UI - 11843637
AU - Cerroni L; Fink-Puches R; Back B; Kerl H
Follicular mucinosis: a critical reappraisal of clinicopathologic
features and association with mycosis fungoides and Sezary syndrome.
SO - Arch Dermatol 2002 Feb;138(2):182-9
AD - Department of Dermatology, University of Graz, Auenbruggerplatz 8,
A-8036 Graz, Austria. firstname.lastname@example.org.
CONTEXT: Beginning in 1957, patients have been described with localized
alopecia characterized histopathologically by mucin deposition within
hair follicles (follicular mucinosis [FM]). At least 2 distinct
diagnostic entities have been proposed: one occurring in children and
young adults without association with other diseases ("idiopathic" FM),
the other occurring in elderly patients and associated with mycosis
fungoides or Sezary syndrome ("lymphoma-associated" FM). OBJECTIVE: To
determine whether idiopathic and lymphoma-associated FM are distinct or
related entities. DESIGN: Case series. SETTING: Department of
Dermatology, University of Graz, Graz, Austria. PATIENTS: Forty-four
patients with FM were divided into 2 groups. Group 1 comprised 16
patients (mean age, 37.5 years) with no associated mycosis fungoides or
Sezary syndrome; group 2 was made up of the other 28 (mean age, 52.2
years), who had clinicopathologic evidence of cutaneous T-cell lymphoma.
RESULTS: Mean age was lower in patients with idiopathic FM, but a
considerable overlapping among the 2 groups was present. Location on the
head and neck region was common in both groups, but most patients with
lymphoma-associated FM had lesions also on other body sites. In fact,
solitary lesions at presentation were common in patients with idiopathic
FM (11 [68.8%] of 16 patients), but uncommon in those with
lymphoma-associated FM (2 [7.1%] of 28 patients). Histopathologic
findings did not allow clear-cut differentiation of the 2 groups.
Finally, a monoclonal rearrangement of the T-cell receptor gamma gene
was demonstrated by polymerase chain reaction analysis in about 50% of
tested cases from each group. CONCLUSIONS: Criteria previously reported
to differentiate idiopathic from lymphoma-associated FM proved
ineffective. In analogy to localized pagetoid reticulosis
(Woringer-Kolopp disease), small-plaque parapsoriasis, and so-called
solitary mycosis fungoides, idiopathic FM may represent a form of
localized cutaneous T-cell lymphoma.
UI - 11843638
AU - van Doorn R; Scheffer E; Willemze R
Follicular mycosis fungoides, a distinct disease entity with or without
associated follicular mucinosis: a clinicopathologic and follow-up study
of 51 patients.
SO - Arch Dermatol 2002 Feb;138(2):191-8
AD - Department of Dermatology, B1-Q-93, Leiden University Medical Center,
Albinusdreef 2, 2333 ZA Leiden, The Netherlands.
OBJECTIVE: To determine the clinicopathologic features and the disease
course of patients with follicular mycosis fungoides (MF). DESIGN: A
multicenter, 14-year, retrospective cohort analysis. SETTING: Dutch
Cutaneous Lymphoma Group. PATIENTS: Fifty-one patients with the
clinicopathologic features of follicular MF with (n = 49) or without (n
= 2) associated follicular mucinosis. Follow-up data were compared with
those of 158 patients with the classic epidermotropic type of MF,
including 122 patients with generalized plaque-stage MF (T2 N0 M0) and
36 patients with tumor-stage MF (T3 N0 M0). OBSERVATIONS: Characteristic
clinical features not or rarely observed in classic MF were the
preferential localization of the skin lesions in the head and neck
region (45 of 51 patients), the presence of follicular papules,
alopecia, acneiform lesions, mucinorrhoea, and often severe pruritus.
