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T-cell lymphomas

 

 About NHL or Types of Lymphoma > T-Cell Types

Last updated: 12/21/2009

TOPICS
Introduction
| Types of T-cell Lymphomas | Clinical TrialsOutcome Abstracts   

TOPIC SEARCH: PubMed: Diagnosis | Review | Therapies | Prognosis  
ASCO | ASH | Medscape 

Introduction

T-cell and NK-cell Lymphomas

Cancers of t-cells or NK cell origin - (lymphocytes), which are are a family of blood cells that normally protect the body from disease, such as by eliminating our own cells that are infected with virus.

What is lymphoma?  See Lymphoma Simplified 

Incidence: "Non-Hodgkin's Lymphomas caused by malignant (cancerous) 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.

Comment on type of lymphoma and goal of therapy:   There are many types of t-cell lymphomas, based on the cell of origin and areas of presentation.  An accurate diagnosis will allow you to choose the most appropriate therapy.  For example,  for a lymphoma type with an aggressive clinical course, therapy with curative intent can be most appropriate. Otherwise, a management approach might be chosen - treating as needed with less toxic protocols.   See also Factors that Influence Treatment

Types of T-cell and Natural Killer Cell lymphomas

based on stage and area of presentation (adapted from WHO)

Review article: Surviving Adult T-cell Leukemia/Lymphoma  http://bit.ly/surv-t-cell

By Developmental stage 
of cell of origin

Subtypes

Early-stage (precursor)
cell types

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Blastic NK lymphoma Lymphoblastic  Lymphoma (aggressive) 

NK = natural killer cell 
Blastic = presenting in blood
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Precursor T/NK-cell Lymphoma (aggressive)

Mature-stage
cell types

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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Primary cutaneous (skin) 
CD30+ disorders: 

Anaplastic large-cell lymphoma (ALCL) (aggressive?)

Lymphomatoid papulosis

Extranodal  
presenting outside lymphatic system

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Intestinal: Enteropathy type  lymphoma (aggressive)
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Liver/spleen: Hepatosplenic T-cell Lymphoma (aggressive) PubMed
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Unspecified: Peripheral T/NK-cell lymphoma  (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
 

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)

Other names

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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)
Related 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

Related Articles
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Technical: Commentary on the 2008 WHO classification of mature T- and NK-cell neoplasms http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2725280&tool=pmcentrez 
CLASSIFYING non-Hodgkin's lymphomas makes sense for several reasons:
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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All studies
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Newly diagnosed or untreated 
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Recurrent
By Subtype (by request):
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Anaplastic Large Cell type: 
Newly diagnosed or  previously untreated
  AND Recurrent
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Angioimmunoblastic type | with stem cell transplant
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Cutaneous T-cell lymphomas
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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 professionals pdf 
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Antibodies for T-Cell NHL
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T-cell lymphomas UMDNJ Hematopathology  | LymphomaInfo.net 
About normal t-cells and NK cells (cells of origin)
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Normal T-cells  
GSU.edu
| Maturation - Virginia.eduand Cellular Immunity - Merck Manual 
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Normal NK cells  Users.rcn.com/jkimball.ma.ultranet
RESEARCH NEWS
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T-cell lymphomas  ASCO Topic Search

Adult T-cell Lymphoma/leukemia

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

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About  demon.co.uk 
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NEW 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
Anaplastic Large Cell Lymphoma
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We have moved this item here

Also see Childhood NHL - Anaplastic Large Cel


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)

 


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   


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.comLymphomaInfoNet 
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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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Treatment of Angioimmunoblastic T-Cell Lymphoma (AILD) with Cyclosporine. Session Type: Oral Session  ASH 2003
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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


Lymphoblastic Lymphoma 
(predominantly t-cell) / Blastic Lymphoma

T-cell lymphoblastic Lymphoma 

TOPIC SEARCH: 
ASCO
| Cancer.gov | ClinicalTrials.gov | Medscape | 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.  More commonly affects males than females. 

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

Presentation: 

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

Signs  (common to other conditions): 

bulletPainless swelling in the neck, armpit or groin, caused by enlarged lymph nodes.
bulletLoss of appetite and tiredness.

 
Resources

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About  cancerbackup.org.uk
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Treatment best practice NCCN.org  PDF
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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

 

 
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. 

CTCL


Enteropathy type T-cell lymphoma

 Also see Peripheral T- cell lymphoma below
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"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." Source: DoctorsDoctor

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About  DoctorsDoctor | hmds.org.uk
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A case study  phenopath.com 
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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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Enteropathy-type intestinal T-cell lymphoma: clinical features and treatment of 31 patients in a single center.  http://jco.ascopubs.org
Clinical Trials
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Phase II Trial of Depsipeptide in Patients with Cutaneous T-Cell Lymphoma and Relapsed Peripheral T-Cell Lymphoma Number: 01-C-0049  NIH.gov
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Expression of the granzyme B inhibitor, protease inhibitor 9, by tumor cells in patients with non-Hodgkin and Hodgkinlymphoma: a novel protective mechanism for tumor cells to circumvent the immune system?  BLOOD  (Scroll down to study in list.)

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 

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 
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
 
Estimating outcome & Survival
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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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For all medical concerns,  you should always consult your doctor. 
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