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Common Types | Resources |
Research News
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SEARCH: ASCO
| Cancer.gov
| Clinical Trials
| Medscape
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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. 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)
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Mycosis
Fungoides MF (WHO and EORTC)
Variants of MF: Folliculotropic Mycosis Fungoides | Granulomatous
Slack Skin
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Sézary
syndrome (WHO and EORTC)
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| Subcutaneous
panniculitis-like (WHO and EORTC)
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| Anaplastic large cell
lymphoma (WHO) - PC CD30+ lymphoproliferative disorders, LyP/PCALCL
/ CD30+ Large cell lymphoma (EORTC) / Lymphomatoid
Papulosis (EORTC)
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Primary
cutaneous peripheral T-cell lymphoma, unspecified (WHO) /
Pleomorphic cell lymphomas (EORTC)
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| Primary
Cutaneous Anaplastic Large Cell Lymphoma
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| Extranodal
NK/T-cell (CD56+) lymphoma, nasal type (WHO) / CD30- large
cell lymphomas (EORTC)
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OTHER
TYPES:
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lymphomatoid
granulomatosis
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| granulomatous slack skin disease, |
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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
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IIB:
Cutaneous tumors (T3) |
|
III:
Erythroderma (T4) |
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IVA: Node
biopsy positive |
Advanced
state
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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
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| Tumor
Burden Index in Cutaneous T-Cell Lymphoma - Medal.org
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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:
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Protocols for Refractory Disease - PAL
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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.
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| 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"
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| 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
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| 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
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| 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
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|
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| Extracorporeal photopheresis PubMed
articles | ClinicalTrials.gov
Is thought to induce antigen processing cell activation and apoptosis
of CTCL cells
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| 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
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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.
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| 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.
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Lenalidomide / Revlimid (investigational) PubMed
articles | ClinicalTrials.gov
A potent immune modulating agent.
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|
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| 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."
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Resources
PubMed Queries on This Subtype of Lymphoma
Clinical Trials
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
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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.
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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.
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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
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|
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.
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| 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
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| 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
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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 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
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| 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
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|
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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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
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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
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Treatment of cutaneous
T cell lymphoma: current status and future directions.
Am J Clin Dermatol. 2002;3(3):193-215. Review.
PMID: 11978140 PubMed
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| 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
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| Treatment of cutaneous T cell lymphoma: current status and future directions. Am J Clin Dermatol. 2002;3(3):193-215. Review. PMID: 11978140
PubMed
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