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Clinical manifestations, pathologic features, and diagnosis of angioimmunoblastic T-cell lymphoma
Authors
Arnold S Freedman, MD
Jon C Aster, MD
Section Editor
Andrew Lister, MD, FRCP, FRCPath, FRCR
Deputy Editor
Rebecca F Connor, MD
Last literature review version 18.2: mayo 2010 | This topic last updated: noviembre 18, 2009 (More)
INTRODUCTION — The peripheral T-cell lymphomas (PTCL) are a heterogeneous group of generally aggressive neoplasms that constitute less than 15 percent of all non-Hodgkin lymphomas (NHLs) in adults [1-5]. (See "Classification of the hematopoietic neoplasms".)

Among these, in decreasing frequency of occurrence, are: …ver más…

AITL can be associated with autoimmune phenomena including hemolytic anemia, vasculitis, polyarthritis, rheumatoid arthritis, and thyroid disease [18]. Cold agglutinins and cryoglobulins can also be seen. These autoimmune features are thought to stem from immune dysregulation of both B cells and T cells, which together result in immunodeficiency and risk of opportunistic infections both before and during treatment.

PATHOLOGIC FEATURES
Morphology
Growth pattern — Involved lymph nodes demonstrate partial or complete effacement of the normal architecture by a predominantly paracortical infiltrate of atypical lymphoid and inflammatory cells [4]. Follicles may be hyperplastic, depleted, or absent. Peripheral sinuses are typically open and even dilated, and the abnormal infiltrate usually does not extend beyond the capsule into perinodal tissue.

There is prominent neovascularization with arborizing high endothelial venules (HEV), many of which show thickened or hyalinized walls. Clusters of epithelioid histiocytes and numerous eosinophils and plasma cells may be present. Expanded aggregates of follicular dendritic cells, visible on immunostained sections, surround the proliferating blood vessels, and may have the appearance of "burned-out" germinal centers.

Cell morphology — The infiltrating lymphoid cells are a mixture