Characteristic histologic findings were the presence of perifollicular
neoplastic infiltrates with a variable degree of folliculotropism, but
generally no epidermotropism, follicular mucinosis (49 of 51 cases), and
often a considerable admixture of eosinophils and plasma cells. Response
on initial treatment, risk of disease progression (development of
extracutaneous disease and/or death from lymphoma), and disease-specific
and overall survival of patients with follicular MF were worse than in
classic MF patients. The actuarial disease-specific survival was 68% at
5 years and 26% at 10 years. CONCLUSIONS: Follicular MF shows
distinctive clinicopathologic features, is more refractory to treatment,
and has a worse prognosis than the classic type of MF; it should be
considered a distinct type of cutaneous T-cell lymphoma. Based on these
results and those of other studies, we suggest the term follicular MF
for cases with or without associated follicular mucinosis.
UI - 11843640
AU - Dummer R; Kamarashev J; Kempf W; Haffner AC; Hess-Schmid M; Burg G
Junctional CD8+ cutaneous lymphomas with nonaggressive clinical
behavior: a CD8+ variant of mycosis fungoides?
SO - Arch Dermatol 2002 Feb;138(2):199-203
AD - Department of Dermatology, University Hospital, Gloriastrasse 31,
CH-8091 Zurich, Switzerland. email@example.com
OBJECTIVE: To evaluate the clinical and prognostic features in primary
cutaneous CD8+ T-cell lymphomas, which are rare and considered to be
aggressive cutaneous lymphoproliferative disorders. DESIGN:
Single-center retrospective study. SETTING: Lymphoma clinic (referral
center) of a university hospital. PATIENTS: Three patients presented
with CD8+ cutaneous lymphoma characterized by a patchlike pattern and
hyperpigmentation. RESULTS: Histological analysis revealed a CD3+, CD8+
small-cell infiltrate showing a remarkable affinity to the
dermoepidermal junction zone. Clonality for the T-cell receptor gamma
chain was detected by polymerase chain reaction followed by denaturing
gradient gel electrophoresis. The clinical presentation lasted several
years (6 and 9 years, respectively) before the correct diagnosis was
made. Treatment with nontoxic approaches (UV-B and local steroids) was
successful. Aggressive clinical behavior was not observed. CONCLUSIONS:
Our 3 cases of junctional CD8+ cutaneous T-cell lymphomas were
characterized by hyperpigmentation and nonaggressive clinical behavior.
This type of lymphoma, which can be considered a CD8+ mycosis fungoides
variant, must be distinguished from other types of cutaneous CD8+
lymphomas so that overtreatment can be avoided.
UI - 11843646
AU - Sangueza OP; Lu D
Mycosis fungoides: new insights into an old problem.
SO - Arch Dermatol 2002 Feb;138(2):244-6
UI - 11917593
AU - Kayao J; Tamaru J; Wakita H; Saitou T; Kuzuu Y; Mikata A; Tanaka N
Anaplastic large cell lymphoma of the mediastinum diagnosed by
transbronchial scratch cytology. A case report.
SO - Acta Cytol 2002 Mar-Apr;46(2):405-11
AD - Departments of Pathology and Internal Medicine, Clinical Laboratory,
Narita Red Cross Hospital, 90-1 Iida-cho, Narita, Chiba 286-8523, Japan.
BACKGROUND: Anaplastic large cell lymphoma (ALCL) is a subtype of
non-Hodgkin's lymphoma characterized by CD30 antigen-positive, large
neoplastic cells. We describe a case of ALCL suggested by cytologic
examination of the tumor cells obtained from bronchial scratch
preparations. CASE: A 26-year-old woman had had a dry cough since
in the mediastinum extending to the pulmonary hilar region. The patient
lesion in the anterior mediastinum invading the right lung.
Transbronchial scratch cytology revealed large, atypical lymphoid cells
expressing CD30 and CD3 on immunocytochemical examination. A
transcutaneous mediastinal biopsy was performed and a diagnosis of ALCL
made. CONCLUSION: Differentiation from Hodgkin's disease was the most
difficult point in this case. Detailed cytologic observation and
CD3-positive immunocytology led to the correct diagnosis. The cell
transfer technique of Sherman et al was very useful for immunocytologic
staining. Thus, transbronchial scratch cytology was an especially
valuable and effective procedure in this case.
