A case of catastrophic anti phospholipid syndrome with superimposed aspergillus infection

Elvina Lingas,Maria del Mar Lucio Paredes

doi:10.3823/2637

Abstract

Catastrophic anti phospholipid syndrome or CAPS for short, is a rare, devastating form of antiphospholipid syndrome that is characterized by successive multiple thrombi and multiorgan failure. One of the complications of antiphospholipid syndrome is nonbacterial thrombotic endocarditis or marantic endocarditis. Due to the nonspecific presentation of multiorgan failure it is difficult to have an early diagnosis. We are presenting a case of catastrophic anti phospholipid syndrome with superimposed aspergillus infection

Highlights

Anti-phospholipid syndrome is a hematological disorder most commonly affecting young females that is characterized by multiple thrombosis and/or recurrent miscarriages alongside with persistence of lupus anticoagulant (LAC); anti-β2-glycoprotein I and/or anti-cardiolipin antibodies
CAPS (Catastrophic Anti Phospholipid Syndrome) is an extremely rare (
Probable CAPS would include either all four criteria, except for only two organs, systems, and/or sites of tissue involvement or all four criteria, except for the laboratory confirmation at least six weeks apart due to the early death of a patient never tested for aPL before the catastrophic APS; or development of the events with positive antibodies without histopathological confirmation

Summary

Introduction

Anti-phospholipid syndrome (or abbreviated as APLS) is a hematological disorder most commonly affecting young females that is characterized by multiple thrombosis and/or recurrent miscarriages alongside with persistence of lupus anticoagulant (LAC); anti-β2-glycoprotein I (anti-β2GPI) and/or anti-cardiolipin (aCL) antibodies. It was first described in 1984 in a patient who developed rapidly worsening kidney failure and systemic thrombi. The term nonbacterial thrombotic endocarditis (NBTE), or marantic endocarditis, refers to sterile vegetations on previously undamaged heart valves (most often aortic and mitral) in the absence of a bloodstream bacterial infection. NBTE can dislodge from the site and cause a recurrent thromboembolic event; despite having not been studied sufficiently to determine prognosis; from clinical observation its prognosis is considered poor. [4] A Russian study that aimed to further characterize NBTE in APLS showed that 54% of patients developed CNS embolic event. [5] Despite being exceedingly rare, this disease entity carries high mortality risk

Case Presentation

Findings

Discussion