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14 - T-cell non-Hodgkin lymphoma
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- By Sheetal M. Kircher, Northwestern University, Beverly P. Nelson, Northwestern University, Steven T. Rosen, Northwestern University, Andrew M. Evens, Tufts University
- Edited by Robert Marcus, King's College London, John W. Sweetenham, University of Utah, Michael E. Williams
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- Book:
- Lymphoma
- Published online:
- 18 December 2013
- Print publication:
- 05 December 2013, pp 226-253
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Summary
Introduction
T-cell non-Hodgkin lymphomas (NHLs) are uncommon malignancies, representing approximately 10–12% of all lymphomas in the United States. Varied geographic frequencies of T-cell NHL have been documented, ranging from 18% of all NHLs diagnosed in Hong Kong to 1.5% in Vancouver, British Columbia. This may in part reflect increased exposure to pathogenic factors such as human T-cell leukemia virus I (HTLVI) and Epstein–Barr virus (EBV) in Asian nations. Immunophenotypic, cytogenetic, and molecular analyses have significantly enhanced the diagnostic capabilities as well as improved classification and prognostication for T-cell NHL. It is advocated that all cases of T-cell NHL be reviewed by an expert hematopathologist.
The current World Health Organization (WHO)/European Organization for Research and Treatment of Cancer (EORTC) classification recognizes seven distinct clinicopathologic non-cutaneous peripheral T-cell NHLs (Table 14.1). They include peripheral T-cell lymphoma (PTCL), not otherwise specified (NOS); angioimmunoblastic T-cell lymphoma (AITL); adult T-cell leukemia/lymphoma (ATLL); NK-/T-cell lymphoma (NK/TCL), nasal type; enteropathy-associated T-cell lymphoma; hepatosplenic T-cell lymphoma; and anaplastic large-cell lymphoma (ALCL), anaplastic lymphoma kinase-positive (ALK+) subtype; additionally, ALCL ALK- subtype is a provisional subtype in the most recent WHO classification. Subcutaneous panniculitis-like T-cell lymphoma and cutaneous γδ T-cell lymphoma are also discussed herein since these diagnoses portend similar prognoses to many of the above non-cutaneous T-cell NHLs and treatment paradigms are similar. Cutaneous T-cell lymphoma (CTCL) and the other primary cutaneous lymphomas are reviewed elsewhere.
22 - Mycosis fungoides and Sézary syndrome
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- By Christiane Querfeld, Section of Dermatology, Department of Medicine, University of Chicago, Chicago, IL, USA, Steven T. Rosen, Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Chicago, IL, USA
- Edited by Susan O'Brien, Julie M. Vose, Hagop M. Kantarjian
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- Book:
- Management of Hematologic Malignancies
- Published online:
- 10 January 2011
- Print publication:
- 18 November 2010, pp 432-448
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Summary
Introduction
Cutaneous T-cell lymphomas (CTCL) represent a clinically and biologically heterogeneous group of non-Hodgkin lymphomas with clonal proliferation of malignant T lymphocytes homing into the skin according to the new revised World Health Organization and European Organization for Research and Treatment of Cancer (WHO–EORTC) consensus classification for cutaneous lymphomas. Mycosis fungoides (MF) and the aggressive and leukemic variant Sézary syndrome (SS), collectively referred to as CTCL, are the most common and best studied entities. CTCL include other entities such as lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large T-cell lymphoma (ALCL), which belong to the CD30+ lymphoproliferative disorders and other rare diseases with distinct clinical features (Table 22.1).
MF disease is characterized by a chronic, slowly progressing course. The clinical manifestations typically include erythematous patches, evolving into plaques and/or tumors (Figure 22.1a–c). Patients usually present with a prolonged history of a skin rash in sun-protected areas such as lower abdomen, upper thighs, buttocks, and breasts in women. Patients with cutaneous patch/plaque disease have an excellent prognosis and a normal life expectancy compared with age-matched individuals. However, 20% of patients progress into more aggressive and advanced disease with either cutaneous or extracutaneous tumor manifestations. Three major variants have been recognized in the new WHO–EORTC classification including granulomatous slack skin, clinically characterized by the development of erythematous lax skin folds and histologically by a granulomatous T-cell infiltrate and loss of elastic fibers, folliculotropic MF, characterized by involvement of hair follicles with or without mucin deposition often leading to alopecia, and pagetoid reticulosis (Woringer–Kolopp disease), presenting as a slowly enlarging, solitary psoriasiform patch with intraepidermal involvement of atypical (pagetoid) T cells expressing a CD4−CD8+ phenotype.
