No abstract available.
Anemia, Hemolytic, Congenital* ; Diagnosis*

No abstract available.
Anemia, Hemolytic, Autoimmune* ; Diagnosis*

Autoimmune hemolytic anemia (AIHA) can be considered in the differential diagnosis of hemolytic anemia with a concomitant cerebral infarction. We report a 79-year-old woman who was stuporous at presentation, and ultimately diagnosed with AIHA and cerebral infarction. Microembolic signals (MES) were detected by transcranial Doppler monitoring on the first hospitalization day. MES disappeared on the sixth hospitalization day following treatment with steroid and anticoagulation. This case represents a rare arterial ischemic complication of AIHA possibly associated with a hypercoagulable state.
Aged ; Anemia, Hemolytic ; Anemia, Hemolytic, Autoimmune* ; Cerebral Infarction ; Diagnosis, Differential ; Female ; Hospitalization ; Humans ; Stroke* ; Stupor ; Thrombophilia

To understand the hemolytic anemia (HA) in children, the diagnostic approach and management of hereditary and acquired HA are described. The hereditary hemolytic anemia (HHA) can be classified according to the pathogenesis into three types:RBC membrane defects, hemoglobinopathies, and RBC enzymopathies. Clinical characteristics, laboratory findings and molecular defects of these three types are presented briefly. In Korea, HHA due to the RBC membrane defect, hereditary spherocytosis had been reported often but HHA due to hemoglobinopathies and RBC enzymopathies had been thought to be relatively rare. With recent development in the molecular diagnosis, beta thalassemia, mostly heterozygote, G6PD and pyruvate kinase deficiency have been reported with gene characterization. If the patients with microcytic hypochromic anemia show unproportionally low MCV or MCH or refractory to the iron therapy, hemoglobin electrophoresis and gene analysis for thalassemia or other unstable hemoglobinopathies need to be done accordingly. The global movement of the population especially from the region prevalent of hemoglobinopathies or enzymopathies to Korea warrants considering broad spectrum of etiology for the diagnosis of HHA. Aquired HA resulting from extracellular factors such as autoimmune HA from warm antibody, cold agglutinin and paroxysmal cold hemoglobinuria as well as nonimmune HA are described briefly.
Anemia, Hemolytic* ; Anemia, Hemolytic, Autoimmune ; Anemia, Hemolytic, Congenital ; Anemia, Hypochromic ; beta-Thalassemia ; Child ; Diagnosis ; Electrophoresis ; Hemoglobinopathies ; Hemoglobinuria, Paroxysmal ; Heterozygote ; Humans ; Iron ; Korea ; Membranes ; Pediatrics* ; Pyruvate Kinase ; Thalassemia

Drug-induced immune hemolytic anemia (DIIHA) is rare condition that is often very difficult to diagnose. For proper diagnosis of DIIHA, careful interpretation of laboratory findings as well as correlation between those findings with the patient's history is important. Therefore, the role of the laboratory physician is critical. DIIHA can be diagnosed using a stepwise approach, from suspicion of hemolytic anemia in the patient to confirmation of serologic tests. Prompt diagnosis is necessary since an essential part of DIIHA treatment is to cease drug administration, and many cases of hemolysis can be improved without further intervention. Furthermore, distinction between the mechanisms of DIIHA is important, as clinical manifestation, treatment options, and prognosis of the disease can differ according to the main mechanism involved in the process of hemolysis.
Anemia, Hemolytic* ; Diagnosis ; Hemolysis ; Humans ; Prognosis ; Serologic Tests

Sjögren's syndrome(SS)is a chronic autoimmune disease that affects exocrine glands, especially salivary and lacrimal glands. The main clinical manifestations are dry mouth and dry eyes, but also multi-organ and multi-system can be involved. Cold agglutinin disease(CAD)is an autoimmune disease characterized by red blood cell agglutination in the blood vessels of extremities caused by cold agglutinin at low temperature, resulting in skin microcirculation disturbance, or hemolytic anemia. Cold agglutinin disease is divided into two categories, primary cold agglutinin disease and secondary cold agglutinin disease. Primary cold agglutinin disease is characterized with cold agglutinin titer of 1 ∶4 000 or more and positive Coomb's test. However, the Coomb's test is not necessarily positive and the cold agglutinin titer is between 1 ∶32 and 1 ∶4 000 in secondary cold agglutinin disease. Here, we reported an elderly patient admitted to hospital due to fever. He was diagnosed with respiratory infection, but he showed incompletely response to the anti-infection treatment. Further laboratory tests showed the patient with positive ANA and anti-SSA antibodies. Additionally, the patient complained that he had dry mouth and dry eyes for 1 year. Schirmer test and salivate gland imaging finally confirmed the diagnosis Sjogren's syndrome. During the hospital stay, the blood clots were found in the anticoagulant tubes. Hemolytic anemia was considered as the patient had anemia with elevated reticulocytes and indirect bilirubin. In addition, further examination showed positive cold agglutination test with a titer of 1 ∶1 024, and cold agglutinin disease was an important type of cold-resistant autoimmune hemolytic anemia. Furthermore, the patient developed cyanosis after ice incubating at the tip of the nose. Hence, the patient was diagnosed as CAD and he was successfully treated with glucocorticoids instead of anti-infection treatments. Hence, the patient was diagnosed with SS combined with secondary CAD. SS combined CAD are rarely reported, and they are both autoimmune diseases. The abnormal function of B lymphocytes and the production of autoantibodies might be the common pathogenesis of them. Cold agglutinin disease can lead to severe hemolytic anemia, even life-threatening. In clinical practice, timely recognizing and dealing with CAD might promote the prognosis of the patient.
Male ; Humans ; Aged ; Anemia, Hemolytic, Autoimmune/diagnosis* ; Sjogren's Syndrome/diagnosis* ; Anemia, Hemolytic/complications* ; Dry Eye Syndromes/complications* ; Autoantibodies

