title: Successful control of refractory and life-threatening autoimmune hemolytic anemia with intravenous immunoglobulins in a man with the primary antiphospholipid syndrome.
abstract: A 58-year old man with a history of hypothyroidism and primary antiphospholipid syndrome (with recurrent thromboembolic disease and therapy-refractory autoimmune thrombocytopenic purpura) presented with a life-threatening crisis of warm autoimmune hemolytic anemia (AIHA) while under chronic low-dose steroid therapy. The exacerbation was eventually controlled with a 5-day course of intravenous immunoglobulin (IVIG, Sandoglobulin) (400 mg/kg per day) but hemolysis rapidly recurred, despite therapy with steroids, azathioprine, and cyclosporin, necessitating a second course of IVIG. Control of packed cell transfusion needs for about 7 months was achieved by weekly administration of IVIG (800 mg/kg), although there is no direct evidence that IVIG therapy reduced the production of anticardiolipin or RBC antibodies. Three months after discontinuation of IVIG and change to maintenance with intermediate-dose corticosteroids plus cyclosporin A, the patient succumbed to duodenal perforation with peritonitis and invasive pulmonary aspergillosis. The case illustrates that IVIG therapy may be helpful in selected life-threatening and refractory cases of AIHA. It also sadly illustrates the long-term toxicity of standardly used therapeutics in refractory AIHA.
authors.affiliation: Laboratory for Experimental Hematology, Faculty of Medicine, University of Leuven, Belgium.
authors.forename: P
authors.initials: P
authors.lastname: Vandenberghe
authors.forename: P
authors.initials: P
authors.lastname: Zachee
authors.forename: S
authors.initials: S
authors.lastname: Verstraete
authors.forename: H
authors.initials: H
authors.lastname: Demuynck
authors.forename: M A
authors.initials: MA
authors.lastname: Boogaerts
authors.forename: G E
authors.initials: GE
authors.lastname: Verhoef
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications* / Q000517:prevention & control*; D000753:Anemia, Refractory / Q000150:complications* / Q000517:prevention & control*; D016736:Antiphospholipid Syndrome / Q000150:complications*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged
chemical_list: D016756:Immunoglobulins, Intravenous