NCI CANCERLIT^® Search: Cutaneous T-cell Lymphoma - September 2001

Last Modified: November 1, 2001

A case of adult T-cell leukaemia/lymphoma characterized by multiplex-fluorescence in situ hybridization, comparative genomic hybridization, fluorescence in situ hybridization and cytogenetics.
CD30+ lymphoproliferative disorder: primary cutaneous anaplastic large cell lymphoma followed by lymphomatoid papulosis.
Phagocytized apoptotic cells in subcutaneous panniculitis-like T-cell lymphoma.
Sezary syndrome in a Malay--case report and literature review.
Primary cutaneous CD30+ T-cell lymphomas: hypothesis for a highly distinctive clinicobiological behaviour.
Neutrophil-rich anaplastic large cell lymphoma of the skull presenting after head trauma.
Therapeutic advances in biological response modifiers in the treatment of cutaneous T-cell lymphoma.
Inability to culture the dominant T-cell clone from the skin of primary cutaneous T-cell lymphoma as proven by TCR gamma-chain gene sequencing.
[Ki-1 lymphoma of the skin in a patient with Crohn's disease undergoing treatment with azathioprine]
Evaluation of a new paraffin-reactive CD7 T-cell deletion marker and a polymerase chain reaction-based T-cell receptor gene rearrangement assay: implications for diagnosis of mycosis fungoides in community clinical practice.
CD45RA+ immunophenotype in mycosis fungoides: clinical, histological and immunophenotypical features in 22 patients.

CancerMail from the National Cancer Institute

1
UI - 21347115
AU - Mao X; Lillington DM; Czepulkowski B; Young BD; Russell-Jones R; Whittaker S
TI - A case of adult T-cell leukaemia/lymphoma characterized by multiplex-fluorescence in situ hybridization, comparative genomic hybridization, fluorescence in situ hybridization and cytogenetics.
SO - Br J Dermatol 2001 Jul;145(1):117-22
AD - Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, London SE1 7EH, UK. mxmayo@hotmail.com
Adult T-cell leukaemia/lymphoma (ATLL) is a neoplasm of mature helper (CD4) T lymphocytes. Little is known, however, about the chromosome aberrations associated with the pathogenesis of this malignancy. Using molecular cytogenetic techniques we, therefore, investigated a 44-year-old man who had a 7-year history of ATLL with cutaneous involvement mimicking primary cutaneous T-cell lymphoma. Conventional cytogenetics revealed gross chromosomal changes with chromosome numbers ranging from 71 to 82. There were structural abnormalities of chromosomes 7 and 9, partial deletions of chromosomes 1, 3, 5 and 6, and loss of chromosomes 2, 4, 9, 11--14, 21 and 22. Multiplex-fluorescence in situ hybridization (M-FISH) identified two derivative chromosomes, der(6)t(6;7)(q16;q21) and der(7)t(6;7)(q16;q21)ins(6;12)(q2?;?), and a deletion of chromosome 1p. Conventional FISH confirmed the M-FISH findings. Comparative genomic hybridization of the blood revealed gains of DNA copy number at 1q12--25, 6p24--25, 9p23, 16p13--q13, 17q11--21, 19p13 and 20q13 and loss at 11p15 while lymph nodes showed gains at 3p22--24, 3q27--29, 7q36 and 15q26 and losses at 2p24--25, 2q37, 10p14--15, 11p15, 13q33--34 and 16p13.3. No DNA copy number changes were seen in a skin lesion. These results show the extent of genetic abnormalities within this malignancy.

2
UI - 21347116
AU - Aoki M; Niimi Y; Takezaki S; Azuma A; Seike M; Kawana S
TI - CD30+ lymphoproliferative disorder: primary cutaneous anaplastic large cell lymphoma followed by lymphomatoid papulosis.
SO - Br J Dermatol 2001 Jul;145(1):123-6
AD - Department of Dermatology, Nippon Medical School Main Hospital, 1-1-5, Sendagi, Bunkyo-ku, Tokyo, Japan 113. mikan@nms.ac.jp
CD30+ large anaplastic lymphoid cells are seen in anaplastic large cell lymphoma (ALCL), and also in lymphomatoid papulosis (LyP) and other lymphoproliferative disorders. It can be difficult precisely to categorize these disorders with CD30+ cells. We report a case of primary cutaneous CD30+ ALCL with systemic metastases in whom the clinical disease subsequently evolved into LyP. The patient was initially administered cisplatin and etoposide and made a good response. Eighteen months later, recurrent, self-healing cutaneous small nodules appeared around the original tumour site without any systemic involvement. Histopathological examination of the recurrent lesions revealed infiltration with a mixture of cells that included neutrophils, eosinophils and CD30+ large anaplastic cells cytologically identical with those in the primary lesion. The anaplastic cells in both the primary and recurrent lesions were positive for monoclonal antibodies CD30, CD25 and a monoclonal antibody directed against the chimeric protein p80(NPM-ALK). These observations suggest the possibility that the ALCL and the subsequent LyP represent different clinical manifestations of proliferation of the same clone.