UI - 11902983
AU - Breneman D; Duvic M; Kuzel T; Yocum R; Truglia J; Stevens VJ
Phase 1 and 2 trial of bexarotene gel for skin-directed treatment of
patients with cutaneous T-cell lymphoma.
SO - Arch Dermatol 2002 Mar;138(3):325-32
AD - Department of Dermatology, University of Cincinnati, OH, USA.
OBJECTIVE: To evaluate the safety, dose tolerance, and efficacy of
topical bexarotene gel in patients with early-stage cutaneous T-cell
lymphoma (CTCL). DESIGN: Phase 1 and 2, open-label, dose-escalation
clinical trial of bexarotene gel. SETTING: Three university-based
clinics. PARTICIPANTS: Sixty-seven adults with early-stage (TNM stages
IA-IIA) CTCL. INTERVENTIONS: Bexarotene gel, 0.1%, 0.5%, and 1.0%,
applied in incremental dose adjustments from 0.1% gel every day to 1.0%
gel 4 times daily or the maximal tolerated dose. MAIN OUTCOME MEASURES:
Patients were followed for efficacy and safety, and treatment continued
as long as they benefited. Response (> or =50% improvement) was
evaluated by the Physician's Global Assessment of cutaneous disease and
by an overall severity assessment of cutaneous disease, including signs
of CTCL and area involved. RESULTS: Most patients tolerated topical
bexarotene at 1% gel twice daily for routine use. Adverse events were
generally mild to moderate in severity and were confined to treatment
sites. Treatment-limiting toxic effects were associated with skin
irritation and increased with gel exposure. Patients achieved an overall
response rate of 63% and a clinical complete response rate of 21%.
Median projected time to onset of response was 20.1 weeks (range,
4.0-86.0 weeks), and the estimated median response duration from the
start of therapy was 99 weeks. Patients with no previous therapy for
mycosis fungoides responded at a higher rate (75%) than those who
previously underwent topical therapies (67%). CONCLUSIONS: Bexarotene
gel was well tolerated, was easily self-applied, and had a substantial
response rate in treating patients with early-stage CTCL.
UI - 11902993
AU - Liu HL; Kim YH
Bexarotene gel: a Food and Drug Administration-approved skin-directed
therapy for early-stage cutaneous T-cell lymphoma.
SO - Arch Dermatol 2002 Mar;138(3):398-9
UI - 10640924
AU - Pujol RM; Gallardo F; Llistosella E; Blanco A; Bernado L; Bordes R;
Nomdedeu JF; Servitje O
Invisible mycosis fungoides: A diagnostic challenge.
SO - J Am Acad Dermatol 2000 Feb;42(2 Pt 2):324-8
AD - Hospital Princeps d'Espanya, Barcelona, Spain. firstname.lastname@example.org
We describe a 76-year-old woman who presented persistent generalized
pruritus as the only cutaneous manifestation of a cutaneous T-cell
lymphoma (mycosis fungoides). No cutaneous lesions were observed
throughout the patient's course. Skin biopsies obtained from
normal-looking pruritic skin revealed a discrete perivascular
lymphocytic infiltrate in the upper dermis and focal intraepidermal
clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR
analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement.
Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR
gene rearrangement. This observation illustrates the existence of a
peculiar and exceedingly rare form of mycosis fungoides characterized
only by persistent pruritus unresponsive to several therapeutic
approaches. The diagnostic difficulties of this rare variant are
UI - 11464202
AU - Hwong H; Nichols T; Duvic M
"Invisible" mycosis fungoides?
SO - J Am Acad Dermatol 2001 Aug;45(2):318
UI - 11464203
AU - Dereure O; Guilhou JJ
Invisible mycosis fungoides: a new case.
SO - J Am Acad Dermatol 2001 Aug;45(2):318-9
UI - 11720171
AU - Shohat M; Hodak E; Sredni B; Shohat B; Sredni D; David M
Cytokine profile of patients with mycosis fungoides and the
immunomodulatory effect of AS101.
