Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thrombocytopenia (< 150,000/microliter) is a common finding, occurring in 7-8% of pregnancies. Some conditions, such as gestational thrombocytopenia pose no maternal or fetal risks. Idiopathic thrombocytopenic purpura (ITP) is an acquired haematologic disorder, common among children and adults, with unknown etiology and autoimmune pathogenesis. The incidence of severe fetal and neonatal thrombocytopenia is very rare, and neonatal intracranial hemorrhage is unlikely to be related to the mode of delivery. Alloimmune thrombocytopenia occurs with an incidence of 1/1,000 livebirths and is induced by a maternal alloimmunization against fetal platelet antigens. The incidence of intracranial haemorrhage in the fetus and neonate is the highest for any immune thrombocytopenia. The HELLP syndrome is a severe, unpredictable and life-threatening complication of preeclampsia, characterized by a triad of hemolysis, elevated liver enzymes and low platelet counts. HELLP syndrome develops in the third trimester but can occur postpartum. Hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are syndromes of microangiopathic hemolytic anemia, and thrombocytopenia. During pregnancy, TTP usually presents in the second trimester, whereas HUS develops in the postpartum period. Heparin-induced thrombocytopenia type II is a serious, immune-mediated complication of heparin therapy.
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PMID:[Differential diagnosis of thrombocytopenia in pregnancy]. 1126 38

Common variable immunodeficiency (CVI) is a primary immunodeficiency characterized by deficient antibody production. The cause of this immunodeficiency is unknown; several in vitro studies have revealed a significant number of alterations that could explain the hypogammaglobulinemia present in this syndrome. Among those described are primary B cell alterations, numerical and functional T cell abnormalities, and defects in the interaction between accessory cells. The alteration typical of CVI is the failure of B lymphocytes to differentiate from antibody-producing cells, resulting in deficient immunoglobulin secretion. Among the T cell abnormalities described are a diminished proliferative response to mitogens and antigens, alterations in the level of production of several cytokines, especially reduction in the production of IL-2, diminished antigen-specific T cells and increase basal apoptosis after stimulation. Antigen presenting cells, monocytes and dendritic cells can also present alterations and contribute to deficient antigen response. The clinical manifestations of these patients is variable; most present recurrent bacterial infections due to encapsulated bacteria, especially sinusitis, otitis, bronchitis, and pneumonias. A few patients can present mycobacterial or fungal infection and occasionally Pneumocystis carinii. Viral infection is uncommon in these patients although some suffer recurrent herpes zoster infection. Clinical features of septicemia and central nervous system infections are less frequent. The incidence of digestive tract infections in these patients is high. The most common cause of diarrhea is Giardia lamblia; Salmonella, Shigella and Campylobacter are also common pathogens. Autoimmune disease is also more prevalent in these patients than in the general population. The most frequently associated diseases are hemolytic anemia, idiopathic thrombocytopenic purpura and autoimmune neutropenia. Cancer is also frequently associated with CVI, the most common forms being lymphoproliferative syndromes, especially non-Hodgkin's lymphoma. Granulomas are a unusual manifestation in some patients with CVI; their localization varies but the most commonly affected organs are the spleen and lungs. Some authors have compared these granulomas with those characterizing sarcoidosis, especially when appearing in the lung. Diagnosis of CVI is usually by exclusion of other diseases, such as cystic fibrosis, immotile cilia syndrome or allergic processes. CVI should be suspected in all patients with recurrent bacterial infections especially those localized in the respiratory tract. Other primary immunodeficiencies which present clinical findings similar to CVI and which should be ruled out are selective IgG subclass deficiency, IgA deficiency and selective deficiency in the response to polysaccharide antigens with normal immunoglobulin levels. The serum hypogammaglobulinemia present in all patients with CVI provides the diagnostic key. The age at which clinical manifestations appear, the absence of familial antecedents and the presence of circulating B lymphocytes form the basis of the differential diagnosis between X-linked agammaglobulinemia and autosomal recessive forms. The treatment of choice of patients with CVI is treatment with human gamma-globulin. Currently, the most common route of administration is intravenous; these molecules have a half-life of approximately 21 days and a high degree of safety concerning the possible transmission of viral infections. Adverse reactions are generally few and clinically unimportant. The most frequently used doses oscillate between 200 and 400 mg/kg body weight every 2-4 weeks. Both the dose and its frequency should be personalized for each patient. Early diagnosis of patients with CVI, application of treatment with appropriate antibiotics for infections and treatment with gamma-globulins prevent long-term complications of this disease and dramatically improve the quality of life and life expectancy of these patients.
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PMID:[Common variable immunodeficiency. Review]. 1143 84

