Lymphomatoid granulomatosis (LYG) is an EBV-positive B-cell lymphoproliferative disorder responsive to alpha-interferon (Ifn) (Meeting abstract).

Important note: Information in this article was accurate in 1994. The state of the art may have changed since the publication date.

Lymphomatoid granulomatosis (LYG) is an EBV-positive B-cell lymphoproliferative disorder responsive to alpha-interferon (Ifn) (Meeting abstract).

Proc Annu Meet Am Soc Clin Oncol; 13:A1294 1994. Unique Identifier : AIDSLINE ICDB/94601290
Wilson WH; Kingma DW; Wittes R; Greiner TC; Jaffe ES; Medicine Branch, NCI, Bethesda, MD

Abstract: LYG is characterized by an angiocentric and angiodestructive infiltration of predominantly T-lymphocytes with varying amounts of tissue necrosis. It typically presents in extranodal tissues, and is divided into 3 grades (grd) depending on the extent of tissue necrosis and cytologic atypia. Up to 30% of pts with grd I and 60% with grd II may develop an aggressive lymphoma. We treated 4 patients (pts) 1 grd I and 3 grd II, with Ifn doses from 4-25 (x 10(6) u TIW). Sites of LYG included lung (4), liver (2), kidney (2), skin (1) and CNS (1). Three pts were previously untreated and 1 had failed CHOP chemotherapy. CD4 counts were 383 and 230 in 2 pts measured, and all 4 pts were HIV negative. EBNA and EBV-IgG serology were positive in 2 of 4 pts. All 4 pts responded to continuous Ifn therapy and are without evidence of active LYG at 10, 15, 17 and 35 mo. Immunohistochemistry (IH) on biopsies from 3 pts showed a predominance of T-cells. Combined rH using anti-CD20 or anti-CD45RO/CD3 and in situ hybridization for EBV RNA showed that EBV infected cells were B cells in all 3 cases. PCR for Ig and TCR rearrangements failed to show a monoclonal population in 3 cases. We postulate that LYG falls within the spectrum of EBV-lymphoproliferative disorders but in a host with only partial immunodeficiency. EBV negative cells predominate and may be mediating the vascular damage. Attempts to boost immune function and control EBV infection with Ifn offers a new approach to this disorder and warrants further investigation.

Keywords: Dose-Response Relationship, Drug Follow-Up Studies Herpesviridae Infections/PATHOLOGY/*THERAPY *Herpesvirus 4, Human Human Infusions, Intravenous Interferon-alpha/*ADMINISTRATION & DOSAGE Lymphoma, B-Cell/PATHOLOGY/*THERAPY Lymphomatoid Granulomatosis/PATHOLOGY/*THERAPY Neoplasm Staging Tumor Virus Infections/PATHOLOGY/*THERAPY ABSTRACT
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