title: Essential thrombocytosis and antiphospholipid antibody syndrome causing chronic Budd-Chiari syndrome.
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.
authors.affiliation: Department of Pediatrics, Lady Hardinge Medical College and associated Kalawati Saran Children's Hospital, New Delhi, India.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Yadav
authors.forename: Jagdish
authors.initials: J
authors.lastname: Chandra
authors.forename: Sunita
authors.initials: S
authors.lastname: Sharma
authors.forename: Varinder
authors.initials: V
authors.lastname: Singh
mesh_terms: D000293:Adolescent; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000150:complications*; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D002908:Chronic Disease; D003906:Developing Countries; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D013920:Thrombocythemia, Essential / Q000150:complications*; D014057:Tomography, X-Ray Computed; D014859:Warfarin / Q000627:therapeutic use
chemical_list: D000925:Anticoagulants; D014859:Warfarin