title: Risk factors for thromboembolism and pulmonary artery hypertension following splenectomy in children with hereditary spherocytosis.
abstract: BACKGROUND
  The aim was to study risk-factors for vascular thrombosis and incidence of pulmonary artery hypertension (PAH) in splenectomized children with hereditary spherocytosis (HS) at a single center.
  
  
  METHODS
  Pre- and post-splenectomy hemoglobin and platelet counts were recorded. Post-splenectomy lipid-profile, fibrinogen, D-dimer, CRP and anti-coagulant-protein levels were compared to established controls. Echo-Doppler was performed for PAH.
  
  
  RESULTS
  Twenty-six children with HS had undergone splenectomy; the mean age at surgery was 7.9 ± 3.7 years. Nineteen of the 26 were prospectively investigated at a median duration of 4.5 years (range: 4 months to 19 years) following splenectomy. Thrombocytosis was observed in 19 (73%), whereas no patient had erythrocytosis at the last follow-up visit. Total cholesterol, LDL-C, HDL-C, and triglyceride levels were not deranged (P ≥ 0.3). Mean CRP levels (males: 2.8 ± 0.5; females: 2.1 ± 0.5 mg/L) were significantly higher than described for normal children (P < 0.001). Six (23%) patients had a positive D-dimer assay. Protein S, anti-thrombin-III and fibrinogen were in range. A single patient had a borderline low protein C activity. Lupus anticoagulant and anti-cardiolipin antibody assays were negative. The mean tricuspid regurgitant jet velocity (TRJV) was 1.8 ± 0.55 meter per second (range: 0-2.4). None had a TRJV ≥2.5 meter per second to suggest PAH.
  
  
  CONCLUSIONS
  There was no evidence of PAH, dyslipidemia, elevation of fibrinogen or a reduction in anti-coagulant proteins, at a median follow-up duration of 4.5 years following splenectomy in children with HS. However, elevated CRP level (42%), persistent thrombocytosis (73%) and elevated D-dimer levels (23%) were observed. These have been recognized as risk factors for cerebrovascular and coronary heart disease.
authors.affiliation: Pediatric Hematology-Oncology Unit, Department of Pediatrics, Advanced Pediatrics Center, Chandigarh, India.
authors.forename: Anirban
authors.initials: A
authors.lastname: Das
authors.forename: Deepak
authors.initials: D
authors.lastname: Bansal
authors.forename: Jasmina
authors.initials: J
authors.lastname: Ahluwalia
authors.forename: Reena
authors.initials: R
authors.lastname: Das
authors.forename: Manoj K
authors.initials: MK
authors.lastname: Rohit
authors.forename: Savita V
authors.initials: SV
authors.lastname: Attri
authors.forename: Amita
authors.initials: A
authors.lastname: Trehan
authors.forename: Ram K
authors.initials: RK
authors.lastname: Marwaha
mesh_terms: D000293:Adolescent; D002097:C-Reactive Protein / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000032:analysis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000209:etiology; D015994:Incidence; D008297:Male; D012307:Risk Factors; D013103:Spherocytosis, Hereditary / Q000097:blood / Q000601:surgery*; D013156:Splenectomy / Q000009:adverse effects*; D013923:Thromboembolism / Q000453:epidemiology* / Q000209:etiology; D055815:Young Adult
chemical_list: D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D; D002097:C-Reactive Protein
keywords: C-reactive protein; echocardiography; hemolytic anemia; low-density lipoprotein; spleen