Humans ; Adult ; Anemia, Hemolytic, Autoimmune/therapy* ; Anemia, Hemolytic

Cold agglutinin disease is a kind of autoimmune hemolytic anemia, caused by cold agglutinin, serum autoantibodies activated at reduced body temperatures to produce red blood cell agglutination and hemolysis. In this paper we described a case of severe hemolytic anemia in a cold agglutinin disease patient treated with therapeutic plasma exchange. Therapeutic plasma exchanges were performed four times every other day. Over the same period, a total of 8 units of washed red blood cells were transfused. Then hemoglobin was increased from 4.0 g/dL to 7.8 g/dL. On the 12th hospital day hemoglobin level was decreased again to 4.2 g/dL and fludarabine chemotherapy was started on the 14th hospital day. The patient's symptoms were relieved and she was discharged on the 30th hospital day. As in this case, therapeutic plasma exchange could be considered as secondary therapy for temporary improvement of acute severe hemolytic anemia in cold agglutinin disease.
Agglutination ; Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune* ; Autoantibodies ; Body Temperature ; Drug Therapy ; Erythrocytes ; Hemolysis ; Humans ; Plasma Exchange*

A 34-year-old lady presenting with features of cold agglutinin disease during the course of systemic lupus erythematosus is described. Cold antibody titer was very high (1 in 4096) with specificity for 'I' antigen. Even though she had poor prognostic factors like high titer of cold antibodies with low thermal amplitude, she responded well to prednisolone.
Adult ; Anemia, Hemolytic, Autoimmune/immunology ; Anemia, Hemolytic, Autoimmune/etiology* ; Anemia, Hemolytic, Autoimmune/drug therapy ; Antibodies/analysis ; Case Report ; Female ; Human ; Lupus Erythematosus, Systemic/complications* ; Prednisolone/therapeutic use ; Prognosis

The purpose of this study was to review the clinical hematological, immunological features and treatment responsiveness in children with autoimmune hemolytic anemia (AHA). Eight children with AHA and positive Coombs' test was evaluated. Seven patients presented with acute onset of symptoms and histories of infection. One case was diagnosed as Evans syndrome, one as a chromosomal anomaly, and one case was combined with the Guillain-Barre syndrome. Among 8 the patients, 4 exhibited warm antibodies and the remainder had cold antibodies. The patients were given washed packed red blood cells, prednisolone or immunosuppressive drugs (6-MP or cyclophosphamide). Five patients responded well to transfusion and/or prednisolone, one patient died and one patient showed no response in 5 months of follow up.
Adolescent ; Anemia, Hemolytic, Autoimmune/blood ; Anemia, Hemolytic, Autoimmune/diagnosis* ; Anemia, Hemolytic, Autoimmune/therapy ; Child ; Child, Preschool ; Coombs' Test ; Female ; Human ; Infant ; Male ; Retrospective Studies

In order to screen the compatible red cells by using extracorporal hemolysis test for acute autoimmune hemolytic anemia (AIHA) patients who were difficult to be matched by automatic microcolumn gel indirect antiglobulin test. Twenty-six cases of AIHA were chosen as control group, to whom the same type of donor red blood cells were infused with the weakest blood agglutination; 12 cases of acute AIHA patients were chosen as test group, these patients were difficult to be matched by automatic microcolumn gel indirect antiglobulin test, and the donor red cells without hemolysis by extracoral hemolysis test were transfused for them. The results showed that compared with the control group,the effect of transfusion was better in test group (P < 0.01), with 2.26 U leukocyte-depleted erythrocyte suspension in average, whose hemoglobin, reticulocyte and total bilirubin levels were changed significantly compared with those before blood transfusion (P < 0.01) . It is concluded that the compatible red blood cells for the acute AIHA patients can be screened by the extracorporal hemolysis test, when it is difficult to screen by the automatic microcolumn gel indirect antiglobulin test.
Anemia, Hemolytic, Autoimmune ; therapy ; Blood Transfusion ; Coombs Test ; Erythrocyte Count ; Erythrocytes ; Hemolysis ; Humans ; Platelet Transfusion

Anemia, Hemolytic, Autoimmune ; drug therapy ; Drugs, Chinese Herbal ; Female ; Humans ; Middle Aged

Adrenal Cortex Hormones ; therapeutic use ; Anemia, Hemolytic, Autoimmune ; diagnosis ; drug therapy ; Female ; Humans ; Infant ; Treatment Outcome

Anemia, Hemolytic, Autoimmune ; therapy ; Antibodies, Monoclonal, Murine-Derived ; therapeutic use ; Female ; Humans ; Infant ; Rituximab

IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosis disease characterized by elevated serum IgG4 and tissues as well as organs infiltrated with IgG4-positive cells, resulting in swelling and damage.It is currently treated as first-line treatment with glucocorticoids. Autoimmune hemolytic anemia (AIHA) is also a relatively rare disease that caused by autoreactive erythrocyte antibodies. Although both are autoimmune-related diseases, they rarely overlap. The relationship between them is not clear. A case of IgG4-RD combined with AIHA is reported. The patient has shortness of breath, cough, and sputum after physical activity. Physical examination showed appearance of anemia, yellow staining of skin and sclera, palpable neck and multiple swollen lymph nodes. Laboratory examination, bone marrow biopsy, and lymph node biopsy confirmed the diagnosis. Therefore, clinicians should develop ideas and raise awareness of such diseases.
Anemia, Hemolytic, Autoimmune ; diagnosis ; drug therapy ; Autoimmune Diseases ; complications ; Biopsy ; Humans ; Immunoglobulin G ; Immunoglobulin G4-Related Disease ; complications ; diagnosis

A pediatric patient with combined primary thrombocytopenic purpura and acquired hemolytic anemia (Evans syndrome), whose condition did not respond to treatment with prednisolone, has enjoyed long-term remission following a period of treatment with 6-Mercaptopurine.
6-Mercaptopurine/therapeutic use* ; Anemia, Hemolytic, Autoimmune/drug therapy* ; Child ; Human ; Male ; Purpura, Thrombocytopenic/drug therapy* ; Syndrome