SO - Acta Derm Venereol 2001 Aug-Sep;81(4):255-7
AD - Department of Dermatology, Rabin Medical Center, Petah Tiqva, Israel.
Cytokines are known to play a major role in the pathogenesis of mycosis
fungoides, a cutaneous malignant neoplasm of CD 4 T cells. In the
present study, we investigated the effect of AS101, a tellurium-based
compound with immunomodulating properties, on the pattern of lymphokine
production by peripheral blood mononuclear cells (PBMCs) from patients
with mycosis fungoides. PBMCs were isolated from 35 patients with
mycosis fungoides stage IA and IB before initiation of treatment and
from 20 healthy sex and age-matched controls. Unstimulated and
phytohaemagglutinin-stimulated PBMCs were tested with and without the
addition of AS101. The production of interferon-gamma, interleukin 2
(IL-2), IL-2 receptor (IL-2R), interleukin 5 (IL-5) and interleukin 10
(IL-10) was determined by enzyme-linked immunosorbent assays. The
effects of AS-101 on mycosis fungoides PBMCs were compared to those of
healthy donor PBMCs. Significantly higher levels of IL-2R, IL-5 and
IL-10 and significantly lower levels of interferon-gamma were found in
the patients compared to the controls. There was no significant
difference between the groups in the production of IL-2. AS101 inhibited
the production of IL-2R, IL-5 and IL-10 and induced a significant
increase in IL-2 levels in the mycosis fungoides PBMCs. These findings
may have important clinical implications for the possible therapeutic
benefit of AS101 in mycosis fungoides.
UI - 11730054
AU - Burg G; Kempf W; Dummer R
Diagnostic signs of cutaneous lymphomas.
SO - J Eur Acad Dermatol Venereol 2001 Jul;15(4):358-60
UI - 11730060
AU - Hazneci E; Aydin NE; Dogan G; Turhan IO
Primary cutaneous anaplastic large cell lymphoma in a young girl.
SO - J Eur Acad Dermatol Venereol 2001 Jul;15(4):366-7
UI - 11862481
AU - Wollina U; Graefe T; Feldrappe S; Andre S; Wasano K; Kaltner H; Zick Y;
Galectin fingerprinting by immuno- and lectin histochemistry in
SO - J Cancer Res Clin Oncol 2002 Feb;128(2):103-10
AD - Department of Dermatology and Dermatological Allergology,
Friedrich-Schiller-University, Jena, Germany. Wollina-Uw@khdf.de
Owing to their relevance for growth regulation and cell adhesion
monitoring the expression of galectins (endogenous
beta-galactoside-binding lectins) and their binding sites has relevance
for tumor biology. Using galectin-type-specific reagents
(non-crossreactive antibodies for proto-type galectin-1, chimera-type
galectin-3 and tandem-repeat-type galectins-4 and -8, and labeled
galectins-1, -3, and -4) we determined galectin expression in cutaneous
T cell lymphomas (CTCL) and controls. In addition to commonly studied
galectins-1 and -3, tandem-repeat-type galectins could be detected,
i.e., galectin-8 in six from 15 cases and galectin-4 in one of 15 cases.
In view of relevant ligands such as bcl-2 or integrins the presence of
galectins-3 and -8 seems to be possibly related to loss of proliferation
control and change in cell adhesion properties that are involved in
clonal expansion and epidermal spread of malignant T cell clones.
Successful chemotherapy of CTCL alters galectin expression selectively
as shown for liposomal doxorubicin.
