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

PURPOSE: Anti-D immunoglobulin has recently emerged as a theraputic option for the treatment of patients with idiopathic thrombocytopenic purpura (ITP). There is no report about anti-D treatment in our country. In this report, the efficacy and adverse reactions of anti-D immunoglobulin in children with ITP were evaluated. METHPDS: From August, 1997 to September, 1998, 7 courses of anti-D treatment were applied in 4 children who had persisting thrombocytopenia and frequent bleeding episodes despite use of intravenous immunoglobulin and corticosteroid. They were Rh-positive and non-splenectomized patients. They received 43~60 mug/kg/dose of anti-D (WinRho ) twice with 7 days interval at out patient department. To evaluate the efficacy and adverse reactions of anti-D, platelet, reticulocyte, hemoglobin, bilirubin and haptoglobin counts were observed weekly. RESULTS: Median age and pretreatment duration after diagnosis were 22 months (15~77 months) and 7 months (2~46 months), respectively. Bleeding decreased significantly after anti-D treatment. Platelet count increased median 4.11 folds (1.85~13.67 folds) and response was maximal at day 7. Duration of platelet increase more than 2 folds was 5 weeks (0~10 weeks). No significant adverse reactions other than mild hemolytic anemia was present. Hemoglobin decreased to minimal 88% (79.5~95.9%) of pretreatment value. Duration of hemoglobin lower than 90% of pretreatment value was 1 week (0~4 weeks). After 10 weeks, platelet and hemoglobin returned to pretreatment value. CONCLUSION: Although anti-D is not a curative treatment for ITP, it is safe and repeated infusions of anti-D can be used to maintain the platelet count at a level of sufficient to provide adequate hemostasis and may enable children to postpone or even avoid splenectomy.
Anemia, Hemolytic ; Bilirubin ; Blood Platelets ; Child ; Diagnosis ; Haptoglobins ; Hemorrhage ; Hemostasis ; Humans ; Immunoglobulins ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic* ; Reticulocytes ; Splenectomy ; Thrombocytopenia

Autoimmune hemolytic anemia associated with an ovarian teratoma is a very rare disease. However, treating teratoma is the only method to cure the hemolytic anemia, so it is necessary to include ovarian teratoma in the differential diagnosis of autoimmune hemolytic anemia. We report herein on a case of a young adult patient who had severe autoimmune hemolytic anemia that was induced by an ovarian teratoma. A 25-yr-old woman complained of general weakness and dizziness for 1 week. The hemoglobin level was 4.2 g/dL, and the direct and indirect antiglobulin tests were all positive. The abdominal computed tomography scan revealed a huge left ovarian mass, and this indicated a teratoma. She was refractory to corticosteroid therapy; however, after surgical resection of the ovarian mass, the hemoglobin level and the reticulocyte count were gradually normalized. The mass was well encapsulated and contained hair and teeth. She was diagnosed as having autoimmune hemolytic anemia associated with an ovarian teratoma. To the best of our knowledge, this is the first such a case to be reported in Korea.
Teratoma/*complications/diagnosis/surgery ; Ovarian Neoplasms/*complications/diagnosis/surgery ; Humans ; Female ; Diagnosis, Differential ; Blood Transfusion ; Anemia, Hemolytic, Autoimmune/diagnosis/*etiology/therapy ; Adult ; Adrenal Cortex Hormones/therapeutic use

A patient with warm autoimmune hemolytic anemia (AIHA) due to predominance of immunoglobulin A (IgA) with an Rh specificity, considered to be the first case in Korea, is described. A 13-yr-old male patient with severe hemolytic anemia showed a weak reactivity (1+) in the direct antiglobulin test (DAT) by using anti-IgG antiglobulin reagent. This finding, however, could not fully explain the patient's severe AIHA. When anti-IgA reagent was used for the DAT, strong reactivity (4+) was observed and free anti-E and anti-c autoantibodies were also detected by anti-IgA and anti-IgG reagents. The patient's hemoglobin began to rise with the administration of steroids. Because RBCs coated with multiple types of immunoglobulins are associated with more severe hemolysis than those only with IgG, the DATs using anti-IgA and other reagents are needed for the correct diagnosis when the result of DAT is not compatible with patient's clinical manifestations.
Adolescent ; Anemia, Hemolytic, Autoimmune/diagnosis/*immunology ; Antibody Specificity ; Autoantibodies/*blood ; Coombs' Test ; Humans ; Immunoglobulin A/*blood ; Korea ; Male ; Rh-Hr Blood-Group System/immunology

