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NCI CANCERLIT® Search: Cutaneous T-cell Lymphoma - October 2002
National Cancer Institute®
Last Modified: October 1, 2002
- Follicular mucinosis associated with early stage cutaneous T-cell lymphoma: successful treatment with interferon alpha-2b and acitretin.
- A T-cell receptor gamma polymerase chain reaction assay using capillary electrophoresis for the diagnosis of cutaneous T-cell lymphomas.
- Mycosis fungoides involving the oral cavity.
- Treatment of stage IA cutaneous T-Cell lymphoma with topical application of the immune response modifier imiquimod.
- The interferon inhibiting cytokine IK is overexpressed in cutaneous T cell lymphoma derived tumor cells that fail to upregulate major
- Medical pearl: new views through the microscope.
- Induction of apoptosis by bexarotene in cutaneous T-cell lymphoma cells: relevance to mechanism of therapeutic action.
- Total skin electron beam therapy in mycosis fungoides. Our experience from 1985 to 1999.
- Primary cutaneous lymphoblastic lymphoma presenting in an 8-week old infant.
- Treatment of childhood mycosis fungoides with topical PUVA.
- Cold urticaria in a patient with mycosis fungoides.
- Assessment of tumor burden and treatment response by 18F-fluorodeoxyglucose injection and positron emission tomography in
- A prospective study on the evolution of the T-cell repertoire in patients with Sezary syndrome treated by extracorporeal photopheresis.
- Phase II trial of interferon-alpha-2a plus psolaren with ultraviolet light A in patients with cutaneous T-cell lymphoma.
- [Cutaneous T-cell lymphoma, cutaneous hyperpigmentation and paraneoplastic pemphigus]
- Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru.
Table of Contents
1
UI - 12243671
AU - Kontochristopoulos GJ; Exadaktylou D; Hatziolou E; Tassidou A;
TI -
Zakopoulou N
Follicular mucinosis associated with early stage cutaneous T-cell
lymphoma: successful treatment with interferon alpha-2b and acitretin.
SO - J Dermatolog Treat 2001 Jun;12(2):117-21
AD - 2nd Department of Dermatology, A Syngros Hospital, Athens, Greece.
BACKGROUND: Follicular mucinosis (FM) is a rare dermatosis characterized
by mucin deposits in the pilosebaceous units. It is divided into a
primary-benign type and a secondary type associated mostly with
lymphomas. No standard effective therapy is available for the primary FM
while in the secondary form treatment is aimed against the underlying
disease. METHODS: We report a case of secondary FM in which a cutaneous
T-cell lymphoma was detected 6 years after the initial eruption.
RESULTS: Complete remission was achieved with combination therapy of
interferon alpha-2b at a dose of 6 million U subcutaneously three times
a week, and acitretin 35 mg/day, for 6 months. CONCLUSION: Regular
clinical and histopathological evaluation is suggested for all patients
with FM. For cases associated with cutaneous T-cell lymphoma the
combination of interferon alpha and acitretin seems to be a good
therapeutical approach.
2
UI - 12045708
AU - Lukowsky A; Richter S; Dijkstal K; Sterry W; Muche JM
TI -
A T-cell receptor gamma polymerase chain reaction assay using capillary
electrophoresis for the diagnosis of cutaneous T-cell lymphomas.
SO - Diagn Mol Pathol 2002 Jun;11(2):59-66
AD - Department of Dermatology and Allergy, Medical Faculty (Charite),
Humboldt-University of Berlin, Germany. ansgar.lukowsky@charite.de
Detection of clonal T-cell receptor gamma rearrangements by polymerase
chain reaction (TCRgamma PCR) followed by high-resolution
electrophoresis has now become a valuable tool in the diagnosis of
cutaneous T-cell lymphoma (CTCL). The identification of clonal TCRgamma
PCR products by fluorescent fragment analysis (FFA) on a capillary DNA
sequencer is described here and compared with an established
hetero-duplex temperature gradient gel electrophoresis (HD-TGGE). Of 55
CTCL derived lesional skin samples, clonality was obtained in 46 samples
by FFA (83.6%) and in 45 samples by HD-TGGE (81.8%). Of 35 control skin
specimens from various nonmalignant dermatoses, two samples (pityriasis
lichenoides chronica) showed clonality by both methods, one sample
(chronic dermatitis) only by FFA. The sensitivity of FFA was established
using three clonal T-cell lines and peripheral blood mononuclear cells.
