title: Prolonged treatment response in aggressive natural killer cell leukemia.
abstract: We describe a case of natural killer (NK) cell leukemia with acute presentation, systemic symptoms and hepatosplenomegaly. The uniform and aberrant phenotype of NK cells with infiltration of bone marrow and spleen was in keeping with a malignant diagnosis. Aggressive presentation was demonstrated by marked constitutional symptoms and significant tumor burden (liver, spleen, blood, bone marrow). The subsequent clinical course has been indolent, but this may have been influenced by treatment. Treatment consisted sequentially of splenectomy, intravenous pentostatin and the combination of cyclosporine A and recombinant human erythropoietin and has resulted in survival of over 48 months. We discuss the difficulties in the diagnosis of this condition, explore possible causes of cytopenia(s), and highlight the role of immunosuppression in controlling disease manifestations in large granular lymphocyte proliferative disorders.
authors: Section of Haemato-Oncology, Royal Marsden NHS Trust/Institute of Cancer Research, London, UK.
authors: N
authors: N
authors: Osuji
authors: E
authors: E
authors: Matutes
authors: A
authors: A
authors: Morilla
authors: I
authors: I
authors: Del Giudice
authors: A
authors: A
authors: Wotherspoon
authors: D
authors: D
authors: Catovsky
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D016572:Cyclosporine / Q000008:administration & dosage; D004921:Erythropoietin / Q000008:administration & dosage; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D007694:Killer Cells, Natural / Q000473:pathology*; D007938:Leukemia / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D015649:Pentostatin / Q000008:administration & dosage; D011994:Recombinant Proteins; D013156:Splenectomy
chemical_list: D011994:Recombinant Proteins; D004921:Erythropoietin; D015649:Pentostatin; D016572:Cyclosporine