15 - T-cell lymphoma
- from Part II - LYMPHOMA SUBTYPES
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- By Mujahid A. Rizvi, Division of Hematology/Oncology, Department of Medicine, Northwestern University, Feinberg School of Medicine, 303, E Superior Street, Chicago IL, 60611, USA, Andrew M. Evens, Division of Hematology/Oncology, Department of Medicine, Northwestern University, Feinberg School of Medicine, 303, E Superior Street, Chicago, IL, 60611, USA, Beverly P. Nelson, Section of Hematopathology, Department of Pathology, Northwestern University, Feinberg School of Medicine, 303, E Superior Street, Chicago, IL, 60611, USA, Steven T. Rosen, Robert H. Lurie Comprehensive Cancer Center, Northwestern University, Feinberg School of Medicine, 303, E Superior Street, Chicago, IL, 60611, USA, Andrew Wotherspoon, Department of Histopathology, Royal Marsden Hospital, Fulham Road, London, SW3 6JJ, UK, Andreas Rosenwald, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany, German Ott, Institute of Pathology, University of Würzburg, Josef-Schneider-Str, 2, Würzburg, 97080, Germany
- Edited by Robert Marcus, John W. Sweetenham, Case Western Reserve University, Ohio, Michael E. Williams, University of Virginia
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- Book:
- Lymphoma: Pathology, Diagnosis and Treatment
- Published online:
- 05 March 2010
- Print publication:
- 21 June 2007, pp 215-232
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Summary
INTRODUCTION
T-cell non-Hodgkin's lymphomas (NHL) are uncommon malignancies, representing approximately 12% of all lymphomas. Various geographic frequencies of T-cell NHL have been documented, ranging from 18.3% of NHL diagnosed in Hong Kong to 1.5% in Vancouver, Canada. This may in part reflect increased exposure to pathogenic factors such as human T-cell leukemia virus 1 (HTLV-1) and Epstein–Barr virus (EBV) in Asian nations. T-cell NHL commonly presents with extranodal disease and often contains varying amounts of necrosis/apoptosis on biopsy specimens, making differentiation between a reactive process and lymphoma challenging. Immunophenotypic, cytogenetic and molecular analyses have enhanced diagnostic capabilities as well as improved classification and prognostication for T-cell NHL.
The current World Health Organization/European Organisation for Research and Treatment of Cancer (WHO/EORTC) classification recognizes nine distinct clinicopathologic peripheral T-cell NHLs. The broad spectrum of pathologic subtypes with varied clinical behavior poses a challenge to the systematic study of these diseases. Furthermore, these distinct T-cell NHL subtypes have unique characteristics and often warrant individualized diagnostic and therapeutic treatment strategies. The primary cutaneous T-cell lymphomas are reviewed in Chapter 16. Here we review the etiology, pathology, diagnosis and treatment strategies for patients with peripheral T-cell lymphomas (Table 15.1).
ETIOLOGY
Genetic alterations involved in lymphoma oncogenesis include chromosome rearrangements, disruption of tumor suppressor genes and an increase in the number of copies of genes (gene amplification). Moreover, infection of cells by viruses and bacteria such as HTLV-I, human herpesvirus 8 (HHV-8), hepatitis C and Helicobacter pylori may also contribute to lymphomagenesis.
The 5-HTTPR Polymorphism Confers Liability to a Combined Phenotype of Psychotic and Aggressive Behavior in Alzheimer Disease
- Robert A. Sweet, Bruce G. Pollock, Danielle L. Sukonick, Benoit H. Mulsant, Jules Rosen, William E. Klunk, Kari B. Kastango, Steven T. DeKosky, Robert E. Ferrell
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- Journal:
- International Psychogeriatrics / Volume 13 / Issue 4 / December 2001
- Published online by Cambridge University Press:
- 10 January 2005, pp. 401-409
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Background: Psychotic symptoms in subjects with Alzheimer disease (AD+psychosis, AD+P) are a marker for a distinct phenotype characterized by more rapid cognitive and functional decline and a liability to aggressive behaviors. We recently found that AD subjects homozygous for long alleles (l) of an insertion/deletion polymorphism in the promoter region of the serotonin transporter (5-HTTPR) had elevated rates of aggressive behavior. Objective: To examine whether the 5-HTTPR ll genotype confers an increased risk of AD+P, and of the combined AD+P/aggressive phenotype. Methods: The 5-HTTPR genotype was determined in 332 subjects diagnosed with possible or probable AD. All subjects received structured psychiatric assessments and were categorized with regard to their history of aggressive behaviors and psychotic symptoms. Results: Consistent with other reports, AD+P was associated with a significant increased risk for aggressive behavior. AD+P and aggression were both significantly associated with 5-HTTPR ll genotype and with an increased l allele frequency. Subjects with the combined behavioral phenotype (AD+P and aggressive behavior) had the highest rate of ll genotype and highest l allele frequency. Conclusion: The 5-HTTPR l allele appears to confer risk for the combined AD+P/aggressive phenotype. Confirmation of this association in a similar behaviorally well-characterized independent sample is needed.
Effects of a History of Heavy Alcohol Consumption on Alzheimer's Disease
- Jules Rosen, Angela Colantonio, James T. Becker, Oscar L. Lopez, Steven T. Dᴇkosky, Howard B. Moss
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- Journal:
- The British Journal of Psychiatry / Volume 163 / Issue 3 / September 1993
- Published online by Cambridge University Press:
- 02 January 2018, pp. 358-363
- Print publication:
- September 1993
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Neuropsychological and psychiatric evaluations were made of 39 subjects with possible Alzheimer's disease and a history of excessive alcohol consumption (AD + ETOH), who had been abstinent or had drunk minimally for at least three months before evaluation, and 225 patients with probable Alzheimer's disease (PAD) of comparable age, years of education, and baseline global impairment. At baseline, there were no significant differences between the groups in terms of age of onset of dementia, neuropsychological test scores, or current behavioural or psychiatric symptoms. One year later, no differences in rates of decline between 20 abstinent AD + ETOH patients and 88 PAD subjects could be shown. Thus, past heavy alcohol consumption does not appear to modify the presentation of dementia of the Alzheimer's type, nor does it modify progression over a one-year interval.