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NCI CANCERLIT® Search: Cutaneous T-cell Lymphoma - January 2002
National Cancer Institute®
Last Modified: January 1, 2002
Table of Contents
1
UI - 11049975
AU - Delfau-Larue MH; Laroche L; Wechsler J; Lepage E; Lahet C; Asso-Bonnet
TI -
M; Bagot M; Farcet JP
Diagnostic value of dominant T-cell clones in peripheral blood in 363
patients presenting consecutively with a clinical suspicion of cutaneous
lymphoma.
SO - Blood 2000 Nov 1;96(9):2987-92
AD - Departments of Immunobiology, Pathology, Biostatistics and Medical
Informatics, and Dermatology, Hospital Henri-Mondor, Assistance
Publique-Hopitaux de Paris, Creteil, France.
marie-helene.delfau@ap-hop-paris.fr
It is now widely accepted that polymerase chain reaction (PCR) analysis
of cutaneous T-cell clonality is of diagnostic value in cutaneous T-cell
lymphomas (CTCLs) and most helpful in the diagnosis of mycosis fungoides
(MF). However, the diagnostic and prognostic value of circulating clonal
T cells remains unclear. We studied T-cell clonality in the peripheral
blood (PB) and the cutaneous lesion, sampled at the same time, in 363
consecutively seen patients with a clinical suspicion of cutaneous
lymphoma. Using a PCR technique providing a specific imprint of T-cell
clones (PCRgamma-denaturing gradient gel electrophoresis), we found that
detection of identical circulating and cutaneous T-cell clones was
associated with the diagnosis of CTCL (P <.001). Detection of
circulating tumor cells in patients with MF was infrequent (12.5%),
except in those with erythrodermic MF (42%; P =.003). Moreover, among
the 46 patients who had identical circulating and cutaneous T-cell
clones, 25 (56%) had erythroderma. The finding of a dominant clone in
the PB but not in the skin was frequent, regardless of the
clinicohistologic classification; it occurred in 30% of patients with
CTCL, 41% with non-CTCL malignant infiltrates, and 34% with benign
infiltrates. This pattern was significantly more frequent in patients
over 60 years of age (P <.002), even in the CTCL group (P <. 01). In
conclusion, dominant T-cell clones detected in the PB of patients with
MF by using a routine PCR technique are rarely tumoral and are more
often related to age. A multicenter prospective study is under way to
establish the prognostic value of circulating tumor cells.
2
UI - 11411914
AU - Topar G; Zelger B; Schmuth M; Romani N; Thaler J; Sepp N
TI -
Granulomatous slack skin: a distinct disorder or a variant of mycosis
fungoides?
SO - Acta Derm Venereol 2001 Jan-Feb;81(1):42-4
AD - Departments of Dermatology, University of Innsbruck, Austria.
Gerda.Topar@uibk.ac.at
About 75% of cutaneous lymphomas belong to the group of T-cell
lymphomas. Mycosis fungoides is the most common entity in this group.
Granulomatous slack skin is a rare form of cutaneous T-cell lymphoma
closely related to mycosis fungoides. We present here a patient with
areas of lax skin for several years who developed a generalized
erythroderma with associated immunoactivation and a deterioration in his
general condition. This report discusses clinically and histologically
the differential diagnoses, namely granulomatous slack skin and
granulomatous mycosis fungoides, and suggests that these 2 disorders are
only variants in the broad spectrum of a single disease.
3
UI - 11432667
AU - Higa M; Kinjo T; Miyagi J; Sakumoto N; Iwamasa T; Kishaba M; Sunakawa H
TI -
Differences in EBNA2 and LMP-1 carboxy terminal region sequences of
Epstein-Barr virus type A between the tumors in a multiple cancer
patient.
SO - Pathol Res Pract 2001;197(6):401-9
AD - Department of Pathology, Ryukyu University School of Medicine,
Nishihara, Okinawa, Japan.
Using PCR, type A Epstein-Barr virus (EBV) infection was demonstrated in
a squamous cell carcinoma of the maxilla (in a 52-year-old man) and the
tongue of the same patient 18 years later (at the age of 70).
Furthermore, at the age of 72, this patient developed an EBV-infected
anaplastic large cell lymphoma. Analysis of the terminal regions of the
EBV genome revealed a monoclonal proliferation of EBV-infected lymphoma
cells. However, sequence analysis of the EBV revealed a slight
difference in the EBNA2 regions between the virus-infected lymphoma and
the squamous cell carcinomas. The mutations at 48991 (G-->T) and 48998
(C-->A) were demonstrated in the lymphoma. Although the squamous cell
carcinoma of the tongue occurred after an interval of 18 years, the
mutation site in the carcinomas was the same, 49137 (A-->G), as compared
with B95-8 strain EBV EBNA2. The mutations at 48991 and at 49137 were
associated with amino acid changes, Arg-->Met and Thr-->Ala,
respectively, but the alteration at 48998 was a silent mutation.
Thirty-bp deletion in the LMP-1 carboxy terminal region was demonstrated
in the virus-infected lymphoma, but not in the squamous cell carcinomas.
