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Night sweats and swollen glands
CASE REPORT
Case report
Night sweats and swollen glands D R McCluskey, M R E Buckley, W G McCluggage A previously well 63-year-old man presented in March, 1997, with drenching night sweats, spiking pyrexia, weight loss, and lethargy for 18 months. He was thin with inguinal and axillary lymphadenopathy and indurated skin swellings. Haemoglobin was 9·9 g/dL with signs of iron deficiency. Erythrocyte sedimentation rate was 122 mm/h, C-reactive protein 120 mg/L, IgG 20 g/L, and IgM 3·76 g/L. There was splenomegaly, and a radiograph showed a small shadow in the apex of his right lung. Sputum analysis, computed tomography scans, and bronchoscopy were normal. Bacterial, viral, and fungal cultures were negative, as was the search for a cancer. Empirical antituberculous therapy was given for 4 weeks with no improvement. A skin biopsy specimen showed a dermal lymphocytic infiltrate. A lymph-node biopsy specimen showed peripheral lymphoid follicles, prominent germinal centres (figure, top) and pronounced vascular spaces packed with mature plasma cells (figure, bottom). Immunohistochemical staining confirmed a polyclonal population in keeping with the plasma-cell variant of Castleman’s disease. Prednisolone 60 mg daily was begun with rapid improvement: night sweats and pyrexia resolved within 48 h , and blood tests returned to normal within 1 week. Steroids were gradually reduced to a maintenance dose of 20 mg daily. He was well when last seen in January, 1998. In 1956 Benjamin Castleman described mediastinal lymphoid masses with follicular hyperplasia.1 Although the disorder was originally thought to be indolent and localised, there have been subsequent reports of multifocal disease. Histologically, Castleman’s disease is characterised by lymph-node hyperplasia with vascular proliferation and endothelial hyperplasia. The hyalinevascular type (90% of cases), generally benign, occurs in younger patients with a localised mediastinal mass— surgical excision is curative. Plasma-cell variant (10% of cases), localised or multicentric, is generally aggressive, and occurs in older patients with generalised lymphadenopathy, hepatosplenomegaly, systemic symptoms, anaemia, elevated inflammatory indices, and a polyclonal rise in immunoglobulins.1 This case highlights the long clinical course of the disease and the importance of doing tissue biopsies in patients with pyrexia of unknown origin. There are suggestions that human Lancet 1998; 351: 722 Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA, UK (D R McCluskey FRCP, M R E Buckley MB, W G McCluggage MRCPath) Correspondence to: Dr D R McCluskey
722
Histology of lymph node Top: low-power view of lymph node showing lymphoid follicles with prominent germinal centres. Bottom: high-power view showing numerous mature plasma cells.
herpesvirus 8, Epstein-Barr virus,2 or dysregulation of interleukin 6 may be involved in the aetiology of this disorder.3 Surgery and radiotherapy for localised lesions have shown good response rates, and interferon, cimetidine,4 chemotherapy, and interleukin antibody5 have had variable success. In the treatment of multicentric plasma-cell variant, steroids and immunosuppressive agents relieve symptoms but are not curative and their effect on preventing the later development of malignancy is still unclear. In the absence of any demonstrable infection or malignancy, Castleman’s disease should be considered in patients with pyrexia of unknown origin. Although rare, it is an important diagnosis to consider, because the localised hyaline vascular type is usually cured by surgery and the multicentric plasma-cell variant requires prompt treatment and close clinical follow-up because of the risk of malignant transformation. We thank Kathleen McCartan for typing the paper.
References 1 2
3
4
5
Shahidi H, Myers JL, Kvale P. Castleman’s disease. Mayo Clin Proc 1995; 70: 969–77. Corbellino M, Poirel L, Aubin JT, et al. The role of human herpes virus 8 and Epstin-Barr virus in the pathogenesis of giant lymph node hyperplasia (Castleman’s disease). Clin Infect Dis 1996; 22: 1120–21. IschIyama T, Koike M, Nakamura S, Kakimoto T, Akimoto Y, Tsuruoka N. Interleukin 6 receptor expression in the peripheral B cells of patients with muilticentric Castleman’s disease. Ann Haematol 1996; 73: 179–82. Barbounis V, Efremidid A. A plasma cell variant of Castleman’s disease treated successfully with cimetidine: case report and review of literature. Anticancer Res 1996; 16: 545–48. De Heer-Groen TA, Prakken ABH, Bax NMA, Van Dijken PJ. Iron therapy resistant microcytic anaemia in a 13 year old girl with Castleman’s disease. Eur J Pediatr 1996; 155: 1015–17.
