title: Von Willebrand Factor and Factor VIII levels in Egyptian children with newly diagnosed acute lymphoblastic leukemia in relation to peripheral blast cells and steroid therapy.
abstract: BACKGROUND
  Endothelial dysfunction has been reported in children with acute lymphoblastic leukemia (ALL). The aim of this study is to assess Von Willebrand Factor antigen (VWF antigen) and Factor VIII (FVIII) in newly diagnosed ALL patients, in relation to peripheral blast (PB) cells, steroid therapy, and any prognostic potential.
  
  
  METHODS
  VWF antigen and FVIII were assessed initially (D0) and at day 8 (D8) steroid therapy for 32 newly diagnosed ALL patients with and without peripheral blood blast cells.
  
  
  RESULTS
  At diagnosis, patients with PBs had a significantly higher levels of VWF antigen (102.7 ± 22.9% vs. 56.9 ± 8%, P<0.001) and FVIII (93.4 ± 15.9% vs. 6 62.6 ± 18.1%, P<0.001) than those without. Following steroid therapy, both factors decreased in those with PBs, whereas an increase above baseline was observed in those without PBs. Furthermore, there was a significant positive correlation between PBs and both VWF antigen (P<0.001) and FVIII levels (P=0.002). High-risk patients were comparable with standard-risk group in mean values of VWF antigen (P=0.234) and FVIII (P=0.891) at diagnosis. After 12 months from diagnosis, all patients without PB achieved and maintained complete remission. Those with initial PB reported relapse (12.5%) or death (4.2%) during follow-up.
  
  
  CONCLUSIONS
  Markers of endothelial dysfunction namely VWF and FVIII were related to circulating blast cells and steroids therapy through lysis of lymphoblasts results in reduction of both factors, with risk of thrombosis during induction with marked disintegration of malignant cells.
authors.affiliation: *Pediatric Hematology/Oncology Unit, Children's Hospital ‡Clinical Pathology Department, Ain Shams University, Nasr City †15 May Hospital, Cairo, Egypt.
authors.forename: Nayera H K
authors.initials: NH
authors.lastname: El Sherif
authors.forename: Marian F G
authors.initials: MF
authors.lastname: Narouz
authors.forename: Tahany A
authors.initials: TA
authors.lastname: Elkerdany
authors.forename: Safinaz A
authors.initials: SA
authors.lastname: El Habashy
mesh_terms: D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D003907:Dexamethasone / Q000008:administration & dosage / Q000009:adverse effects; D004534:Egypt; D005169:Factor VIII / Q000378:metabolism*; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage / Q000009:adverse effects; D006801:Humans; D007223:Infant; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism; D011379:Prognosis; D011446:Prospective Studies; D012074:Remission Induction; D018570:Risk Assessment; D013256:Steroids / Q000008:administration & dosage* / Q000009:adverse effects*; D013927:Thrombosis / Q000139:chemically induced / Q000175:diagnosis / Q000378:metabolism; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D015415:Biomarkers; D005938:Glucocorticoids; D013256:Steroids; D014841:von Willebrand Factor; D003907:Dexamethasone; D005169:Factor VIII