UI - 11451328
AU - Georgala S; Katoulis AC; Symeonidou S; Georgala C; Vayopoulos G
Persistent pigmented purpuric eruption associated with mycosis
fungoides: a case report and review of the literature.
SO - J Eur Acad Dermatol Venereol 2001 Jan;15(1):62-4
AD - National University of Athens, Department of Dermatology and
Venereology, A. Sygros Hospital, Greece.
A purpuric eruption may be an unusual early manifestation of mycosis
fungoides (MF). On the other hand, persistent pigmented purpuric
dermatoses (PPPD) may, occasionally, evolve to cutaneous T-cell
lymphoma. Coexistence of these two conditions has been reported, but it
is extremely rare. We present the case of an elderly woman with a
long-standing pruritic, pigmented purpuric eruption. On 1-year
follow-up, histological features suggesting early MF were observed and
molecular analysis of the rearrangement of T-cell receptor genes
revealed clonality. Our patient may represent a case of PPPD evolving to
MF, a case of MF clinically featuring PPPD, or an intermediate condition
in a nosological continuity extending from PPPD to MF. A persistent
pigmented purpuric eruption may rarely be a harbinger of cutaneous
T-cell lymphoma. Therefore, vigilant long-term follow-up of PPPD is
UI - 11553063
AU - Bowles KM; Tooze R; Marcus RE
Large-cell lymphoma. An unusual late relapse.
SO - Clin Lab Haematol 2001 Jun;23(3):197-9
AD - Department of Haematology, Addenbrookes Hospital, Cambridge, UK.
We report a case of a 40-year-old man with a stage 4, anaplastic,
large-cell lymphoma. He had been diagnosed 13 years before as having a
liposarcoma, at which point he was treated with combination
chemotherapy, which included anthracycline. On review of the
histopathology from 13 years before, the original diagnosis of
liposarcoma was revised to that of an anaplastic large-cell lymphoma. A
diagnosis of relapsed anaplastic large-cell lymphoma was made. A MUGA
scan showed a reduced ejection fraction of 46%. Our patient responded
initially to combination chemotherapy, which included anthracycline,
without further reduction in his ejection fraction. This was followed by
high-dose chemotherapy and peripheral blood stem-cell transplantation.
Twenty months later he is well and remains in complete remission.
UI - 12056952
AU - McGinnis KS; Shapiro M; Junkins-Hopkins JM; Smith M; Lessin SR; Vittorio
CC; Rook AH
Denileukin diftitox for the treatment of panniculitic lymphoma.
SO - Arch Dermatol 2002 Jun;138(6):740-2
AD - firstname.lastname@example.org
UI - 12056954
AU - Yudle L
Healing from the inside out: one person's path with cutaneous T-cell
SO - Arch Dermatol 2002 Jun;138(6):748-50
AD - email@example.com
UI - 12065565
AU - Sarris AH; Phan A; Duvic M; Romaguera J; McLaughlin P; Mesina O; King K;
Medeiros LJ; Rassidakis GZ; Samuels B; Cabanillas F
Trimetrexate in relapsed T-cell lymphoma with skin involvement.
SO - J Clin Oncol 2002 Jun 15;20(12):2876-80
AD - Department of Lymphoma and Myeloma, University of Texas M.D. Anderson
Cancer Center, Houston, TX, USA. firstname.lastname@example.org
PURPOSE: Methotrexate (MTX) is active against lymphomas, but transport
or polyglutamylation mutations confer MTX resistance. Because
trimetrexate (TMTX) enters cells by passive diffusion and is not
polyglutamylated, its activity in relapsed T-cell lymphoma was
investigated. PATIENTS AND METHODS: Eligible patients had histologically
confirmed relapsed T-cell lymphoma involving the skin, had received more
than one previous regimen, were older than 16 years, had normal organ
function, and had no CNS disease or serious infections, including human
immunodeficiency virus. TMTX (200 mg/m(2)) was given intravenously every
14 days without topical or systemic corticosteroids. Patients who
responded received up to 12 doses. RESULTS: Twenty patients were
assessable for response. Median age was 59 years (range, 45 to 87
years); 13 patients were men. Three patients had anaplastic large-cell
lymphoma, 15 had mycosis fungoides or Sezary syndrome (14 with
large-cell transformation), and two had peripheral T-cell lymphoma.
