ABSTRACT
Angioimmunoblastic lymphadenopathy (AILD) is a systemic disease clinically characterized by fever, generalized lymphadenopathy, hepatosplenomegaly, polyclonal gamma-globulinemia and Cooms' positive hemolytic anemia. The lymph node in AILD reveals a polymorphic feature consisting of a proliferation of small vessels, immunoblasts and plasma cells and acidophilic interstitial material. Progression into immunoblastic sarcoma is reported as high 35% of the patient with AILD. Nathwani et al have observed not only malignant transformation of AILD in sequential tissue examination, but also the coexistence of AILD and immunoblastic lymphoma in the same lymph node or at different sites in the same patient. Multiple clusters or islands of compactly arranged large lymphoid cells constitute the initial histologic evidence of immunoblastic sarcoma. Immunoblastic sarcoma is a large cell lymphoma conceptually related to transformed T-and B-lymphocytes of the extrafollicular compartment of the immune system, which proignosis is poor. We have recently experienced a case of immuno blastic sarcoma arising in angioimmunoblastic lymphadenopathy in a 24-year-old woman. She had history of multiple enlarged lymph nodes in the inguinal, axilla and supraclavicular areas. Previous lymph node biopsies revealed reactive change. Six month later, right axillary lymph node biopsy reveled AILD with focal clusters of immunoblasts. Subsequent lymph node biopsy at the same site revealed diffuse immunoblasic sarcoma, B-cell type. A case presentation with histologic findings and a brief review of literature were done.