3
UI - 21386500
AU - Ikeda E; Endo M; Uchigasaki S; Baba S; Suzuki H; Kinukawa N; Nemoto N
TI - Phagocytized apoptotic cells in subcutaneous panniculitis-like T-cell lymphoma.
SO - J Eur Acad Dermatol Venereol 2000 Mar;15(2):159-62
AD - Department of Dermatology, Surugadai Nihon University Hospital, Tokyo, Japan.
We reported on a case of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with multiple erythematous nodular lesions on the extremities, trunk and face. Histological examination of an excised lesion revealed a dense infiltrate of markedly atypical T-lymphoid cells expressing the CD8+ phenotype located in the subcutaneous tissue with histiocyte-phagocytizing apoptotic cells. The 'bean-bag' histiocytic cells, the characteristic finding of SPTCL, are considered to be products of haemophagocytosis. In our case the 'bean-bag' cells were produced by phagocytosis of apoptotic bodies, as confirmed by electron microscopy. It is suspected that 'bean-bag' cells are related not to haemophagocytosis but to phagocytosis of apoptotic cells in the CD8+ T-cell type of SPTCL.

4
UI - 21403966
AU - Yuen P; Chan HL; Lee YS
TI - Sezary syndrome in a Malay--case report and literature review.
SO - Singapore Med J 2001 May;42(5):224-7
AD - Department of Medicine, National University Hospital, Singapore.
Sezary syndrome is a rare form of primary cutaneous T cell lymphoma. It is a distinct systemic variant of mycosis fungoides, marked by erythroderma, lymphadenopathy and circulating cerebriform lymphocytes in the peripheral blood. We report a case of Sezary syndrome in a 61-year-old Malay man with a five-year history of indurated plaques, ulcers and tumours on the head and trunk, with characteristic findings on physical examination, skin biopsy, electron microscopy, immunophenotyping and peripheral blood film. A literature review on Sezary syndrome is presented.

5
UI - 21386487
AU - Pimpinelli N
TI - Primary cutaneous CD30+ T-cell lymphomas: hypothesis for a highly distinctive clinicobiological behaviour.
SO - J Eur Acad Dermatol Venereol 2000 Mar;15(2):108-9

6
UI - 21334613
AU - Parker JR; Lopez-Terrada D; Gresik MV; Vogel H; Baumgartner JE; Finegold MJ
TI - Neutrophil-rich anaplastic large cell lymphoma of the skull presenting after head trauma.
SO - Pediatr Dev Pathol 2001 Jul-Aug;4(4):397-401
AD - Department of Pathology, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA.
The presentation of anaplastic large cell lymphoma in bone is uncommon. We report a case of anaplastic large cell lymphoma of the skull that was diagnosed after head trauma. Biopsy revealed significant destruction of the outer table of the frontal bone. Histopathologically, the initial evaluation suggested osteomyelitis because of a mixed inflammatory infiltrate with large numbers of neutrophils. However, several clusters and individual mononuclear cells were atypical. The tumor cells had large, pleomorphic nuclei; these cells stained positively with antibodies to Ki-1 (CD 30), ALK-1, and EMA. Fluorescence in situ hybridization (FISH) showed rearrangement of the ALK gene, which usually results from the t(2;5) translocation, present in most anaplastic large cell lymphomas. There was no evidence of systemic disease. The patient has tolerated chemotherapy and is free of disease 12 months later.

7
UI - 21411406
AU - Vittorio CC; Rook AH; French LE; Shapiro M; Lehrer MS; Junkins-Hopkins JM
TI - 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.

8
UI - 21256879
AU - Harwix S; Gunzl HJ; Blaschke V; Zachmann K; Neumann C
TI - Inability to culture the dominant T-cell clone from the skin of primary cutaneous T-cell lymphoma as proven by TCR gamma-chain gene sequencing.
SO - Arch Dermatol Res 2001 Mar;293(3):139-46
AD - Department of Dermatology, Von-Siebold-Str. 3, Gottingen University, 37075 Gottingen, Germany.
Molecular analysis of T-cell receptor (TCR) chain rearrangement has recently become an attractive tool for demonstrating the clonal origin of cutaneous T-cell lymphoma (CTCL) and for identifying the malignant clone at the molecular level. Over the past decade a number of attempts have been made to culture malignant CTCL cells using standard procedures and these attempts have resulted in several cell lines from the peripheral blood of Sezary syndrome, mycosis fungoides and CD30+ lymphoma patients. However, so far it has not been proven by sequence analysis that the cultured T cells truly represent the malignant cells. Aiming to functionally analyze the malignant T cells at a clonal level, we generated a total of 150 T-cell clones (TCC) from lesional skin and peripheral blood of three patients with mycosis fungoides and one patient with a CD30+ lymphoma. Cells were grown either in the presence of autologous irradiated peripheral blood feeder cells using various conditions for T-cell stimulation by direct outgrowth or from skin specimens with various cytokine combinations. In order to identify the malignant TCC we used N-region-specific PCR and compared TCR gamma-chain sequences from clones of lesional skin with in vitro-generated TCC. With the methods employed, none of the 150 established cell lines was found to be identical to the malignant TCC which was readily detected in lesional skin. Our results indicate that standard cell culture methods are not suitable for growing low-grade CTCL cells from the skin but give rise only to benign infiltrating T cells.