Serum lactate dehydrogenase was high in 35%, and beta-2 microglobulin
was more than 3.0 mg/L in 35% of patients. The median number of previous
regimens was three (range, two to 15) and included MTX in five patients.
Disease was refractory to the regimen immediately preceding TMTX in 85%
of patients. Responses were complete in one and partial in eight
patients (overall response rate, 45%). Two of five patients previously
treated with MTX responded. Grade 3 or 4 mucositis was observed after
4%, infection after 3%, neutropenic fever after 6%, neutrophils less
than 100/microL after 4%, and platelets less than 10,000/microL after 3%
of TMTX doses. CONCLUSION: TMTX is active with acceptable toxicity in
this population and merits further investigation.
UI - 12033965
AU - Pereira EM; Maeda SA; Reis-Filho JS
Sarcomatoid variant of anaplastic large cell lymphoma mimicking a
primary breast cancer: a challenging diagnosis.
SO - Arch Pathol Lab Med 2002 Jun;126(6):723-6
AD - Salomao & Zoppi Associated Pathologists, Porto, Portugal.
The sarcomatoid variant of anaplastic large cell lymphoma is one of the
rarest histologic variants of this neoplasm. Due to its sarcomatoid
features, it is frequently misdiagnosed as a poorly differentiated
sarcoma, anaplastic carcinoma, or melanoma. We report the case of a
92-year-old woman with a sarcomatoid anaplastic large cell lymphoma
mimicking a primary breast neoplasm. The patient presented with a
rapidly enlarging lump in the left breast and nodules in the right
axilla. The immunohistochemical profile showed reactivity for leukocyte
common antigen, UCHL-1, vimentin, and CD30, but immunoexpression of
anaplastic lymphoma kinase was lacking. Anaplastic large cell lymphomas
are lymphoid neoplasms of T-cell/null-cell lineage that consistently
express the activation marker CD30 and usually carry a gene
rearrangement of the anaplastic lymphoma kinase gene. To the best of our
knowledge, this is the first reported case of sarcomatoid anaplastic
large cell lymphoma presenting as a primary breast neoplasm in which
anaplastic lymphoma kinase expression was assessed.
UI - 12090418
AU - Creager AJ; Geisinger KR; Bergman S
Neutrophil-rich Ki-1-positive anaplastic large cell lymphoma: a study by
fine-needle aspiration biopsy.
SO - Am J Clin Pathol 2002 May;117(5):709-15
AD - Department of Pathology, Wake Forest University, Baptist Medical Center,
Winston-Salem, NC, USA.
Fine-needle aspiration biopsy (FNAB) is an accurate, cost-effective
method of evaluating lymphomas. The neutrophil-rich variant of
anaplastic large cell lymphoma (NR-ALCL) is a rare non-Hodgkin lymphoma.
To our knowledge, we present thefirst study of NR-ALCL by FNAB cytology.
Histologic confirmation was available for both patients. Both cases were
positive for Ki-1 (CD-30) and were either T-cell or null-cell phenotype.
FNAB specimens were highly cellular with a single-cell pattern composed
of pleomorphic tumor cells, "hallmark" tumor cells, and a background
rich in neutrophils that occasionally obscured tumor cells. Diagnosis on
FNAB is difficult owing to the rarity of this tumor, its resemblance to
Hodgkin lymphoma and other non-Hodgkin lymphomas that express CD30, its
similarity to an infectious process, and its occasional confusion with
metastatic carcinoma and melanoma. Reproducible cytologic features
usually are present, and the diagnosis can be made conclusively by FNAB
in conjunction with ancillary studies.
UI - 11121132
AU - Bagot M; Martinvalet D; Echchakir H; Chabanette-Schirm F; Boumsell L;
Bensussan A; Martinvallet D
Functional inhibitory receptors expressed by a cutaneous T cell
lymphoma-specific cytolytic clonal T cell population.