The detection limit for clonal material was approximately 1% to 2.5% in
mixtures of DNA and 1% to 3% in cell dilutions. For cell dilution
series, we confirmed a linear correlation between the clonal/polyclonal
peak-size ratios and the portion of clonal cells up to about 10%. Thus,
the initial ratio between mono-and polyclonal template is correctly
displayed by FFA within that concentration range. In conclusion, FFA on
capillary DNA sequencer is a well-suited separation technique in
TCRgamma PCR-based clonality analysis also exhibiting quantitative
properties.
3
UI - 12196250
AU - Chua MS; Veness MJ
TI -
Mycosis fungoides involving the oral cavity.
SO - Australas Radiol 2002 Sep;46(3):336-9
AD - Department of Radiation Oncology, Westmead Hospital, Sydney, Australia.
Mycosis fungoides is a malignant T-cell lymphoproliferative disease with
a predilection for cutaneous involvement. Extracutaneous disease is
uncommon and oral mucosal involvement is rare. We describe a case of
mycosis fungoides involving the hard palate treated with radiotherapy.
The relevant literature on this topic is reviewed.
4
UI - 12224972
AU - Suchin KR; Junkins-Hopkins JM; Rook AH
TI -
Treatment of stage IA cutaneous T-Cell lymphoma with topical application
of the immune response modifier imiquimod.
SO - Arch Dermatol 2002 Sep;138(9):1137-9
AD - Department of Dermatology, Hospital of the University of Pennsylvania, 2
Rhoads Pavilion, 3600 Spruce St, Philadelphia, PA 19104-2399, USA.
arook@mail.med.upenn.edu
5
UI - 11407974
AU - Willers J; Haffner A; Zepter K; Storz M; Urosevic M; Burg G; Dummer R
TI -
The interferon inhibiting cytokine IK is overexpressed in cutaneous T
cell lymphoma derived tumor cells that fail to upregulate major
histocompatibility complex class II upon interferon-gamma stimulation.
SO - J Invest Dermatol 2001 Jun;116(6):874-9
AD - Department of Dermatology, University Hospital of Zurich, Zurich,
Switzerland.
Cutaneous T cell lymphomas are characterized by an accumulation of
malignant clonal lymphocytes in the skin and occasionally in the blood.
We compared gene transcription profiles from cultured clonal lymphocytes
with autologous healthy blood lymphocytes by microarray hybridization.
Cutaneous T cell lymphoma derived cells transcribed high amounts of an
interferon inhibiting cytokine factor. The presence of an interferon
inhibiting cytokine factor was confirmed in 12 skin biopsies of mycosis
fungoides and Sezary syndrome derived blood lymphocytes by reverse
transcriptase-polymerase chain reaction. The presence of interferon
inhibiting cytokine factor mRNA in Sezary syndrome derived lymphocytes
was associated with a lack of HLA class II upregulation after
stimulation with interferon-alpha and interferon-gamma. This was not due
to a loss of the interferon signaling cascade as the presence of
interferon-signaling components was confirmed by reverse
transcriptase--polymerase chain reaction on the transcriptional level.
The elevated constitutive interferon inhibiting cytokine factor
expression observed in cutaneous T cell lymphoma derived cells was
insensitive to interferon-gamma stimulation, but was enhanced in normal
peripheral blood mononuclear cells. We suggest that interferon
inhibiting cytokine factor contributes to the lack of HLA class II
upregulation in lymphoma cells. Interferon inhibiting cytokine factor
may participate in providing a microenvironment at the tumor site
insensitive to interferon-gamma stimulation and thus prevents an
efficient local immune response.
6
UI - 11423845
AU - Smoller BR
TI -
Medical pearl: new views through the microscope.
SO - J Am Acad Dermatol 2001 Jul;45(1):120-1
7
UI - 12006543
AU - Zhang C; Hazarika P; Ni X; Weidner DA; Duvic M
TI -
Induction of apoptosis by bexarotene in cutaneous T-cell lymphoma cells:
relevance to mechanism of therapeutic action.
