About
NHL or Types of Lymphoma > T-Cell
Types Last updated: 05/03/2009 |
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Introduction
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of T-cell Lymphomas |
Outcome Abstracts
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.
Four major types of T cell lymphoma: are peripheral
T cell lymphoma, T cell lymphoblastic lymphoma, cutaneous T cell
lymphoma, and adult T cell lymphoma. See Types
below.
What is lymphoma?
Briefly, lymphomas result when DNA
damage or changes occurs to an immune cell (a lymphocyte) that
alters the behavior of the cells. The damage to DNA results in the
abnormal production of proteins that prevents the cells from dying
when they should, or causes sustained rapid cell division that
produces more of its kind. These malignant cells then may accumulate
to form tumors in areas where the normal counterparts of the cell type
reside - lymph nodes, skin, mucosal linings, etc.
See Lymphoma Simplified for more detail.
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.
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Types of T-cell
and Natural Killer Cell lymphomas
We
have recently reworked the classifications and are developing resource
pages for each subtype.
We develop resources in response to visitor
questions
Resources for T-Cell and Natural Killer Cell
Lymphomas
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Cutaneous Types:
Cutaneous - not a subtype, but an
area (skin) of presentation,
such as Sézary's
syndrome (leukemia form of Mycosis fungoides)
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Subcutaneous Panniculitis-like T-cell lymphoma - thedoctorsdoctor.com
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OTHER
T cell and NK-cell neoplasms (Mature cell stage unless
"precursor cell" is indicated)
Lymphomatoid T-cell lymphoma (uncertain malignant status)
Extra nodal NK/T-cell lymphoma, nasal type
Primary cutaneous anaplastic large cell lymphoma
CLASSIFIED AS LEUKEMIAS
T-cell large granular lymphocytic leukemia
Precursor T-lymphoblastic lymphoma/leukemia (precursor cell)
"exhibits an unexplained, chronic (> 6 months) 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"
Wikipedia.org
T-cell prolymphocytic leukemia
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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.
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CLINICAL TRIALS
All T-cell Types:
By Subtype (by request):
RESOURCES
About normal t-cells and NK cells (cells of origin)
RESEARCH NEWS
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| Adult T-cell Lymphoma/leukemia |
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TOPIC
SEARCH: ASCO
| Cancer.gov
| Clinical Trials
| Medscape
| PubMed
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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
Cel
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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:
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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
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| 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
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"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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NEW: 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
"In multivariate analysis, only male gender (p=0.004),
mediastinal lymphadenopathy (p=0.041) and anemia (p=0.042)
adversely affected overall survival.
... In conclusion, AITL is a morphologically heterogeneous T-cell
lymphoma commonly expressing CXCL13 and CD10 and carrying few
prognostic factors. It portends a poor prognosis even when treated
intensively. However, not always lethal as 30% of patients are
alive at 7 years."
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Outcomes: 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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[626] 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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Cytologic findings of
angioimmunoblastic T-cell lymphoma: analysis of 16 fine-needle
aspirates over 9-year period. Cancer. 2002 Jun 25;96(3):166-73.
PMID: 12115305 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
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ClinicalTrials.gov listings of clinical trials:
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T-cell lymphoblastic Lymphoma
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T-cell lymphoblastic Lymphoma
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TOPIC
SEARCH: ASCO
| Cancer.gov
| ClinicalTrials.gov
| Medscape
| PubMed
A high grade lymphoma which arise from precursors of
B or T-cells. The vast majority (90%) are of immature T-cell lineage.
Most of these cases occur in children and adolescents.
| 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
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Blastic cell origin - T-cell lymphoma
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"Blastic"
generally refers to immature-looking cells that resemble cells known
as a blasts.
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Genomic Alterations in Blastic Natural Killer/Extranodal Natural Killer-Like T Cell Lymphoma with Cutaneous Involvement - abstract ~
www.jidonline.org
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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
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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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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
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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.)
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Hepatosplenic
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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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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
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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
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
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TOPIC SEARCH:- PubMed
Outcome abstracts:
| 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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