Primary mixed-type autoimmune hemolytic anemia concomitant with acute splanchnic venous thrombosis of idiopathic origin in a young woman: an unexplained association

نویسندگان

  • Laura Scaramucci
  • Marco Giovannini
  • Pasquale Niscola
  • Alessio Perrotti
  • Paolo de Fabritiis
چکیده

which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Primary mixed-type autoimmune hemolytic anemia concomitant with acute splanchnic venous thrombosis of idiopathic origin in a young woman: an unexplained association TO THE EDITOR: Mixed autoimmune hemolytic anemia (AIHA) is defined by the presence of both warm and cold types of autoantibodies [1, 2]. The diagnosis is based on the detection of these autoantibodies by using a monospecific direct antiglobulin test; a positive test result shows a pattern of IgG and complement C3d along with the presence of cold agglutinins. Mixed AIHA may be of idiopathic origin or associated with an underlying disease [1]. Venous throm-boembolism is a common complication of AIHA, although it is not often identified [3, 4]. In some instances, its occurrence may be related to coexistent antiphospholipid anti-bodies. However, the association of primary mixed AIHA with idiopathic acute splanchnic venous thrombosis (SVT), as we recently observed, represents an exceptional occurrence ; thus far, it has been reported for AIHA only in conjunction with splenectomy [5], but has never been reported as a concomitant manifestation. In the present report, we describe the case of a 19-year-old woman who presented with diffuse abdominal pain and severe anemia. At admission, she presented with life-threatening medical conditions due to severe anemia and critical abdominal features. Her medical history was unremarkable. Findings on clinical images and laboratory parameters were consistent with an acute and severe hemolytic disorder; her Hb level had decreased to 3.9 g/dL, whereas the retic-ulocyte count, indirect bilirubin level, and lactate de-hydrogenase concentration were elevated. On admission, the patient was transfused with packed red blood cells (12 units). A strongly positive result on direct antiglobulin test (IgG3 and C3d) was detected; the indirect antiglobulin test indicated a positive result at 4 o C and a negative result at 22 o C and 39 o C in the presence of a cold panagglutinin anti-body (IgM). The concomitant acute abdominal condition was investigated by a comprehensive radiological work-up, including an abdominal echography and a body computed tomography scan, and revealed complete thrombosis of the portal and splenic veins as well as partial occlusion of the superior mesenteric vein. A diagnosis of mixed AIHA associated with SVT was made, and treatment with prednisone (1 mg/kg body weight/day) was promptly initiated; in addition , therapeutic subcutaneous Fraxiparine injections with warfarin were also administered. Additional …

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عنوان ژورنال:

دوره 48  شماره 

صفحات  -

تاریخ انتشار 2013