On the other hand, HTLV-1 proviral DNA (tax, gag and env) was not
detected in the lymphoma, nor was HPV demonstrated in the squamous cell
carcinomas, although Okinawa is known as an HTLV-1 and HPV prevalence
region. The T-cell receptor beta gene rearrangement was demonstrated in
the lymphoma, but the t(2;5) fusion transcript was not detected using
PCR. Cytogenetic analysis of the lymphoma cells showed a complex
hypertriploid karyotype with 76XY. The type A EBV infection might play a
role in the carcinogenesis of the tumors of our patient. Interestingly,
the infected virus genome sequences, the EBNA2 and LMP-1 regions, which
were closely associated with carcinogenesis in the squamous cell
carcinomas and the lymphoma, showed slight differences.
4
UI - 11444511
AU - Cook DL
TI -
Early mycosis fungoides: can the diagnosis be made reliably?
SO - Adv Anat Pathol 2001 Jul;8(4):240-4
AD - Department of Pathology, University of Vermont College of Medicine,
Fletcher Allen Health Care, Burlington, USA.
The diagnosis of early mycosis fungoides is often regarded as difficult
or impossible due to the lack of clear-cut histopathologic criteria.
Many authors have published observations of histologic features seen in
biopsies of patients with mycosis fungoides and have offered parameters
that may be helpful in diagnosis. In the study, undertaken by members of
the EORTC Cutaneous Lymphoma Study Group, the proposed histologic
features are tested for sensitivity and specificity. This commentary
discusses some of the historical issues and the value of the EORTC study
pertaining to the diagnosis of early mycosis fungoides.
5
UI - 11479770
AU - Turbat-Herrera EA; Veillon D; Cotelingam J; Fowler M; Herrera GA
TI -
The ultrastructural and immunohistochemical heterogeneity of
CD-30-positive neoplasms: so-called anaplastic large cell Ki-1
lymphomas.
SO - Med Electron Microsc 2001 Mar;34(1):19-28
AD - Louisiana State University Health Sciences Center, Department of
Pathology, 1501 Kings Highway, Shreveport, LA 71130, USA.
eturba@lsuhsc.edu
Anaplastic large cell lymphoma (ALCL), also referred to as Ki-1
lymphomas, was first recognized as an entity with characteristic light
microscopic appearance in 1985. This tumor is composed of variably
cohesive cells, often with large, markedly atypical, and multinucleated
cellular forms. The recognition of ALCL resulted from the development of
a monoclonal antibody in Kiel, Germany, named Ki-1, which was initially
believed to be a putative marker for Reed-Sternberg cells. This antibody
was later found to be specific against the epitope CD-30. Attempts to
create strict criteria to preserve this neoplasm as a specific entity
have undergone evolution. However, it is now clear that included in this
group are a variety of pleomorphic neoplasms with CD-30
immunoreactivity. Some of these neoplasms are nonlymphoid and show
marked heterogeneity in their immunohistochemical and ultrastructural
profiles. This article aims to highlight the ultrastructural spectrum of
neoplasms exhibiting CD-30 positivity that are within the spectrum of
ALCL. It remains to be determined if there are subgroups of these
CD-30-positive neoplasms that can be segregated on the basis of
ultrastructural and immunohistochemical criteria with corresponding
clinical correlates that may impact on their management, treatment, and
prognosis. We review here the heterogeneity of CD-30-positive neoplasms
(so-called anaplastic large cell Ki-1 lymphomas).
6
UI - 11150254
AU - Egbert PR; Erny BC
TI -
T-cell lymphoma and ophthalmic abnormalities.
SO - Ophthalmology 2001 Jan;108(1):11-2
7
UI - 11561161
AU - Pulford K; Morris SW; Mason DY
TI -
Anaplastic lymphoma kinase proteins and malignancy.
SO - Curr Opin Hematol 2001 Jul;8(4):231-6
AD - Immunodiagnostics Unit, Nuffield Department of Clinical Laboratory
Sciences, John Radcliffe Hospital, Oxford, OX3 9DU, UK.
karen.pulford@ndcls.ox.ac.uk
The anaplastic lymphoma kinase (ALK) gene fuses to the nucleophosmin
(NPM) gene as a result of a (2;5) translocation associated with a
subtype of human lymphoma (initially designated anaplastic large cell
lymphoma [ALCL] or Ki-1/CD30-positive lymphoma). The immunocytochemical
detection of NPM-ALK (and proteins encoded by other ALK fusion genes)
has allowed the definition of a tumor entity, "ALK-positive lymphoma"
(which shows only partial overlap with pathologists' diagnosis of ALCL),
to be defined and is invaluable in distinguishing this disease from
ALK-negative large cell lymphomas. Eight variant ALK fusion proteins
have been identified. Some are expressed only in ALCL, some are found
only in the nonhematopoietic neoplasm inflammatory myofibroblastic tumor
(IMT), and some are present in both types of malignancy. The ALK gene is
silent in adult tissues except for restricted sites within the nervous
system (consequently, patients with ALK-positive lymphoma produce
antibodies to the ALK protein) but is expressed in some neuroblastomas
and rhabdomyosarcomas. Biochemical studies suggest an anti-apoptotic
function of NPM-ALK, and this may contribute to oncogenesis. Although
ALK-positive lymphomas have a generally good prognosis, new therapeutic
regimens are still needed for patients whose disease is refractory to
conventional treatment.
8
UI - 11564061
AU - Falini B
TI -
Anaplastic large cell lymphoma: pathological, molecular and clinical
features.
SO - Br J Haematol 2001 Sep;114(4):741-60
AD - Institute of Haematology, Policlinico Monteluce, University of Perugia,
Perugia, Italy. faliniem@unipg.it
9
UI - 11571710
AU - Luo V; Lessin SR; Wilson RB; Rennert H; Tozer C; Benoit B; Leonard DG
TI -
Detection of clonal T-cell receptor gamma gene rearrangements using
fluorescent-based PCR and automated high-resolution capillary
electrophoresis.
