NONRANDOM CHROMOSOME-ABNORMALITIES IN ANGIOIMMUNOBLASTIC LYMPHADENOPATHY

  • 1 January 1982
    • journal article
    • research article
    • Vol. 60  (4) , 877-887
Abstract
Cytogenetic and pathologic studies were performed on 6 patients with angioimmunoblastic lymphadenopathy (AILD). All 6 had diffuse lymphadenopathy; 5 had fever, 4 had weight loss and 4 had a diffuse erythematous rash. All patients except 1 had a polyclonal elevation of Ig. All patients had diagnostic findings in lymph node (LN) and bone marrow (BM) biopsies. Two patients died of progressive AILD; 1 patient died after transformation of AILD to immunoblastic sarcoma (IBS); 1 patient died of gastrointestinal bleeding of unknown cause. The remaining 2 patients, who have achieved complete remission with intensive chemotherapy, are alive 20 and 8 mo. after the diagnosis; one of these had AILD and the other, both AILD and IBS. Despite diagnostic BM biopsy findings, none of the patients had chromosome abnormalities in their BM cells. In studying LN cells of 5 patients, chromosome abnormalities were found in each; clonal abnormalities were detected in 2, both clonal and nonclonal abnormalities in 2, and only nonclonal single-cell abnormalities in 1. An extra chromosome 3, seen in 4 patients, was clonal in 2 and nonclonal in the 2 others. Cells with +5, +15, +19, +21, +22 were seen in 2 patients. All patients had .gtoreq. 50% or more normal dividing cells in their LN. The mosaicism of unrelated abnormal karyotypes that was seen in 4 patients suggests that this malignant tumor is not necessarily monoclonal in its early stages but that 1 clone may be selected and predominate in the late stage. Because nonrandom acquired clonal chromosome abnormalities are a consistent feature of malignancies, the data suggest that AILD may be a malignant disease despite its original description as a benign proliferative process. Therefore, it may require aggressive chemotherapy.