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Peripheral T-cell lymphoma

  

About Lymphoma > Types of Lymphoma > T-cell > Peripheral T-cell Lymphoma

Last update: 05/29/2008

Resources | Topic Searches  | Clinical Trials | Research News

Peripheral T-cell lymphomas describes a diverse group of blood cancers that originate from T-cells, which may be at various stages of  development.  These lymphomas usually presents at diagnosis in stage III or IV, and often has an aggressive clinical course requiring prompt treatment.  As with all lymphomas the molecular characteristics of the cells, and the stage of development are variable and therefore clinical course can be unique. (See About Lymphoma, simplified.)

Gene expression profiling identifies molecular subgroups among nodal peripheral T-cell lymphomas. Oncogene. 2006 Mar 9;25(10):1560-70. PMID: 16288225

Incidence: More frequent in adults than children. Low incidence - approximately 15-20% of non-Hodgkin's lymphoma.  Uncommon in North America. Peripheral T-Cell Lymphoma Unspecified is the most common group. Also see specific variants (subtypes), below.

Staging: Staging refers to the how widespread the disease is. Imaging tests (CT MRI, PET, Gallium). 
See Staging for more detail.

Diagnosis requires analysis of tissue sample using a variety of tests to identify the cell type.  T-cell markers will "express CD4 or CD8, but not both." 1

Prognosis:  Each lymphoma is unique and the prognosis can depend on many clinical
factors, including the unique molecular and biological characteristics of the tumor, the patient's age, general health and immune status, areas of involvement, how widespread the disease is at diagnosis (stage), and if so-called b-symptoms are present. 

Also see Prognostic Markers for articles on biomarkers that may predict risk of disease

Treatment:  Combination chemotherapy, such as CHOP.  For investigational approaches, see below.  

References and resources
 

  1. Treatment policy prepared by Dr. Kirsty Tompkins  www.tsrcc.on.ca T-Cell.pdf 

  2. About: DoctorsDoctor | ASCO slide presentation | cancerbacup.org.uk

  3. Medscape Topic search:  Medscape (free login required)

  4. Extranodal types  hmds.org.uk

Also consider reading Guidelines at diagnosis for a checklist 
that pertains to all types of lymphoma.

Peripheral T-Cell Lymphoma, Examples of specific variants (subtypes)

Adult T-cell lymphoma/leukemia (with human T-cell leukemia virus type 1 [HTLV-1])
Aggressive NK-cell leukemia
Anaplastic large cell lymphoma, primary systemic type
Anaplastic large cell lymphoma, primary cutaneous type
Angioimmunoblastic T-cell lymphoma
CLL/prolymphocytic lymphoma
Granular lymphocytic leukemia
Hepatosplenic gamma/delta T-cell lymphoma  PubMed abstracts
Extranodal T-cell/NK-cell lymphoma, nasal type
Enteropathy-type intestinal T-cell lymphoma - See above
Mycosis fungoides/Sézary syndrome
Subcutaneous panniculitis-like T-cell lymphoma
Unspecified type (57%)

Resources 

PDQ -- for Health Professionals  NIH
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PubMed queries on this subtype of lymphoma

 Diagnosis | Review | Therapies | Prognosis
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Clinical Trials

ClinicalTrials.gov studies for T-cell lymphomas
Refractory disease - protocols for t-cell lymphomas, resistant to standard therapies
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Research News

 
NEW Insight: Gene expression analysis of peripheral T cell lymphoma, unspecified, reveals distinct profiles and new potential therapeutic targets jci.org (full text)

Notably, both phosphorylation of PDGFRα and sensitivity of cultured PTCL cells to imatinib (as well as to an inhibitor of histone deacetylase) were found. These results, which might be extended to other more rare PTCL categories, provide insight into tumor pathogenesis and clinical management of PTCL/U.