SO - Mol Diagn 2001 Sep;6(3):169-79
AD - Molecular Pathology Laboratory, Department of Pathology and Laboratory
Medicine, University of Pennsylvania, Philadelphia, PA 19104-4283, USA.
BACKGROUND: Analysis of T-cell receptor gamma (TCR gamma) gene
rearrangements by PCR is a powerful tool for detecting clonal T-cell
populations for the diagnosis of lymphoid neoplasms. We report a method
for TCR gamma PCR analysis using capillary electrophoresis (CE). METHODS
AND RESULTS: To define the threshold for identification of a predominant
monoclonal population within a polyclonal background, we developed a
novel objective parameter of the peak height ratio (Rn) of the peak of
interest and the average of the two immediate flanking peaks. After
evaluation of monoclonal, reactive, and normal T-cell populations, an Rn
of 3.0 or greater was determined to be consistent with a monoclonal
population, whereas an Rn between 1.9 and 3.0 was considered an
intermediate range. This CE method was compared with the standard
denaturing gradient gel electrophoresis (DGGE) method using previously
evaluated clinical specimens. Eleven of 12 clinical specimens (92%) with
a definitive diagnosis of T-cell lymphoma were monoclonal by CE, with
100% concordance with the DGGE method. Of nine specimens morphologically
suspicious for T-cell lymphoma, five specimens were positive by CE
analysis compared with four specimens by DGGE. In addition, 14 specimens
for staging from patients with known T-cell lymphoma were studied using
both the CE and DGGE methods, with a concordance of 86%. CONCLUSION: CE
is a powerful and efficient method for analysis of clonality by TCR
gamma PCR.
10
UI - 11589730
AU - Wu KD; Hansen ER
TI -
Shortened telomere length is demonstrated in T-cell subsets together
with a pronounced increased telomerase activity in CD4 positive T cells
from blood of patients with mycosis fungoides and parapsoriasis.
SO - Exp Dermatol 2001 Oct;10(5):329-36
AD - Department of Dermatology, Marselisborg Hospital, University of Aarhus,
8000 Aarhus C, Denmark. wuk@mskcc.org
We have recently demonstrated that telomerase activity is increased and
telomere length shortened in lymphocytes from peripheral blood of
patients with cutaneous T-cell lymphoma. In order to determine which
cell type has increased telomerase activity and shortened telomere
length, CD4+, CD8+, CLA+ CD3+ and CLA- CD3+ T cells were isolated from
peripheral blood of 25 patients, including 15 patients with mycosis
fungoides and 10 patients with parapsoriasis. Eleven healthy individuals
were used as controls; CD19+ B cells were separated from each individual
as an internal control. The results showed that the increased telomerase
activity was significantly predominating in the CD4+ T-cell subset.
Significantly shortened telomere length was found in CD4+ and CD8+
T-cell subsets from the patients compared with the same cell subsets
obtained from healthy individuals. However, no difference was observed
between the subsets; CD19+ B cells collected from patients and healthy
control individuals had similar telomerase activity and telomere length
which was significantly different from the values found in T cells. The
telomere length was significantly shorter in CLA+ CD3+ subset than in
CLA- CD3+ subset. Interestingly, increased telomerase activity and
shortened telomere length was also detected in CD4+ T cells from
patients with parapsoriasis indicating that alteration of telomerase
activity and telomere length in CD4+ T cells is an early event in the
pathogenesis of cutaneous T-cell lymphoma. Thus, the results indicate
that a significant high level of telomerase activity and shortened
telomere length frequently occur in T cells of patients with CTCL and
may reflect tumorigenesis.
11
UI - 11586017
AU - Albes B; Bazex J; Bayle-Lebey P; Bennet A; Lamant L
TI -
Primary hyperparathyroidism and cutaneous T-cell lymphoma: fortuitous
association?
SO - Dermatology 2001;203(2):162-4
AD - Department of Dermatology, Hospital of Purpan, Toulouse, France.
This is the third report of an association between T-cell cutaneous
lymphoma (mycosis fungoides) and primary hyperparathyroidism (adenoma).
Some studies support the concept that hyperparathyroidism may have
promotional activity for the development of certain malignant tumors. A
high risk for successive or concurrent neoplasms has been reported in
patients with parathyroid adenomas. Primary hyperparathyroidism in a
neoplastic context may be underreported. Patients with tumor-associated
hypercalcemia should be evaluated for the possibility of primary
hyperparathyroidism. Copyright 2001 S. Karger AG, Basel
12
UI - 11606919
AU - Kashani-Sabet M; McMillan A; Zackheim HS
TI -
A modified staging classification for cutaneous T-cell lymphoma.
SO - J Am Acad Dermatol 2001 Nov;45(5):700-6
AD - Cutaneous Oncology Division, University of California San Francisco
Cancer Center, 4th Floor, 1600 Devisadero Street, San Francisco, CA
94115, USA.