RhoD immune globulin intravenous (anti-D IGIV) increases platelet counts in patients who have not undergone splenectomy and are positive for RhoD with idiopathic thrombocytopenic purpura. After treatment, in most patients, anemia develops as a result of immune-mediated red cell destruction in the spleen. Although intravascular hemolysis (IVH) is not expected, life-threatening IVH has been recently reported by the Food and Drug Administration, and physicians are encouraged to report their experience with patients with idiopathic thrombocytopenic purpura in whom IVH develops after anti-D administration. Severe IVH was observed after treatment with anti-D IGIV in two adolescent girls with acute thrombocytopenia related to Epstein-Barr virus. They did not have hemolytic anemia before treatment. The authors believe that anti-D IGIV triggered an unusual virus-induced immune response causing hemolysis; therefore, anti-D IGIV should not be used in patients with Epstein-Barr virus-related thrombocytopenia, particularly during the acute phase of infection.
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PMID:Intravenous anti-D immune globulin-induced intravascular hemolysis in Epstein-Barr virus-related thrombocytopenia. 1187 83

In order to investigate the clinical characteristics of hematological abnormality in patients with systemic lupus erythematosus (SLE) and inquire into the basis for differential diagnosis, the hematological data of 92 cases with lupus erythematosus-related hematological disorder (SLERHD) were retrospectively analyzed by use of SPSS/PC software. The results showed that these patients were short of specificity in clinical manifestation and hemogram, however, all cases possessed multiple SLE-related autoantibodies, increase of serum globulin level and varying extent dermal and arthral signs. The incidence of primary or initial symptom in the 92 cases was as follow: 65 anemia (72.8%), 39 purpura (42.4%), 17 hemolytic anemia (18.5%), 56 leukopenia (60.9%), 54 thrombocytopenia (58.7%), and 41 pancytopenia (44.6%). The bone marrow examinations showed that the cellularity of nucleated cells was mostly normal, and active proliferation in 57 cases (61.9%) and hypercellularity in 35 cases (38.1%); the G/E ratio was normal in majority, and G/E ratio > 3 in 59 cases (64.1%) and < 3 in 33 cases (35.9%) and G/E < 1 in 17 cases with hemolytic anemia Coombs' test positive; megakaryocyte counts were normal in 11 cases (11.9%), increase in 80 cases (86.9%) and lower than 7/marrow smear in 1 case (1.1%). Neutrophil alkaline phosphatase staining was negative in all of the cases. From above data it is concluded that patients with SLERHD are varied in clinical and blood pictures, but all patients are provided with multiple SLE-related autoantibodies, globulinemia and dermal and arthral signs. It is easy to identify SLERHD from aplastic anemia, myelodysplastic syndrome, idiopathic thrombocytopenic purpura, autoimmune hemolytic anemia and Evans' syndrome by comprehensive and detailed clinical and laboratory examinations.
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PMID:[Clinical features of hematological abnormality in systemic lupus erythematosus-related hematological disorders]. 1251 74

