UI - 11952563
AU - Modschiedler K; Altenhoff J; von den Driesch P
SO - Br J Dermatol 2002 Mar;146(3):529-31
UI - 11950640
AU - del Carpio-O'Donovan R; Freeman C
Brainstem involvement with mycosis fungoides: an unusual central nervous
SO - AJNR Am J Neuroradiol 2002 Apr;23(4):533-4
AD - Department of Diagnostic Radiology, McGill University Health Centre,
McGill University, Montreal, Canada.
Changes in the brainstem were demonstrated with MR imaging in a patient
with mycosis fungoides. Previous reports of CNS involvement in this rare
disease have not had similar findings.
UI - 11859306
AU - Rampino M; Ragona R; Monetti U; Mussano A; Guarneri A; Sannazzari GL
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.
UI - 11914622
AU - El-Shabrawi-Caelen L; Cerroni L; Medeiros LJ; McCalmont TH
Hypopigmented mycosis fungoides: frequent expression of a CD8+ T-cell
SO - Am J Surg Pathol 2002 Apr;26(4):450-7
AD - Department of Dermatology, University of Texas-M.D. Anderson Cancer
Center, Houston, Texas, USA. firstname.lastname@example.org
Hypopigmented mycosis fungoides (MF) is a form of cutaneous T-cell
lymphoma in which hypopigmentation occurs in the absence of classic
lesions of MF. Hypopigmented MF predominantly affects people with dark
complexions. The natural history of this variant of cutaneous T-cell
lymphoma is similar to that of conventional MF, although the disease
onset is usually in childhood or adolescence. In a retrospective study
we evaluated the clinical, histopathologic, immunohistochemical, and
molecular characteristics of hypopigmented MF in 15 patients. Similar to
other reports, the disease onset occurred in childhood and adolescence
in most of the cases. The survival rate was comparable with that of
classic MF. We did not observe progression to systemic disease or lymph
node involvement. Histopathologically hypopigmented lesions were
indistinguishable from hyperpigmented or erythematous patches. On
immunohistochemical analysis a predominantly CD8+ infiltrate was
detected in the majority of cases (nine of 15 patients). To determine
whether epidermotropic CD8+ T cells represent the malignant T-cell clone
or whether these cells are innocent, tumor-infiltrating T lymphocytes,
we performed microdissection of epidermotropic CD8+ T cells and analyzed
T-cell receptor-gamma chain gene for rearrangements. The epidermotropic
CD8+ T lymphocytes showed clonal T-cell receptor gene rearrangement and
therefore represented the malignant T-cell clone. We conclude that
hypopigmented MF tends to occur in young people and that it belongs to
the group of CD8+ cutaneous T-cell lymphomas in the majority of cases.
UI - 11978575
AU - Bouwhuis SA; McEvoy MT; Davis MD
Sustained remission of Sezary syndrome.
SO - Eur J Dermatol 2002 May-Jun;12(3):287-90
AD - Department of Dermatology, Mayo Clinic, 200 First Street SW Rochester,
Minnesota 55905, USA.
Sezary syndrome, an aggressive form of cutaneous T-cell lymphoma, is a
devastating, highly symptomatic form of non-Hodgkin lymphoma. Malignant
clones of mature helper CD4 T cells containing large, convoluted nuclei
known as Sezary cells circulate in the blood and infiltrate the skin.
Clinical features include exfoliative erythroderma, generalized
lymphadenopathy, alopecia, onychodystrophy, palmoplantar hyperkeratosis,
and ectropion. Patients often have severe pruritus, burning sensations,
pain, bleeding from excoriations, and disfigurement. Extracorporeal
photopheresis, an immunomodulatory therapy, has become a primary therapy
for these patients. This pheresis-based therapy uses psoralen and
ultraviolet A radiation-mediated photochemotherapy to induce immune
responses. The effects of extracorporeal photopheresis vary
considerably. We report sustained remission (2 years) in a patient with
Sezary syndrome. Previously he had received extracorporeal photopheresis
and interferon alfa-2b injections. He is the only one of 55 patients
with Sezary syndrome treated at Mayo Clinic (Rochester, Minnesota, USA)
to achieve sustained remission on extracorporeal photopheresis alone.
UI - 10989648
AU - Edelman J; Meyerson HJ
Diminished CD3 expression is useful for detecting and enumerating Sezary
SO - Am J Clin Pathol 2000 Sep;114(3):467-77
AD - Department of Pathology, Case Western Reserve University and University
Hospitals of Cleveland, OH 44106, USA.
