Angiotropic Lymphoma: An Immunophenotypically and Clinically Heterogeneous Lymphoma

Abstract

Angiotropic lymphoma (AL) is an uncommon lymphoma often presenting with nonspecific clinical features and having a high mortality rate. Although not specifically recognized by the Revised European-American Classification of Lymphoid Neoplasms, it likely will appear as a subtype of diffuse large B-cell lymphoma in the upcoming WHO classification. Some authors may also consider it to be a subtype of cutaneous lymphomas. Recent studies have reported an immunophenotypic heterogeneity of AL, and in rare instances, an association with other NHL. To further characterize AL, we studied the immunophenotype by immunohistochemistry for CD5, CD10, CD20, bcl-2, and bcl-6 in 18 cases of B-cell AL identified at three medical centers in North America. Bcl-2 gene rearrangement status by polymerase chain reaction and Epstein Barr virus status by in situ hybridization also were evaluated.Eight men and 10 women were identified with AL (median age 71 years). Eleven patients were diagnosed in life and seven were diagnosed at autopsy. Neurologic symptoms were the most common presentation, seen in six patients. Skin was the most commonly biopsied site. All showed classic intravascular localization; in two cases, there was also a minor diffuse large cell lymphoma component observed in some organs. Most (89%) of the cases expressed bcl-2 protein; CD10, bcl-6 and CD5 were each expressed in 22% of cases. Based on CD5 and CD10 expression, three major groups were evident: CD5؊, CD10؊ (11 cases); CD5؉, CD10؊ (3 cases), and CD5؊, CD10؉ (3 cases). Even though a follicle center lymphoma preceded the AL in one patient, we did not detect bcl-2 gene rearrangement in any of these cases. All cases were negative for Epstein Barr virus. Of the five treated with chemotherapy, two achieved a complete remission.Based on these findings, we conclude that ALs are clinically and immunophenotypically heterogeneous and may represent more than one pathogenetic entity. In some instances AL may be preceded by another lymphoproliferative disorder, raising the possibility that some cases of AL may represent a transformation from another type of lymphoma. Cutaneous manifestations of AL are common; however, it appears to be a systemic lymphoma. Although often fatal, patients with AL who are diagnosed early and treated with chemotherapy may achieve remission.

CD5+ Epstein-Barr virus-positive intravascular large B-cell lymphoma in the uterus co-existing with huge myoma

“…In contrast to the previous report by Yegappan et al [8], EBV was detected in the lymphoma cells of our patient. It has been reported that DLBCL in immunocompromised patients are more frequently EBV + than sporadic DLBCL [12,13].…”

“…She had shown neither neurological abnormalities nor skin lesions, which are typically found in classical IVLBL [6][7][8], but she had also never shown the clinical features of Asian variant/B-LAHS [9,10]. Therefore, we diagnosed her as having classical IVLBL which originated in the uterus and was found incidentally at the time of hysterectomy for the huge myoma.…”

“…Yamaguchi et al reported that such patients with CD5 + DLBCL showed a higher age distribution, female predominance, and was more closely associated with many aggressive clinical features or parameters subsequently resulting in poorer prognosis compared with the characteristics of those with CD5 À DLBCL [18]. Concerning IVLBL, several investigators, including us, also examined the CD5 expression in patients with classical IVLBL or Asian variant [7,8,10,19,20]. According to these reports, about 20-50% of classical IVLBL and about 30-60% of Asian variant expressed CD5 + antigen, the incidence of which seemed rather high compared with DLBCL, as described previously [15][16][17].…”

“…This DLBCL subtype affects mainly adult patients and is usually disseminated in various extranodal sites such as skin, brain, lung, or kidney [6][7][8]. On the other hand, Murase et al recently proposed an Asian variant of IVLBL which was characterized by bone marrow invasion with hemophagocytosis (B-cell lymphomaassociated hemophagocytic syndrome, B-LAHS) but usually lacked skin lesions and neurological abnormalities in contrast to classical IVLBL [9,10].…”

Primary Bilateral Adrenal Intravascular Large B-cell Lymphoma Associated with Adrenal Failure

“…The survival of patients with CD5+DLBCLis significantly worse than that of patients with CD5" DLBCL. Moreover, several patients with CD5+ IVL have been described (17,18).…”