BACKGROUND: Despite refinements in the diagnosis of cutaneous T-cell
lymphoma (CTCL), since 1979 there have been no changes to the staging of
CTCL used to classify mycosis fungoides and Sezary syndrome. OBJECTIVE:
We reviewed the current staging of CTCL and examined the usefulness of a
new staging scheme for mycosis fungoides and Sezary syndrome. METHODS:
We determined overall survival of 450 patients with mycosis fungoides
and Sezary syndrome using the current and modified staging
classifications. RESULTS: There were no significant differences between
survival of patients with stage IB (patches/plaques involving greater
than 10% body surface area) and IIA (peripheral adenopathy) disease and
of patients with stage IIB (tumor) and III (erythroderma) disease. There
was a significant difference in survival between patients with extensive
patch versus extensive plaque stage disease. Modification of the current
classification by splitting T2 into patch versus plaque stage disease
and incorporating tumors and erythroderma into stage III proved superior
to the current scheme in predicting overall survival. CONCLUSION:
Modification of the current staging classification for CTCL yields
subgroups useful in the prognostic assessment of CTCL.
13
UI - 11673693
AU - Nabhan C; Krett N; Gandhi V; Rosen S
TI -
Gemcitabine in hematologic malignancies.
SO - Curr Opin Oncol 2001 Nov;13(6):514-21
AD - Division of Hematology/Oncology, Robert H. Lurie Comprehensive Cancer
Center, Northwestern University Medical School, Chicago, Illinois 60611,
USA. c-nabhan@northwestern.edu
Gemcitabine is a pyrimidine analogue that showed significant activity in
solid malignancies. Gemcitabine acts by inhibiting DNA synthesis through
chain termination and ribonucleotide reductase inhibition. During
initial phase I and II studies, gemcitabine had a low toxicity profile
and was well tolerated as a single agent and in combination therapy.
Recently, there has been more interest in studying the activity of
gemcitabine in hematologic malignancies. Gemcitabine demonstrated good
activity in refractory Hodgkin disease patients, non-Hodgkin lymphoma,
cutaneous T-cell lymphoma, and acute leukemias. There is a preponderance
of evidence on the activity of gemcitabine in vitro in myeloma and
leukemic cell lines. The activity of gemcitabine in these disorders will
pave the way for incorporating this agent into the early phases of
therapy.
14
UI - 11675351
AU - Qin JZ; Zhang CL; Kamarashev J; Dummer R; Burg G; Dobbeling U
TI -
Interleukin-7 and interleukin-15 regulate the expression of the bcl-2
and c-myb genes in cutaneous T-cell lymphoma cells.
SO - Blood 2001 Nov 1;98(9):2778-83
AD - Department of Dermatology, University Hospital of Zurich, Switzerland.
Interleukin-7 (IL-7) and IL-15 have been recently identified as growth
factors for cutaneous T-cell lymphoma (CTCL) cells, and they protect
these cells from cell death. Using the CTCL cell line SeAx as a test
system now shows that IL-7 and IL-15 are indeed necessary to maintain
high levels of bcl-2. The up-regulation of bcl-2 was paralleled by
increased DNA-binding activities of the transcription factors STAT2,
STAT5, STAT6, and c-Myb to bcl-2 gene promoter-enhancer elements.
Because STAT5 and c-Myb positively regulate bcl-2, IL-7 and IL-15 may
mediate some of their effects on cell death survival gene expression
through these 2 factors. Constitutive activities of the 3 STAT factors
and c-Myb were found in the IL-7- and IL-15-independent CTCL cell lines
HUT78 and MyLa 2059. The c-Myb protein was also present in CTCL cells of
the skin lesions of all investigated patients. These results indicate
that IL-7 and IL-15 may increase bcl-2 expression in CTCL cells by the
activation of c-myb and STAT factors.
15
UI - 11675364
AU - Piekarz RL; Robey R; Sandor V; Bakke S; Wilson WH; Dahmoush L; Kingma
TI -
DM; Turner ML; Altemus R; Bates SE
Inhibitor of histone deacetylation, depsipeptide (FR901228), in the
treatment of peripheral and cutaneous T-cell lymphoma: a case report.
SO - Blood 2001 Nov 1;98(9):2865-8
AD - Medicine Branch, National Cancer Institute, National Institutes of
Health, Bethesda, MD, USA. rpiekarz@nih.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.
16
UI - 11676833
AU - Beylot-Barry M; Sibaud V; Thiebaut R; Vergier B; Beylot C; Delaunay M;
TI -
Chene G; Dubus P; Merlio JP
Evidence that an identical T cell clone in skin and peripheral blood
lymphocytes is an independent prognostic factor in primary cutaneous T
cell lymphomas.
SO - J Invest Dermatol 2001 Oct;117(4):920-6
AD - Equipe Histologie et Pathologie Moleculaire 12406, Universite Victor
Segalen Bordeaux 2, France. marie.beylot-barry@histo.u-bordeaux2.fr
The monoclonality of the T cell receptor gamma-chain gene was analyzed
by polymerase chain reaction in skin and blood specimens of 85 patients
with cutaneous T cell lymphomas including 67 mycosis fungoides, seven
Sezary syndromes, and 11 CD30- nonepidermotropic cutaneous T cell
lymphomas. A cutaneous T cell clone was detected in 69% of mycosis
fungoides and 100% of Sezary syndromes. This frequency varied according
to the clinical stage: 57% in early stages (Ia-IIa) to 96% in advanced
stages (IIb-IV, Sezary syndrome). A peripheral blood T cell clone was
detected in 42% of early stages and in 74% of late stages but was
identical to the cutaneous one in 15% and in 63%, respectively. A
significant association between initial clinical stage and T cell
monoclonality was observed. In nonepidermotropic cutaneous T cell
lymphomas, T cell monoclonality was detected in 55% of skin and 36% of
blood samples. Univariate and multivariate analyses showed that, besides
the initial clinical stage, an identical cutaneous and blood T cell
clone was an independent prognostic factor for disease progression of
mycosis fungoides/Sezary syndrome (hazard ratio 3.4, 95% confidence
interval 1.4-9.9). Parallel polymerase chain reaction study of skin and
blood specimens may therefore provide an initial prognostic marker that
could help to monitor therapeutic strategies. A fully prospective study,
with simultaneous therapeutic trials, needs to be done to confirm our
findings and to include treatment variables in the statistical analysis.
