title: The use of recombinant human activated protein C (rhAPC) in the treatment of severe sepsis in immunosuppressed patients in the course of hematological diseases.
abstract: BACKGROUND
  Treatment of hemopoietic system neoplasias involves severe complications associated with immunosuppression. We present two cases of treating severe sepsis utilizing recombinant human activated protein C (rhAPC) in the course of bilateral pneumonia in patients with hairy cell leukemia (HCL) and T-cell acute lymphoblastic leukemia (ALL). RhAPC, limited the coagulation cascade by inactivating FVa and FVIIIa, directly and indirectly limiting systemic inflammatory response syndrome (SIRS), and improved the fibrinolysis process. These actions break the pathomechanism of sepsis and improve survival.
  
  
  METHODS
  Besides intensive, multidirectional treatment of severe sepsis, Xigris (activated drotrecogin alfa) was administered on the second day in both cases. The infusion continued, as recommended, for 96 hours without complications. During treatment the patients' general condition and respiratory efficiency improved, allowing respirator weaning on days 5 and 8 of therapy. These cases of severe sepsis and immunosuppression indicate a high therapeutic efficacy of drotrecogin alfa (activated). Treatment outcome was uncertain because of the patients' hematological condition, so rapid restoration of respiratory efficiency and no disease progression after discontinuing treatment was a great success, possibly due to implementing Xigris at a relatively early stage of sepsis and the intensive therapy conducted according to Surviving Sepsis Campaign guidelines.
  
  
  CONCLUSIONS
  Patient survival in severe sepsis directly depends on early diagnosis and institution of treatment. 1. These cases confirm the effectiveness of drotrecogin alfa in severe sepsis as part of multidirectional therapy. 2. Microorganisms causing atypical pneumonia should be considered in diagnosing infections in patients treated with cytostatic agents.
authors.affiliation: Department of Anesthesiology and Intensive Care, Silesian Medical University, Katowice, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Kunsdorf-Wnuk
authors.forename: Ewa
authors.initials: E
authors.lastname: Marzec-Lewenstein
authors.forename: Danuta
authors.initials: D
authors.lastname: Arct-Danielak
authors.forename: Ewa
authors.initials: E
authors.lastname: Musioł
authors.forename: Romuald
authors.initials: R
authors.lastname: Bohatyrewicz
authors.forename: Rafał
authors.initials: R
authors.lastname: Becht
mesh_terms: D000328:Adult; D004789:Enzyme Activation; D006801:Humans; D007108:Immune Tolerance; D007943:Leukemia, Hairy Cell / Q000150:complications* / Q000276:immunology / Q000628:therapy; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications* / Q000276:immunology / Q000628:therapy; D011486:Protein C / Q000627:therapeutic use*; D011994:Recombinant Proteins / Q000627:therapeutic use; D018805:Sepsis / Q000150:complications* / Q000188:drug therapy*
chemical_list: D011486:Protein C; D011994:Recombinant Proteins