Fatal pulmonary embolism following splenectomy in a patient with Evan’s syndrome: case report and review of the literatureReport as inadecuate




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Thrombosis Journal

, 15:18

First Online: 03 July 2017Received: 04 May 2017Accepted: 26 June 2017

Abstract

BackgroundEvans syndrome ES is a rare disease characterized by simultaneous or sequential development of autoimmune hemolytic anemia AIHA and immune thrombocytopenia ITP with or without immune neutropenia. Splenectomy is one of the treatment options for disease refractory to medical therapy. Venous thromboembolism VTE following splenectomy for hematological diseases has an incidence of 10%.

Case presentationHere we describe a case report of a young patient hospitalized with severe hemolytic anemia with Hgb 4.8 g-dl. He developed thrombocytopenia with platelet nadir of 52,000-mm, thus formally diagnosed with ES. He failed standard medical therapy. He underwent splenectomy and had a fatal outcome. Autopsy confirmed the cause of death as pulmonary embolism PE.

ConclusionsThis case report and review of the literature highlight important aspects of the association between VTE, splenectomy, and hemolytic syndromes including the presence of thrombocytopenia. The burden of the disease is reviewed as well as various pathophysiologic mechanisms contributing to thromboembolic events in these patients and current perioperative prophylactic anticoagulation strategies. Despite an advancing body of literature increasing awareness of VTE following splenectomy, morbidity and mortality remains high. Identifying high risk individuals for thromboembolic complications from splenectomy remains a challenge. There are no consensus guidelines for proper perioperative and post-operative anti-coagulation. We encourage future research to determine which factors might be playing a role in increasing the risk for VTE in real time with hope of forming a consensus to guide management.

KeywordsVenous thromboembolism Splenectomy Evan’s Syndrome Reactive thrombocytosis Case report AbbreviationsACCPAmerican College of Chest Physicians

AIHAAutoimmune hemolytic anemia

BMIBody mass index

CMSCenters for Medicaid and Medicare Services

DVTDeep vein thrombosis

EKGElectrocardiogram

ESEvan’s Syndrome

ETEssential thrombocythemia

ITPImmune thrombocytopenia

IVIGIntravenous immunoglobulin

LDHLactate dehydrogenase

MCVMean corpuscular volume

MPNMyeloproliferative neoplasm

PEPulmonary embolism

PMPPlatelet microparticles

PSVTPortal or splenic vein thrombosis

RNARibonucleic acid

RPReticulated platelets

RP%Reticulated platelet percentage

RTReactive thrombocytosis

SCDsSequential compression devices

VTEVenous thromboembolism

w-AIHAWarm autoimmune hemolytic anemia

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Author: Varun Monga - Seth M. Maliske - Usha Perepu

Source: https://link.springer.com/article/10.1186/s12959-017-0141-5







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