Clinical trial:  Gleevec (imatinib) in Relapsed/Refractory T Cell Non-Hodgkin's Lymphoma ClinicalTrials.gov
PTCL Prognostic marker: Expression of CYP3A4 as a predictor of response to chemotherapy in peripheral T-cell lymphomas. Blood. 2007 Nov 1;110(9):3345-51. Epub 2007 Jul 18. PMID: 17634410 

In conclusion, a high CYP3A4 tumoral expression could be useful to predict poor response to the standard PTCL chemotherapy; in these cases alternative chemotherapy combinations or doses should be explored.
Interim Response and Safety Analyses Support Continuation of PDX (pralatrexate) in Patients with Peripheral T-Cell Lymphoma  www.earthtimes.org

Results of the interim analysis of patient response data exceeded the pre-specified threshold for continuation of the trial, which required a minimum of four responses (complete or partial) out of the first 35 evaluable patients, as determined by independent oncology review.
Frontline Autologous Stem Cell Transplantation (Asct) In High-Risk Peripheral T-Cell Lymphoma (PTCL): A Prospective Study From The Gel-Tamo Study Group  blackwell-synergy.com 

Results: CR was achieved by 12 patients (46%) after 3 courses of MegaCHOP and 12 patients received IFE as a salvage therapy. After the ASCT (n=19), 89% of patients achieved CR. In contrast, 6 patients (23%) did not receive the transplant due to the progression of the disease (n=5) or lethal toxicity (n=1). One patient in first-line CR refused ASCT. 

After a median follow-up of 35 months, the OS and PFS at 3 years was 73% and 53%, respectively. Moreover, the OS, PFS and DFS were 84%, 56% and 63%, respectively 2 years after transplant in the patients who received ASCT consolidation (n=19). 

Conclusions: Early salvage therapy for patients with high-risk aggressive nodal PTCL that do not achieve CR after 3 courses of chemotherapy and ASCT frontline consolidation for chemosensitive patients may improve treatment outcome.
Long-term follow-up of patients with peripheral T-cell lymphomas treated up-front with high-dose chemotherapy followed by autologous stem cell transplantation.
Leukemia. 2006 Sep;20(9):1533-8. Epub 2006 Jul 27. PMID: 16871285 

... our findings indicate (1) up-front high-dose therapy and ASCT are feasible, but could induce a high rate of long-term CR only in patients with ALK-positive ALCL and (2) the achievement of CR before autografting is a strong predictor of better survival."
Autologous transplantation for relapsed or primary refractory peripheral T-cell lymphoma.
Br J Haematol. 2006 Jun 6; PMID: 16759221
Marker Expression in Peripheral T-Cell Lymphoma: A Proposed Clinical-Pathologic Prognostic Score.
J Clin Oncol. 2006 Apr 24; PMID: 16636342
Gene expression profiling identifies molecular subgroups among nodal peripheral T-cell lymphomas.
Oncogene. 2006 Mar 9;25(10):1560-70. PMID: 16288225
Direct comparisons of peripheral T-cell lymphoma with diffuse B-cell lymphoma of comparable histological grades--should peripheral T-cell lymphoma be considered separately? jco.org 
Stem cell transplantation for peripheral T-cell lymphomas. 
Leuk Lymphoma. 2004 Mar;45(3):441-6. Review. PMID: 15160904
Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients. Blood. 2003 Sep 15;102(6):2213-9. Epub 2003 May 15. PMID: 12750155 | More
Peripheral T-cell lymphoma (excluding anaplastic large-cell lymphoma): results from the Non-Hodgkin’s Lymphoma Classification Project  Annals of Oncology 13:140-149, 2002
Clinical features of peripheral T-cell lymphomas in 78 patients diagnosed according to the Revised European-American lymphoma (REAL) classification.
Eur J Cancer. 2002 Jan;38(1):75-81. PMID: 11750843  PubMed
Peripheral T-cell lymphoma [NK-type] Curr Oncol Rep. 2002 Sep;4(5):434-42. Review. PMID: 12162919  PubMed
Inhibitor of histone deacetylation, depsipeptide (FR901228), in the treatment of peripheral and cutaneous T-cell lymphoma: a case report.  Blood. 2001 Nov 1;98(9):2865-8. PMID: 11675364  PubMed

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For all medical concerns,  you should always consult your doctor. 
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