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


METHODS
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.


METHODS
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.


RESULTS
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.
Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, The Netherlands. r.fritsch@umcutrecht.nl
Ruth D E
RD
Fritsch-Stork
Roos J
RJ
Leguit
Ronald H W M
RH
Derksen
D001379:Azathioprine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D004317:Doxorubicin / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D015481:HTLV-I Antibodies / Q000097:blood; D015368:Human T-lymphotropic virus 1 / Q000276:immunology / Q000302:isolation & purification; D006801:Humans; D016867:Immunocompromised Host; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use
D005938:Glucocorticoids; D015481:HTLV-I Antibodies; D007166:Immunosuppressive Agents; D004317:Doxorubicin; D003520:Cyclophosphamide; D001379:Azathioprine; D011241:Prednisone