Although a pathogenic mechanism of hemolytic anemia complicated with viral hepatitis is unknown, it is suggested that there are four mechanisms; 1) In the individual who has predisposition to hemolytic anemia, viral infection accelerates the red cell destruction & hemolysis become obvious. 2) Directly, virus itself injures to the red cell membrane. 3) The serious liver failure & hypersplenism induce the hemolysis. 4) Autoimmune hemolytic anemia because of immunological abnormality caused by viral infection. We experienced a case of autoimmune hemolytic anemia in 33-year-old male patient who was diagnosed as chronic lobular hepatitis B with biopsy. Diagnosis was estabilished by clinical features, blood cell count, routine urinalysis, direct & indirect Coombs test, liver function test, immunoglobulin quantitations, hepatitis B marker, bone marrow aspiration, and liver biopsy. This case was treated with corticosteroid and transfusion. During follow-up, he has been well tolerated.
Adult ; Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune* ; Biopsy ; Blood Cell Count ; Bone Marrow ; Cell Membrane ; Coombs Test ; Diagnosis ; Follow-Up Studies ; Hemolysis ; Hepatitis B* ; Hepatitis* ; Humans ; Hypersplenism ; Immunoglobulins ; Liver ; Liver Failure ; Liver Function Tests ; Male ; Urinalysis

Anemia is defined as an insufficient amount of RBC mass to adequately deliver oxygen to peripheral tissues. For practical purposes, however, the measurements of three parameters that can be obtained from the complete blood count (CBC) are enough to establish the presence of anemia; hemoglobin (Hb) concentration, hematocrit, and RBC number. Among these, the Hb level is the most convenient parameter to establish the diagnosis of anemia. Anemia is not a disease by itself but mostly a consequence of the underlying acquired or genetic abnormality. Although the clues to the cause of anemia may be found from the history and physical examination, three parameters from CBC provide most critical information for the differential diagnosis of anemia; mean corpuscular volume (MCV), red cell distribution width (RDW), and the reticulocyte count. MCV provides information on the size of the red cell. Values greater than 100 fL usually signify a nuclear maturation defect resulting in macrocytic anemias, while values less than 80 fL are diagnostic of hemoglobin synthesis defect causing microcytic anemias. Meticulous evaluation of the serum iron status and body iron storage is essential to the differential diagnosis of microcytic anemias. RDW is a measure of the red cell size variation. It is increased by the appearance of microcytic or macrocytic cells, or both. The reticulocyte count is a useful laboratory measurement of effective red cell production. Hemolytic anemia or acute bleeding can increase the reticulocyte count. There are four clinically useful laboratory measurements indicating the presence of hemolytic process; the reticulocyte count, the serum bilirubin, the serum lactate dehydrogenase (LDH), and the serum haptoglobin concentration. Once the presence of hemolytic anemia is established, laboratory assessment to differentiate between intravascular and extravascular hemolysis is important because clinical conditions producing intravascular hemolysis may be anticipated in certain clinical situations, which may be complicated by acute renal failure or disseminated intravascular coagulation that needs immediate interventions. If the definitive cause of anemia cannot be established by examining the peripheral blood, a bone marrow study may be helpful.
Acute Kidney Injury ; Anemia* ; Anemia, Hemolytic ; Anemia, Macrocytic ; Bilirubin ; Blood Cell Count ; Bone Marrow ; Cell Size ; Diagnosis ; Diagnosis, Differential ; Disseminated Intravascular Coagulation ; Erythrocyte Indices ; Haptoglobins ; Hematocrit ; Hemolysis ; Hemorrhage ; Humans ; Iron ; L-Lactate Dehydrogenase ; Oxygen ; Physical Examination ; Reticulocyte Count

Hereditary hemolytic anemia comprises a group of disorders in which red blood cells are destroyed faster than they are produced in the bone marrow; various hereditary factors can cause this condition, including production of defective Hb and erythrocyte membrane. Recently, we identified Hb Koriyama, a rare Hb variant that was undetectable in Hb electrophoresis and stability tests, in a patient with severe hemolytic anemia. This is the first study to show the nucleotide-level sequence variations in Hb Koriyama. On the basis of our results, we conclude that unstable Hb may not be detectable by conventional Hb electrophoresis or stability tests. Thus, we suggest further genetic workup in cases of unexplained hereditary hemolytic anemia.
Amino Acid Sequence ; Anemia, Hemolytic/blood/*diagnosis ; Child ; Female ; Gene Duplication ; Hemoglobins, Abnormal/*genetics ; Heterozygote ; Humans ; Molecular Sequence Data ; Mutation ; Sequence Analysis, DNA