SO - Clin Cancer Res 2002 May;8(5):1234-40
AD - Department of Dermatology, The University of Texas M.D. Anderson Cancer
Center, Houston 77030, USA.
PURPOSE: Bexarotene is the first synthetic rexinoid approved for the
treatment of all stages of cutaneous T-cell lymphoma (CTCL) however the
mechanism of bexarotene action is unknown. We examined the effects of
bexarotene on induction of apoptosis and expression of its cognate
receptors in well-established CTCL cell lines (MJ, Hut78, and HH).
EXPERIMENTAL DESIGN: CTCL cells were treated with 0.1, 1, and 10 microM
bexarotene for 24, 48, 72, and 96 h. Apoptosis was determined by
flow-cytometry analysis of sub-G(1) hypodiploid nuclei and annexin V
binding populations. Apoptosis-associated proteins and retinoid
receptors were detected by Western blots. RESULTS: Bexarotene treatment
at 1 and 10 microM for 96 h increased the number of cells with sub-G1
populations and annexin V binding in a dose-dependent manner compared
with vehicle controls (DMSO) in all three cell lines, respectively.
Bexarotene treatment suppressed the expression of retinoid X receptor
alpha and retinoic acid receptor alpha proteins in all three lines
compared with untreated controls. Bexarotene treatment decreased the
protein levels of survivin, activated caspase-3, and cleaved
poly(ADP-Ribose) polymerase, but had no obvious effect on expression of
Fas/Fas ligand and bcl-2 proteins in all three CTCL lines. CONCLUSIONS:
Bexarotene treatment at clinically relevant concentrations causes
apoptosis of CTCL cell lines in association with activation of caspase-3
and cleavage of poly(ADP-Ribose) polymerase, as well as down-regulation
of retinoid X receptor alpha, retinoic acid receptor alpha, and
survivin. These findings support apoptosis as a mechanism for bexarotene
therapy in CTCL.
8
UI - 11859306
AU - Rampino M; Ragona R; Monetti U; Mussano A; Guarneri A; Sannazzari GL
TI -
Total skin electron beam therapy in mycosis fungoides. Our experience
from 1985 to 1999.
SO - Radiol Med (Torino) 2002 Jan-Feb;103(1-2):108-14
AD - Dipartimento di Radioterapia, Universita degli Studi, Turin, Italy.
PURPOSE: The specific goal of this retrospective study is to evaluate
the role of total skin electron beam therapy (TSEBT) in the treatment of
Mycosis Fungoides (MF) and to assess the most significant prognostic
factors in univariate and multivariate analyses. MATERIAL AND METHODS:
technique) were performed on a total of 86 patients (63 with Mycosis
Fungoides, 6 with Sezary Syndrome and 17 with Cutaneous Lymphomas). This
study considers only the Mycosis Fungoides group, which consisted of 60
cases evaluable for response, survival and toxicity. The distribution of
patients by stage (MFCG Staging Classification, 1991) was 21, 5, 12, 22
for stages I, II, III and IV, respectively. RESULTS: The overall
response rate was 96.6% (58/60) with complete response (CR) in 50/60
patients (83.3%) and partial response (PR) in 8 cases (13.3%). The
five-year and ten-year actuarial overall survival (OS) was 50% and 45%,
respectively. Local control, intended as control of the disease in the
skin, was 35% at five years and 20% at ten years, and was correlated
with skin involvement. The prognostic factors confirmed by the
multivariate analysis for both overall survival and local control were:
T (p<0.001) and response after TSEBT (p<0.001). The treatment was very
well tolerated. CONCLUSIONS: Our study showed good results in terms of
response and survival with a long follow-up time (mean value 40 months).
We confirm that TSEBT yields very good results in early-stage MF;
additional trials of combined modality and investigational therapies are
needed to improve the outcome for advanced-stage disease.
9
UI - 12150131
AU - Trupiano JK; Bringelsen K; Hsi ED
TI -
Primary cutaneous lymphoblastic lymphoma presenting in an 8-week old
infant.
SO - J Cutan Pathol 2002 Feb;29(2):107-12
AD - Department of Pathology, Cleveland Clinic Foundation, OH 44195, USA.
