understanding Tumor Biology and Developing Novel Therapies http://clincancerres.aacrjournals.org/content/16/23/5608.full
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Latif and Morris:
Monoclonal Antibody Therapy of T-Cell Leukemia and Lymphoma:  http://bit.ly/WimCgm
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ASH 2012 - T-cell lymphoma abstracts compiled by PAL
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ASCO 2012 - T-cell lymphoma abstracts compiled by PAL
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ISRN Hematol. 2011; A Systematic Review and Meta-Analysis of Front-line Anthracycline-Based Chemotherapy Regimens for Peripheral T-Cell Lymphoma
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T-cell - Cancer: Increasing incidence of Enteropathy-associated T-cell lymphoma in  US, 1973-2008.
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T-Cell - Weill Cornell, Dr. Ruan: ASH 2011: New Treatment for T-Cell Lymphoma
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2010 - Biol Blood Marrow Transplant: Long-Term Results Of Autologous Hematopoietic Cell Transplantation For Peripheral T Cell Lymphoma: The Stanford Experience  

"The pre-transplant factors that impacted survival were disease status and the number of prior regimens. Histology, age, sex, stage, B symptoms, bone marrow involvement, and duration of first response did not significantly affect PFS or OS.

Based on these results, AHCT as consolidation therapy in first complete or partial response may offer a durable survival benefit.

However, AHCT with conventional salvage chemotherapy has minimal durable benefit in patients with relapsed or refractory PTCL, and thus novel strategies and/or allogeneic HCT should be more aggressively explored in lieu of AHCT for relapsed/ refractory PTCL."
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Technical: Commentary on the 2008 WHO classification of mature T- and NK-cell neoplasms
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Surviving Adult T-cell Leukemia/Lymphoma 
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ASH 2010 Abstracts  


Centers

Engaged in Cooperative Research in T-cell Lymphomas

North America

Europe

Asia

 

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North American Centers
 
Engaged in Cooperative Research: T-cell Lymphomas

Australia,  Radiation Oncology, Peter MacCallum Cancer Centre, East Melbourne

Canada British Columbia Cancer Agency, Vancouver, BC

Canada Medical Oncology and Hematology, Princess Margaret Hospital, Toronto

Canada Medical Oncology, British Columbia Cancer Agency, Vancouver, BC

Canada Pathology, British Columbia Cancer Agency, Vancouver, BC

CA City of Hope National Medical Center, Duarte

CA Stanford School of Medicine, Stanford

CA UCLA, Los Angeles

CT Yale Medical Oncology, New Haven

FL H. Lee Moffitt Cancer Center and Research Institute, Tampa

GA Emory University Hospital, Atlanta

GA Winship Cancer Institute, School of Medicine, Emory University, Atlanta

IA University of Iowa, Iowa City

MA Dana-Farber Cancer Institute, Boston

MO Washington University School of Medicine, St. Louis

NE Hematology/Oncology, University of Nebraska Medical Center, Omaha

NE Pathology, University of Nebraska Medical Center, Omaha

NJ The John Theurer Cancer Center at Hackensack University Medical Center, Hackensack

NY Department of Medicine, NYU Cancer Institute, NYU Langone Medical Canter, New York

NY Hematology/Oncology, New York Univ. Clinical Care Center, New York

NY Hematology/Oncology, NYU Langone Medical Center, New York

NY Memorial Sloan-Kettering Cancer Center, New York

NY New York University Cancer Institute, New York

NY Univ. of Rochester Med. Ctr., Rochester

PA Fox Chase Cancer Center, Philadelphia

TX Dermatology, UT MD Anderson Cancer Center, Houston

TX Institute for Drug Development, CTRC at UT Health Science Center at San Antonio

TX University of Texas MD Anderson Cancer Center, Houston

WA University of Washington, Seattle

 


