Acute hemolysis of transfused A2 red cells by an auto-HI antibody.

Background: Anti-HI is a common cold autoantibody that complicates serologic testing for underlying alloantibodies and has only rarely been associated with hemolysis. An unusual case of an acute hemolytic transfusion reaction (AHTR) due to an anti-HI autoantibody in a subgroup A1 patient transfused with A2 red blood cells (RBCs) is reported.

Case Report: A 56-year-old man presented to the hospital with anemia and gastrointestinal tract bleeding.

Current usage of intravenous immune globulin and the rationale behind it: the Massachusetts General Hospital data and a review of the literature.

Background: Intravenous immune globulin (IVIG) has been approved by the Food and Drug Administration (FDA) for use in 6 conditions: immune thrombocytopenic purpura (ITP), primary immunodeficiency, secondary immunodeficiency, pediatric HIV infection, Kawasaki disease, prevention of graft versus host disease (GVHD) and infection in bone marrow transplant recipients. However, most usage is for off-label indications, and for some of these comprehensive guidelines have been published.

Study Design And Methods: We retrospectively reviewed all approved IVIG transfusions at Massachusetts General Hospital in 2004 to identify the current usage pattern and completed a literature review.

Rituximab can be combined with daily plasma exchange to achieve effective B-cell depletion and clinical improvement in acute autoimmune TTP.

Much of idiopathic thrombotic thrombocytopenic purpura (TTP) is attributed to the presence of an autoantibody to ADAMTS13, the metalloprotease that degrades ultralarge von Willebrand protein multimers. Most patients respond to treatment with therapeutic plasma exchange (TPE), which replaces the missing protease and removes the circulating inhibitor. However, a substantial fraction of idiopathic TTP cases (10%-20%) might not respond to TPE alone, and, therefore, interest has been gathering around the use of the novel immunosuppressive anti-B-cell antibody, rituximab.

Hyperhemolysis syndrome in anemia of chronic disease.

Background: Occasional cases of delayed hemolytic transfusion reaction (DHTR) demonstrate severe and persistent hemolysis and are referred to as hyperhemolysis syndrome. This syndrome usually occurs in patients with sickle cell disease and possibly thalassemia who receive multiple transfusions. There are few such clinical reports in patients without hemoglobinopathies.

Nephrolithiasis as primary symptom in sarcoidosis.

A 30-year-old black woman presented with a 1-year history of repeated episodes of worsening weight loss and fatigue. Physical examination showed no skin lesions and a chest roentgenogram was normal. Workup showed no obvious cause of recurrent renal stones.