BACKGROUND: We report a case of primary cutaneous lymphoblastic lymphoma
(LBL) presenting in an 8-week-old infant. METHODS: Histopathology and
flow cytometric analysis confirmed the diagnosis of a lymphoblastic
lymphoma. The cells expressed CD19, CD20, CD34 and surface
immunoglobulin (sIg). RESULTS: The cells were negative for TdT and CD99.
This unusual immunophenotype has been described as "transitional
pre-B-cell", in that the cells express both immature markers (CD34) and
mature markers (sIg). CONCLUSIONS: To our knowledge, only five other
cases of sIg positive precursor B-cell LBL have been reported in the
literature. This may represent the youngest reported case of primary
cutaneous LBL, occurring at 8weeks of age.
10
UI - 12271301
AU - Pabsch H; Rutten A; Von Stemm A; Meigel W; Sander CA; Schaller J
TI -
Treatment of childhood mycosis fungoides with topical PUVA.
SO - J Am Acad Dermatol 2002 Oct;47(4):557-61
AD - Department of Dermatology, Dermatohistological Unit, St Barbara
Hospital, Duisburg, Germany.
Mycosis fungoides is the most common type of cutaneous T-cell lymphoma,
which is usually observed in mid to late adulthood. We report 5 cases of
mycosis fungoides in children, all presenting as patch- and plaque-stage
disease most commonly involving the buttocks. Histologic examination
showed in every case the typical features of mycosis fungoides. In 4 of
the 5 cases, the infiltrating lymphocytes were characterized by the
T-cell phenotype CD3(+), CD4(+), CD8(+); and in 3 cases, a monoclonal
rearrangement of the T-cell receptor gamma (TCR-gamma) gene was found.
Three children received topical PUVA treatment, and the other two were
treated with mid-potency topical corticosteroids, resulting in complete
clinical remission. A management approach to mycosis fungoides with
topical PUVA may be appropriate for children.
11
UI - 12271309
AU - Koay J; Jones D; Duvic M
TI -
Cold urticaria in a patient with mycosis fungoides.
SO - J Am Acad Dermatol 2002 Oct;47(4):608-10
AD - Baylor College of Medicine, Houston, Texas 77030, USA.
We report what we believe to be the first documentation of a patient
with both cold urticaria and mycosis fungoides. The patient described a
marked worsening of his long-standing lesions of mycosis fungoides at
the same time as the onset of cold sensitivity. We believe this suggests
a possible association between these 2 rare diseases.
12
UI - 12271315
AU - Shapiro M; Yun M; Junkins-Hopkins JM; Vittorio CC; Schulman N; Saidman
TI -
BH; Fried RG; Rook AH; Alavi A
Assessment of tumor burden and treatment response by
18F-fluorodeoxyglucose injection and positron emission tomography in
patients with cutaneous T- and B-cell lymphomas.
SO - J Am Acad Dermatol 2002 Oct;47(4):623-8
AD - Department of Dermatology, University of Pennsylvania Health System,
Philadelphia, USA.
18F-Fluorodeoxyglucose (FDG)-positron emission tomography (PET) is a
unique functional/metabolic imaging modality that is efficacious in
nodal staging and detection of extranodal involvement for a variety of
lymphomas. We report its novel use in evaluating tumor burden and
response to therapy in two patients with cutaneous lymphomas. A
24-year-old woman with aggressive subcutaneous panniculitic T-cell
lymphoma associated with fever, arthralgias, lymphadenopathy, mild
anemia, and widespread painful lesions refractory to multiple treatment
strategies exhibited intense uptake of a glucose analogue at sites of
clinically apparent (and clinically imperceptible) disease. Denileukin
diftitox therapy resulted in clinical remission, and a repeat PET scan
failed to detect residual foci of malignancy. A 38-year-old man with a
more indolent multifocal primary cutaneous follicle center B-cell
lymphoma characterized by few systemic symptoms and slowly evolving
lesions demonstrated only mild glucose analogue uptake at sites of
disease. Remission was achieved by radiotherapy and intravenous
rituximab, and confirmed by a repeat PET scan. Extracutaneous disease
was not evident in either patient by this technique. These preliminary
data suggest that FDG-PET may be useful in determining disease activity
at the time of initial diagnosis, after treatment, and evaluating a
suspected recurrence.