European Centers
Engaged in Cooperative Research in T-cell Lymphomas

Department of Cancer Epidemiology, Lund University, Lund, Sweden
Department of Haematology, Midwestern Regional Hospital, Limerick. Ireland, Limerick, Ireland
Department of Hematology, Aarhus University Hospital, Aarhus C, Denmark
Department of Hematology, Karolinska Institutet, Stockholm, Sweden
Department of Internal Medicine, Vejle Hospital, Vejle, Denmark
Department of Medicine, Kuopio University Hospital, Kuopio, Finland
Department of Oncology, Karolinska University Hospital, Karolinska, Sweden
Department of Oncology, Lund University Hospital, Lund, Sweden
Department of Oncology, Oslo University Hospital, Oslo, Norway
Department of Oncology, Oulu University Hospital, Oulu, Finland
Department of Oncology, St. Olav University Hospital Tronheim, Tronheim, Norway
Department of Oncology, Ullevål Oslo University Hospital, Ullevål, Norway
Department of Oncology, Umeå University Hospital, Umeå, Sweden
Department of Oncology, Uppsala University Hospital, Uppsala, Sweden
Department of Pathology, Copenhagen University Hospital, Copenhagen, Denmark
Department of Pathology, Oslo University Hospital, Oslo, Norway
Department of Pathology, Tampere University Hospital, Tampere, Finland
Dept. of Hematology, Copenhagen University Hospital, Copenhagen, Denmark
Dept. of Pathology, Uppsala University Hospital, Uppsala, Sweden
Hospices Civils de Lyon, Lyon, France
Hospital Universitario, Salamanca, Spain
Institue of Cancer Resch/Royal Marsden, Sutton, Surrey, England
Institue of Cancer Resch/Royal Marsden, Sutton, Surrey, England
Klinikum Nuernberg, Nuernberg, Germany
Royal North Shore Hospital, St Leonards, Australia
TopoTarget, Copenhagen, Denmark
University Hospital Cologne, Cologne, Germany
University Hospital of Lille, Lille, France
University Hospital Rigshospitalet, Copenhagen, Denmark

 

Asians Centers
Engaged in Cooperative Research in T-cell Lymphomas

Clinical Pathology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
Department of Hematology and Molecular Medicine, Atomic Bomb Disease Institute, Nagasaki University Hospital, Nagasaki, Japan
Department of Hematology and Oncology, Nagoya Daini Red Cross Hospital, Nagoya, Japan
Department of Hematology, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan
Department of Medical Oncology and Immunology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
Dept. of Hematology, Imamura Bun-in Hospital, Kagoshima, Japan
Development Division, Kyowa Hakko Kirin Co., Ltd., Chiyoda-ku, Japan
Graduate School of Med. Bioregulatory Medicine, Ehime University, Toon, Japan
Hematology & Cell Therapy, Aichi Cancer Center, Nagoya, Japan
Hematology and Immunology, NTT West Kyushu Hsp, Kumamoto, Japan
Hematology and Stem Cell Transplantation Division, National Cancer Center Hospital, Tokyo, Japan
Hematology, Kumamoto Medical Ctr, Kumamoto, Japan
Hematology, Nagasaki Medical Ctr, Omura, Japan
Hematology, Oita Prefectural Hsp, Oita, Japan
Int Med, Japanese Red Cross Nagasaki Genbaku Hsp, Nagasaki, Japan
Internal Medicine, Division of Hematology and Oncology, Fukuoka University, Fukuoka, Japan
Medicine and Biosystemic Science, Kyushu University Hospital, Fukuoka, Japan
School of Medicine Dept. of Hem., Kumamoto Univ., Kumamoto, Japan

 

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CLASSIFYING  lymphomas:
The categories appear to correspond to biological entities that behave distinctly. 
Gives the clinician important guidance for treating the lymphoma
Helps assess prognosis.
Helps  pathologist to recognize a lymphoma.
Helps discover important biological principles that underlie their appearance and behavior.
SOURCE: - 
UMDNJ Hematopathology
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CLINICAL TRIALS

All T-cell Types:
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Newly diagnosed or untreated 
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Recurrent
By Subtype:
Anaplastic Large Cell type: 
Newly diagnosed or  previously untreated
  AND Recurrent
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Angioimmunoblastic untreated  | relapsed | with stem cell transplant
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Cutaneous T-cell lymphoma untreated  | relapsed
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NK Cell 
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PTCL (Peripheral T-cell Lymphoma) 
 


RESOURCES

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Non-Hodgkins Lymphomas Clinical Practice Guidelines in Oncology – v.1.2006  NCCN.org  PDF  
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Antibodies for T-Cell NHL
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T-cell lymphomas UMDNJ Hematopathology  
About normal t-cells and NK cells (cells of origin)
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Normal T-cells  
 