UI - 11874489
AU - Scarisbrick JJ; Woolford AJ; Calonje E; Photiou A; Ferreira S; Orchard
G; Russell-Jones R; Whittaker SJ
Frequent abnormalities of the p15 and p16 genes in mycosis fungoides and
SO - J Invest Dermatol 2002 Mar;118(3):493-9
AD - Skin Tumor Unit, St. John's Institute Dermatology, St. Thomas' Hospital,
Lambeth Palace Road., London, UK. email@example.com
There are few data on the molecular pathogenesis of cutaneous T cell
lymphomas. A recent allelotyping study by our group identified frequent
allelic loss on 9p, 10q, and 17p including losses on 9p21 in 16% of
patients with mycosis fungoides and 46% with Sezary syndrome. The P15
and P16 genes are intricately linked on 9p21 and can be inactivated in
melanoma and non-Hodgkin's lymphoma. We have therefore studied 76
patients with either mycosis fungoides or Sezary syndrome for
abnormalities of these genes. DNA samples were analyzed for loss of
heterozygosity, homozygous deletion, intragenic mutations, and promoter
methylation. In addition P15 and P16 protein expression was assessed.
Microsatellite analysis was informative in 73 of 76 cases: allelic loss
on 9p21 was identified in 18 patients (25%), including 12 of 57 with
mycosis fungoides (21%) and six of 16 with Sezary syndrome (37%). Single
strand conformation polymorphism analysis of the entire coding regions
of both genes did not identify any mutations, although two polymorphisms
were identified including C613A, which has not previously been
described. P15 and P16 gene promoter methylation was found in 45% and
29% of patients, respectively. Furthermore aberrant P15 protein
expression was detected in 85% of patients analyzed with P15 gene
abnormalities and abnormal P16 expression in 59% with P16 gene
abnormalities. These abnormalities were not dependent on cutaneous stage
of disease. This study suggests that abnormalities of the P15 and P16
genes are common in both early and advanced stages of mycosis fungoides
and Sezary syndrome and that these genes may be inactivated by allelic
loss and aberrant promoter methylation.
UI - 11862472
AU - Wollina U; Graefe T; Fuller J
Granulomatous slack skin or granulomatous mycosis fungoides -- a case
report. Complete response to percutaneous radiation and interferon
SO - J Cancer Res Clin Oncol 2002 Jan;128(1):50-4
AD - Department of Dermatology, Hospital Dresden-Friedrichstadt, PO Box
120706, 01008 Dresden, Germany. Wollina-Uw@khdf.de
A 46-year-old man presented with circumscribed inflammatory
poikilodermatic lesions of loose skin on the upper arm. Histologic
examination disclosed a heavy lymphocytic infiltrate of the whole dermis
and the upper part of the subcutaneous fat tissue with a predominant
T-helper phenotype and about 10% of Mac 387-positive macrophages and
some scattered multinucleated giant cells. Lymphocytic cells were
aligned along the epidermal basement membrane and showed focal
epidermotropism. In part these cells had multilobular nuclei. The
diagnosis of granulomatous mycosis fungoides versus early granulomatous
slack skin was made. The patient was treated with a combination of
radiotherapy (total dose 36 Gy) and interferon-alpha as a maintenance
treatment which resulted in complete remission and disease-free survival
of 27 months up to now.
UI - 10906662
AU - Railan D; Fivenson DP; Wittenberg G
Capillary leak syndrome in a patient treated with interleukin 2 fusion
toxin for cutaneous T-cell lymphoma.
SO - J Am Acad Dermatol 2000 Aug;43(2 Pt 1):323-4
UI - 11208829
AU - Olsen E; Duvic M; Frankel A; Kim Y; Martin A; Vonderheid E; Jegasothy B;
Wood G; Gordon M; Heald P; Oseroff A; Pinter-Brown L; Bowen G; Kuzel T;
Fivenson D; Foss F; Glode M; Molina A; Knobler E; Stewart S; Cooper K;
Stevens S; Craig F; Reuben J; Bacha P; Nichols J
Pivotal phase III trial of two dose levels of denileukin diftitox for
the treatment of cutaneous T-cell lymphoma.
SO - J Clin Oncol 2001 Jan 15;19(2):376-88
AD - Duke University Medical Center, Durham, NC 27710, USA.