SO - J Invest Dermatol 2000 Dec;115(6):994-9
AD - INSERM U448, Paris XII University, Hopital Henri Mondor, AP-HP, Creteil,
Inhibitory receptors on natural killer cells and on a minority of T
lymphocytes are major histocompatibility complex class Ia or Ib
specific. We have previously reported several tumor-specific cytotoxic T
cell clones infiltrating a CD4(+) V beta 13(+) cutaneous T cell
lymphoma. These clones mediated a specific major histocompatibility
complex class I-restricted cytotoxic activity toward the uncultured
tumor cells and autologous long-term tumor T cell lines. In this study,
we cultured with interleukin-2 the peripheral blood lymphocytes of the
same patient a few weeks before invasion of the blood by tumor cells. We
report the rapid and selective expansion of a CD8(+) V beta 13(+)
lymphoid population. This population was clonal, as it expressed a
unique T cell receptor-V beta junctional region. V beta 13(+) tumor
cells and V beta 13(+) reactive T cells were shown to have different
junctional sequences. The CD8(+) reactive clone was functional, as it
had a specific autologous tumor-specific, human leukocyte antigen-A2
restricted, cytotoxic activity. This clone coexpressed high levels of
CD158a, CD158b, p70, and CD94/NKG2A inhibitory receptors. Interestingly,
we found that anti-CD158a and anti-CD158b monoclonal antibodies could
inhibit anti-CD3 redirected cytotoxicity mediated by the reactive clonal
population. Further, an anti-human leukocyte antigen-B/C monoclonal
antibody enhanced the specific cytotoxic activity of the clone against
autologous tumor cells. These results are the first evidence that
inhibitory receptor expression can lead to the inhibition of cutaneous T
cell lymphoma-specific T cell responses.
UI - 11982647
AU - Bassiri-Tehrani S; BA BA; Cohen DE
Treatment of cutaneous T-cell lymphoma with alitretinoin gel.
SO - Int J Dermatol 2002 Feb;41(2):104-6
AD - Department of Dermatology, New York University School of Medicine, New
York 10016, USA.
UI - 11982650
AU - Ozdemir M; Demirkesen C; Arzuhal N; Tuzun Y
Mucin-poor follicular mycosis fungoides.
SO - Int J Dermatol 2002 Feb;41(2):112-4
AD - Department of Dermatology, Istanbul University Cerrahpasa Medical
Faculty, Istanbul, Turkey. email@example.com
UI - 12081166
AU - Chang SE; Huh J; Choi JH; Sung KJ; Moon KC; Koh JK
A case of hyper-IgE syndrome complicated by cutaneous, nodal, and liver
peripheral T cell lymphomas.
SO - J Dermatol 2002 May;29(5):320-2
UI - 12031034
AU - Braam P; Sanders CJ; Canninga-Van Dijk MR
Neurofibromatosis type 1 and mycosis fungoides.
SO - Int J Dermatol 2002 Apr;41(4):236-8
AD - Department of Dermatology, University Medical Center, Heidelberglaan
100, 3584 CX Utrecht, The Netherlands.
UI - 12077596
AU - Masood N; Russell KJ; Olerud JE; Sabath DE; Sale GE; Doney KC; Flowers
ME; Fefer A; Thompson JA
Induction of complete remission of advanced stage mycosis fungoides by
allogeneic hematopoietic stem cell transplantation.
SO - J Am Acad Dermatol 2002 Jul;47(1):140-5
AD - Fred Hutchinson Cancer Research Center, University of Washington School
of Medicine, Seattle, WA 98109-1023, USA.
Advanced mycosis fungoides (MF) is incurable with conventional
treatments. High-dose chemoradiotherapy with autologous bone marrow
transplantation has induced remissions in a small number of patients
with MF, but this modality is limited by a high relapse rate. We report
induction of complete remission in a 37-year-old woman with rapidly
progressive stage IV MF with allogeneic stem cell transplantation (Allo
SCT). She remains in continuous complete remission 2 years after
transplant. Allo SCT for MF is theoretically attractive, because there
is no contamination of the graft by malignant cells, and because of the
possibility of graft-versus-tumor effect. Although the results in this
patient are encouraging, more patients and longer follow-up are needed
to define the usefulness of Allo SCT in the treatment of MF.
