Medical and Pediatric Oncology 18333-335 (1990) zy A Successfully Treated Case of Muhicentric Angiofollicular Hyperplasia With Oral Chemotherapy (Castleman’s Disease) Nicholas A. Pavlidis, MD, Fotini N. Skopouli, MD, Mary C. Bai, MD, and Constantinos zyxwvu L. Bourantas, MD zyxwvu A case of angiofollicular lymph node hy- ambucil and corticosteroids and has re- perplasia of plasma cell type in a 60-year-old mained free of disease for the last 30 woman is reported. The patient presented months. The authors review and discuss the with lymphadenopathy, splenornegaly, and systemic cytotoxic treatment of this clinico- fever. She responded dramatically to chlor- pathologic entity. Key words: lymph node hyperplasia, chlorambucil, corticosteroids INTRODUCTION zyxwvutsrq Angiofollicular hyperplasia was first described in 1956 by Castleman et al, and subsequently has been termed “Castleman’s disease” [I]. Since then several names have been given to this distinct entity such as lymph nodal hamartoma, benign giant lymphoma, angi- omatous lymphoid hamartoma, and follicular lymphore- ticuloma [2]. This tumor was initially considered to be a benign lesion affecting predominantly the mediastinurn, but later two different histological variants were recognized. The hyaline-vascular, is the most common and less ag- gressive type, and the plasma-cell variant is character- ized by a more unfavorable course [2]. Localized forms can be cured by surgical resection; however, unresect- able or disseminated tumors can be partially treated with either radiotherapy or chemotherapy. In this paper, we report on a multicentic case of an- giofollicular hyperplasia of the plasma-cell type in which the patient achieved complete remission after oral che- motherapy and exhibited prolonged disease-free survival. CASE REPORT A 60-year-old woman was admitted to the Department of Medicine, Ioannina School of Medicine, in July 1987 because of a 12-month history of intermittent high fevers up to 39°C. On examination, bilateral supraclavicular, axillary, and inguinal lymph node enlargement as well as moderate splenomegaly were present. Laboratory work-up revealed Hct 41%, WBC 4.100/ zyxwvu 0 1990 Wiley-Liss, Inc. mm3 with normal differential, platelets 200.000/mm3, and erythrocyte sedimentation rate 78 m d h r . Liver and renal function tests as well as serum protein electro- phoresis were within normal limits. Autoantibodies, cryoglobulins, toxoplasma, and several antiviral antibod- ies were all negative. Chest X-ray was normal. Abdomi- nal CT-scan and ultrasound showed moderate splenom- egaly . Bone biopsy was negative. Supraclavicular lymph node biopsy was compatible with the diagnosis of angio- follicular hyperplasia of the plasma-cell variant. (Fig. 1). Oral treatment with chlorambucil 10 mg and pred- nisone 40 mg daily for 2 weeks every 28 days was started. After two courses fever, lymphadenopathy, and splenomegaly resolved. The first 11 cycles were given on a monthly basis up to June 1988 followed by the same regimen on a 2-month interval. Repeated restaging eval- uations revealed no evidence of active disease. She re- mains in complete remission and free of disease for the last 30 months. DISCUSSION We report a successfully treated case of multicentric angiofollicular hyperplasia of the plasma-cell type in a 60-year-old female patient. From the Oncology (N.A.P.) and Hematology (M.C.B.) Sections, Department of Medicine and Pathology (N.A.P., F.N.S., M.C.B., C.L.B.), University of Ioannina, Ioannina 45 110, Greece. Address reprint requests to Dr. N. Pavlidis, Department of Medicine, Medical School, Univeristy of Ioannina, 451 10 Ioannina, Greece.