13
UI - 12200382
AU - Ingen-Housz-Oro S; Bussel A; Flageul B; Michel L; Dubertret L; Kourilsky
TI -
P; Gachelin G; Bachelez H; Musette P
A prospective study on the evolution of the T-cell repertoire in
patients with Sezary syndrome treated by extracorporeal photopheresis.
SO - Blood 2002 Sep 15;100(6):2168-74
AD - Institut de Recherche sur la Peau, Institut National de la Sante et de
la Recherche Medicale (INSERM) U532, Hopital Saint-Louis, 75475 Paris,
France.
Sezary syndrome is a leukemic form of epidermotropic cutaneous T-cell
lymphoma related to the malignant proliferation of clonal CD4(+) T
cells. Extracorporeal photochemotherapy may induce a transient
improvement of the clinical signs, but its efficiency is discussed. To
investigate the frequency of the T-cell clone in the peripheral blood of
patients with Sezary syndrome and to monitor its evolution in patients
treated using extracorporeal photopheresis or chemotherapy, we used the
immunoscope technique. In one patient, we observed a decrease of the
relative frequency of the clone from 15.6% to 0%, paralleling a complete
remission of the clinical disease and a disappearance of the circulating
Sezary cells. In the other cases, the evolution of the relative
frequency paralleled the initial improvement of the clinical status and
the absence of long-term efficiency in patients treated with
extracorporeal photopheresis or chemotherapy. We observed a quick-acting
direct cytotoxicity of the association 8MOP + UVA on the T-cell clone.
The immunoscope technique appears to be an efficient tool to appreciate
the amount of tumoral cells and to monitor the evolution of the clonal
component in the Sezary syndrome.
14
UI - 12209749
AU - Chiarion-Sileni V; Bononi A; Fornasa CV; Soraru M; Alaibac M; Ferrazzi
TI -
E; Redelotti R; Peserico A; Monfardini S; Salvagno L
Phase II trial of interferon-alpha-2a plus psolaren with ultraviolet
light A in patients with cutaneous T-cell lymphoma.
SO - Cancer 2002 Aug 1;95(3):569-75
AD - Division of Medical Oncology, Azienda Ospedaliera-Universita, Via
Giustiniani 2, 35123 Padua, Italy. mgaliz@tiscalinet.it
PURPOSE: To evaluate the efficacy and side effects of psolaren with
ultraviolet light A (PUVA) and interferon-alpha-2a (IFN-alpha-2a) in
patients with mycosis fungoides (MF) and Sezary syndrome (SS). PATIENTS
all stages of MF and SS were treated in a prospective Phase II trial
with systemic escalating doses of IFN-alpha-2a combined with PUVA for 1
year, followed by indefinite PUVA maintenance in complete responding
patients. RESULTS: Sixty-three patients were enrolled (Stage IA, n = 6;
IB, n = 37; IIA, n = 3; IIB, n = 3; III, n = 12; IVA, n = 2). Ten
patients had received previous therapy. The median follow-up duration
for the entire cohort is 37 months. Of 63 patients, 51 achieved a
complete response (CR; 74.6%) or partial response (PR; 6%) to therapy.
The median response duration is 32 months. The 5-year overall survival
rate is 91% and the 5-year disease-free survival rate is 75%. No
life-threatening side effects were observed. Five patients stopped
IFN-alpha-2a therapy due to toxicity. Eighty-four percent of the
patients received more than 75% of the planned dose (12 million units
three times a week). CONCLUSIONS: This combination of IFN-alpha-2a and
phototherapy is an effective and safe therapy for patients with
symptomatic MF. Copyright 2002 American Cancer Society.
15
UI - 12218920
AU - De Saint Paul G; Albes B; Bayle P; Lamant L; Bazex J
TI -
[Cutaneous T-cell lymphoma, cutaneous hyperpigmentation and
paraneoplastic pemphigus]
SO - Ann Dermatol Venereol 2002 Jun-Jul;129(6-7):896-900
AD - Service de Dermatologie, Hopital Nord, Marseille, France.