17
UI - 11679011
AU - Kreuter A; Gambichler T; Hoffmann A; von Kobyletzki G; Altmeyer P;
TI -
Hoffmann K
Forced extracorporeal photopheresis (forced-ECP) in Sezary syndrome.
SO - Int J Dermatol 2001 Jul;40(7):476-8
18
UI - 11703301
AU - Chang SE; Choi JH; Sung KJ; Moon KC; Koh JK
TI -
Cutaneous nasal-type CD56+ natural killer/T-cell lymphoma preceded by
Epstein-Barr virus antigenaemia.
SO - Br J Dermatol 2001 Oct;145(4):668-9
19
UI - 11703305
AU - Amo Y; Ohta Y; Hamada Y; Katsuoka K
TI -
Serum levels of interleukin-18 are increased in patients with cutaneous
T-cell lymphoma and cutaneous natural killer-cell lymphoma.
SO - Br J Dermatol 2001 Oct;145(4):674-6
20
UI - 11703521
AU - Pielop JA; Jones D; Duvic M
TI -
Transient CD30+ nodal transformation of cutaneous T-cell lymphoma
associated with cyclosporine treatment.
SO - Int J Dermatol 2001 Aug;40(8):505-11
AD - Baylor College of Medicine, Houston, Texas, USA.
BACKGROUND: Mycosis fungoides (MF) may evolve from pre-existing chronic
atopic or psoriasiform dermatitis and the histology can be equivocal.
Early patch and plaque lesions of MF may evolve into tumors, disseminate
to lymph nodes, bone marrow, and internal organs, and/or undergo
transformation to a large cell size. METHODS: A patient with a history
of "atopic dermatitis" followed by "psoriasis" rapidly developed
exfoliative erythroderma and axillary lymphadenopathy following
treatment with cyclosporine. At presentation, biopsy specimens of skin
lesions and lymph nodes and staging were obtained. We present the
treatment and follow-up of this patient and review the medical
literature for similar cases. RESULTS: Multiple skin biopsy specimens
from lesions revealed changes consistent with low-grade, cutaneous,
T-cell lymphoma (MF) without evidence of large cell transformation and
psoriasiform epidermal hyperplasia. CD30+ large cell transformation was
present in the lymph node. Adenopathy and erythroderma resolved without
systemic therapy following discontinuation of cyclosporine and treatment
with psoralen/ultraviolet A (PUVA), isotretinoin, interferon-alpha, and
antimicrobials. CONCLUSIONS: This case documents a close relationship
between atopy, psoriasis, and the development of cutaneous T-cell
lymphoma, and illustrates that an immunosuppressive agent, cyclosporine,
can dramatically alter the course of the disease.
21
UI - 11701409
AU - Quaglino P; Osella-Abate S; Novelli M; Lisa F; Comessatti A; Bernengo MG
TI -
Peripheral blood involvement in a mycosis fungoides patient with limited
skin lesions: phenotypical features and homing molecule pattern.
SO - Eur J Dermatol 2001 Nov-Dec;11(6):560-3
AD - Department of Medical and Surgical Specialties, Section of Dermatology,
1st Dermatologic Clinic, University of Turin, via Cherasco 23, 10126
Turin, Italy.
Peripheral blood involvement in mycosis fungoides (MF) patients is more
frequent in the advanced stages and is associated with a worse
prognosis. We report a MF patient with limited patch lesions on her
shoulders, upper chest and thighs (T2N0M0) and peripheral blood
involvement. Clonality in the peripheral blood was demonstrated by the
PCR assay and confirmed by the expansion of the same restricted variable
region of the TCR beta-chain (vbeta17) expressed in the cutaneous
infiltrate. The patient was treated with fludarabine achieving a
complete hematological response followed by an early relapse, whilst the
cutaneous lesions remained unchanged. The soluble interleukin-2 receptor
levels showed a decrease from baseline levels down to normal values at
hematological remission, followed by a further increase. The low
sLex/CLA expression in the cutaneous lymphoid infiltrate could have
given rise to a higher recruitment in the peripheral blood.
22
UI - 11736912
AU - Kurokawa M; Amano M; Miyaguni H; Tateyama S; Ogata K; Idemori M;
TI -
Setoyama M
Eccrine poromas in a patient with mycosis fungoides treated with
electron beam therapy.
SO - Br J Dermatol 2001 Nov;145(5):830-3
AD - Department of Dermatology, Miyazaki Medical College, Kiyotake, Miyazaki,
889-1692, Japan. mkurokawa@post1.miyazaki-med.ac.jp
We describe a 72-year-old man with mycosis fungoides (MF) followed up at
our hospital for more than 20 years, who has developed 14 eccrine
poromas (EPs) in the past 12 years. Twelve of these tumours were
ascertained as EP histopathologically without any findings of malignancy
and the other two were clinically diagnosed and are not yet resected. To
our knowledge, this is the first case report of MF complicated with
multiple EPs. In our patient, all EPs arose in skin areas previously
irradiated with an electron beam for the treatment of MF, suggesting
that electron beam irradiation might have an effect on the development
of EPs.
