Extranodal Marginal Zone Lymphoma-like Presentations of Angioimmunoblastic T-Cell Lymphoma: A T-Cell Lymphoma Masquerading as a B-Cell Lymphoproliferative Disorder. 2015

Benjamin Kaffenberger, and Brad Haverkos, and Kelly Tyler, and Henry K Wong, and Pierluigi Porcu, and Alejandro Ariel Gru
*Division of Dermatology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH; †Division of Hematology, Department of Internal Medicine, The Ohio State University Wexner Medical Center, Columbus, OH; and ‡Division of Dermatopathology, Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH.

Angioimmunoblastic T-cell lymphoma (AITL) is the second most common type of peripheral T-cell lymphoma worldwide, and in some countries, it is the most common form. Clinically, AITL usually presents with systemic symptoms, diffuse lymphadenopathy, hepatosplenomegaly, and common laboratory abnormalities such as hypergammaglobulinemia. Rashes are seen in 50%-80% of patients. AITL derives from follicular T-helper cells (TFH), that express germinal center markers and produce hyperactivation of B-cells seen in AITL. Although the histological features of AITL in the skin could be similar to pathological findings present in lymph node biopsies, herein, we present 2 cases of AITL with histological and immunophenotypic features that were somewhat suggestive of extranodal marginal zone lymphoma. Caution is urged to exclude the possibility of a systemic T-cell lymphoma such as AITL in cutaneous and lymph node B-cell proliferations.

UI MeSH Term Description Entries
D007119 Immunoblastic Lymphadenopathy A disorder characterized by proliferation of arborizing small vessels, prominent immunoblastic proliferations and amorphous acidophilic interstitial material. Clinical manifestations include fever, sweats, weight loss, generalized lymphadenopathy and frequently hepatosplenomegaly. Angioimmunoblastic Lymphadenopathy,Lymphadenopathy, Immunoblastic,Angioimmunoblastic Lymphadenopathies,Immunoblastic Lymphadenopathies,Lymphadenopathies, Angioimmunoblastic,Lymphadenopathies, Immunoblastic,Lymphadenopathy, Angioimmunoblastic
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D016410 Lymphoma, T-Cell, Cutaneous A group of lymphomas exhibiting clonal expansion of malignant T-lymphocytes arrested at varying stages of differentiation as well as malignant infiltration of the skin. MYCOSIS FUNGOIDES; SEZARY SYNDROME; LYMPHOMATOID PAPULOSIS; and PRIMARY CUTANEOUS ANAPLASTIC LARGE CELL LYMPHOMA are the best characterized of these disorders. Granulomatous Slack Skin,T-Cell Lymphoma, Cutaneous,Cutaneous T-Cell Lymphoma,Lymphoma, T Cell, Cutaneous,Cutaneous T Cell Lymphoma,Cutaneous T-Cell Lymphomas,Lymphoma, Cutaneous T-Cell,Lymphomas, Cutaneous T-Cell,Slack Skin, Granulomatous,T Cell Lymphoma, Cutaneous,T-Cell Lymphomas, Cutaneous
D018442 Lymphoma, B-Cell, Marginal Zone Extranodal lymphoma of lymphoid tissue associated with mucosa that is in contact with exogenous antigens. Many of the sites of these lymphomas, such as the stomach, salivary gland, and thyroid, are normally devoid of lymphoid tissue. They acquire mucosa-associated lymphoid tissue (MALT) type as a result of an immunologically mediated disorder. Lymphoma, Mucosa-Associated Lymphoid Tissue,MALT Lymphoma,Marginal Zone B-Cell Lymphoma,Lymphoma of Mucosa-Associated Lymphoid Tissue,Mucosa-Associated Lymphoid Tissue Lymphoma,Lymphoma of Mucosa Associated Lymphoid Tissue,Lymphoma, MALT,Lymphoma, Mucosa Associated Lymphoid Tissue,Lymphomas, MALT,MALT Lymphomas,Marginal Zone B Cell Lymphoma,Mucosa Associated Lymphoid Tissue Lymphoma

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