The first study on nucleotide-level identification of Hb Koriyama in a patient with severe hemolytic anemia

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.     

Hemolytic anemia

Hemolysis presents as acute or chronic anemia, reticulocytosis, or jaundice. The diagnosis is established by reticulocytosis, increased unconjugated bilirubin and lactate dehydrogenase, decreased haptoglobin, and peripheral blood smear findings. Premature destruction of erythrocytes occurs intravascularly or extravascularly. The etiologies of hemolysis often are categorized as acquired or hereditary. Common acquired causes of hemolytic anemia are autoimmunity, microangiopathy, and infection. Immune-mediated hemolysis, caused by antierythrocyte antibodies, can be secondary to malignancies, autoimmune disorders, drugs, and transfusion reactions. Microangiopathic hemolytic anemia occurs when the red cell membrane is damaged in circulation, leading to intravascular hemolysis and the appearance of schistocytes. Infectious agents such as malaria and babesiosis invade red blood cells. Disorders of red blood cell enzymes, membranes, and hemoglobin cause hereditary hemolytic anemias. Glucose-6-phosphate dehydrogenase deficiency leads to hemolysis in the presence of oxidative stress. Hereditary spherocytosis is characterized by spherocytes, a family history, and a negative direct antiglobulin test. Sickle cell anemia and thalassemia are hemoglobinopathies characterized by chronic hemolysis.     

胃癌骨髓转移并血栓性血小板减少性紫癜一例报告

目的提高对血栓性血小板减少性紫癜(thrombotic thrombocytopenic punpura,TTP)的认识,以减少误诊。方法回顾分析1例胃癌骨髓转移并TTP的临床资料。结果患者以发热、贫血原因待查入院,入院后出现呕血,胃镜检查确诊为胃癌。病程中出现进行性意识障碍、血小板减少及溶血性贫血,血涂片可见裂片红细胞,确诊为胃癌骨髓转移并TTP。结论对非血液系统恶性肿瘤患者出现进行性贫血、血小板减少等表现时,应注意合并TTP。     

Pulmonary aspergillosis and central nervous system hemorrhage as complications of autoimmune hemolytic anemia treated with corticosteroids

Warm, active antibody adult autoimmune hemolytic anemia is the most common form of hemolytic anemia not related to drug therapy. Mortality in adult autoimmune hemolytic anemia is related to the inability to successfully treat patients' underlying disease, or the infectious complications of splenectomy and prolonged steroid therapy. Predisposing factors for invasive aspergillosis are neutropenia and steroid therapy. We present a fatal case of aspergillosis complicating a nonneutropenic case of warm active antibody adult autoimmune hemolytic anemia treated with prolonged steroid therapy.     

Hemolytic anemias. Failure of the red cell membrane.

The normal erythrocyte membrane is composed of nearly equivalent amounts of lipid and protein. The lipid portion of the membrane has been well studied. Even though de novo synthesis of lipid does not occur in human red cells, many biochemical pathways exist which facilitate detoxification of lipid breakdown products and lipid renewal. Rare defects in these processes are associated with hemolytic disorders. Recent studies have revealed that the membrane proteins are diverse and suggest that protein dysfunction may also account for clinical disease. Protein and lipid are entwined in a physicochemical relationship which is probably best depicted by the classic lipid bilayer with interspersed proteins in both the inner and outer surfaces and also spanning the bilayer. Membrane failure results in hemolytic anemia. This failure can be intrinsic, caused by abnormal lipid or protein constituents; or extrinsic, with a normal membrane being unable to counteract physical, chemical or immunologic stress. Clinical examples of membrane failure and hemolytic anemia can be separated into three groups according to the predominant mechanism of the hemolysis: fragmentation, whole-cell lysis, and filtration and entrapment. Although these mechanisms can act separately or in concert, the final hemolytic destruction of the cell can usually be traced to a failure of membrane function.     

Doppler超声检测胎儿溶血性贫血

胎儿溶血性贫血在临床上多见,其围生期结局大多不良,以前产前诊断胎儿溶血性贫血的方法属于有创性检测,大大增加了孕妇与胎儿的风险性。目前,采用Doppler超声产前诊断胎儿溶血性贫血取得了不错的进展。本文着重介绍超声在产前检测胎儿溶血性贫血方面的应用。     

Autoimmune hemolytic anemia associated with a hypernephroma

This report describes a case of warm-antibody-mediated hemolytic anemia associated with a hypernephroma. Only four patients with renal cell carcinoma and Coombs'-positive hemolytic anemia have been reported previously. Although rare, the occurrence of autoimmune hemolytic anemia in association with a hypernephroma should be considered one of the possible hematologic complications of this tumor.     

