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Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis


Background. A 39-year-old woman with no relevant medical or family history was admitted to hospital with episodic fever, which persisted despite antibiotic therapy. Other notable findings at admission were splenomegaly, pancytopenia, hyponatremia, elevated levels of liver enzymes, hyperferritinemia and hypofibrinogenemia.

Investigations. Physical examination, laboratory tests, rheumatic marker serology, pathogen detection assays, complete blood counts, measurement of levels of ferritin, fibrinogen, triglycerides and soluble CD25, natural killer cell functional studies, PRF1 mutation analysis, renal biopsy, bone marrow biopsy, CT imaging of the chest and abdomen.

Diagnosis. Idiopathic, atypical hemophagocytic lymphohistiocytosis.

Management. Initial treatment with antibiotics was followed by immunosuppressive therapy (including intravenous immunoglobulin, ciclosporin, infliximab, corticosteroids and etoposide). Remission was achieved by treatment with the anti-CD52 monoclonal antibody, alemtuzumab, after which allogeneic stem-cell transplantation (with reduced-intensity conditioning treatment and graft-versus-host disease prophylaxis) resulted in a definitive cure.

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Figure 1: Changes in white blood cell counts and ferritin levels during the patient's treatment.
Figure 2: Chest and abdominal CT images of the patient at diagnosis.
Figure 3: Histology of the patient's bone marrow biopsy sample at diagnosis.


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M. P. Strout is a Fellow of the Leukemia and Lymphoma Society and a Scholar of the American Society of Hematology. Written consent for publication was obtained from the patient.

Charles P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

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Strout, M., Seropian, S. & Berliner, N. Alemtuzumab as a bridge to allogeneic SCT in atypical hemophagocytic lymphohistiocytosis. Nat Rev Clin Oncol 7, 415–420 (2010).

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