Abstract:
BACKGROUND:Thalassemia is a group of hereditary hemoglobinopathies caused by decreased or absent synthesis of α and/or β globin chains. Studies have shown that hypercoagulability and thrombosis are common clinical symptoms in β-thalassemia, especially β-thalassemia intermedia, but little is known about in α-thalassemia. This study aims to examine phosphatidylserine (PS) levels, platelet activation, and coagulation markers in splenectomized (S) and nonsplenectomy (NS) patients with hemoglobin (Hb) H disease. METHODS:The NS group comprised 20 patients (median age 15.0 years, range, 14-16.5 years), and the S group consisted of 11 patients (median age 16.4 years, range, 14-19.9 years) with Hb H disease; the control group consisted of 20 normal subjects. Hematological parameters were collected. Flow cytometry was used to measure PS exposure on red blood cells. The levels of intercellular adhesive molecule (ICAM)-1, tumor necrosis factor α (TNFα), β-thromboglobulin (TG) and prothrombin fragment 1 + 2 (F1.2) were determined using ELISA test kits. RESULTS:Significant increases in the levels of PS, ICAM-1, TNFα, β-TG, and F1.2 were observed in both patient groups compared to normal controls (p < 0.01). CONCLUSION:This observation indicates blood coagulation, endothelial injury, chronic low-grade inflammation, platelet activation, and thrombin generation are present in Hb H disease; these findings merit further assessment in a larger prospective cohort to establish possible links with thrombotic manifestations.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Chansai S,Fucharoen S,Fucharoen G,Jetsrisuparb A,Chumpia Wdoi
10.1159/000486157subject
Has Abstractpub_date
2018-01-01 00:00:00pages
47-51issue
1eissn
0001-5792issn
1421-9662pii
000486157journal_volume
139pub_type
杂志文章abstract::A modified cytochemical method was developed for demonstrating magnesium-activated adenosine triphosphatase in haemic cells. The enzyme was shown in a variety of normal and leukaemic, unfixed cells. The high sensitivity of this enzyme to all fixatives tried in this study hampered further exploration and characterizati...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205983
更新日期:1987-01-01 00:00:00
abstract:OBJECTIVE:We investigated the prevalence of opportunistic infections in HIV-infected women according to transferrin (TF) phenotype. METHODS:We conducted a cross-sectional study among 200 HIV-positive women in the Butare University Teaching Hospital in Rwanda. TF phenotypes were determined using starch gel electrophore...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000338262
更新日期:2012-01-01 00:00:00
abstract:BACKGROUND:It is a common belief among women that iron compounds have unpleasant gastrointestinal side effects. OBJECTIVE:To assess the gastrointestinal side effects of iron prophylaxis in pregnancy. METHODS:A randomized, double-blind study comprising 404 healthy pregnant women allocated to four groups taking ferrous...
journal_title:Acta haematologica
pub_type: 杂志文章,随机对照试验
doi:10.1159/000089466
更新日期:2006-01-01 00:00:00
abstract::We have retrospectively analyzed a series of 19 patients with hepatitis C virus (HCV) infection and chronic thrombocytopenia not attributable to hypersplenism or to other causes. Antiplatelet antibodies were present in 81% of cases. Response to prednisone was observed in 6 of 7 patients and 1 of 3 patients responded t...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040842
更新日期:1998-01-01 00:00:00
abstract::The most common single genetic disorder and a major public health issue in Greece and other Mediterranean countries is beta-thalassemia. Current therapeutic approaches for homozygous beta-thalassemia entail blood transfusions and iron chelation therapy with deferoxamine or deferiprone for preventing tissue hemosideros...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000077551
更新日期:2004-01-01 00:00:00
abstract::We describe in this paper a female patient affected by chronic granulomatous disease with all the features of the classic X-linked form of the disease and presenting a mild form of the disease, the major clinical manifestation being a granulomatous cheilitis. The capability of the patient's phagocytes to undergo a res...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205028
更新日期:1990-01-01 00:00:00
abstract::Iron overload is a constant and the more important complication in thalassemia. Serum ferritin concentration accurately reflects body iron stores. A total of 245 thalassemic patients aged 12-55 years were examined, 71 having Hb H disease and 174 beta-thalassemia/Hb E disease. The patients received minimal or no blood ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207129
更新日期:1981-01-01 00:00:00
abstract::A 29-year-old man with acute myelomonocytic leukemia had an initial leukocyte count of 192 X 10(9) cells/l and 84% blasts. During the initial treatment with hydroxyurea, 1 g/m2 orally tid, he developed pulmonary leukostasis which responded rapidly to whole-lung radiation with 1.5 Gy in one fraction. Pulmonary leukosta...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205987
更新日期:1987-01-01 00:00:00
abstract::Iron overload due to regular transfusions of packed red cells can cause multiple organ damage. Iron chelation therapy (ICT) is important in patients with aplastic anemia (AA) who require blood transfusions as supportive management. With the introduction of the oral iron chelator deferasirox, ICT has become more widely...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000342772