Evans' syndrome: possible benefit from plasma exchange

Because of previous reports of benefit of plasma exchange in immunologic disorders, we plasmapheresed a 45-year-old woman with immune thrombocytopenia and autoimmune hemolytic anemia (Evans' syndrome) who appeared to be dying despite splenectomy, prednisone, immunosuppressives and transfusions. Nine liters of plasma were removed over a 12-day period. Prior to plasma exchange, the patient's hematocrit remained below 16 per cent despite six units of red blood cells over a three-day period. Following plasma exchange, the hematocrit rose to 29 per cent; the platelet count gradually rose from 14,500/microliter to 272,000/microliter; and the transfusion requirements declined to only two units of red blood cells over the next 37 days. We conclude that plasmapheresis should be considered in the management of patients with refractory immune thrombocytopenia and autoimmune hemolytic anemia.     

Diagnostic d’une anémie hémolytique en réanimation

Hemolytic anemia is not an exceptional situation in adults. The abrupt onset of hemolysis and the severity of anemia may sometimes be life-threatening and require admission to the intensive care unit. Establishing the hemolytic mechanism of an anemia is rather easy, but finding its etiology can be quite difficult. The diagnosis of severe hemolytic anemia requires a rapid multiple-step procedure taking into account patient and family history, a careful analysis of the blood smear as well as the result of a direct antiglobulin test. While management is mainly supportive, some causes of hemolytic anemia may require “specific” and emergent therapy, based only on a rapid diagnosis procedure.     

Immune hemolytic anemia associated with teicoplanin

BACKGROUND: Several drugs can cause immune hemolytic anemia. Here a patient who developed hemolytic anemia after treatment with teicoplanin is described. CASE REPORT: Owing to a two-vessel disease, a 68-year-old white man underwent coronary artery bypass grafting. He was readmitted for superficial sternal wound infection and sternal instability. Rewiring was required and worsening anemia characterized the course after the reoperation. Drugs used in the second admission were gentamycin, teicoplanin, paracetamol, and codeine. They were considered as a possible cause of drug-induced hemolytic anemia. RESULTS: The DAT was positive for complement and IgG. Autoanti-e was identified in the patient's undiluted serum sample. The eluate was reactive with all RBCs tested only after adding teicoplanin; when diluted 1:4, anti-e specificity was observed in the presence of teicoplanin. CONCLUSION: To our knowledge, this is the first report of immune hemolytic anemia owing to teicoplanin.     

Variations in the Agglutinability of Red Blood Cells from Patients with Hematologic Disorders

The agglutinability of red blood cells from patients with various hematologic disorders was compared with that of erythrocytes from normal persons by quantitative hemagglutination in the ABH blood group system. Red blood cells from six patients with hypoplastic anemia or metastatic marrow disease as well as normal reticulocytes (five experiments) were less agglutinable than normal mature cells. Erythrocytes from one patient with lymphoma and an acquired hemolytic anemia showed enhanced agglutinability. Our data suggest that changes in erythrocyte reactivity may be more common than suspected, although usually undetected by less sensitive methods.     

Application of therapeutic plasma exchange in hematology

Therapeutic plasma exchange (TPE) was used in 146 patients with hematologic disorders: hyperviscosity syndrome, 74; cryoglobulinemia, 53; porphyria, 9; immune complex disease, 3; cold agglutinin disease, 1; hemolytic uremic syndrome, 1; autoimmune hemolytic anemia, 1; autoimmune thrombocytopenia, 1; autoimmune neutropenia, 1; Clq deficiency, 1; and secondary immunodeficiency, 1. It was shown that TPE applied in patients with hyperviscosity syndrome resulted in rapid reduction of paraprotein concentrations, and normalization or significant decrease of serum viscosity associated with marked clinical improvement (regression of neurologic, renal, hematologic, visual and other disturbances). Application of TPE in patients with cryoglobulinemia resulted in plasma cryoglobulin reduction and clear clinical effects (blood flow improvement, skin ulcer healing, reversal of impaired renal function and disappearance of purpura and other abnormalities). Very good results were obtained in patients with porphyria (decreased sensitivity to sunlight) and also in patients with Clq deficiency. Satisfactory clinical improvement and better laboratory findings were also seen in patients with immune complex disease, autoimmune hemolytic anemia, autoimmune thrombocytopenia and hemolytic uremic syndrome.     

B-Prolymphocytic leukemia: a case study.

The case study reported is of a patient initially misdiagnosed as chronic lymphocytic leukemia. Immunophenotyping studies ultimately identified the nature of the disease as B-cell prolymphocytic leukemia with concomitant warm autoimmune hemolytic anemia. The leukemia and hemolytic anemia were refractory to all conventional treatments administered. The patient survived a significantly shorter period of time than the median time of three years reported in the literature. The patient expired from complications resulting from B-cell PLL, warm autoimmune hemolytic anemia, and combination chemotherapy.     