PURPOSE: The objective of this phase III study was to determine the
efficacy, safety, and pharmacokinetics of denileukin diftitox
(DAB389IL-2, Ontak [Ligand Pharmaceuticals Inc, San Diego, CA]) in
patients with stage Ib to IVa cutaneous T-cell lymphoma (CTCL) who have
previously received other therapeutic interventions. PATIENTS AND
METHODS: Patients with biopsy-proven CTCL that expressed CD25 on > or =
20% of lymphocytes were assigned to one of two dose levels (9 or 18
microg/kg/d) of denileukin diftitox administered 5 consecutive days
every 3 weeks for up to 8 cycles. Patients were monitored for toxicity
and clinical efficacy, the latter assessed by changes in disease burden
and quality of life measurements. Antibody levels of antidenileukin
diftitox and anti-interleukin-2 and serum concentrations of denileukin
diftitox were also measured. RESULTS: Overall, 30% of the 71 patients
with CTCL treated with denileukin diftitox had an objective response
(20% partial response; 10% complete response). The response rate and
duration of response based on the time of the first dose of study drug
for all responders (median of 6.9 months with a range of 2.7 to more
than 46.1 months) were not statistically different between the two
doses. Adverse events consisted of flu-like symptoms (fever/chills,
nausea/vomiting, and myalgias/arthralgias), acute infusion-related
events (hypotension, dyspnea, chest pain, and back pain), and a vascular
leak syndrome (hypotension, hypoalbuminemia, edema). In addition, 61% of
the patients experienced transient elevations of hepatic transaminase
levels with 17% grade 3 or 4. Hypoalbuminemia occurred in 79%, including
15% with grade 3 or 4 changes. Tolerability at 9 and 18 microg/kg/d was
similar, and there was no evidence of cumulative toxicity. CONCLUSION:
Denileukin diftitox has been shown to be a useful and important agent in
the treatment of patients whose CTCL is persistent or recurrent despite
other therapeutic interventions.
UI - 11174402
AU - Acosta M; Teitelbaum A
Capillary leak syndrome in a patient treated with interleukin 2 fusion
toxin for cutaneous T-cell lymphoma.
SO - J Am Acad Dermatol 2001 Feb;44(2):312-3
UI - 11270155
AU - Bell TJ
T-cell lymphoma products.
SO - Nurse Pract 2001 Mar;26(3):19
UI - 11520254
AU - Vittorio CC; Rook AH; French LE; Shapiro M; Lehrer MS; Junkins-Hopkins
Therapeutic advances in biological response modifiers in the treatment
of cutaneous T-cell lymphoma.
SO - BioDrugs 2001;15(7):431-7
AD - Department of Dermatology, University of Pennsylvania School of
Medicine, Philadelphia, Pennsylvania 19104, USA.
Cutaneous T-cell lymphoma (CTCL) is most often a skin-infiltrating
malignancy of clonal CD4+ T-cells. Therapy is based on staging and the
likelihood of progression. Biological response modifiers and
chemotherapeutic agents are used to preserve the integrity of the host
antitumour response while selectively targeting the malignant cells. The
biological response-modifying treatment options currently used to treat
CTCL are bexarotene, denileukin diftitox, interferon-alpha,
interferon-gamma and interleukin-12, as well as extracorporeal
photopheresis and phototherapy. A combination therapy approach maximises
response in patients with advanced CTCL. Biological response modifiers
in combination with photopheresis are used for patients with the
leukaemic phase of the disease. Among the majority of patients with
advanced stage disease so treated, immune response augmentation appears
to prolong survival. Future areas of research should assess not only
survival and optimal treatment combinations, but also quality of life
during the treatment period.
UI - 11705358
AU - Apisarnthanarax N; Duvic M
Cutaneous T-cell lymphoma. New immunomodulators.
SO - Dermatol Clin 2001 Oct;19(4):737-48
AD - Division of Internal Medicine, Department of Dermatology, University of
Texas, MD Anderson Cancer Center, Houston, Texas, USA.