The authors present an account on their own experience with laparoscopic splenectomy which is used at the First Surgical Clinic, First Medical Faculty and General Faculty Hospital in Prague since 1996. In 1996 to 2002 the authors performed at the Surgical Clinic a total of 66 splenectomies. This number included on account of injury of the spleen, tumour or cyst 12 splenectomies by the open route. The remaining ones were indicated for haematological reasons in collaboration with the haematological department of the First Medical Clinic of the First Medical Faculty and General Faculty Hospital in Prague and the Institute of Haematology and Blood Transfusion in Prague. 23 splenectomies for haematological reasons were made by the classical open route. In 31 patients the splenectomy was performed by the laparoscopic route. In all haematological reasons were involved. In the group operated at the First Surgical Clinic LSE was indicated because of ITP 15x, for spherocytosis 8x, for haemolytic anaemia 7x, for eliptocytosis 1x. In eight patients at the same time laparoscopic cholecystectomy was performed. In the group subjected to classical splenectomy infection in the surgical wound was recorded in 11%, re-operations on account of a suppurative complications in the abdominal cavity were made in 13% and on account of haemorrhage in 11%. In the group of laparoscopic splenectomies the authors did not record infection at the site of the inserted trocars, there were no suppurative complications in the abdominal cavity. In four laparoscopic operations the authors converted the operation on account of haemorrhage four times (11%), once on account of major adiposity of the omentum, in five postoperative revision on account of haemorrhage was necessary (16%), incl. one case of profuse haemorrhage. Therefore the authors sought a way how to prevent haemorrhage. Based on their own experience they recommend to apply clamps to the trunk of the lienal artery. The time of operation was reduced by the application of clamps to the lienal artery to 60-80 mins. and the peroperative blood losses dropped to 20-30 ml. The morbidity declined and the patients are discharged into domiciliary care on the 4th day after operation, to be on the safe side. No late complications of the operation were recorded.
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PMID:[Laparoscopic splenectomy--personal experience]. 1256 93

Laparoscopic operations of the spleen, incl. splenectomy, are increasingly accepted worldwide. The most frequent indications for this type of surgical treatment are haematological diseases (some types of haemolytic anaemia, thrombocytopenia, myeloproliferative syndrome). Another indication for surgery is hypersplenism from other causes such as vascular anomalies or isolated thrombosis of the lienal vein. The reason for urgent or planned laparoscopy may be also traumatic damage of the spleen (haemorrhage, pseudocysts). In the submitted work the authors present their experience with 21 patients where a laparoscopic operation of the spleen was performed. Of 21 children four were laparoscopically operated on account of a mesothelial cyst or posttraumatic pseudocyst of the spleen (ratio 1:3). In another 15 children laparoscopoc splenectomy was performed for haematological reasons (11 x spherocytosis, 3 x idiopathic thrombocytopenic purpura, 1 x autoimmune haemolytic anaemia). Twice the operation was indicated on account of isolated thrombosis of the lienal vein. In three instances a concurrently, also laparoscopic cholecystectomy was performed and in two liver biopsy. On operation 3-5 troacars were used and the patient was placed in a supine position. In no instance preoperative embolization of the lienal arteries was performed. On treatment of cystic structures of the spleen the authors made in three instances total resection of a cyst--in the upper pole of the spleen, in case of a central localization, only 2/3 resection and fixation of the omentum. In resections recently coagulation and Argon spraying was used. The spleen was extracted by morcellation in a plastic bag or via a 5 cm left-sided subcostal incision (2x). The median size of the spleen was 14.4 cm (10-20 cm), of cystic formations 7.25 cm (6-8 cm). The mean period of operation in splenectomy was 146 minutes. In two patients a blood transfusion was necessary (1x splenectomy, 1x resection of the lower third of the spleen). As to complications the authors recorded once major haemorrhage after splenectomy--resolved by the use of Endo GIA stapler. Conversion was not necessary in any of the operations. The mean period of hospitalization was 6 days.
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PMID:[Laparoscopic surgery of the spleen in children]. 1266 80