23
UI - 11727269
AU - Kadin ME; Drews R; Samel A; Gilchrist A; Kocher O
TI -
Hodgkin's lymphoma of T-cell type: clonal association with a CD30+
cutaneous lymphoma.
SO - Hum Pathol 2001 Nov;32(11):1269-72
AD - Department of Pathology, Beth Israel Deaconess Medical Center, Boston,
MA 02215, USA.
The derivation of Reed-Sternberg cells in Hodgkin's lymphoma has been a
subject of great interest. In most cases, Reed-Sternberg cells seem to
be derived from germinal center B cells. In few sporadic cases, a T-cell
origin has been shown. This article supports the concept of a T-cell
derivation for rare cases of Hodgkin's lymphoma and provides evidence of
a novel mechanism of pathogenesis from chronic inflammation in the skin.
Copyright 2001 by W.B. Saunders Company
24
UI - 11731515
AU - Vermeer MH; van Doorn R; Dukers D; Bekkenk MW; Meijer CJ; Willemze R
TI -
CD8+ T cells in cutaneous T-cell lymphoma: expression of cytotoxic
proteins, Fas Ligand, and killing inhibitory receptors and their
relationship with clinical behavior.
SO - J Clin Oncol 2001 Dec 1;19(23):4322-9
AD - Department of Dermatology, Free University Hospital, Amsterdam, the
Netherlands. m.h.vermeer@lumc.nl
PURPOSE: We investigated the number, phenotype, and prognostic
significance of CD8+ T cells in patients with mycosis fungoides (MF) and
CD30- primary cutaneous large T-cell lymphoma (PCLTCL). PATIENTS AND
METHODS: Immunohistochemical stainings for CD8, granzyme B (GrB), T
cell-restricted intracellular antigen (TIA-1), Fas ligand (FasL), and
killer-cell inhibitory receptors (KIRs; CD95, CD158a, and CD158b) were
performed on 83 first-diagnostic biopsy samples obtained from patients
with plaque-stage MF (n = 42), tumor-stage MF (n = 20), and CD30- PCLTCL
(n = 21). RESULTS: Serial sections and double-staining experiments
showed that the large majority of CD8+ T cells in MF and CD30- PCLTCL
expressed TIA-1 and FasL, whereas only a minority expressed GrB, which
suggested that these CD8+ T cells were partly activated cytotoxic T
lymphocytes (CTLs). These CD8+ CTLs never or rarely expressed KIRs. This
phenotype was a constant feature of CD8+ CTLs and did not alter with
disease progression. In contrast, the median percentage of CD8+ CTLs in
plaque-stage MF (22%), tumor-stage MF (7%), and CD30- PCLTCL (3%)
differed significantly (P < .0001) and was associated with a significant
decrease in 5-year survival. Also within the group of tumor-stage MF, a
significant relation between CD8+ CTLs and survival was found.
Multivariate analysis in the total group of MF demonstrated that both
skin stage and percentage of CD8+ CTLs were independent parameters of
survival. CONCLUSION: Our results demonstrated that partly activated
CD8+ CTLs were present in CTCL and that high proportions of these cells
correlated with a better prognosis. This suggested that these CD8+ CTLs
could play an important role in the antitumor response in these
conditions.
25
UI - 11712057
AU - Seckin D; Demirhan B; Oguz Gulec T; Arikan U; Haberal M
TI -
Posttransplantation primary cutaneous CD30 (Ki-1)-positive large-cell
lymphoma.
SO - J Am Acad Dermatol 2001 Dec;45(6 Suppl):S197-9
AD - Department of Dermatology, Baskent University Faculty of Medicine,
Ankara, Turkey.
We describe the case of a 51-year-old female renal transplant recipient
with primary cutaneous CD30-positive large-cell lymphoma of T-cell
origin. Cutaneous T-cell lymphomas are rarely reported in organ
transplant recipients, and we believe they should be considered in the
differential diagnosis of cutaneous neoplastic and infectious diseases
affecting this patient group.
26
UI - 10406618
AU - Cook BE Jr; Bartley GB; Pittelkow MR
TI -
Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma.
SO - Ophthalmology 1999 Jul;106(7):1339-44
AD - Department of Ophthalmology, Mayo Clinic and Mayo Foundation, Rochester,
Minnesota 55905, USA.
OBJECTIVE: To determine the frequency of ophthalmic abnormalities in
patients with cutaneous T-cell lymphoma (mycosis fungoides and Sezary
syndrome) and T-cell lymphoma involving the skin and to describe the
clinical course of the disease with selected examples. DESIGN:
Retrospective, clinic-based, cross-sectional study. PARTICIPANTS: A
computerized diagnostic retrieval system was used to identify all
patients with T-cell lymphoma involving the skin who were examined at
the Mayo Clinic (Rochester, Minnesota) between January 1, 1976 and
December 31, 1990. The medical records of affected patients were
reviewed. MAIN OUTCOME MEASURES: Identification of ophthalmic
abnormalities. RESULTS: During the 15-year interval from 1976 through
1990, cutaneous T-cell lymphoma was diagnosed in 2155 patients. Of
these, 42 (1.95%; 26 male and 16 female) had at least one ophthalmic
abnormality attributable to the disease. The diagnoses in these 42
patients were mycosis fungoides in 19, clinical variants of T-cell
lymphoma of the skin (most commonly peripheral T-cell lymphoma) in 11,
and Sezary syndrome in 12. Cicatricial eyelid ectropion was the most
common finding, affecting 17 (40.4%) of the 42 patients. Thirty-seven
patients had findings that, although probably not a direct consequence
of cutaneous T-cell lymphoma, have been cataloged in previous studies.