During the most recent decades, much knowledge has been gained
concerning the immunologic and pathologic mechanisms of CTCL. The
development of immunomodulators aimed at correcting aberrations in
immunology and cellular growth and differentiation reflects this
increased understanding. This review of the currently available
immune-response modifying drugs shows that recombinant forms of natural
cytokines and retinoids can be developed with tolerable toxicity
profiles and substantial efficacy. Although milestone drugs such as
bexarotene have been approved by the FDA- for treatment of CTCL, other
agents such as IL-12 may also have a place in treatment of the disease.
Even though unapproved, IFN-alpha may be the most active single
immunomodulating agent against CTCL. It seems that further delineation
of CTCL cytokine profile changes and immunologic aberrations are key in
developing effective immunomodulators that are able to reverse these
UI - 11891807
AU - Awaya N; Mori S; Takeuchi H; Mori S; Sugano Y; Kamata T; Takeuchi T; Abe
CD30 and the NPM-ALK fusion protein (p80) are differentially expressed
between peripheral blood and bone marrow in primary small cell variant
of anaplastic large cell lymphoma.
SO - Am J Hematol 2002 Mar;69(3):200-4
AD - The Second Department of Internal Medicine, Saitama Medical Center,
Saitama Medical School, Saitama, Japan. firstname.lastname@example.org
The t(2;5)(p23;q35) translocation results in the formation of a unique
chimeric NPM-ALK protein (p80). Expression of this protein is considered
to be one of the clinical features of anaplastic large cell lymphoma
(ALCL). Recently recognized as one clinical subtype of ALCL, the small
cell variant is prone to have a leukemic presentation. Although the
small cell variant has been recognized as a subtype of ALCL, the
clinical properties of this subtype, especially the immunophenotype of
lymphoma cells in peripheral blood, have not yet been fully described.
This report shows that neither CD30 nor p80 is detected by
immunostaining in the predominant small cell malignant clone and also in
large lymphoma cells in peripheral blood, while large cells and
occasionally observed small cells in bone marrow were found to be
positive for CD30 and p80. Our findings suggest that differential
expression of CD30 and p80 between peripheral blood and bone marrow
lymphoma cells is a property of the small cell variant of ALCL.
UI - 11809604
AU - Roustan G; Yus ES; Simon A
Nevoid hyperkeratosis of the areola with histopathological features
mimicking mycosis fungoides.
SO - Eur J Dermatol 2002 Jan-Feb;12(1):79-81
AD - Department of Dermatology, Clinica Universitaria Puerta de Hierro,
Madrid, Spain. email@example.com
Hyperkeratosis of the areola is a rare benign condition of unknown
etiology characterized by slowly growing verrucous thickening and brown
pigmentation of the areola or/and the nipple. It may be presented as
isolated nevoid form or associated with other skin diseases. We
described a 21-year-old woman with characteristic lesions of nevoid
hyperkeratosis in both areolas but with histopathological findings
resembling mycosis fungoides. Cutaneous lesions have remained unchanged
after two years of follow-up.
UI - 11843294
AU - Fukuda N; Shinohara K; Ota I; Muraki K; Shimohakamada Y
Therapy-related myelodysplastic syndrome in a case of cutaneous adult
SO - Int J Hematol 2002 Jan;75(1):67-71
AD - Department of Medicine, Yamaguchi Prefecture Central Hospital, Hofu,
We report a case of therapy-related myelodysplastic syndrome (t-MDS) in
adult T-cell lymphoma. A 69-year-old man suffered from cutaneous adult
T-cell lymphoma, which was treated with radiation to the skin and
combination chemotherapy of CHOP-V-MMV and VEPA-B. After 14 months of
these therapies, anemia and thrombocytopenia appeared, and bone marrow
aspiration smears showed immature myeloblasts, dysplastic erythroblasts,
and micromegakaryocytes. Therapy-related MDS of refractory anemia with
an excess of blasts was diagnosed. Cytogenetic study of the bone marrow
cells showed 5q- and additional abnormalities. Rearrangement of the MLL
gene was observed in the bone marrow cells. Mutations of N-ras codons at
12,13, and 61, p53 tumor suppressor gene, and monoclonal integration of
human T-lymphotrophic virus -1 provirus DNA were not observed in the
bone marrow cells. The patient died of pneumonia 21 months after
diagnosis of cutaneous adult T-cell lymphoma.
