NATURE REVIEWS | RHEUMATOLOGY VOLUME 5 | MAY 2009 | 283 CASE STUDY Department of Rheumatology and Clinical Immunology (RDE Fritsch-Stork, RHWM Derksen), Department of Pathology (RJ Leguit), University Medical Center Utrecht, The Netherlands. Correspondence: RDE Fritsch-Stork, Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, PO Box 85500, 3508GA Utrecht, The Netherlands r.fritsch@umcutrecht.nl Rapidly fatal HTLV-1-associated T-cell leukemia/ lymphoma in a patient with SLE Ruth D. E. Fritsch-Stork, Roos J. Leguit and Ronald H. W. M. Derksen Background. A 57-year-old Afro-Jamaican woman with an 8-year history of systemic lupus erythematosus, including lupus nephritis, was admitted to hospital with intractable back pain accompanied by fever and severe malaise. At the time of presentation she was receiving immunosuppressive treatment with glucocorticoids and azathioprine. She also had gout, hypertension and type II diabetes. Investigations. Physical and neurological examination and laboratory analyses, including biochemical, hematological and electrophoresis tests, X-ray of the lumbar spine, pelvis and chest, mammography, MRI of the lumbar spine, thoracic and abdominal CT, and biopsy of a peripheral lymph node and bone marrow with immunohistochemistry and serology for human T-cell lymphotrophic virus (HTLV) 1 and 2. Diagnosis. HTLV-1-associated acute adult T-cell leukemia/lymphoma with bone marrow infiltration and hypercalcemia. Reaching the correct diagnosis was difficult and only possible through close collaboration with the pathologist and with consideration of the patient’s ethnic and geographical background. Management. Chemotherapy with high-dose prednisone and adjusted doses of cyclophosphamide and doxorubicin. The patient developed tumor lysis syndrome and died 3 weeks after the diagnosis was made. Fritsch-Stork, R. D. E. et al. Nat. Rev. Rheumatol. 5, 283–287 (2009); doi:10.038/nrrheum.2009.49 The case At the age of 49 years, an obese (BMI 30.6 kg/m 2 ) Afro- Jamaican woman was diagnosed with systemic lupus erythematosus (SLE) on the basis of the presence of poly- arthritis, diffuse proliferative lupus nephritis and anti- nuclear, anti-double-stranded (ds)-DNA and anti-Sm antibodies. Following an initial infusion of intravenous pulse methylprednisolone 1 g per day for 3 days at the time of diagnosis, immunosuppressive treatment con- sisted exclusively of daily oral prednisone (10–20 mg per day) and azathioprine (150 mg per day; cumulative dose approximately 400 g). The patient was also treated with inhibitors of angiotensin-converting enzyme, diuretics and statins. Apart from hypertension, which was dif- Apart from hypertension, which was dif- ficult to treat, and type II diabetes mellitus, which was controlled with oral anti-diabetics (metformin) starting 5 years after her diagnosis of SLE, the patient did well. She developed gout, which was treated with the uricos- uric drug benzbromarone, 7 years after the diagnosis of SLE. Her creatinine clearance rate was stable and normal at 1.92 ml/s, and proteinuria did not exceed 1.0 g per day when measured at several points during the 8 years following the diagnosis of SLE. Eight years after the diagnosis of SLE the patient was admitted to hospital for the evaluation of complaints of nonfebrile malaise, diarrhea, nausea, abdominal pain and gouty arthritis (confirmed by microscopy) in the left foot and knee over the previous several months. Proteinuria had increased to 4 g per day with cylindruria; the patient’s anti-dsDNA antibody titer was stable and her serum complement level was normal. Relevant laboratory values are given in Table 1. Abdominal CT was normal; in par- ticular, no lymphadenopathy was detected (Figure 1a). A kidney biopsy showed membranous lupus nephritis, and stool cultures confirmed infection with norovirus. The dose of prednisone was increased to 60 mg per day, and insulin was added to her treatment. The patient’s abdominal complaints and arthritis disappeared within several weeks, and she was discharged after 1 month. The daily dose of prednisone was gradually tapered to the preadmission dose of 10 mg per day. Ten weeks after discharge the patient was readmitted with fever and, for the first time, a raised C-reactive protein (CRP) level was detected (129 mg/l; normal range 0–10 mg/l). No infectious cause was found in multiple cul- tures, serology and polymerase chain reaction analyses of the patient’s serum, synovial fluid, urine and stool. As the fever and raised CRP level were transient, and no cause other than a recurrent gouty attack could be identified, the patient was discharged after 10 days with an increased dosage of benzbromarone. Twelve days after discharge, she was admitted again with severe malaise and new, progres- sive lumbar pain. Physical examination revealed a fever (38.1 °C), tachycardia (100 beats per min), local pain upon pressure in the lumbar region and difficulty walking owing to lumbar pain. Neurological examination was normal and there were no clinical signs of SLE activity. Levels of complement protein and anti-dsDNA titer were normal. Competing interests The authors declared no competing interests. © 2009 Macmillan Publishers Limited. All rights reserved