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    Enteropathy-Type T Cell Lymphoma

    Definition

    • T cell lymphoma involving the gastrointestinal tract, expressing cytotoxic granule associated proteins, frequently associated with enteropathic changes

    Alternate/Historical Names

    • Intestinal T cell lymphoma
    • Enteropathy associated T cell lymphoma
    • Malignant histiocytosis of the intestine

    Diagnostic Criteria

    • Forms an ulcerated intestinal mass
    • Wide range of histologic appearances
      • Ranges from regular small to medium size cells to large pleomorphic cells
      • Frequent inflammatory cells including histiocytes and eosinophils
    • Cytotoxic T lineage
    • Frequently associated with enteropathic changes
      • Adjacent epithelium abnormal in 75% of cases
      • Villous atrophy with crypt hyperplasia
      • Intraepithelial lymphocytes
      • Lamina propria lymphoplasmacytic infiltrate

    Dita Gratzinger MD PhD
    Yasodha Natkunam MD PhD
    Robert V Rouse MD

    Department of Pathology
    Stanford University School of Medicine
    Stanford CA 94305-5342

    Initial posting : September 1, 2007

    Supplemental studies

    Immunohistology and Flow

    • T lineage markers
      • CD3 90%
      • CD8 30%
        • 80% of CD56+ cases express CD8
      • CD4, CD5 negative
    • Cytotoxic protein markers
      • TIA1, perforin, Granzyme B positive in nearly all cases
    • CD103 positive in most cases
      • Lymphoid adhesion molecule of enteric T cell subset
    • CD56 20% positive
      • More often in small to medium sized cases
    • CD30 40%
      • All in CD56+ subset
    • EMA 16%
      • All in CD56+ subset
    • CD79a reported in 3/52 cases
      • All expressed cytotoxic markers
      • All were CD20 negative
    • Negative:
      • CD20
      • CD57
      • ALK

    Genetic analysis

    • EBV EBER variably reported
      • 0% in US patients (Ilas et al.)
      • 7% in German patients (Chott et al.)
      • 100% in Mexican patients (Quintanilla-Martinez et al.)
    • T cell receptor beta and gamma genes clonally rearranged
      • Same clonal rearrangements found in adjacent mucosa
      • Cases of refractory sprue may show clonal rearrangements, suggesting a precursor lesion
    • Various chromosomal gains and losses
      • Most common is gain of 9q33-34 in 64% of cases
        • Very rare in other lymphomas

    Differential Diagnosis

    Extranodal NK/T Cell Lymphoma Enteropathy Type T Cell Lymphoma
    CD56 100% CD56 20%
    EBV 100% in nasal sites but variable in other sites and outside endemic areas EBV variable, possibly by geography
    No adjacent enteropathy Adjacent enteropathy 75%
    Both may involve the GI tract and both express cytotoxic T markers

    Primary Systemic Anaplastic Large Cell Lymphoma Enteropathy Type T Cell Lymphoma
    Large anaplastic cells Wide variety of histologic appearances
    No adjacent enteropathy Adjacent enteropathy 75%
    ALK 85% ALK negative
    CD56 negative CD56 20%
    Both may involve the GI tract, both may express CD30 and EMA

    Extranodal Marginal Zone Lymphoma Enteropathy Type T Cell Lymphoma
    Generally predominantly small cell pattern Wide variety of patterns
    B lineage T lineage
    Both may involve the GI tract

    Enteropathy-type T Cell Lymphoma Ulcerative Jejunoileitis
    Identification of a monomorphic population of atypical T cells Lacks a monomorphic population

    • Clonal T cell populations are present in both
    • Some believe that ulcerative jejunoileitis is an early form of enteropathy-type T cell lymphoma

    Clinical

    • 75% of cases show enteropathy in adjacent mucosa
    • Usually involves jejunum or ileum
      • Forms ulcerated mass
      • May perforate or cause obstruction
      • Rarely involves other GI sites
      • Most reurrences are intestinal
        • Other sites include mesenteric nodes, liver, spleen, marrow, lung, skin
    • Mostly adults
    • Poor prognosis

