Immunologic and Electronmicroscopic Characteristics of a Case of Immunoblastic Lymphadenopathy

Abstract

A new disease entity of the lymphoid system has recently been reported by Lukes and Tindle as immunoblastic lymphadenopathy and by Frizzera and associates as angioimmunoblastic lymphadenopathy with dysproteinemia. Reported herein are cytologic, histologic, ultrastructural and immunologic studies of several tissues of a patient with this disorder. In addition to confirming the previous observations that the main cellular constituents in the affected tissue consist of immunoblast, plasmacytoid lymphocytes and plasma cells, the authors have demonstrated a profound deficiency of T lymphocytes in this patient. The previously described amorphous interstitial material appears to represent multiple small cytoplasmic fragments. The histiocytic component, which appears in variable amounts in this disease, in the reported case was very active in phagocytizing cellular and nuclear debris, some of which appeared to be lymphocytic in origin. A rubella infection preceded the onset of the disorder by 3 months.

Studies of the pathogenesis of angioimmunoblastic lymphadenopathy.

“…Previous studies have demonstrated spontaneous Ig production by MNC from AILD patients (8). Our findings are consistent with those reports in that AILD T cells plus AILD B cells led to proliferation and Ig production (7,10,11).…”

“…Although the clinical and pathologic features of patients with AILD are becoming better understood, there is considerable confusion regarding the cellular abnormalities that underlie this disorder (7)(8)(9)(10)(11)(12)(13). Most patients have an abnormal composition of their peripheral blood leukocytes.…”

Ultrastructural Study of Immunoblastic Lymphadenopathy

“…As has been pointed out by Moore and co-workers (1976) , the possibility of a viral cause cannot be excluded. It is noteworthy that rubella infection has preceeded the onset of immunoblastic lymphadenopathy by 3 months in a case reported by Palutke et al (1976). Of another interest is the case described by Schlutz and Yunis (1975).…”

Angioimmunoblastic lymphadenopathy with dysproteinemia

“…Palutke et a1. 17 however, considered the interstitial eosinophilic material to be composed of cellular debris. The plethora of small blood vessels, which is a characteristic feature of this lesion, may reflect the action of lymphocyteinduced angiogenesis.…”