CONCLUSION: Although ophthalmic abnormalities in patients with cutaneous
T-cell lymphoma are relatively uncommon, the manifestations of the
disease are diverse and frequently difficult to treat.
27
UI - 11482045
AU - Claros Gonzalez I; Santamaria Giron L; Saenz de Santamaria Gutierrez I;
TI -
Otero Diez J; Florez Garcia LJ; Perez Suarez A
[Gastric perforation as first manifestation of extracutaneous
dissemination of mycosis fungoides]
SO - Rev Esp Enferm Dig 2001 Jun;93(6):403-4
28
UI - 11712039
AU - French LE; Shapiro M; Junkins-Hopkins JM; Vittorio CC; Rook AH
TI -
Regression of multifocal, skin-restricted, CD30-positive large T-cell
lymphoma with interferon alfa and bexarotene therapy.
SO - J Am Acad Dermatol 2001 Dec;45(6):914-8
AD - Department of Dermatology, University of Pennsylvania, Philadelphia,
USA. french@cmu.unige.ch
We report the case of a 43-year-old male patient with persistent
multifocal, skin-restricted, CD30-positive, large T-cell lymphoma.
Combination therapy of systemic interferon alfa and oral bexarotene was
initiated on an experimental basis in the hope of circumventing
therapies such as methotrexate, radiotherapy, or multiple-agent
chemotherapy that may be required in such cases. This treatment was
associated with rapid and marked regression of the patient's cutaneous
lesions.
29
UI - 11712043
AU - Bowman PH; Hogan DJ; Sanusi ID
TI -
Mycosis fungoides bullosa: report of a case and review of the
literature.
SO - J Am Acad Dermatol 2001 Dec;45(6):934-9
AD - Dermatology Section, Louisiana State University Medical Center,
Shreveport 71130-3932, USA.
Mycosis fungoides, the most common type of cutaneous T-cell lymphoma,
can manifest in a variety of clinical and histologic forms. Presentation
with vesiculobullous lesions is extremely rare. We report the ninth
documented case of mycosis fungoides bullosa in which other concomitant
autoimmune blistering diseases were ruled out by negative
immunofluorescence. All previously reported cases in the world
literature since the first in 1887 are reviewed. We recommend the
following defining criteria for the disease: (1) clinically apparent
vesiculobullous lesions, with or without typical mycosis fungoides
lesions (patches, plaques, tumors); (2) typical histologic features of
mycosis fungoides (atypical lyphoid cells, epidermotropism, Pautrier's
microabscesses) with intraepidermal or subepidermal blisters; (3)
negative immunofluorescence (both direct and indirect, if possible) to
rule out concomitant autoimmune bullous diseases; (4) negative
evaluation for other possible causes of vesiculobullous lesions (eg,
medications, bacterial or viral infection, porphyria, phototherapy).
30
UI - 11499537
AU - Tran D; Kwok YK; Goh CL
TI -
A retrospective review of PUVA therapy at the National Skin Centre of
Singapore.
SO - Photodermatol Photoimmunol Photomed 2001 Aug;17(4):164-7
AD - National Skin Centre, Singapore.
BACKGROUND: Photochemotherapy (PUVA) is beneficial for the treatment of
various dermatoses. The introduction of ultraviolet B and narrow-band
ultraviolet B phototherapy has had a significant impact on the role of
PUVA in dermatology. This study aims to assess the current role of PUVA
in treating dermatoses, in a predominantly Asian population, at the
National Skin Centre in Singapore. MATERIALS AND METHODS: We reviewed
the clinical data of 115 patients who were started on PUVA treatment at
the National Skin Centre, Singapore in 1998. We analysed the
epidemiology data, the clinical response rate and the adverse effects of
PUVA therapy. All of the patients continued to have ongoing maintenance
treatment. RESULTS: Most of our patients were Chinese (74.8%) and male
(58.3%), ranging from the ages of 4 to 74 years. Vitiligo (60.9%) was
the commonest skin disorder treated with PUVA, followed by psoriasis
(20.9%), endogenous eczema (11.3%), mycosis fungoides (3.5%), lichen
amyloidosis (2.6%) and prurigo nodularis (0.9%). Most of our patients
received paint PUVA (50.4%), 33.9% oral PUVA and 15.7% bath PUVA. The
best clinical response--those patients achieving a greater that 50%
clearance--was observed in patients with endogenous eczema, psoriasis
and mycosis fungoides (76.8%, 73.9% and 60%, respectively). Of the
vitiliginous patients, 54.3% experienced a poor response to PUVA
therapy. The main side effects were mild erythema and pruritus.
CONCLUSION: PUVA remains a valuable, well-tolerated therapeutic option
for a variety of dermatoses.
31
UI - 11745245
AU - Fraser-Andrews EA; Russell-Jones R; Woolford AJ; Wolstencroft RA; Dean
TI -
AJ; Whittaker SJ
Diagnostic and prognostic importance of T-cell receptor gene analysis in
patients with Sezary syndrome.