GSU.edu | Maturation - Virginia.edu |
and Cellular Immunity - Merck Manual 
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Normal NK cells  Users.rcn.com/jkimball.ma.ultranet

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In the News:

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New concept:
Off-the-shelf anti-CD7 CAR T-cells for T-cell malignancies - 
www.youtube.com
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Belinostat in Relapsed or Refractory Peripheral T-Cell #Lymphoma: Results of the Pivotal Phase II BELIEF Study bit.ly/1V9Y1by
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2015 Blood Journal |
Engineered T cells CAN fight malignant T cells http://bit.ly/1Udu30z  more study needed however.
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Cheah: Novel Treatments for T-Cell Lymphoma | 2015 Educational Book | Meeting Library 2015 http://bit.ly/1Q6ulJK

Table of Novel agents for t-cell lymphoma From meetinglibrary.asco.org 2015: http://bit.ly/1Q6tFUQ

Table of doublet studies for t-cell lymphoma From meetinglibrary.asco.org 2015: http://bit.ly/1ePpY57

 

Adult T-cell Lymphoma/leukemia

 
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About  demon.co.uk 
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Encouraging report: Arsenic trioxide, interferon alpha, and zidovudine in newly diagnosed adult T-cell leukemia/lymphoma (ATL). http://bit.ly/3t5gvg 

n = 10 newly diagnosed ATL

An impressive 100 percent response rate was observed including 7 complete remissions, 2 complete remissions but with more than 5% circulating atypical lymphocytes, and 1 partial response. Responses were rapid and no relapse was noted. Side effects were moderate and mostly hematologic. In conclusion, treatment of chronic ATL with arsenic, interferon-alpha, and zidovudine is feasible and exhibits an impressive response rate with moderate toxicity.

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Bay 11-7082 inhibits transcription factor NF-kappaB and induces apoptosis of HTLV-I-infected T-cell lines and primary adult T-cell leukemia cells. Blood. 2002 Sep 1;100(5):1828-34. PMID: 12176906  PubMed

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Anaplastic Large Cell Lymphoma

 
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We have moved this item here

Also see Childhood NHL - Anaplastic Large Cell


T-cell large granular lymphocytic leukemia (LGL)

 
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"LGL exhibits an unexplained, chronic elevation in large granular lymphocytes (LGLs) in the peripheral blood.

It is also known by the following terms: Proliferation of large granular lymphocytes (LGLs), LGL leukemia, Tγ-lymphoproliferative disorder, T-cell chronic lymphocytic leukemia"  1 

LGL has an indolent clinical course mortality is uncommon (rarely fatal).2

"Some patients with mild cytopenias (low blood counts) and/or lymphocytosis remain asymptomatic for a long period. Spontaneous remissions have been reported in some studies. In symptomatic cases (majority) treatment should be initiated. Being a rare malignancy, treatment recommendations are based on data coming from case reports and retrospective studies. Recurrent neutropenia and infections are the main concern in treatment of this disease." 3

LGL is "a clonal proliferation of cytotoxic T cells, which causes neutropenia, anemia, and/or thrombocytopenia. This condition is often associated with autoimmune disorders, especially rheumatoid arthritis, and other lymphoproliferative disorders". 4

Resources:

  1. Wikipedia.org 
  2. atlasgeneticsoncology.org/ 
  3. doctorslounge.com 
  4. theoncologist 

    (most comprehensive description)

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Angiocentric Lymphoma (nasal T-cell Lymphoma)

 
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TOPIC SEARCH: 
ASCO | Cancer.gov |
Clinical Trials | Medscape | PubMed

"Angiocentric lymphoma is a type of T-cell lymphoma that is associated with Epstein Barr virus (EBV) infection. It includes cancers that previously were labeled "lethal midline granulomas," "nasal T-cell lymphoma," and "lymphatoid granulomatosis." 

Angiocentric lymphoma typically is characterized by a destructive nasal or midline facial tumor that is accompanied by invasive infiltrate, a substance that penetrates the spaces between tissues. 

The patient's palate (roof of mouth) may be destroyed by the tumor, and he or she may have prominent swelling of the eyes and face. 

