Ask Question
Sign Guest book

 
About Lymphoma
| Advocacy & Art | CAM & Life Style | Clinical trials | Doctors & Centers  | Guidelines  at  Diagnosis | How  to   Help  | Research | Side Effects  | Support | Symptoms  | Tests | Treatments


WebCasts

T-cell subtype - cutaneous

  

About Lymphoma > Types > T-cell > Cutaneous (CTCL) / Mycosis Fungoides

Last update: 06/21/2008

Common Types | Resources | Research News

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

"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. Its symptoms vary. Often it is confined to the skin and can be treated and cured. Aggressive forms can extend beyond the skin to the lymph nodes, blood, and internal organs."  Source: Dermatology Channel

"T-cell lymphomas positive for the CD4 receptor constitute around 5% of Non Hodgkin's Lymphomas. The prevalence of mycosis fungoides (MF) is estimated at 16,000 to 20,000 in the US. MF patients tend to have a lifespan of 10 to 30 years and therefore may need treatment several times during the disease progression. 

Non-cutaneous T-cell lymphomas that are positive for the CD4 receptor are predominantly of the nodal subtype. This includes peripheral and Angioimmunoblastic T-cell lymphomas of which 75% are CD4 positive and anaplastic large cell lymphomas of which 20% are CD4 positive. 

The combined incidence of these lymphomas is approximately 2,770 in the US and Canada and 3,280 in industrialized Europe. Their prevalence in Europe is approximately 10,000 and in US and Canada it ranges from 8,000 to 10,000." prnewswire


Common Types  (by WHO-EORTC classification systems) 

Mycosis Fungoides MF (WHO and EORTC)
 
Variants of MF: Folliculotropic Mycosis Fungoides | Granulomatous Slack Skin

About - Medscape
 

Sézary syndrome  (WHO and EORTC)
 

About - Medscape 
 

Subcutaneous panniculitis-like (WHO and EORTC)
 

About - Medscape 
 

Anaplastic large cell lymphoma (WHO) - PC CD30+ lymphoproliferative disorders, LyP/PCALCL
/ CD30+ Large cell lymphoma (EORTC) / Lymphomatoid Papulosis (EORTC)

About - Medscape
 

Primary cutaneous peripheral T-cell lymphoma, unspecified (WHO) / Pleomorphic cell lymphomas (EORTC)
 

About - Medscape
 

Primary Cutaneous Anaplastic Large Cell Lymphoma
 

About - Medscape
 

Extranodal NK/T-cell (CD56+) lymphoma, nasal type (WHO) / CD30- large cell lymphomas (EORTC)
 

About - Medscape

OTHER TYPES:

lymphomatoid granulomatosis

About - Medscape 

granulomatous slack skin disease,

adult T-cell leukemia/lymphoma, 

pagetoid reticulosis 

Source: clfoundation.org

CTCL Staging

Early stage

IA : < 10% patch/plaque

IB: > 10% BSA patch/plaque

IIA: palpable adenopathy

Intermediate stage

IIB: Cutaneous tumors (T3)

III: Erythroderma (T4)

IVA: Node biopsy positive

Advanced state

Visceral involvement - "referring to the viscera, the internal organs of the body, specifically those within the chest (as the heart or lungs) or abdomen (as the liver, pancreas or intestines)." http://www.medterms.com 

 

Recommended Resources

The Cutaneous Lymphoma Foundation
About - eMedicine | dermnetnz.org | clfoundation.org | NCI PDQ | Review Article 
Tumor Burden Index in Cutaneous T-Cell Lymphoma - Medal.org

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

Treatments

Skin-directed treatments 
(2006 CAC NYC - Foss presentation)

Lesional skin only

High potency topical steriods

Bexaratene gel

Other: Excision; Imiquimod; Photdynamic; UVB laser

Lesional skin only

UVB

Topical HN2 or BCNU

Photochemotherapy

Radiotherapy

Related treatment resources:
Protocols for Refractory Disease  - PAL

 

Agents and Therapeutics
NEW:  Pegylated liposomal doxorubicin effective in poor prognosis CTCL  medwire-news

Of the 10 patients with Sézary syndrome, six (60%) patients achieved an objective response at the end of treatment, including one complete response and five partial responses. Five (50%) of 10 patients with transformed CTCL achieved an objective response, including one patient who achieved a complete response and was still disease-free after 3 years.
Phase 2 Data for Romidepsin Showing Durable Response in Refractory CTCL businesswire.com 

"Romidepsin is a novel, cyclic peptide, pan-HDAC inhibitor under investigation for hematologic malignancies"
ASH: Forodesine HCl active single oral agent for advanced refractory CTCL  abstracts2view.com  | ClinicalTrials.gov