SO - Cancer 2001 Oct 1;92(7):1745-52
AD - Skin Tumor Unit, St. John's Institute of Dermatology, St. Thomas'
Hospital, Lambeth Palace Road, London, SE1 7EH, United Kingdom.
efraser-andrews@doctors.org.uk
BACKGROUND: Sezary syndrome (SS) is characterized by erythroderma,
peripheral lymphadenopathy, and circulating Sezary cells and is
clinically heterogeneous. METHODS: T-cell receptor (TCR) gene analysis
was performed using DNA extracted from peripheral blood mononuclear
cells from 74 patients, and the results were correlated with a variety
of other diagnostic parameters and patient outcomes. RESULTS: Two groups
were identified: 66 patients with clonal TCR gene rearrangement (clonal
patients) detected with Southern blot analysis and/or polymerase chain
reaction/single-strand conformational polymorphism analysis and 8
patients with no clonal rearrangement detected (nonclonal patients)
using either technique. Clonal patients were compared with nonclonal
patients. The following median blood parameters were significantly
greater in the clonal group: total white cell count (13.7 10(9)/L vs.
9.6 10(9)/L), lymphocyte count (4.9 10(9)/L vs. 2.2 10(9)/L), absolute
Sezary count (3.22 10(9)/L vs. 0.99 10(9)/L), CD4 count (3.17 10(9)/L
vs. 1.36 10(9)/L), and CD4:CD8 ratio (15.86 vs. 3.21). An expanded
population of T-cells of a specific TCR variable beta subset was
detected in 7 of 36 clonal patients and in 1 of 4 nonclonal patients.
Cytogenetic analysis of peripheral blood from 1 nonclonal patient and 6
clonal patients was normal. The median survival from the time of
diagnosis was 45 months in the clonal group, and 40 of 49 deaths were
cutaneous T-cell lymphoma (CTCL)-related, whereas 3 deaths in the
nonclonal group were unrelated to CTCL (P < 0.01; log-rank test).
Multivariate proportional hazards analysis showed that the absolute
Sezary count and lymph node status were independent prognostic variables
(P = 0.016 and P = 0.036, respectively). CONCLUSIONS: TCR gene analysis
defines a distinct clinicopathologic group of patients with SS. Clonal
patients have a poor prognosis and are likely to die from
leukemia/lymphoma, whereas nonclonal patients may have a reactive,
inflammatory T-cell disorder. The authors suggest that the definitive
diagnostic criteria for patients with SS should include the presence of
a clonal TCR gene rearrangement. Copyright 2001 American Cancer Society.
32
UI - 11715400
AU - Schmook T; Hofbauer G; Kempf W; Burg G; Dummer R
TI -
[Granulomatous slack skin: partial remission following intralesional
administration of interferon-alpha and PUVA]
SO - Hautarzt 2001 Oct;52(10 Pt 2):985-8
AD - Dermatologische Klinik, Universitatsspital Zurich, Gloriastrasse 31,
CH-8091 Zurich.
Granulomatous slack skin (GSS) is a rare cutaneous T-cell lymphoma which
typically runs a protracted and indolent course. On histopathological
assessment lymphoid infiltrates with multinucleated giant cells in the
dermis and subcutis with elastophagocytosis can be observed. Skin
lesions are characterized by pendulous folds. We report on the
successful response of the lesions to intralesional interferon alpha
combined with PUVA.
33
UI - 11757458
AU - Schley G; Kauffold D; Guse E; Slucka S; Jeschke M
TI -
[Angioendotheliomatosis proliferans systematizata]
SO - Hautarzt 2001 Nov;52(11):1030-4
AD - Hautklinik Schwerin, Werderstrasse 30, 19049 Schwerin.
Angioendotheliomatosis proliferans systematisata comprises two different
entities; a malignant and benign (reactive) form. The more common
malignant form with a fatal prognosis should be termed intravascular or
angiotropic lymphoma and is a multifocal systemic disease, which affects
especially the small and middle-sized blood vessels of the skin and
central nervous system. Reactive angioendotheliomatosis is a benign
disease, often associated with an infectious disease (predominantly with
subacute bacterial endocarditis). We report a 38-year-old woman with a
reactive angioendotheliomatosis and a 68-year-old man diagnosed as
having the rare T-cell type of intravascular lymphoma.
34
UI - 11745897
AU - Thomson AB; McKenzie KJ; Jackson R; Wallace WH
TI -
Subcutaneous panniculitic T-cell lymphoma in childhood: successful
response to chemotherapy.
SO - Med Pediatr Oncol 2001 Dec;37(6):549-52
AD - Royal Hospital for Sick Children, Edinburgh, Scotland, United Kingdom.
35
UI - 11558874
AU - Edstrom DW; Porwit A; Ros AM
TI -
Photodynamic therapy with topical 5-aminolevulinic acid for mycosis
fungoides: clinical and histological response.
SO - Acta Derm Venereol 2001 Jun-Jul;81(3):184-8
AD - Department of Dermatology, Karolinska Hospital, Stockholm, Sweden.
desiree.edstrom@ks.se
There is no curative treatment for mycosis fungoides (MF), the most
common primary cutaneous T-cell lymphoma. The aim of this study was to
investigate the response of single lesions to photodynamic therapy
(PDT). The study included 10 plaque MF lesions and 2 tumour MF lesions
from 10 patients. First, 20% 5-aminolevulinic acid was applied topically
to the lesion and adjacent skin for 5-6 h. The lesion was then exposed
to red light at around 630nm. Skin b
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