We evaluated 44 peripheral blood, bone marrow, and lymph node specimens
from 23 patients with the clinical diagnosis or suspicion of cutaneous
T-cell lymphoma by 4-color flow cytometry for the presence of altered
CD3 expression and compared the results with light microscopic evidence
of involvement by mycosis fungoides (MF) or Sezary syndrome (SS). In 19
specimens (15 peripheral blood, 3 lymph node, 1 bone marrow) from 6
patients, we noted reduced CD3 expression compared with normal T
lymphocytes. Diminished CD3 expression correlated with morphologic
evidence of MF/SS cells in all cases, although the enumeration of MF/SS
cells differed. Comparison of the enumeration of MF/SS cells in the
peripheral blood by CD3+CD7- phenotyping with CD3dim phenotyping
suggested that CD3dim phenotyping was the most accurate method for
counting MF/SS cells. Therefore, diminished CD3 expression as shown by
flow cytometry may be particularly useful for detecting and enumerating
MF/SS cells in hematopoietic tissue of patients with cutaneous T-cell
UI - 11952676
AU - Chave TA; Graham-Brown RA
Mycosis fungoides masquerading as tinea of the axilla.
SO - Clin Exp Dermatol 2002 Jan;27(1):66-7
UI - 10911796
AU - De Wolf-Peeters C; Achten R
Anaplastic large cell lymphoma: what's in a name?
SO - J Clin Pathol 2000 Jun;53(6):407-8
UI - 10911802
AU - ten Berge RL; Oudejans JJ; Ossenkoppele GJ; Pulford K; Willemze R;
Falini B; Chott A; Meijer CJ
ALK expression in extranodal anaplastic large cell lymphoma favours
systemic disease with (primary) nodal involvement and a good prognosis
and occurs before dissemination.
SO - J Clin Pathol 2000 Jun;53(6):445-50
AD - Department of Pathology, University Hospital Vrije Universiteit,
Amsterdam, The Netherlands.
AIMS: In anaplastic large cell lymphoma (ALCL), the site of origin has
been described as an important prognostic factor. Recently, a fusion
protein containing anaplastic lymphoma kinase (ALK) was described in
systemic nodal ALCL, and shown to be associated with a good prognosis.
The aims of this study were to investigate whether the presence of ALK
protein differs between ALCL of different sites of origin; to determine
whether ALK expression occurs before dissemination to other sites; and,
finally, to investigate whether the site of origin remains a prognostic
parameter in ALK negative ALCL. METHODS: ALK expression, as detected by
immunohistochemistry using the monoclonal antibodies ALK1 and ALKc, was
studied in 85 ALCLs from different sites of origin. In 22 patients, ALK
expression was studied in multiple biopsies from different sites
(including 13 skin, 16 lymph node, and nine other). Overall survival
time was analysed using the Kaplan Meier method. RESULTS: ALK expression
was found in 20 of 51 systemic ALCLs with (primary) nodal involvement.
No ALK expression was found in 15 primary cutaneous, 14
gastrointestinal, and five nasal ALCLs. Multiple and subsequent biopsies
of patients showed ALK expression to be identical to that seen in the
primary diagnostic biopsy. Kaplan Meier survival curves showed that in
ALK negative ALCLs originating from different sites, primary cutaneous
cases are associated with an excellent overall survival, whereas the
other cases show a comparable five years survival of less than 40%.
CONCLUSIONS: If present, ALK expression favours systemic ALCL with
(primary) nodal involvement, and can be used in differentiating between
extranodal involvement of systemic (nodal) ALCL and primary extranodal
ALCL. ALK is expressed consistently in multiple biopsies of a given
patient, indicating that the chromosomal abnormality leading to aberrant
ALK expression occurs before dissemination to other sites. Finally, in
ALK negative non-cutaneous ALCLs, different sites of origin show
comparable poor survival.
UI - 11376029
AU - Ten Berge RL; Oudejans JJ; Dukers DF; Meijer CJ
Anaplastic large cell lymphoma: what's in a name?
SO - J Clin Pathol 2001 Jun;54(6):494-5
UI - 11952816
AU - Breccia M; Petti MC; D'Elia GM; D'Andrea M; Carmosino I; Alimena G
Cutaneous pleomorphic T-cell lymphoma coexisting with myelodysplastic
syndrome transforming into acute myeloid leukemia: successful treatment
with a fludarabine-containing regimen.
SO - Eur J Haematol 2002 Jan;68(1):1-3
AD - Department of Cellular Biotechnology and Hematology, University La
Sapienza, Via Benevento 6, Rome, Italy. email@example.com
The coexistence of a primary myelodysplastic syndrome (MDS) and a T-cell
cutaneous non-Hodgkin's lymphoma is an extremely rare event, which has
so far only been reported in a single instance in the literature. We
describe herein an additional case in which the lymphoid disease was
combined with an MDS at the time of its evolution into acute myeloid
leukemia (AML). Both diseases were successfully treated with a regimen
containing fludarabine. We discuss possible pathogenetic mechanisms and
suggest the use of nonalkylating drugs, such as fludarabine, for the
treatment of this rare association of malignancies usually characterized
by a very poor response to therapy.
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