    Grading / Staging / Report

    Grading not applicable

    Ann Arbor Staging System

    • Stage I
      • I if involvement of a single lymph node region
      • IE if involvement of a single extralymphatic organ or site
    • Stage II
      • II if two or more lymph node regions on same side of diaphragm
      • IIE if localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm
    • Stage III
      • III if Involvement of lymph node regions on both sides of the diphragm
      • IIIS if spleen involved
      • IIIE if extralymphatic site involved
    • Stage IV
      • Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement
    • Systemic Symptoms in 6 months preceding admission
      • Fever, night sweats, 10% weight loss
      • A = absent
      • B = present
    • Extranodal sites are also designated
      • M+ = marrow
      • L+ = lung
      • H+ = liver
      • P+ = pleura
      • O+ = bone
      • D+ = skin and subcutaneous tissue
    • Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also used for non-Hodgkin lymphomas.

    The pathology report should contain the following information:

    • Diagnosis in the World Health Organization (WHO) classification
      • Equivalent diagnosis in other classifications used by relevant clinicians
    • Results of supplementary studies if performed
    • The presence or absence of associated enteropathy
    • Relationship to other specimens from the same patient
    • Information relevant to staging if available

     

    Lists

    Mature T and NK cell neoplasms (WHO classification)

    Gastrointestinal tract lymphomas

    Bibliography

    • Warnke RA, Weiss LM, Chan JKC, Cleary ML, Dorfman RF . Tumors of the Lymph Nodes and Spleen, Atlas of Tumor Pathology, AFIP Third Series, Fascicle 14, 1995
    • Jaffe ES, Harris NL Stein H, Vardiman JW eds. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, World Health Organization Classification of Tumours 2001
    • Murray A, Cuevas EC, Jones DB, Wright DH. Study of the immunohistochemistry and T cell clonality of enteropathy-associated T cell lymphoma. Am J Pathol 1995 Feb;146(2):509-19
    • de Bruin PC, Connolly CE, Oudejans JJ, Kummer JA, Jansen W, McCarthy CF, Meijer CJ. Enteropathy-associated T-cell lymphomas have a cytotoxic T-cell phenotype. Histopathology 1997 Oct;31(4):313-7
    • Ilyas M, Niedobitek G, Agathanggelou A, Barry RE, Read AE, Tierney R, Young LS, Rooney N. Non-Hodgkin's lymphoma, coeliac disease, and Epstein-Barr virus: a study of 13 cases of enteropathy-associated T- and B-cell lymphoma. J Pathol 1995 Oct;177(2):115-22
    • Cellier C, Delabesse E, Helmer C, Patey N, Matuchansky C, Jabri B, Macintyre E, Cerf-Bensussan N, Brousse N. Refractory sprue, coeliac disease, and enteropathy-associated T-cell lymphoma. French Coeliac Disease Study Group. Lancet 2000 Jul 15;356(9225):203-8
    • Chott A, Haedicke W, Mosberger I, Fodinger M, Winkler K, Mannhalter C, Muller-Hermelink HK. Most CD56+ intestinal lymphomas are CD8+CD5-T-cell lymphomas of monomorphic small to medium size histology. Am J Pathol 1998 Nov;153(5):1483-90
    • Blakolmer K, Vesely M, Kummer JA, Jurecka W, Mannhalter C, Chott A. Immunoreactivity of B-cell markers (CD79a, L26) in rare cases of extranodal cytotoxic peripheral T- (NK/T-) cell lymphomas. Mod Pathol 2000 Jul;13(7):766-72
    • Quintanilla-Martinez L, Lome-Maldonado C, Ott G, Gschwendtner A, Gredler E, Angeles-Angeles A, Reyes E, Fend F. Primary intestinal non-Hodgkin's lymphoma and Epstein-Barr virus: high frequency of EBV-infection in T-cell lymphomas of Mexican origin. Leuk Lymphoma 1998 Jun;30(1-2):111-21
    • Isaacson PG, Du MQ. Gastrointestinal lymphoma: where morphology meets molecular biology. J Pathol. 2005 Jan;205(2):255-74.
    • Zettl A, deLeeuw R, Haralambieva E, Mueller-Hermelink HK. Enteropathy-type T-cell lymphoma. Am J Clin Pathol. 2007 May;127(5):701-6.
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