BACKGROUND: Immune hemolysis secondary to ABO minor incompatibility is a rare graft versus host disease in renal recipients, secondary to anti-ABO antibody produced by lymphocytes of donor origin that reacts against recipient RBCs. METHODS: To investigate the incidence and clinical features of immune hemolysis secondary to ABO minor incompatibility in renal allograft recipients, clinical records of 358 renal transplantation performed in Maryknoll Hospital since 1991 were analyzed retrospectively. RESULTS: Fifty four (15%) of 358 renal transplants were ABO minor incompatible. Immune hemolysis secondary to anti-ABO antibody developed in 5 (9.2%) of 54 ABO minor incompatible renal transplant recipients. Immune hemolysis occurred in 3 (13.6%) patients among 22 allografts from blood type O donor to A recipients and 2 (10%) patients among 20 from blood type O donor to B recipients. All 5 patients received cyclosporin with prednisolone, and MMF was administered to one patient additionally. Immune hemolysis developed on 14+/-3 days after renal transplantation and lasted for about 10+/-3 days. The maximum reduction of hemoglobin was 3.3+/-1.0 g/dL. All patients required donor type (blood type O) washed RBCs transfusion (5.0+/-2.6 units per patient) and plasmapheresis were performed in 3 patients (4.0+/-1.0 per patient). All patients recovered without deterioration of graft function. Age, number of HLA mismatch, creatinine at 1 year after transplantation, frequency of acute rejection and serum cyclosporin level during first 2 weeks were not significantly different between hemolysis group (N=5) and non-hemolysis group (N=49). Living unrelated transplantation is associated with increased incidence of immune hemolysis compared with living related transplantation (p<0.01). CONCLUSION: Although immune hemolysis secondary to ABO minor incompatibility is uncommon, we experienced cases with marked reduction of hemoglobin that required a large amount of transfusion. Therefore, this type of immune hemolysis needs to be considered as a differential diagnosis of posttransplant hemolysis. As our center routinely performs donor specific transfusion (DST), the incidence may be higher than that of other centers where DST is not usually given.
Allografts ; Anemia, Hemolytic* ; Blood Group Incompatibility ; Creatinine ; Cyclosporine ; Diagnosis, Differential ; Graft vs Host Disease ; Hemolysis ; Humans ; Incidence ; Kidney Transplantation ; Lymphocytes ; Plasmapheresis ; Prednisolone ; Retrospective Studies ; Tissue Donors ; Transplantation ; Transplants

OBJECTIVETo evaluate the clinic value of micro-column gel cards direct Coombs test (gel test) in diagnosis of autoimmune hemolytic anemia (AIHA).

METHODSSpecimens of 128 suspected AIHA patients were performed direct Coombs test by conventional tube or gel cards. The results of the two tests were compared. The hemoglobin concentrations, proportions of reticulocyte, serum levels of bilirubin and free hemoglobin were detected simultaneously and compared in subgroups.

RESULTSThe positive detection rate of direct Coombs test performed by gel test or tube were 88.4% and 37.7%, respectively.

CONCLUSIONCompared with the tube tests, gel test assay is more sensitive, easy to perform and standardized in diagnosis of AIHA, and the gel card can be stored for a long time. The gel test is valuable for the diagnosis of AIHA.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Anemia, Hemolytic, Autoimmune ; blood ; diagnosis ; Child ; Child, Preschool ; Coombs Test ; Female ; Humans ; Infant ; Male ; Middle Aged ; Young Adult

This study was aimed to analyze the serological characteristics, efficacy and safety of incompatible RBC transfusion in patients with autoimmune hemolytic anemia (AIHA). The patients with idiopathic or secondary AIHA were analyzed retrospectively, then the serological characteristics and the incidence of adverse transfusion reactions were investigated, and the efficacy and safety of incompatible RBC transfusion were evaluated according to the different autoantibody type and infused different RBC components. The results showed that out of 61 cases of AIHA, 21 cases were idiopathic, and 40 cases were secondary. 8 cases (13.1%) had IgM cold autoantibody, 50 cases (82.0%) had IgG warm autoantibody, and 3 cases (4.9%) had IgM and IgG autoantibodies simultaneously. There were 18 cases (29.5%) combined with alloantibodies. After the exclusion of alloantibodies interference, 113 incompatible RBC transfusions were performed for 36 patients with AIHA, total efficiency rate, total partial efficiency rate and total inefficiency rate were 56.6%, 15.1% and 28.3%, respectively. Incompatible RBC transfusions were divided into non-washed RBC group and washed RBC group. The efficiency rate, partial efficiency rate and inefficiency rate in non-washed RBC group were 57.6%, 13.0% and 29.4%, respectively. The efficiency rate, partial efficiency rate and inefficiency rate in washed RBC group were 53.6%, 21.4% and 25.0%, respectively. There was no significant difference of transfusion efficacy (P > 0.05) in two groups. Incompatible RBC transfusions were also divided into IgM cold autoantibody group and IgG warm autoantibody group. The efficiency rate, partial efficiency rate and inefficiency rate in IgM cold autoantibody group were 46.2%, 30.8% and 29.4%, respectively. The efficiency rate, partial efficiency rate and inefficiency rate in IgG warm autoantibody group were 56.7%, 13.4% and 29.9%, respectively. There was no significant difference of transfusion efficacy (P > 0.05 ) in two groups. Hemolytic transfusion reaction was not observed in all incompatible RBC transfusions. It is concluded that the same ABO type of non-washed RBC transfusion and O type washed RBC transfusion are all relatively safe for the AIHA patients with severe anemia after the exclusion of alloantibodies interference. There is no significant difference of transfusion efficacy in two groups. The same ABO type of non-washed RBC transfusion is more convenient and efficient than washed RBC transfusion, and excessive use of type O RBCs can also be avoided.
Adult ; Aged ; Aged, 80 and over ; Anemia, Hemolytic, Autoimmune ; diagnosis ; immunology ; therapy ; Blood Grouping and Crossmatching ; Erythrocyte Transfusion ; Female ; Humans ; Isoantibodies ; Male ; Middle Aged ; Treatment Outcome ; Young Adult