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Essential Thrombocytosis and Antiphospholipid Antibody Syndrome Causing Chronic Budd-Chiari Syndrome

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Abstract

Essential thrombocytosis is extremely rare in children. However, when present, it is associated with increased prevalence of antiphospholipid antibodies and thrombo-hemorrhagic complications. The authors report here a child with Budd-Chiari Syndrome resulting from essential thrombocytosis and associated antiphospholipid antibodies. A 13- y-old boy presented with microcytic hypochromic anemia, hepatosplenomegaly and thrombocytosis. CT scan demonstrated calcified thrombus in inferior vena cava (IVC). Diagnosis of essential thrombocytosis was considered in view of persistent thrombocytosis, antiphospholipid antibodies, bone marrow showing increased number, clusters and giant forms of megakaryocytes and IVC thrombosis. He was started on warfarin prophylaxis and did not have thrombotic recurrence on follow up.

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Contributions

DY, JC: Diagnosis and management of patient, reviewed the literature and drafted the manuscript. SS, VS: Helped in diagnostic work up and critical review of the manuscript.

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Correspondence to Jagdish Chandra.

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Yadav, D., Chandra, J., Sharma, S. et al. Essential Thrombocytosis and Antiphospholipid Antibody Syndrome Causing Chronic Budd-Chiari Syndrome. Indian J Pediatr 79, 538–540 (2012). https://doi.org/10.1007/s12098-011-0550-6

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  • DOI: https://doi.org/10.1007/s12098-011-0550-6

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