A 72-year-old man with general weakness visited the outpatient clinic of the hematology department. The patient had been treated under the diagnosis of autoimmune hemolytic anemia for 2 years. His hemoglobin level at the time of the visit was 6.3 g/dL, and a blood transfusion was requested to treat his anemia. The patient's blood type was A, RhD positive. Antibody screening and identification test showed agglutination in all reagent cells with a positive reaction to autologous red blood cells (RBCs). He had a prior transfusion history with three least incompatible RBCs. The patient returned home after receiving one unit of leukoreduced filtered RBC, which was the least incompatible blood in the crossmatching test. After approximately five hours, however, fever, chills, dyspnea, abdominal pain, and hematuria appeared and the patient returned to the emergency room next day after the transfusion. The anti-Fy(a) antibody, which was masked by the autoantibody, was identified after autoadsorption using polyethylene glycol. He was diagnosed with an acute hemolytic transfusion reaction due to anti-Fy(a) that had not been detected before the transfusion. In this setting, it is necessary to consider the identification of coexisting alloantibodies in patients with autoantibodies and to become more familiar with the method of autoantibody adsorption.
Abdominal Pain ; Adsorption ; Aged ; Agglutination ; Ambulatory Care Facilities ; Anemia ; Anemia, Hemolytic, Autoimmune* ; Autoantibodies ; Blood Transfusion ; Chills ; Diagnosis ; Dyspnea ; Emergency Service, Hospital ; Erythrocytes ; Fever ; Hematology ; Hematuria ; Humans ; Isoantibodies ; Masks ; Mass Screening ; Methods ; Polyethylene Glycols ; Transfusion Reaction*

While imported falciparum malaria has been increasingly reported in recent years in Korea, clinicians have difficulties in making a clinical diagnosis as well as in having accessibility to effective anti-malarial agents. Here we describe an unusual case of imported falciparum malaria with severe hemolytic anemia lasting over 2 weeks, clinically mimicking a coinfection with babesiosis. A 48-year old Korean man was diagnosed with severe falciparum malaria in France after traveling to the Republic of Benin, West Africa. He received a 1-day course of intravenous artesunate and a 7-day course of Malarone (atovaquone/proguanil) with supportive hemodialysis. Coming back to Korea 5 days after discharge, he was readmitted due to recurrent fever, and further treated with Malarone for 3 days. Both the peripheral blood smears and PCR test were positive for Plasmodium falciparum. However, he had prolonged severe hemolytic anemia (Hb 5.6 g/dl). Therefore, 10 days after the hospitalization, Babesia was considered to be potentially coinfected. A 7-day course of Malarone and azithromycin was empirically started. He became afebrile within 3 days of this babesiosis treatment, and hemolytic anemia profiles began to improve at the completion of the treatment. He has remained stable since his discharge. Unexpectedly, the PCR assays failed to detect DNA of Babesia spp. from blood. In addition, during the retrospective review of the case, the artesunate-induced delayed hemolytic anemia was considered as an alternative cause of the unexplained hemolytic anemia.
Anemia, Hemolytic/chemically induced/*etiology/*pathology ; Anti-Bacterial Agents/therapeutic use ; Antimalarials/therapeutic use ; Artemisinins/adverse effects/therapeutic use ; Atovaquone/therapeutic use ; Azithromycin/therapeutic use ; Babesiosis/complications/*diagnosis/drug therapy/*pathology ; Benin ; Blood/parasitology ; Coinfection/diagnosis/pathology ; Drug Combinations ; France ; Humans ; Korea ; Malaria, Falciparum/complications/*diagnosis/drug therapy/*pathology ; Male ; Middle Aged ; Plasmodium falciparum/*isolation & purification ; Proguanil/therapeutic use ; Travel ; Treatment Outcome

A 26-yr-old male patient reported worsened dyspnea, dizziness one year after an emergency Bentall operation for type A aortic dissection. There was evidence of hemolytic anemia and aortogram revealed a significant stenosis at the distal anastomosis site. During the reoperation, we found the inner felt at the distal anastomosis was inverted causing a significant stenosis. The reoperation successfully resolved this problem. Here, we report a rare case of hemolytic anemia caused by an inverted inner felt after Bentall operation.
Acute Disease ; Adult ; Anastomosis, Surgical ; Anemia, Hemolytic/*diagnosis/*etiology/surgery ; Aneurysm, Dissecting/complications/*surgery ; Aortic Aneurysm/complications/*surgery ; *Blood Vessel Prosthesis ; Blood Vessel Prosthesis Implantation/*adverse effects/instrumentation ; Dizziness/etiology ; Dyspnea/etiology ; Echocardiography ; Humans ; Male ; *Postoperative Complications/surgery ; Reoperation ; Time Factors ; Tomography, X-Ray Computed ; Treatment Outcome

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

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

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

A 37-year-old male presented with fever and jaundice was diagnosed as hepatitis A complicated with progressive cholestasis and severe autoimmune hemolytic anemia. He was treated with high-dose prednisolone (1.5 mg/kg), and eventually recovered. His initial serum contained genotype IA hepatitis A virus (HAV), which was subsequently replaced by genotype IIIA HAV. Moreover, at the time of development of hemolytic anemia, he became positive for immunoglobulin M (IgM) anti-hepatitis E virus (HEV). We detected HAV antigens in the liver biopsy specimen, while we detected neither HEV antigen in the liver nor HEV RNA in his serum. This is the first report of hepatitis A coinfected with two different genotypes manifesting with autoimmune hemolytic anemia, prolonged cholestasis, and false-positive IgM anti-HEV.
Adult ; Anemia, Hemolytic, Autoimmune/*diagnosis/drug therapy/etiology ; Anti-Inflammatory Agents/therapeutic use ; Cholestasis/*diagnosis/drug therapy/pathology ; Coinfection/*diagnosis ; Genotype ; Hepatitis A/complications/*diagnosis/genetics ; Hepatitis E/complications/*diagnosis/genetics ; Humans ; Immunoglobulin M/blood ; Liver/pathology/virology ; Male ; Prednisolone/therapeutic use ; RNA, Viral/blood

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

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

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