Other sites of involvement outside the lymph nodes (extranodal) are the skin, lungs, and central nervous system. This tumor often is very resistant to therapy and has a poor prognosis. It is much more common in Asians than in people of European descent." source:  oncologyChannel 
 

Resources & Research News

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Combined chemotherapy and radiation versus radiation alone in the management of localized angiocentric lymphoma of the head and neck. Radiother Oncol 2001; 61: 261-9. Abstract | Related articles   

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Angioimmunoblastic Lymphoma

 
Also see Peripheral T- cell lymphoma below

"While not always malignant, AILD commonly progresses to an AILD-like T cell lymphoma."

source: hsc.virginia.edu 

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PubMed TOPIC SEARCH 
Diagnosis | Prognosis | Review | Treatment 

Angioimmunoblastic T-cell lymphoma (AILD) is considered a variety of T-cell lymphoma, which usually occurs in adults. 

Symptoms include generalized lymphadenopathy (enlarged lymph nodes) and increased antibody proteins in the blood (hypergammaglobinemia).  Other symptoms are fever, skin rash and weight loss. 

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About emedicine.com  
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High-Dose Therapy (HDT) and Autologous Stem-Cell Transplantation (ASCT) in Angioimmunoblastic Lymphoma: Complete Remission at Transplantation Is the Major Determinant of Outcome  J Clin Oncol. 2007 Dec 10;   PMID: 18071187 

This study shows that HDT and ASCT offers the possibility of long-term disease-free survival to patients with AITL. Early transplantation is necessary to achieve optimal results.
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Clinical, biological and pathological features in 157 patients with angioimmunoblastic T-cell lymphoma treated within the Groupe d'Etude des Lymphomes de l'Adulte (GELA) trials. Blood. 2008 Feb 21; PMID: 18292286
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Angioimmunoblastic lymphadenopathy with dysproteinemia: 
emphasis on pathogenesis and treatment. Acta Haematol. 1998;99(2):57-64. Review. PMID: 9554450   PubMed
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Successful treatment of angioimmunoblastic lymphadenopathy with dysproteinemia with cyclosporin A. Cancer. 1992 May 15;69(10):2567-70. PMID: 1568181   PubMed
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Angioimmunoblastic lymphadenopathy (AILD) may respond to thalidomide treatment: two case reports. Leuk Lymphoma. 2002 Jan;43(1):133-7. PMID: 11908717  PubMed
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Angio-immunoblastic T cell lymphoma (AILD-TL) rich in large B cells and associated with Epstein-Barr virus infection. A different subtype of AILD-TL? Leukemia. 2002 Oct;16(10):2134-41. PMID: 12357368  PubMed

ClinicalTrials.gov listings of clinical trials:

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Angioimmunoblastic type | with stem cell transplant
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TIPS:
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Select Results on Map to find studies in your region for the above queries.
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Because these are uncommon lymphomas, it's important to consult experts at centers that specialize in this research and work cooperatively to do trials.  

See Centers

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Lymphoblastic Lymphoma 
(predominantly t-cell) / Blastic Lymphoma

Lymphoblastic Lymphoma 

TOPIC SEARCH:  PubMed

Lymphoblastic lymphoma is a very rare form of non-Hodgkin lymphoma in adults, accounting for less than 3 in 100 cases overall. However, it accounts for more than 1 in 3 of all cases occurring in children and teenagers.  This lymphoma more commonly affects males than females. 

It's a high grade (aggressive) lymphoma which arise from precursors of B or T-cells.  In 4 out of 5 cases the T-lymphocytes are affected. The vast majority (90%) are of immature T-cell lineage. 

Clinical Trials for Lymphoblastic (b or t cell):
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Previously Untreated
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Relapsed
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Relapsed, but ineligible for SCT
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TIPS:
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Select Results on Map to find studies in your region for the above queries.
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Because these are uncommon lymphomas, it's important to consult experts at centers that specialize in this research and work cooperatively to do trials.  

See Centers

Presentation: 

bullet The thymus gland, or lymph nodes deep within the chest
(the mediastinal lymph nodes) is often affected. 

Signs: (common to other conditions)

bullet Painless swelling in the neck, armpit or groin, caused by enlarged lymph nodes.
 
bullet Loss of appetite and tiredness.

