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Rapidly fatal HTLV-1-associated T-cell leukemia/lymphoma in a patient with SLE

Nature Reviews Rheumatology volume 5, pages 283–287 (2009)Cite this article

Abstract

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.

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Figure 1: Radiologic findings.
Figure 2: Histology of the patient's supraclavicular lymph node.

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Acknowledgements

The authors thank Prof. P. M. Kluin of the University Medical Centre Groningen, The Netherlands, for his invaluable help in the diagnostic procedure.

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Authors and Affiliations

  1. Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, The Netherlands

    Ruth D. E. Fritsch-Stork & Ronald H. W. M. Derksen

  2. Department of Pathology, University Medical Center Utrecht, The Netherlands

    Roos J. Leguit

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  1. Ruth D. E. Fritsch-Stork
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  2. Roos J. Leguit
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  3. Ronald H. W. M. Derksen
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Correspondence to Ruth D. E. Fritsch-Stork.

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The authors declare no competing financial interests.

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Fritsch-Stork, R., Leguit, R. & Derksen, R. Rapidly fatal HTLV-1-associated T-cell leukemia/lymphoma in a patient with SLE. Nat Rev Rheumatol 5, 283–287 (2009). https://doi.org/10.1038/nrrheum.2009.49

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