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

Cytomegalovirus is a common virus that is mostly asymptomatic when infected, but rarely causes life-threatening hemolysis especially in immunocompromised children. We report a case of antiglobulin test negative severe hemolytic anemia caused by cytomegalovirus infection developed in an immune competent 9-year-old girl. The patient's hemoglobin level was 4.8 g/dL on the day of admission. The diagnosis was achieved by exclusion of other causes of hemolytic anemia and serological evidence of recent CMV infection. The patient was successfully treated with anti-viral agents and steroids resulting in recovery from anemia. Clinicians should consider cytomegalovirus infection in the differential diagnosis of hemolytic anemia in pediatric patients.
Anemia ; Anemia, Hemolytic* ; Child ; Coombs Test* ; Cytomegalovirus ; Cytomegalovirus Infections ; Diagnosis ; Diagnosis, Differential ; Female ; Hemolysis ; Humans ; Steroids

The author obtained index of red cell volume distribution width(RDW) and other red cell indices in 210 patients of various hematoncologic conditions and 200 healthy control group using, an automated blood analyzer, Coulter Counter Model S-plus II. This study performed to classify various etiologic anemia based on the MCV and RDW, to evaluate availability to the differential diagnosis in korean anemic distoders somewhat different from etiologies of anemias in foreginers. In the most of cases, the increase or decrease of MCV were always combined the pararell changes of MCH and MCHC: But the values of MCV and RDW were not correlated in control group and patient group. So the terms of heterogenous of homogenous anemia were meaningful morphologic classification than hypochromic or normochromic anemia. The heterogenous microcytic anemia contained iron deficiency anemia. In heterogenous normocytic anemia, myelophthisic anemia, acute leukemia were contained. In heterogenous macrocytic anemia, megaloblastic anemia, hemolytic anemia were contained. The homogenous microcytic anemia was observed in anemia of chronic disorders. In homogenous normocytic anemia, acute blood loss, chronic leukemia, multiple myeloma were contained. The aplastic anemia was belonged to homogenous macrocytic anemia. The diagnostic significance of RDW in hemoglobinopathies is most importhant. But this study was not contained hemoglobinopathies. Instead RDW was very helpful to differential diagnosis of most common anemias, iron deficiency anemia and anemia due to chronic disorders in Korea.
Anemia* ; Anemia, Aplastic ; Anemia, Hemolytic ; Anemia, Iron-Deficiency ; Anemia, Macrocytic ; Anemia, Megaloblastic ; Anemia, Myelophthisic ; Cell Size ; Classification* ; Diagnosis, Differential ; Erythrocyte Indices ; Hemoglobinopathies ; Humans ; Korea ; Leukemia ; Multiple Myeloma

BACKGROUND: The direct antiglobulin test(DAT) is a method detecting red cell-coated antibodies, much of which are related to immune hemolytic anemia. The microcolumn method-direct antiglobulin test(MC-DAT) is known to be more sensitive and convenient than conventional tube method-direct antiglobulin test(T-DAT). We compared the results of both DAT methods and evaluated the relationship between positive DAT result and immune hemolytic anemia. METHODS: Ninety-three subjects were classified into three groups according to clinical diagnosis, hemoglobin level, and serum IgG level; 15 healthy controls(group I), 8 patients without anemia and with total IgG greater than 1800 g/dL(group II), and 69 anemic patients with hemoglobin less than 10 g/dL(group III). DAT was performed on the EDTA-anticoagulated samples of these patients using both microcolumn method and tube method. Additional tests for hemolytic anemia were performed when either the result of MC-DAT or T-DAT was positive, and diagnosis of hemolysis was divided into three categories: hemolysis(category A), undetermined(category B), and no hemolysis(category C). RESULTS: Of total 93 samples, 18 were positive and 48 were negative with both DAT methods. Twenty-seven samples showed positive results by MC-DAT and negative by T-DAT, while none showed to be negative results by MC-DAT and positive by T-DAT. The agglutination strength of MC-DAT was stronger than that of T-DAT. Five samples which could be categorized to category A showed positive results by MC-DAT and negative by T-DAT. Two samples showing positive results with both methods but which were categorized to category C could be found in group II. CONCLUSIONS: The microcolumn method(MC-DAT) seems to be more sensitive than the tube method. However, diagnosis of immune hemolytic anemia based on the DAT result needs much caution because both methods can be influenced by high serum IgG concentration and these can show false positive results.
Agglutination ; Anemia ; Anemia, Hemolytic ; Antibodies ; Coombs Test* ; Diagnosis ; Hemolysis ; Humans ; Immunoglobulin G