Overview of the clinical reference guides for the idiopathic hematopoietic disorders

The research committee for idiopathic hematopoietic disorders, which has been supported by the government over the past 35 years, has recently worked out a series of reference guide for the management of diseases under investigation to provide aids for better understanding of pathophysiology of diseases and appropriate clinical decision making. Such attempts covered aplastic anemia, Fanconi anemia, pure red cell aplasia, autoimmune hemolytic anemia, paroxysmal nocturnal hemoglobinuria, myelodysplastic syndromes and primary myelofibrosis, and included diagnostic criteria and severity classification as well as clinical pictures derived from nationwide survey studies. Therapeutic measures were evaluated according to the concepts of evidence-based medicine, when applicable. This report presents an overview of these guides and summarizes key issues in each disease entity.     

Globin chain analysis: An important tool in phenotype study of hemoglobin disorders

Phenotype studies still occupy a key position in the diagnosis of hemoglobin (Hb) disorders. An additional dimension to the methods for diagnosis of Hb disorders which are mostly based on difference in charge of the Hb molecules may be brought by studying some properties of the globin chains. Among the methods proposed, reversed-phase liquid-chromatography (RP-LC) reveals differences in hydrophobicity allowing to discriminate between variants displaying identical charges. Thus, abnormal Hbs responsible for hematological disorders, such as chronic hemolytic anemia, erythrocytosis, or thalassemia like presentation, but with a charge similar to HbA or to that of a common variant may be revealed. Also RP-LC, which discriminates between the two types of γ chains, may be of interest for diagnosis of hereditary persistence of fetal hemoglobin (HPFH) or for suggesting a haplotype in the case of sickle cell anemia.     

Cefotetan disodium-induced hemolytic anemia.

OBJECTIVE: To report a case of cefotetan disodium-induced hemolytic anemia. DATA SOURCES: Original research articles and case reports. DATA SYNTHESIS: A 46-year-old woman developed fulminant hemolytic anemia following a second exposure to intravenous cefotetan disodium for postoperative prophylaxis. She developed respiratory failure requiring intubation and acute renal failure requiring hemodialysis. The hemolysis was successfully treated with plasmapheresis, but the patient died on the 25th postoperative day. Positive Coomb's tests have been reported in less than three percent of patients receiving cefotetan. To our knowledge, this is the first case of fulminant hemolytic anemia associated with intravenous cefotetan disodium therapy. CONCLUSIONS: Cefotetan should be added to the list of drugs known to cause hemolytic anemia. Monitoring for hemolysis should be considered for patients who receive multiple courses of therapy.     

THE INCREASED SUSCEPTIBILITY TO HEMOLYSIS BY INDOL IN DOGS FED DEFICIENT DIETS

1. Indol is more hemolytic in the presence of a deficiency complex than when a normal diet is fed. 2. The hemolytic effect can be abolished by supplementing the deficient diet with liver extract curative of pernicious anemia in man. 3. The hemolysis affects all hemoglobin-containing cells, including reticulocytes. 4. The repair of the anemia resulting from the administration of indol in the presence of a deficiency represents the cessation of a hemolytic process. 5. An abnormally low rate of production of erythrocytes may well be a factor in the production of the anemia.     

Hemolytic anemia complicating infectious mononucleosis due to the interaction of an IgG cold anti-i and an IgM cold rheumatoid factor

This is the case of a 25-year-old man who presented with infectious mononucleosis complicated by hemolytic anemia and hepatitis. Antibody evaluation revealed a cold-reactive IgG anti-i and an IgM cold rheumatoid factor. The patient's hemolytic anemia improved following treatment with corticosteroids.     

Pathophysiology of Common Hemolytic Syndromes

With increased understanding of the inherited disorders of red cell metabolism, the physician can diagnose hemolytic anemia in a logical fashion. The pathophysiologic aspects of the major categories of hemolysis are discussed here, with attention focused on pyruvate kinase deficiency and glucose-6-phosphate dehydrogenase deficiency. The former is uncommon but highly instructive. The latter is extremely common and equally instructive, and must be carefully searched for in patients who may be the helpless victims of enthusiastic therapy.     

异基因造血干细胞移植后溶血性贫血

目的 探讨异基因造血十细胞移植后溶血性贫血的发生情况.方法 分析溶血性贫血的发生率、发生时间、临床表现、病因以及治疗和预后.结果 121例异某因造血干细胞移植患者有2例出现溶血性贫血,占1.6%,死亡1例.原因有AIHA(1/2),TTP(1/2).供受者血型为A→O.结论 溶血性贫血是异基因造血干细胞移植后严重并发症,死亡率高.需尽快考虑到以上原因予以相应的治疗.