Severe Hypertriglyceridemia Associated with Pancytopenia and Lipoprotein Lipase Deficiency
Authors: P. N. DURRINGTON
Journal: Annals of Internal Medicine (1981)

Brief Reports1 February 1981Severe Hypertriglyceridemia Associated with Pancytopenia and Lipoprotein Lipase DeficiencyP. N. DURRINGTON, M.D., J. E. MacIVER, M.D., G. HOLDSWORTH, Ph.D., D. J. GALTON, M.D.P. N. DURRINGTON, M.D.Search for more papers by this author, J. E. MacIVER, M.D.Search for more papers by this author, G. HOLDSWORTH, Ph.D.Search for more papers by this author, D. J. GALTON, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-94-2-211 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptBoth type I and type V hyperlipoproteinemia are characterized by hyperchylomicronemia (1). In type I hyperlipoproteinemia levels of no other serum lipoproteins are increased, whereas in type V hyperlipoproteinemia very low density lipoprotein levels are also elevated. Recognized complications of both disorders include episodes of abdominal pain, acute pancreatitis, splenomegaly, and splenic infarction (1). We report a middle-aged patient with the type V phenotype who was unusual because she had pancytopenia secondary to hypersplenism, a complication previously believed not to occur with splenomegaly due to hyperchylomicronemia (1). She also had a profound deficiency of lipoprotein lipase activity in both postheparin...References1. FREDRICKSONGOLDSTEINBROWN DJM. Familial hyperlipoproteinemias. In: STANBURY JB, WYNGAARDEN JB, FREDRICKSON DS, eds. The Metabolic Basis of Inherited Disease. 4th ed. New York:McGraw-Hill Book Co.; 1978: 608-55. Google Scholar2. HARLANWINESETTWASSERMAN WPA. Tissue lipoprotein lipase in normal individuals and in individuals with exogenous hypertriglyceridemia and the relationship of this enzyme to assimilation of fat. J Clin Invest. 1967;46:239-47. CrossrefMedlineGoogle Scholar3. KRAUSSLEVYFREDRICKSON RRD. Selective measurement of two lipase activities in postheparin plasma from normal subjects and patients with hyperlipoproteinemia. J Clin Invest. 1974;54:1107-24. CrossrefMedlineGoogle Scholar4. GRETENDEGRELLAKLOSERASCHERDE GENNESGJONE HRGWJE. Measurement of two plasma triglyceride lipases by an immunochemical method: studies in patients with hypertriglyceridemia. J Lipid Res. 1976;17:203-10. CrossrefMedlineGoogle Scholar5. TAYLORHOLDSWORTHGALTON KGD. Lipoprotein lipase in adipose tissue and plasma triglyceride clearance in patients with primary hypertriglyceridemia. Eur J Clin Invest. 1980;10:133-8. CrossrefMedlineGoogle Scholar6. BRECKENRIDGELITTLESTEINERCHOWPOAPST WJGAM. Hypertriglyceridemia associated with deficiency of apolipoprotein C-II. N Engl J Med. 1978;298;1265-73. CrossrefMedlineGoogle Scholar7. BRADY R. Glucosyl ceramide lipidosis: Gaucher's disease. In: STANBURY JB, WYNGAARDEN JB, FREDRICKSON DS, eds. The Metabolic Basis of Inherited Disease. 4th ed. New York: McGraw-Hill Book Co.; 1978:731-46. Google Scholar8. JAEKENCASTEELS-VANDAELEHARVENGT JML. A hyperlipidemia syndrome in infancy with rapidly fatal evolution. Helv Paediatr Acta. 1973;28:67-71. MedlineGoogle Scholar9. HAGBERGHULTQUISTSVENNERHOLMVOSS BGLH. Malignant hyperlipemia in infancy. Am J Dis Child. 1964;107:267-76. MedlineGoogle Scholar10. YAMAMURASUDOISHIKAWAYAMAMOTO THKA. Familial type I hyperlipoproteinemia caused by apolipoprotein CII deficiency. Atherosclerosis. 1979;34:53-65. CrossrefMedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: University of Manchester and Manchester Royal Infirmary Manchester St. Bartholomew's Hospital West Smithfield, London United Kingdom PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byDyslipidaemiaPreserved Endothelial Function in Patients With Severe Hypertriglyceridemia and Low Functional Lipoprotein Lipase ActivityLipoprotein Lipase Activity in the Pathological Metabolism of LipoproteinsMetabolism of apolipoprotein B-100 and of triglyceride-rich lipoprotein particles in the absence of functional lipoprotein lipaseLipolysis of triglyceride-rich lipoproteins activates coagulant factor XII: A study in familial lipoprotein-lipase deficiencyHypertriglyceridemia Associated with Thrombocytopenia and Lipoprotein Lipase DeficiencyL. ROMICS, M.D. 1 February 1981Volume 94, Issue 2Page: 211-212KeywordsAbdominal painAcute pancreatitisInfarctionLipasesLipoproteinsPhenotypesSplenomegalyVery low density lipoprotein Issue Published: 1 February 1981 PDF downloadLoading ...