UI - 11899329
AU - Saxon M
SO - Clin J Oncol Nurs 2000 Nov-Dec;4(6):289, 293
AD - Cancer Centers of the Carolinas, Seneca, SC, USA.
Primary cutaneous T-cell lymphomas (CTCLs) encompass a wide variety of
lymphomas that are characterized by the localization of the malignant
lymphocytes to the skin at presentation. They are slow-growing and rare,
occurring in fewer than 1,000 people annually. Patients may go for
months to years with skin abnormalities before being diagnosed. Mycosis
fungoides and Sezary syndrome are the most common forms of CTCL and are
considered to be indolent diseases. Patients with T1 disease have a
normal life expectancy, whereas patients who undergo transformation to
large cell lymphoma (8%-23% of patients) have a poor prognosis, with
mean survival ranging from 2-19 months.
UI - 11879274
AU - Young SK
New advances in the management of cutaneous T-cell lymphoma.
SO - Cancer Pract 2001 Jan-Feb;9(1):52-4
AD - Mercy Suburban Hospital, Norristown, Pennsylvania, USA.
UI - 11917163
AU - Liu V; McKee PH
Cutaneous T-cell lymphoproliferative disorders: approach for the
surgical pathologist: recent advances and clarification of confused
SO - Adv Anat Pathol 2002 Mar;9(2):79-100
AD - Department of Pathology, Brigham & Women's Hospital, Boston,
Massachusetts 02115, USA.
Cutaneous T-cell lymphoproliferative disorders (CTCLs) remain a subject
of confusion and controversy. In this review, the authors discuss
diagnostic criteria and classification, including the role of
immunohistochemistry and gene rearrangement studies. In addition,
cutaneous T-cell pseudolymphomas, the current status of parapsoriasis
and other premalignant syndromes, and the clinicopathological variants
of mycosis fungoides are discussed. CD30-positive lymphoproliferative
disorders and a number of rare variants of CTCL including granulamatous
slack skin, subcutaneous (panniculitic) T-cell lymphoma, gamma-delta
cutaneous lymphoma, NK/NK-like T-cell lymphoma, and primary cutaneous
CD8-positive epidermotropic cytotoxic T-cell lymphoma are also
UI - 11917454
AU - Bouwhuis S; Davis MD
Sezary syndrome: a summary.
SO - Dermatol Nurs 2001 Jun;13(3):205-7, 230
AD - Mayo Graduate School of Medicine, Rochester, MN, USA.
Sezary syndrome is the leukemic form of primary cutaneous T-cell
lymphoma. It is an aggressive disease, with the lowest reported median
survival of all cutaneous lymphomas. Patients with Sezary syndrome live
with the awareness that they are suffering from an incurable disease.
Having to cope daily with extensive skin care regimens, these patients
can benefit tremendously from the expertise of dermatology nurses, who
can teach them skin selfcare and who are aware of the psychologic impact
of this disease. The symptoms, treatments, and emotional distress
related to Sezary syndrome are summarized.
UI - 11907502
AU - von den Driesch P; Coors EA
Localized cutaneous small to medium-sized pleomorphic T-cell lymphoma: a
report of 3 cases stable for years.
SO - J Am Acad Dermatol 2002 Apr;46(4):531-5
AD - Department of Dermatology, University of Erlangen-Nuremberg, Germany.
Small to medium-sized pleomorphic cutaneous T-cell lymphomas represent a
provisional entity in the new European Organization for Research and
Treatment of Cancer classification. We describe 3 patients with a
localized and outstanding stable variant of this tumor. A median
follow-up period of 50 months did not reveal any spread into regional
lymph nodes or to distant sites in any patient.
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