The treatment of selected refractory autoimmune diseases has been complemented by the use of Protein A (Prosorba column) immunoadsorption. US Food and Drug Administration-approved clinical applications include idiopathic thrombocytopenia purpura (ITP) and rheumatoid arthritis (RA). Other common off label uses include thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). Less common experimental uses in diseases in which efficacy has been reported include autoimmune CNS syndromes, peripheral neuropathies, autoimmune pancytopenia, hemolytic anemia and solid organ transplant rejection. Prosorba column treatment is generally well tolerated but a small proportion of treated patients experience chills, fever, tremor, hypotension and rash. The mechanism of action suggested for the efficacy of the column is the restoration of normal immune balance and normal tolerance. Observations in ITP has suggested that column treatment stimulates a rise in anti-idiotype antibody directed against antiplatelet antibodies, effecting a decrease in pathogenic antiplatelet antibodies and immune complexes.
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PMID:Correcting immune imbalance: the use of Prosorba column treatment for immune disorders. 1291 43

To explore the relationship between T cell markers in hematological diseases and T cell markers in solid tumor, CD3, CD4, CD8 in hematological diseases, malignant and benign tumors were detected by flow cytometry and results were analyzed statistically. The results showed that CD3, CD4, CD8 and CD4/CD8 in chronic leukemia decreased significantly while these markers in acute leukemia and MDS decreased obviously in comparison with normal persons and other hematological diseases (P < 0.0l). Hemolytic anemia markers increased significantly (P < 0.05). CD3, CD4, CD4/CD8 in idiopathic thrombocytopenic purpura decreased and CD8 increased (P < 0.0l). CD3, CD4, CD8 in iron-deficiency anemia, anemia from chronic diseases, benign tumor and other hematological diseases were lower than those in normal persons and hemolytic anemia, but higher than those in acute and chronic leukemia, malignant tumor, granulocytopenia, and MDS (P > 0.05). It is notable that the above markers correlated with the development and prognosis of diseases. In conclusion, expression of CD3, CD4, CD8, CD4/CD8 contributes to diagnosis of hematological diseases and benign or malignant tumors, and is an important indicator for therapeutic strategy.
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PMID:[Expression of T cell markers in hematological diseases and solid tumor]. 1498 75

Autoimmune diseases (AD) are conditions in which there is the development of antibodies against self cells/ organs. AD could either be organ-specific or non-organ specific (systemic) in clinical presentation. Commonly reported ADs includes: Myasthenia gravis, Hashimoto thyroiditis, Guillian-Barre syndrome, vitiligo, type 1 diabetes mellitus, Graves diseases, Goodpastures syndrome, pemphigus, rheumatoid arthritis, systemic lupus erythematosis, Addisons disease, multiple sclerosis, pernicious anaemia, autoimmune haemolytic anaemia, chronic active hepatitis, idiopathic thrombocytopenic purpura. There is paucity of locally documented information on the occurrence of AD in same patient in our environment. We therefore report the case of a 66 year old woman who presented at the University College Hospital (UCH), Ibadan, with a spectrum of the AD, Vitiligo, rheumatoid arthritis, myasthenia gravis, impaired glucose tolerance.
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PMID:Autoimmune diseases in a Nigerian woman--a case report. 1500 10

Modern treatments of autoimmune diseases are based on immunological therapies. Rituximab induces a targeted B-cell depletion in the aim of eradicating autoreactive clones in various autoimmune disorders. Several studies are being undertaken and preliminary reports are very encouraging. The mechanism of action is not evident, but appears to be connected with the lowering of autoantibody levels, in the diseases where relevant antibody titres are relievable. Most of the patients treated were affected by idiopathic thrombocytopenic purpura (ITP) and autoimmune haemolytic anaemia, but also very rare diseases like acquired haemophilia are reported. Best results are described in autoimmune haemolytic anaemia, in many others there is clear evidence for efficacy; in all the diseases the number of complete or partial remission, though temporary, is much greater than 50%. Side effects are rarely reported, and immunosuppression is not a great problem. The persistence of clinical improvement for more than 1 year after B-lymphocyte repopulation supports the hypothesis of a stochastic generation of pathogenic B-cell subsets. Other studies and controlled trials are required to establish when and which patients are to be treated, and find the opportunity of the association of others drugs.
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PMID:Rituximab in autoimmune diseases. 1519 66


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