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Angiocentric immunoproliferative lesions: a clinicopathologic spectrum of
post-thymic T-cell proliferations
EH Lipford , JB Margolick, DL Longo, AS Fauci and ES Jaffe
Hematopathology Section, National Cancer Institute, Bethesda, MD.
Twenty-three patients with angiocentric immunoproliferative lesions (AILs)
including angiocentric lymphoma were evaluated clinically and
pathologically. Pathologic subclassification performed without knowledge of
the clinical outcome divided the cases into three histologic grades on the
basis of cellular atypia and degree of inflammatory background.
Immunophenotypic studies of lesions from six patients demonstrated a mature
T-cell phenotype with a predominance of CD4-positive cells. Abnormalities
of antigenic phenotype were demonstrated in only one case, classified as
grade III. That tumor also demonstrated a clonal rearrangement of the T
beta gene. Progression to malignant lymphoma following initial
immunosuppressive therapy with cyclophosphamide and prednisone occurred in
three of nine patients with grade I lesions and four of six patients with
grade II lesions. The supervening lymphomas were usually refractory to
subsequent aggressive chemotherapy, with only one patient achieving a
complete remission. In contrast, seven of eight patients with grade III
lesions achieved a complete remission with aggressive combination
chemotherapy, two of whom also received supplemental radiation therapy.
These studies support the concept that the AILs represent a spectrum of
post-thymic T- cell proliferations. The single most important prognostic
indicator for ultimate survival is achievement of an initial complete
remission. Patients treated initially with conservative chemotherapy may be
compromised in their ability to achieve a complete remission if they
progress to a higher grade lesion.
Volume 72,
Issue 5,
pp. 1674-1681,
11/01/1988
Copyright © 1988 by The American Society of Hematology
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