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EBV Positive Mucocutaneous Ulcer-A Study of 26 Cases Associated With Various Sources of Immunosuppression

DOJCINOV, Stefan D ; VENKATARAMAN, Girish ; RAFFELD, Mark ; PITTALUGA, Stefania ; JAFFE, Elaine S

The American journal of surgical pathology, 2010-03, Vol.34 (3), p.405-417 [Periódico revisado por pares]

Hagerstown, MD: Lippincott Williams & Wilkins

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Título:
EBV Positive Mucocutaneous Ulcer-A Study of 26 Cases Associated With Various Sources of Immunosuppression
Autor: DOJCINOV, Stefan D ; VENKATARAMAN, Girish ; RAFFELD, Mark ; PITTALUGA, Stefania ; JAFFE, Elaine S
Assuntos: Adult ; Age Factors ; Aged ; Aged, 80 and over ; B-Lymphocytes - drug effects ; B-Lymphocytes - immunology ; B-Lymphocytes - virology ; Biological and medical sciences ; Epstein-Barr Virus Infections - chemically induced ; Epstein-Barr Virus Infections - etiology ; Epstein-Barr Virus Infections - immunology ; Epstein-Barr Virus Infections - pathology ; Epstein-Barr Virus Infections - therapy ; Female ; Gastrointestinal Diseases - chemically induced ; Gastrointestinal Diseases - immunology ; Gastrointestinal Diseases - pathology ; Gastrointestinal Diseases - therapy ; Gastrointestinal Diseases - virology ; Gene Rearrangement, T-Lymphocyte ; Genes, Immunoglobulin ; Hematologic and hematopoietic diseases ; Humans ; Immune Tolerance ; Immunohistochemistry ; Immunosuppressive Agents - adverse effects ; In Situ Hybridization ; Infectious diseases ; Investigative techniques, diagnostic techniques (general aspects) ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Lymphoproliferative Disorders - chemically induced ; Lymphoproliferative Disorders - immunology ; Lymphoproliferative Disorders - pathology ; Lymphoproliferative Disorders - therapy ; Lymphoproliferative Disorders - virology ; Male ; Medical sciences ; Middle Aged ; Mouth Mucosa - drug effects ; Mouth Mucosa - immunology ; Mouth Mucosa - virology ; Oral Ulcer - chemically induced ; Oral Ulcer - immunology ; Oral Ulcer - pathology ; Oral Ulcer - therapy ; Oral Ulcer - virology ; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques ; Polymerase Chain Reaction ; Recurrence ; Remission, Spontaneous ; Skin Diseases, Infectious - chemically induced ; Skin Diseases, Infectious - immunology ; Skin Diseases, Infectious - pathology ; Skin Diseases, Infectious - therapy ; Skin Diseases, Infectious - virology ; Skin Ulcer - chemically induced ; Skin Ulcer - immunology ; Skin Ulcer - pathology ; Skin Ulcer - therapy ; Skin Ulcer - virology ; T-Lymphocytes - drug effects ; T-Lymphocytes - immunology ; T-Lymphocytes - virology ; Treatment Outcome ; Viral diseases
É parte de: The American journal of surgical pathology, 2010-03, Vol.34 (3), p.405-417
Descrição: We describe a series of Epstein Barr virus (EBV)-positive circumscribed, ulcerative lesions associated with various types of immunosuppression (IS). The study group (26 patients) comprised 10 males and 16 females, median age 77 years (range 42 to 101). IS in 9 cases included azathioprine (AZA), methotrexate (MTX) or cyclosporin-A (CyA). Seventeen patients had age-related immunosenescence. Patients presented with isolated sharply circumscribed ulcers involving oropharyngeal mucosa (16), skin (6), and gastrointestinal tract (4). Lesions were histologically characterized by a polymorphous infiltrate and atypical large B-cell blasts often with Hodgkin/Reed-Sternberg (HRS) cell-like morphology. The B cells showed strong CD30 and EBER positivity, some with reduced CD20 expression, in a background of abundant T cells. CD15 was positive in 43% of cases (10/23). The pathologic features were identical regardless of the anatomic site or cause of IS. Polymerase chain reaction revealed 39% (7/18) clonal Ig gene rearrangements with 38% (6/16) and 31% (5/16) clonal and restricted T-cell patterns, respectively. Twenty-five percent of patients (5/20) received standard chemotherapy and/or radiotherapy. Forty-five percent (9/20) regressed spontaneously with no treatment and 15% (3/20) were characterized by a relapsing and remitting course. All of the iatrogenic lesions (6/6) with available follow-up responded to reduction of IS. All patients achieved complete remission with no disease-associated deaths over a median follow-up period of 22 months (range 3 to 72). We propose EBV-positive mucocutaneous ulcer as a newly recognized clinicopathologic entity with Hodgkin-like features and a self-limited, indolent course, generally responding well to conservative management. Association with various forms of IS implies a common pathogenetic mechanism. The localized nature of the disease may be owing to a minimal and localized lapse in immunosurveillance over EBV.
Editor: Hagerstown, MD: Lippincott Williams & Wilkins
Idioma: Inglês

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EBV Positive Mucocutaneous Ulcer-A Study of 26 Cases Associated With Various Sources of Immunosuppression

DOJCINOV, Stefan D ; VENKATARAMAN, Girish ; RAFFELD, Mark ; PITTALUGA, Stefania ; JAFFE, Elaine S

Hagerstown, MD: Lippincott Williams & Wilkins

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