 
Resources

bullet
About Lymphoblastic lymphoma  E-medicine
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Treatment best practice NCCN.org  PDF
(Login required. Now difficult to find on site)

Reports

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Nine-Year Survival of Lymphoblastic Lymphoma Patients (full text report)
ncbi.nlm.nih.gov
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Adult lymphoblastic lymphoma: a retrospective analysis of 92 patients under 61 years included in the LNH87/93 trials 
ncbi.nlm.nih.gov/
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Outcome with the hyper-CVAD regimens in lymphoblastic lymphoma
bloodjournal.hematologylibrary.org

"The results of the hyper-CVAD regimen in LL were very encouraging. Overall, 91% of patients achieved CR, and the estimated 3-year overall survival rate was 70%, with a PFS rate of 66% "

Maintenance included 6-mercaptopurine (6-MP), methotrexate, vincristine, and prednisone (
POMP) for 24 months.
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High-dose therapy and autologous stem-cell transplantation vs. conventional-dose consolidation/maintenance therapy as post-remission therapy for adult patients with lymphoblastic lymphoma ncbi.nlm.nih.gov
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Nelarabine induces complete remissions in adults with relapsed or refractory T-lineage acute lymphoblastic leukemia or lymphoblastic lymphoma: cancer and leukemia group B study 19801. Blood. 2007 Mar 7;
PMID: 17344466  
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FDA Advisory Committee Recommends Accelerated Approval of GlaxoSmithKline's Arranon (nelarabine) Injection  docguide.com
Orphan Drug Under Review for Children and Adults with T-cell Acute Lymphoblastic Leukemia (T-ALL) and T-cell Lymphoblastic Lymphoma (T-LBL)
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Favorable outcome for children and adolescents with T-cell lymphoblastic lymphoma with an intensive ALL-type therapy without local radiotherapy.
Ann Hematol. 2001;80 Suppl 3:B73-6.
PMID: 11757713

Blastic cell origin - T-cell lymphoma

"Blastic" generally refers to immature-looking cells that resemble cells known as a blasts.

  1. Genomic Alterations in Blastic Natural Killer/Extranodal Natural Killer-Like T Cell Lymphoma with Cutaneous Involvement - abstract ~ www.jidonline.org
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TIP:
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Because these are uncommon lymphomas, it's important to consult experts at centers that specialize in this research and work cooperatively to do trials.  

See Centers

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Cutaneous T-cell Lymphoma

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Cutaneous T cell lymphoma (CTCL) is a lymphoma of T cell (a type of white blood cell that is responsible for immune function) origin that affects the skin. 

See CTCL


Enteropathy type T-cell lymphoma

 
Also see
Peripheral T-cell lymphoma below
 

"This aggressive [intestinal] lymphoma almost always arises in the setting of celiac disease. Rarely the lymphoma may arise in extraintestinal sites. The most common location is the jejunum and presents as single or multiple tumors."

For uncommon lymphomas, it's important to consult experts at centers that specialize in this research and work cooperatively to do trials.  

See Centers specializing in T-cell lymphoma research

bullet
Blood 2014: Alison J. Moskowitz, Matthew A. Lunning and Steven M. Horwitz
How I treat the peripheral T-cell lymphomas http://bit.ly/1k8AW1Q

Technical -- but for anyone diagnosed with PTCL ... a very important paper to look over and share with our oncologists - underlining the potential importance of consulting specialists in this area.
 
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Malignant lymphomas in coeliac disease: evidence of increased risks for lymphoma types other than enteropathy-type T cell lymphoma. Gut 2005;54(1):54-59.  Full Text
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Blood 2011: Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project” http://bit.ly/UYpZdm
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Blood Journal | Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: a retrospective study by the EBMT http://bit.ly/1iiUBR9

In this study, the survival curves showed a plateau, suggesting that ASCT could be a curative approach in the majority of EATL patients who receive high-dose therapy in first CR or PR. The OS of 66% observed in patients transplanted in first CR or PR is about the same as OS of 68% at 5 years observed in the UK study in patients who completed the whole treatment schedule including ASCT.
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Combination chemotherapy followed by autologous stem cell transplant for enteropathy-associated T cell lymphoma. Br J Haematol. 2006 Nov 20;  PMID: 17116129 
 
