title: Orthotopic liver transplant for multifocal lymphangioendotheliomatosis with thrombocytopenia.
abstract: An eight-yr-old female with a history of multifocal lymphangioendotheliomatosis and thrombocytopenia presented for MVT. The patient had multiple vascular lesions in the skin and stomach in infancy. Although her cutaneous lesions resolved with vincristine and methylprednisolone, her gastric lesions persisted. Eight yr later, she was diagnosed with portal hypertension and decompensating liver function despite therapy with bevacizumab, propranolol, furosemide, and spironolactone. Upon presentation, she was found to have a Kasabach-Merritt-like coagulopathy in association with multiple lesions in her GI tract and persistent gastric lesions. Although treatment with methylprednisolone and sirolimus normalized her coagulation factors and d-dimer levels, she never developed sustained improvement in her thrombocytopenia. Her liver function continued to deteriorate and she developed hepatorenal syndrome. Given better outcomes after OLT in comparison with MVT, she underwent OLT, with the plan to manage her GI lesions with APC post-transplant. Post-transplant, her liver function and coagulopathy normalized, and GI tract lesions disappeared upon screening with capsule endoscopy. The patient is doing well, without recurrence of either GI lesions or thrombocytopenia, at 18 months after transplantation.
authors.affiliation: Division of Pediatrics, Children's Hospital of Los Angeles, University of Southern California, Los Angeles, CA, USA.
authors.forename: Christine H
authors.initials: CH
authors.lastname: Yang
authors.affiliation: Division of Pathology, Children's Hospital of Los Angeles, University of Southern California, Los Angeles, CA, USA.
authors.forename: Shengmei
authors.initials: S
authors.lastname: Zhou
authors.affiliation: Division of Hepatobiliary Surgery and Abdominal Organ Transplantation, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
authors.forename: Sophoclis
authors.initials: S
authors.lastname: Alexopoulos
authors.affiliation: Division of Pathology, Children's Hospital of Los Angeles, University of Southern California, Los Angeles, CA, USA.
authors.forename: Larry
authors.initials: L
authors.lastname: Wang
authors.affiliation: Pediatric Gastroenterology and Nutrition Associates, Sunrise Children's Hospital, Las Vegas, NV, USA.
authors.forename: Howard I
authors.initials: HI
authors.lastname: Baron
authors.affiliation: Division of Hepatobiliary Surgery and Abdominal Organ Transplantation, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
authors.forename: Yuri
authors.initials: Y
authors.lastname: Genyk
authors.affiliation: Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Los Angeles, University of Southern California, Los Angeles, CA, USA.
authors.forename: Nanda
authors.initials: N
authors.lastname: Kerkar
mesh_terms: D001777:Blood Coagulation; D001779:Blood Coagulation Factors; D002648:Child; D002779:Cholestasis; D005260:Female; D005338:Fibrin Fibrinogen Degradation Products / Q000737:chemistry; D041981:Gastrointestinal Tract / Q000473:pathology; D006530:Hepatorenal Syndrome / Q000150:complications; D006801:Humans; D006975:Hypertension, Portal; D016031:Liver Transplantation / Q000379:methods*; D008202:Lymphangioma / Q000150:complications* / Q000601:surgery*; D008775:Methylprednisolone / Q000008:administration & dosage; D013270:Stomach / Q000473:pathology; D013921:Thrombocytopenia / Q000150:complications* / Q000601:surgery*; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D001779:Blood Coagulation Factors; D005338:Fibrin Fibrinogen Degradation Products; C036309:fibrin fragment D; D014750:Vincristine; D008775:Methylprednisolone
keywords: liver; pediatrics; transplants