Cold agglutinin disease (CAD) is a small group of disorders that is characterized by cold-reactive autoantibodies that bind to erythrocyte carbohydrate antigens, and this causes hemagglutination and complement-mediated hemolysis. Autoimmune hemolytic anemia (AIHA) is an immune disorder that is mediated via auto-antibodies produced by lymphoid B cells against red blood cells. The disorder may be a primary (idiopathic) or secondary disease with an underlying autoimmune disease, a lymphoproliferative disorder or infection. The mainstay of initial treatment is immunosuppression with glucocorticosteroids. For those who do not have satisfactory response to initial glucocorticosteroids or they have a relapse after initially successful treatment, splenectomy or other immunosuppressive agents such as azathioprine, cyclosporine and intravenous immunoglobulin (IVIG) could be the next available options. More recently, rituximab, which is a human-murine chimeric monoclonal antibody specific for the CD20 antigen found on the surface of B lymphocytes, is also available. We report here on the successful use of rituximab for the treatment of a Korean elderly patient with CAD and the patient presented with recurrent AIHA.
Aged ; Anemia, Hemolytic, Autoimmune ; Antibodies, Monoclonal, Murine-Derived ; Antigens, CD20 ; Autoantibodies ; Autoimmune Diseases ; Azathioprine ; B-Lymphocytes ; Cold Temperature ; Cyclosporine ; Erythrocytes ; Hemagglutination ; Hemolysis ; Humans ; Immune System Diseases ; Immunoglobulins ; Immunosuppression ; Immunosuppressive Agents ; Lymphoproliferative Disorders ; Pneumonia, Bacterial ; Recurrence ; Splenectomy ; Rituximab

We report an unusual case of acute myelogenous leukemia in a patient who showed an extramedullary relapse in her uterus, without bone marrow recurrence, two years after an allogeneic bone marrow transplant. She complained of irregular vaginal spotting, and magnetic resonance imaging demonstrated a uterine mass. A biopsy revealed a massive infiltration of immature myeloid cells. A variable number of tandem repeats (VNTR) based on an examination of peripheral blood cells showed full donor chimerism. After receiving chemotherapy, her uterine mass had completely resolved. She has remained in complete remission for more than 6 months. This case suggests that physicians should be aware of the possibility of a uterine relapse in female bone marrow transplant recipients with acute myelogenous leukemia.
Adult ; Female ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Human ; Leukemia, Myelocytic, Acute/*pathology/*therapy ; Neoplasm Recurrence, Local ; Sarcoma, Granulocytic/etiology/*pathology ; Uterine Neoplasms/etiology/*pathology