UI - 12077599
AU - Zackheim HS; Koo J; LeBoit PE; McCalmont TH; Bowman PH; Kashani-Sabet M;
Jones C; Zehnder J
Psoriasiform mycosis fungoides with fatal outcome after treatment with
SO - J Am Acad Dermatol 2002 Jul;47(1):155-7
UI - 12077602
AU - Kimball AB; Turner ML
Mycosis fungoides and serology for human T-cell lymphotropic virus, type
SO - J Am Acad Dermatol 2002 Jul;47(1):159; discussion 159-60
UI - 12077583
AU - Bouwhuis SA; el-Azhary RA; Gibson LE; McEvoy MT; Pittelkow MR
Effect of insulin-dependent diabetes mellitus on response to
extracorporeal photopheresis in patients with Sezary syndrome.
SO - J Am Acad Dermatol 2002 Jul;47(1):63-7
AD - Department of Dermatology, Mayo Clinic, 200 First Street SW, Rochester,
MN 55905, USA.
BACKGROUND: Extracorporeal photopheresis (ECP) has become a primary
therapy for selected forms of cutaneous T-cell lymphoma, especially
Sezary syndrome. Variability in response of patients with Sezary
syndrome to ECP has been reported. OBJECTIVE: Our purpose was to
determine whether underlying medical conditions influence the efficacy
of ECP in patients with Sezary syndrome. METHODS: We retrospectively
reviewed the medical records of 55 patients with Sezary syndrome who
received ECP between 1987 and 2000. Efficacy criteria included decrease
in Sezary cell count, erythroderma, lymphadenopathy, organomegaly, and
pruritus. RESULTS: Thirty-four patients responded well and 10 patients
responded partially to ECP; 11 patients had no response. Nine patients
with no response to ECP had insulin-dependent diabetes mellitus (IDDM).
IDDM was documented in only 2 patients with a good response and in no
patients with a partial response to ECP. CONCLUSION: Patients with
Sezary syndrome and IDDM typically respond poorly to the standard ECP
UI - 12071044
AU - Fazaa B; Goucha S; Zermani R; Zeglaoui F; Zghal M; Kennou H; Mezni F;
Ezzine N; Kharfi M; Mokhtar I; Ben Jilani S; Kamoun MR
[Mycosis fungoides. Study of a series of 11 Tunisian cases]
SO - Tunis Med 2002 Jan;80(1):40-5
AD - Service de Dermatologie, Etablissement Publique de Sante Charles
Mycosis fungoides is an epidermotropic cutaneous T lymphoma. It's a non
Hodgkinian lymphoma. We report the results of a retrospective review of
11 mycosis fungoide seen during 22 years. The frequency of MF was about
39.3% among all cutaneous lymphoma. Six patients were male and 5 were
female; the mean age was about 56 years. Mean delay between diagnostic
and the first manifestation was about 25 months. All patients had the
progressive form: 4 had infiltrate plaques and 7 were at the tumoral
phase. Lymph nodes and medullar metastases were noted respectively in 1
and 2 cases. Treatment was mono or polychemotherapy associated in 6
cases with topical drug. Three patients died of their diseases According
to our experience and after reviewed the literature, we notice that our
patients are slightly younger without male predominance. The diagnostic
was done tardily and this may explain the pejorative prognostic.
UI - 12057060
AU - Siegel RS; Kuzel TM
Cutaneous T-cell lymphoma/leukemia.
SO - Curr Treat Options Oncol 2000 Apr;1(1):43-50
AD - Robert H. Lurie Comprehensive Cancer Center, Northwestern University
Medical School, 676 N. St. Clair, Suite 850, Chicago, IL 60611, USA.