Forodesine is a rationally designed, potent inhibitor of purine nucleoside phosphorylase (PNP) that leads to intracellular accumulation of dGTP and then apoptosis. ... Conclusion: Oral forodesine demonstrates clinical activity in subjects with refractory CTCL, including those with SS, with minimal toxicity to date. 
Treatment of mycosis fungoides using a 308-nm excimer laser: two case studies.
Dermatol Online J. 2006 Dec 10;12(7):11. PMID: 17459297 
506U78 (Nelarabine) Results of a phase II study of 506U78 in cutaneous T-cell lymphoma and peripheral T-cell lymphoma: CALGB 59901. Leuk Lymphoma. 2007 Jan;48(1):97-103. PMID: 17325852 | ClinicalTrials.gov
Anti-cd2 (Siplizumab) antibody (investigational)  ash05 | ClinicalTrials.gov

Siplizumab is a humanized IgG1κ class monoclonal antibody that binds to the CD2 receptor on 
human T- and NK-cells
Anti-cd30 (SGN-30) antibodies (investigational)  PubMed articles | ClinicalTrials.gov  

CD30 is a promising target for antibody-based immunotherapy of Hodgkin lymphoma (HL) and anaplastic large cell lymphoma.   PMID: 12881320 
Bexarotene  PubMed articles | ClinicalTrials.gov  
 
( beks-AIR-oh-teen) a retinoids (RET-i-noyds), applied to the skin Acts by interfering with the growth of cells of the tumor. It may be used after other drugs have been tried, and the tumor is still a problem. - MedlinePlus

Dose may be limited by cholesterol/TG elevations. Pretreatment with anti-lipid agents may be needed
Bexarotene with ONTAK  PubMed articles | ClinicalTrials.gov  
Extracorporeal photopheresis  PubMed articles | ClinicalTrials.gov
Is thought to induce antigen processing cell activation and apoptosis of CTCL cells
HDAC inhibitor: Depsipeptide  PubMed articles | ClinicalTrials.gov 

"Depsipeptide, FR901228, has demonstrated potent in vitro and in vivo cytotoxic activity against murine and human tumor cell lines. In the laboratory, it has been shown to be a histone deacetylase (HDAC) inhibitor. In a phase I trial of depsipeptide conducted at the National Cancer Institute, 3 patients with cutaneous T-cell lymphoma had a partial response, and 1 patient with peripheral T-cell lymphoma, unspecified, had a complete response. Sezary cells isolated from patients after treatment had increased histone acetylation. These results suggest that inhibition of HDAC is a novel and potentially effective therapy for patients with T-cell lymphoma."  PMID: 11675364 
Interleukin 12 Active in Mycosis Fungoides (a T-cell lymphoma)  cancerconsultants.com 

The current study involved 23 patients with mycosis fungoides, all of whom had received more than three prior therapies. These authors reported that 10 patients achieved a partial response (PR), 30% had minor responses and 22% had stable disease.
Immune modulation  PubMed articles | ClinicalTrials.gov
Immune therapy and immune modulation is likely most appropriate as an early treatment; 
not in advanced disease
Immune therapy  PubMed articles | ClinicalTrials.gov 
 
Improves durability of response in responders to combination therapy.
Lenalidomide / Revlimid  (investigational)  PubMed articles | ClinicalTrials.gov
 
A potent immune modulating agent. 
ONTAK (denileukin diftitox)  PubMed articles | ClinicalTrials.gov
Transimmunization (immune therapy)  Clinical trial: med.yale.edu 

"Transimmunization was developed after years of laboratory research advanced the understanding of the underlying principles of ECP (Extracorporeal, photochemotherapy, or photopheresis). 

The scientific basis of ECP is the ability to stimulate the development of powerful stimulators of the immune system called dendritic cells. Transimmunization is a more efficient means by which to bring these dendritic cells in contact with target cancer cells, before they are returned to the body to stimulate an anti-tumor immune response."

Resources 

Clinical abstracts  CTCLconsult.com
Lymphoma of the Skin (Nov_28_02) 
 
Joseph M. Connors, Eric D. Hsi and Francine M. Foss Abstract  asheducationbook.org
Scroll down to Section III for Cutaneous T-Cell Lymphoma
Photopheresis Followed by Immunotherapy Improves Responses in Cutaneous T-Cell Lymphoma www.ufscc.ufl.edu 

About Photopheresis  PAL

T-Cell Lymphoma Presenting as Benign Dermatoses - includes images  aafp.org
PDQ -- for Health Professionals NIH
Return to top

