Journal of Medical Case Reports · 2019 · 13 citations · 29 references
MedicineHematologyInternal MedicineHematopathologyHemostasisSystemic PathologyCoagulation AbnormalitiesCoagulation DisordersNephrotic SyndromePulmonary EmbolismThrombosisThrombotic DisordersFactor Viii AlterationsFactor Viii PhysiologyMénétrier DiseaseProtein Deficiency DisordersRecurrent Pulmonary EmbolismTherapeutic Anticoagulation
BACKGROUND: Acquired thrombophilia is a potential sequela of malignancy, chronic inflammation, and conditions characterized by severe protein deficiency (for example, nephrotic syndrome, protein-losing enteropathy). As such, venous thrombosis is often a feature, and occasionally a presenting sign, of systemic disease. Ménétrier's disease is a rare hyperplastic gastropathy that may lead to gastrointestinal protein loss and hypoalbuminemia. To date, reports of venous thrombosis associated with Ménétrier's disease are exceedingly scarce. CASE PRESENTATION: We report the case of a 40-year-old white man who presented with unprovoked deep venous thrombosis, pulmonary embolism, and renal vein thrombosis. Upon receiving therapeutic anticoagulation, he developed severe gastrointestinal bleeding, and endoscopic evaluation led to a diagnosis of Ménétrier's disease. A laboratory workup revealed deficiency of protein C, protein S, and antithrombin III, as well as markedly elevated levels of factor VIII. He was determined to have an acquired thrombophilia as a direct result of Ménétrier's disease. CONCLUSIONS: This case describes an acquired thrombophilic state in a patient with Ménétrier's disease and profound hypoalbuminemia. Although this association is rarely described, we discuss the probable mechanisms leading to our patient's thrombosis. Specifically, we posit that his gastrointestinal protein loss led to a deficiency of several anticoagulant proteins and a compensatory elevation in factor VIII, as occurs in nephrotic syndrome and inflammatory bowel disease. Of note, this patient's recurrent venous thrombosis was the initial clinical sign of his gastrointestinal pathology.
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Protein S Deficiency Occurs in the Nephrotic Syndrome
Silvana Vigano’D’Angelo · Annals of Internal Medicine · 1987 · 147 citations
Medicine, Biochemistry, Nephrology +15
Coagulation factor abnormalities as possible thrombotic risk factors after Fontan operations
A.H. Cromme-Dijkhuis, C. M. A. Bijleveld, C M A Henkens et al. · The Lancet · 1990 · 144 citations
Medicine, Hematology, Surgery +14
Thrombotic vascular risk factors in inflammatory bowel disease.
Mark Hudson, A Chitolie, R A Hutton et al. · Gut · 1996 · 135 citations · Full text
Medicine, Hematology, Hemostasis +15