Enteropathy-associated T cell lymphoma (EATL) is a rare entity associated with coeliac disease, with a poor prognosis due to perforation and gastro-intestinal bleeding during treatment, and a high relapse risk. Six patients were treated with two cycles of IVE (ifosphamide, etoposide, epirubicin), followed by two cycles of high-dose methotrexate (3 g/m(2)) with folinic acid rescue and a BEAM (carmustine, etoposide, cytarabine, melphalan) autograft. Enteral feeding was given throughout treatment. Four patients remain alive in complete remission at 1.83-4.32 years; two have relapsed. Given the historically poor outcome in these patients, this regimen appears very promising in the treatment of EATL.
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International T-cell lymphoma project. International Peripheral T-cell Lymphoma Project: International peripheral T-cell and NK/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol 2008;26(25):4124-4130. Abstract
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Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center.  http://jco.ascopubs.org

Clinical Trials

bullet
Find studies for this type of lymphoma on ClinicalTrials.gov - All studies

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Hepatosplenic

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About Hepatosplenic gamma-delta T-cell lymphoma  www.thedoctorsdoctor.com
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Long-Term Survival With Allogeneic Stem Cell Transplant and Donor Lymphocyte Infusion Following Salvage Therapy with Anti-CD52 Monoclonal Antibody (Campath) in a Patient with alpha/beta Hepatosplenic T-Cell Non-Hodgkin's Lymphoma. Leuk Lymphoma. 2004;45(8):1673-1675. PMID: 15370223 
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TIP:
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Because these are uncommon lymphomas, it's important to consult experts at centers that specialize in this research and work cooperatively to do trials.  

See Centers


NK-Cell Lymphoma

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 Go NK Cell Lymphoma

 


Peripheral T- cell lymphoma, unspecified

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  Go to Peripheral T-cell

 


Subcutaneous panniculitis-like T-cell lymphoma

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"Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon cutaneous lymphoma that has been proposed as a distinct clinicopathologic entity, but studies of SPTCL are limited."  Source: nci.nih.gov
 
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About  thedoctorsdoctor.com | archderm - PDF 
bullet
TIP:
bullet
Because these are uncommon lymphomas, it's important to consult experts at centers that specialize in this research and work cooperatively to do trials.  

See Centers

 

Resources & Research News

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Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature.
Am J Dermatopathol. 2001 Jun;23(3):206-15. Review. PMID: 11391101  PubMed
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Aggressive subcutaneous panniculitis-like T-cell lymphoma: complete remission with fludarabine, mitoxantrone and dexamethasone. Br J Dermatol. 2000 Aug;143(2):408-10.
PMID: 10951154   PubMed 
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Subcutaneous panniculitic T-cell lymphoma and cytophagic histiocytic panniculitis.
Australas J Dermatol. 2001 Aug;42(3):183-7. Review. PMID: 11488712  PubMed
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Subcutaneous T-cell lymphoma presenting as panniculitis in children: report of two cases. Pediatr Pathol. 1994 Jul-Aug;14(4):595-608. PMID: 7971579  PubMed
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Cutaneous T-cell lymphoproliferative disorders: approach for the surgical pathologist: recent advances and clarification of confused issues. Adv Anat Pathol. 2002 Mar;9(2):79-100. Review. PMID: 11917163  PubMed

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Estimating outcome & Survival

 

TOPIC SEARCH:- PubMed

Outcome abstracts:

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Multicenter study of pegylated liposomal doxorubicin in patients with cutaneous T-cell lymphoma. Cancer. 2003 Sep 1;98(5):993-1001. PMID: 12942567
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A single-centre study of treatment outcomes and survival in 120 patients with peripheral T-cell non-Hodgkin's lymphoma. Ann Hematol. 2002 May;81(5):267-72. PMID: 12029536  PubMed
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T-cell non-Hodgkin's lymphoma in adults: clinicopathological characteristics, response to treatment and prognostic factors. Leuk Lymphoma. 2002 Apr;43(4):805-11. PMID: 12153168  PubMed
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The use of anti-T-cell receptor-Vbeta antibodies for the estimation of treatment success and phenotypic characterization of clonal T-cell populations in cutaneous T-cell lymphomas. Br J Haematol. 2002 Sep;118(4):1019-26. PMID: 12199780  PubMed

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