9
UI - 21306028
AU - Martinez Tirado P; Redondo Cerezo E; Gonzalez Aranda Y; J Cabello Tapia M; Nogueras Lopez F; Gomez Garcia M
TI - [Ki-1 lymphoma of the skin in a patient with Crohn's disease undergoing treatment with azathioprine]
SO - Gastroenterol Hepatol 2001 May;24(5):271-2

10
UI - 21402847
AU - Ormsby A; Bergfeld WF; Tubbs RR; Hsi ED
TI - Evaluation of a new paraffin-reactive CD7 T-cell deletion marker and a polymerase chain reaction-based T-cell receptor gene rearrangement assay: implications for diagnosis of mycosis fungoides in community clinical practice.
SO - J Am Acad Dermatol 2001 Sep;45(3):405-13
AD - Department of Anatomic Pathology and Dermatology, The Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH 44195, USA.
BACKGROUND: T-cell deletion and T-cell receptor (TCR) gene rearrangement studies are helpful in the early diagnosis and subsequent management of mycosis fungoides (MF). However, this often requires fresh-frozen tissue that can be difficult to obtain and evaluate in community clinical practice. A new CD7 antibody, the most sensitive and specific T-cell deletion marker, and a new TCR-gamma gene rearrangement polymerase chain reaction (PCR) assay (TCR-gamma PCR) are now available on routine paraffin-embedded biopsy specimens. OBJECTIVE: Our purpose was to assess the utility of CD7 deletion and TCR-gamma PCR in the diagnosis of MF using routine paraffin-embedded biopsy material. METHODS: Cases of MF (n = 17) with matching frozen tissue immunohistochemistry and benign reactive dermatoses (lichen planus; n = 27) were assessed for CD7 (Clone: CD7-272) deletion and TCR-gamma PCR using paraffin-embedded biopsy specimens. RESULTS: Excellent concordance comparing frozen and paraffin embedded CD7 immunostaining (88%) was observed. CD7 deletion and TCR-gamma PCR was sensitive (94%) and specific (96%) for a diagnosis of MF using paraffin-embedded biopsy specimens. CONCLUSION: In the diagnosis of MF, detection of CD7 deletion and monoclonal TCR rearrangements can be successfully performed in a cost-effective, timely fashion using routine formalin-fixed paraffin-embedded biopsy specimens.

11
UI - 21331831
AU - Fierro MT; Novelli M; Savoia P; Cambieri I; Quaglino P; Osella-Abate S; Bernengo MG
TI - CD45RA+ immunophenotype in mycosis fungoides: clinical, histological and immunophenotypical features in 22 patients.
SO - J Cutan Pathol 2001 Aug;28(7):356-62
AD - Department of Medical and Surgical Specialities, 1st Section of Dermatology, University of Turin, Torino, Italy.
BACKGROUND: Mycosis fungoides (MF) is a cutaneous T-cell lymphoma (CTCL) usually characterized by a T-helper memory phenotype (CD3+, CD4+, CD8-, CD45R0+). Aberrant phenotypes are more commonly seen in the tumor stages. CD45RA expression has so far been documented in only a few cases of CD8+ or TCR gamma delta+ CTCL and in some pagetoid reticulosis cases. METHODS: Two hundred and fifteen MF patients were immunophenotyped in our laboratory between January 1992 and June 2000 and 22 cases of CD45RA+ MF (8.7%) were identified by immunohistochemical analysis. RESULTS: The majority of these CD45RA+ patients (20/22) showed a patch-plaque stage disease and an indolent clinical course, as expected in early-stage MF. The remaining 2 patients presented with stage IIB and IVA MF, and were characterized by an aggressive clinical course, with systemic spread. The immunohistochemical analysis revealed that CD45RA+ neoplastic cells belonged to the memory compartment, displaying a CD62L-, CD11a+, CD29+ phenotype. Most patients showed aberrant phenotypes, with a loss of T-cell lineage markers and expression of cytotoxic molecules or gamma-delta chain of the T-cell receptor. CONCLUSIONS: Our data show that CD45RA+ MF is a rare variant of CTCL and shares with the classic MF cases both the clinical features and disease course, even if it is characterized by a higher incidence of immunopathological abnormalities.