Effective treatment for cutaneous T-cell lymphomas (CTCL) requires an
accurate and specific diagnosis based on the clinical presentation
combined with evaluation of the histopathology, immunophenotyping, and
gene rearrangement studies. Careful clinical and pathologic evaluation
in centers familiar with the diverse forms of CTCL is most valuable for
determining treatment options. The goals of treatment in mycosis
fungoides (MF), which afflicts more than 50% of patients with CTCL, are
the relief of symptoms and improvement in cosmetics. Despite some
uncontrolled clinical trial results that have been reported to suggest
"cures" in this disease, the general perception remains that this
disease is not curable with standard therapies available today.
Treatment is divided into topical (skin-directed) and systemic therapy.
The most active systemic agent for the treatment of MF remains
interferon-alpha, although many new modalities have recently been
approved for the treatment of CTCL.
UI - 11843225
AU - Mahendran R; Grant JW; Hoggarth CE; Burrows NP
Angioimmunoblastic T-cell lymphoma with cutaneous involvement.
SO - J Eur Acad Dermatol Venereol 2001 Nov;15(6):589-90
UI - 12113107
AU - Apisarnthanarax N; Duvic M
Therapy options in cutaneous T-cell lymphoma.
SO - Expert Rev Anticancer Ther 2001 Oct;1(3):403-20
AD - Department of Dermatology, MD Anderson Cancer Center, 1515 Holcombe
Boulevard, Box 434, Houston, TX 77030-4095, USA.
The treatment of cutaneous T-cell lymphoma (CTCL) is continually
evolving, as new and emerging drugs are added to the growing arsenal of
CTCL therapy. The availability of newly approved investigational
therapies, such as bexarotene, denileukin diftitox (DAB389- IL2),
monoclonal antibodies and novel chemotherapeutic agents, adds complexity
to decisions on the management and treatment of CTCL patients. In
formulating a treatment plan, therapeutic options are best approached
through consideration of overall clinical staging (stage IA-IVB) and
skin staging (T1-T4), which affect prognosis and the characteristics of
each individual patient's disease. This article will present and discuss
the optimal therapeutic agents for all clinical stages of CTCL patients,
based on currently available and investigational agents.
UI - 11766294
AU - Dominguez JM; Alegre J; Iglesias D; Recio J; Fernandez de Sevilla T
[Streptococcus pyogenes sepsis in a patient with Ki-1 anaplastic
SO - An Med Interna 2001 Oct;18(10):556-7
UI - 12060388
AU - Dereure O; Levi E; Vonderheid EC; Kadin ME
Infrequent Fas mutations but no Bax or p53 mutations in early mycosis
fungoides: a possible mechanism for the accumulation of malignant T
lymphocytes in the skin.
SO - J Invest Dermatol 2002 Jun;118(6):949-56
AD - Department of Pathology, Beth Israel Deaconess Medical Center-Harvard
Medical School, Boston, Massachusetts 02215, USA.
Mycosis fungoides (MF) is the most frequent manifestation of cutaneous T
cell lymphoma but its cause and pathophysiology remain unclear. Because
progression of lesions is characteristically slow, we hypothesized that
mycosis fungoides originates from an accumulation of lymphocytes due to
defective apoptosis of skin homing T lymphocytes. In this study, we
investigate possible alterations of three molecules regulating
apoptosis, i.e., Fas antigen, Bax, and p53, at the genomic level in skin
lesions from 44 patients with MF, as Fas mediates one of two major
pathways for apoptosis of activated T cells. Fas mutations were found in
six patients using a polymerase chain reaction and single-strand
conformational polymorphism method followed by cloning and sequencing of
abnormal polymerase chain reaction products. The mutations predict for
defective transmission of the death signal in three cases.
Immunohistochemistry demonstrated the lack of Fas protein expression on
dermal lymphocytes in one case with Fas gene mutation predicting for a
truncated death domain, whereas Fas protein was expressed by dermal
lymphocytes in the other investigated cases. By contrast, no mutations
of Bax or p53 were found, whereas immunohistochemistry demonstrated
increased p53 expression in the nucleus of basal keratinocytes above the
neoplastic infiltrate in some MF cases. These results support the
hypothesis that Fas defects may play a role in the pathogenesis of MF.
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