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Risk factors for thromboembolism and pulmonary artery hypertension following splenectomy in children with hereditary spherocytosis
Author(s) -
Das Anirban,
Bansal Deepak,
Ahluwalia Jasmina,
Das Reena,
Rohit Manoj K.,
Attri Savita V.,
Trehan Amita,
Marwaha Ram K.
Publication year - 2014
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.24766
Subject(s) - medicine , splenectomy , hereditary spherocytosis , gastroenterology , thrombosis , reference range , fibrinogen , surgery , cardiology , spleen
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. Procedure 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. Pediatr Blood Cancer 2014;61:29–33. © 2013 Wiley Periodicals, Inc.