PubMed Queries on This Subtype of Lymphoma

 Diagnosis | Review | Therapies | Prognosis
Return to top

Clinical Trials

ClinicalTrials.gov studies for T-cell lymphomas
Return to top

Research News

Assessment
Assessment of tumor burden and treatment response by 18F-fluorodeoxyglucose injection and positron emission tomography in patients with cutaneous T- and B-cell lymphomas. J Am Acad Dermatol. 2002 Oct;47(4):623-8. PMID: 12271315
Treatment
Treatment of mycosis fungoides using a 308-nm excimer laser: two case studies.
Dermatol Online J. 2006 Dec 10;12(7):11. PMID: 17459297 

Our findings confirm previous observations that the 308-nm excimer laser is a safe, effective, and well-tolerated therapy for early stage MF.
Phase II Trial of Oral Vorinostat (Suberoylanilide Hydroxamic Acid, SAHA) for Refractory Cutaneous T-cell Lymphoma (CTCL). Blood. 2006 Sep 7; PMID: 16960145 | Related articles 

Eight (of 31) patients achieved a PR, including 7 with advanced disease and 4 with Sezary syndrome. The median Time to Response, Duration of Response, and Time to Progression for responders were 11.9, 15.1, and 30.2 weeks, respectively. Fourteen of 31 evaluable patients had pruritus relief. The most common drug-related AE were fatigue, thrombocytopenia, diarrhea, and nausea.
International multicenter phase II study of the HDAC inhibitor (HDACi) depsipeptide (FK228) in cutaneous T-cell lymphoma (CTCL): Interim report.  ASCO 2006 

Conclusions: The previously reported efficacy of depsipeptide in CTCL has also been seen in the present study. Duration of response is encouraging. Toxicity is manageable and the study continues to accrue.
About PUVA - Photopheresis is a process where your white blood cells are separated from the rest of your blood. The white blood cells are exposed the methoxsalen and UV light and then reinfused back into you.  rmhonline.com 
T-Cell Lymphoma Successfully Treated with Psoralen Plus UV-A Therapy  cancerconsultants.com
According to a recent article in the Archives of Dermatology, Psoralen plus UV-A (PUVA) is an effective treatment for patients with mycosis fungoides (MF), inducing long-term remissions and in some cases, disease cure.
Immunophenotypic and molecular features, clinical outcomes, treatments, and prognostic factors associated with subcutaneous panniculitis-like T-cell lymphoma: a systematic analysis of 156 patients reported in the literature. Cancer. 2004 Sep 15;101(6):1404-13.  PMID: 15368328 
HuMax-CD4 Phase II cutaneous T-cell lymphoma (CTCL) studies - 55% of Higher Dose Patients Achieve a Clinical Response in Primary Indication  prnewswire Apr 2004
Multicenter study of pegylated liposomal doxorubicin in patients with cutaneous T-cell lymphoma. Cancer. 2003 Sep 1;98(5):993-1001. PMID: 12942567
A complete and durable response to denileukin diftitox in a patient with mycosis fungoides. J Am Acad Dermatol. 2003 Feb;48(2):275-6. PMID: 12582402  PubMed
Enhanced expression of T-cell activation and natural killer cell antigens indicates systemic anti-tumor response in early primary cutaneous T-cell lymphoma. J Invest Dermatol. 1997 May;108(5):743-7. PMID: 9129226 PubMed
EPOCH for refractory T-cell NHL Chemotherapy with etoposide, vincristine, doxorubicin, bolus cyclophosphamide, and oral prednisone in patients with refractory cutaneous T-cell lymphoma. Cancer. 1999 Oct 1;86(7):1368-76. PMID: 10506727  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
Phase II trial of interferon-alpha-2a plus psolaren with ultraviolet light A in patients with cutaneous T-cell lymphoma. Cancer 2002; 95: 569-75.   PubMed
Treatment of relapsing or recalcitrant cutaneous T-cell lymphoma with pegylated liposomal doxorubicin. J Am Acad Dermatol. 2000 Jan;42(1 Pt 1):40-6. PMID: 10607318  PubMed
Treatment of cutaneous T cell lymphoma: current status and future directions. 
Am J Clin Dermatol. 2002;3(3):193-215. Review. PMID: 11978140  PubMed
Treatment of advanced mycosis fungoides by allogeneic stem-cell transplantation with a nonmyeloablative regimen. Bone Marrow Transplant. 2003 Apr;31(8):663-6. PMID: 12692606  PubMed
Treatment of cutaneous T cell lymphoma: current status and future directions. Am J Clin Dermatol. 2002;3(3):193-215. Review. PMID: 11978140  PubMed  
Return to top
 
Disclaimer:  The information presented on Lymphomation.org is not intended to be a substitute for 
professional medical advice or to replace your relationship with a physician.
For all medical concerns,  you should always consult your doctor. 
Patients Against Lymphoma, Copyright © 2004,  All Rights Reserved.