THE LANCET • Vol 351 • March 7, 1998
Case report
Night sweats and swollen glands D R McCluskey, M R E Buckley, W G McCluggage A previously well 63-year-old man presented in March, 1997, with drenching night sweats, spiking pyrexia, weight loss, and lethargy for 18 months. He was thin with inguinal and axillary lymphadenopathy and indurated skin swellings. Haemoglobin was 9·9 g/dL with signs of iron deficiency. Erythrocyte sedimentation rate was 122 mm/h, C-reactive protein 120 mg/L, IgG 20 g/L, and IgM 3·76 g/L. There was splenomegaly, and a radiograph showed a small shadow in the apex of his right lung. Sputum analysis, computed tomography scans, and bronchoscopy were normal. Bacterial, viral, and fungal cultures were negative, as was the search for a cancer. Empirical antituberculous therapy was given for 4 weeks with no improvement. A skin biopsy specimen showed a dermal lymphocytic infiltrate. A lymph-node biopsy specimen showed peripheral lymphoid follicles, prominent germinal centres (figure, top) and pronounced vascular spaces packed with mature plasma cells (figure, bottom). Immunohistochemical staining confirmed a polyclonal population in keeping with the plasma-cell variant of Castleman’s disease. Prednisolone 60 mg daily was begun with rapid improvement: night sweats and pyrexia resolved within 48 h , and blood tests returned to normal within 1 week. Steroids were gradually reduced to a maintenance dose of 20 mg daily. He was well when last seen in January, 1998. In 1956 Benjamin Castleman described mediastinal lymphoid masses with follicular hyperplasia.1 Although the disorder was originally thought to be indolent and localised, there have been subsequent reports of multifocal disease. Histologically, Castleman’s disease is characterised by lymph-node hyperplasia with vascular proliferation and endothelial hyperplasia. The hyalinevascular type (90% of cases), generally benign, occurs in younger patients with a localised mediastinal mass— surgical excision is curative. Plasma-cell variant (10% of cases), localised or multicentric, is generally aggressive, and occurs in older patients with generalised lymphadenopathy, hepatosplenomegaly, systemic symptoms, anaemia, elevated inflammatory indices, and a polyclonal rise in immunoglobulins.1 This case highlights the long clinical course of the disease and the importance of doing tissue biopsies in patients with pyrexia of unknown origin. There are suggestions that human Lancet 1998; 351: 722 Royal Victoria Hospital, Grosvenor Road, Belfast BT12 6BA, UK (D R McCluskey FRCP, M R E Buckley MB, W G McCluggage MRCPath) Correspondence to: Dr D R McCluskey
722
Histology of lymph node Top: low-power view of lymph node showing lymphoid follicles with prominent germinal centres. Bottom: high-power view showing numerous mature plasma cells.
herpesvirus 8, Epstein-Barr virus,2 or dysregulation of interleukin 6 may be involved in the aetiology of this disorder.3 Surgery and radiotherapy for localised lesions have shown good response rates, and interferon, cimetidine,4 chemotherapy, and interleukin antibody5 have had variable success. In the treatment of multicentric plasma-cell variant, steroids and immunosuppressive agents relieve symptoms but are not curative and their effect on preventing the later development of malignancy is still unclear. In the absence of any demonstrable infection or malignancy, Castleman’s disease should be considered in patients with pyrexia of unknown origin. Although rare, it is an important diagnosis to consider, because the localised hyaline vascular type is usually cured by surgery and the multicentric plasma-cell variant requires prompt treatment and close clinical follow-up because of the risk of malignant transformation. We thank Kathleen McCartan for typing the paper.
References 1 2
3
4
5
Shahidi H, Myers JL, Kvale P. Castleman’s disease. Mayo Clin Proc 1995; 70: 969–77. Corbellino M, Poirel L, Aubin JT, et al. The role of human herpes virus 8 and Epstin-Barr virus in the pathogenesis of giant lymph node hyperplasia (Castleman’s disease). Clin Infect Dis 1996; 22: 1120–21. IschIyama T, Koike M, Nakamura S, Kakimoto T, Akimoto Y, Tsuruoka N. Interleukin 6 receptor expression in the peripheral B cells of patients with muilticentric Castleman’s disease. Ann Haematol 1996; 73: 179–82. Barbounis V, Efremidid A. A plasma cell variant of Castleman’s disease treated successfully with cimetidine: case report and review of literature. Anticancer Res 1996; 16: 545–48. De Heer-Groen TA, Prakken ABH, Bax NMA, Van Dijken PJ. Iron therapy resistant microcytic anaemia in a 13 year old girl with Castleman’s disease. Eur J Pediatr 1996; 155: 1015–17.
THE LANCET • Vol 351 • March 7, 1998