BACKGROUND: Paraneoplastic pemphigus is an autoimmune mucocutaneous
disease usually associated with neoplasia as lymphoid proliferations. We
report the original case of a patient who had developed a mycosis
fongoide preceded by a paraneoplastic pemphigus. To our knowledge, this
association has never been reported before. Cutaneous manifestations of
mycosis fongoide as pigmentary change are known. This mycosis fongoide
was particular in its progressive cutaneous hyperpigmentation. CASE
REPORT: A 70-year-old male patient developed a mycosis fongoide with
CD30 positive cells in the dermis several months after the diagnosis of
a paraneoplastic pemphigus. Simultaneously, a cutaneous
hyperpigmentation was predominantly noticed on photo-exposed areas,
which improved after chemotherapy. DISCUSSION: Paraneoplastic pemphigus
may precede the cancer, as is shown by our present case. This
paraneoplastic pemphigus is singular because of the lack of oral
erosions, patient's prolonged survival and its association with a
mycosis fongoide. The diagnosis of mycosis fongoide with CD30 + cells
was finally established together with its relationship to the cutaneous
hyperpigmentation. Indeed, a few cases of pigmentary changes in mycosis
fongoide have already been reported. The pathogenesis is still unknown,
but the role of mast cell and stem cell factor in hyperpigmented mycosis
fongoide has been proposed.
16
UI - 11893040
AU - Barrionuevo C; Anderson VM; Zevallos-Giampietri E; Zaharia M; Misad O;
TI -
Bravo F; Caceres H; Taxa L; Martinez MT; Wachtel A; Piris MA
Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular
genetic study of 16 pediatric cases from Peru.
SO - Appl Immunohistochem Mol Morphol 2002 Mar;10(1):7-14
AD - Department of Pathology, The Institute of Neoplastic Diseases, Lima,
Peru.
Hydroa-like cutaneous T-cell lymphoma (hydroa-like CTCL) is an unusual
pediatric malignancy with a poor prognosis. An impressive cutaneous rash
characterized by edema, blisters, ulcers, crusts, and scars, resembling
hidroa vacciniforme, is seen mainly on the face and sometimes on the
extremities. The lesion consists of lymphomatous T-cell infiltration of
the skin and subcutis with variable exocytosis and angiocentricity. It
has been also called edematous, scarring vasculitic panniculitis and
hydroa-like lymphoma. An association with Epstein-Barr virus has been
suggested. The differential diagnosis includes other cutaneous
lymphomas, particularly the cutaneous nasal type T/natural killer-cell
lymphoma, mycosis fungoides, precursor T-cell lymphoblastic lymphoma,
nonspecific peripheral T-cell lymphoma, cutaneous anaplastic large cell
lymphoma, and subcutaneous panniculitic T-cell lymphoma. Other
differential diagnoses are inflammatory dermatopathies and
panniculitides. Based on a series of 16 such cases referred to the
Institute of Neoplastic Diseases, the objective of this report is not
only to provide a better clinicopathologic understanding of this entity
but also a reappraisal of it as a malignancy. The male/female frequency
ratio was 1:1. The median age was 10 years old. All cases showed
predominant facial involvement with edema, blisters, ulcers, crusts, and
scars. Chemotherapy and/or radiotherapy had little or no benefit. The
prognosis was usually dismal. The lymphoma extended from the epidermis
to the subcutis, with frequent angiocentric and periadnexal array.
Lymphoma cells were mostly of intermediate size with dense
hyperchromatic nuclei, inconspicuous nucleoli, and infrequent mitosis. A
scanty and variable inflammatory background was found. The lymphoma
cells displayed T-cell cytotoxic phenotype. In addition, they were
negative for the natural killer cell antigens CD56 and CD57.
Epstein-Barr virus in situ hybridization was positive in the six cases
in which it was assayed. T-cell receptor gamma (TCRgamma) displayed
monoclonal-type rearrangement in four cases studied. Our findings
indicate that hydroa-like CTCL is an independent clinicopathologic
entity that affects children. Consequently, it should be considered an
independent subset of CTCLs and be included as such in the
classification of neoplastic diseases of the lymphoid tissues.
The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.
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