更新日期:2013-01-01 00:00:00
abstract::A case of isolated lambda-light chain proteinemia and proteinuria is reported. During a 42-month follow-up, no sign of myelomatosis or amyloidosis developed and Bence Jones proteinuria remained nearly of the same magnitude. This case of apparently idiopathic Bence Jones gammopathy, the first lambda-type so far reporte...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207409
更新日期:1980-01-01 00:00:00
abstract::Serum soluble interleukin-6 receptor (sIL-6R) concentrations were measured in 50 patients with plasma cell dyscrasias using a commercially available immunoenzymatic assay kit. There were 40 patients with multiple myeloma (MM), 5 patients with monoclonal gammopathy of undetermined significance (MGUS), 3 patients with s...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203682
更新日期:1997-01-01 00:00:00
abstract::6 patients with acute leukemia are described. All of them suffered from high temperature and malaise, and showed negative urine and blood cultures. High doses of gentamicin, cephalothin and cerebenicillin failed to lower the fever. The temperature became normal after administration of chloramphenicol (CAP) 2.0 g/day. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207275
更新日期:1980-01-01 00:00:00
abstract:OBJECTIVE:The aim of this investigation was to study the effect of vitamin E treatment in oxidative stress of red and white cells of beta-thalassaemia intermedia patients. METHODS:Nine patients undergoing occasional transfusions (5 females/4 males), median age 39 years (range 15-74), were recruited for oral daily admi...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000201988
更新日期:2008-01-01 00:00:00
abstract::15 patients with aplastic anemia were prospectively followed after having measurements of myeloid progenitor cells in bone marrow and blood. Treatment included androgens, low or high dose steroids and standardized supportive care. The median length of survival was 5.8 months. When patients were grouped according to th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206263
更新日期:1985-01-01 00:00:00
abstract::In 33 children with cyanotic congenital heart disease the platelet function has been studied. The most significant changes were reduced platelet adhesiveness to glass and impaired availability of platelet factor 3 in nearly 50% of the patients. Although clot retraction was poor in 84% of them, thrombocytopenia and pro...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208017
更新日期:1976-01-01 00:00:00
abstract::A case of systemic mastocytosis with unusual clinical manifestations, appearing as an isolated splenohepatomegaly, is described. The proliferative character is evident from the cytological characteristics of immaturity and the presence of a moderate percentage of mast cells in the peripheral blood. These cells make up...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207846
更新日期:1977-01-01 00:00:00
abstract::Acute myeloid leukemia (AML) is a stem cell malignancy that originates in the bone marrow and involves the peripheral blood. Extramedullary AML is rarer, but it is most commonly associated with the former French-American-British (FAB) subtypes M4 or M5 of AML. AML cells may also home to the central nervous system and ...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000508526
更新日期:2020-08-19 00:00:00
abstract::We present the case of a woman undergoing treatment with acenocoumarol for deep vein thrombosis, who maintained an international normalized ratio (INR) of between 2.5 and 4 for 2 months. Seven days after the introduction of amoxycillin (500 mg/8 h) for a probable respiratory infection, the patient developed spontaneou...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204457
更新日期:1993-01-01 00:00:00
abstract::Alpha-thalassemia is very common in the Kuwaiti population, but its influence on anemia of pregnancy has not been previously investigated. We have screened a group of 59 anemic (Hb < 11 g/dl) pregnant women for the alpha-thal-2 (-alpha-3.7 kb) deletion which is the commonest alpha-thal allele in this community, using ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000039757
更新日期:2000-01-01 00:00:00
abstract::Eleven patients with acute myeloid leukaemia (AML) in first complete remission (CR) were treated with alfa-2a-interferon (for short 'interferon') maintenance therapy, at a dose of 3 MU twice to thrice weekly subcutaneously. Adjustments were made to maintain neutrophil counts > 1 x 10(9)/l and platelet counts > 100 x 1...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204473
更新日期:1993-01-01 00:00:00
abstract::The effect of methylprednisolone on fresh cells from patients with chronic lymphocytic leukaemia (CLL) has been studied using the differential staining cytotoxicity (DiSC) assay resulting in LC90s of < or = 0.2 to 2,000 micrograms/ml. Cells from previously treated patients were, on average, significantly more sensitiv...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000204115
更新日期:1995-01-01 00:00:00
abstract::Residues 124 and 125 of the beta-chain of human haemoglobin are prolyl-prolyl. In the delta-chain, one of these positions is occupied by a glutaminyl, and there has been uncertainty as to which of the two residues is the prolyl and which the glutaminyl. The sequence has now been established to be delta124 Pro - 125 Gl...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207923
更新日期:1976-01-01 00:00:00
abstract::10 patients with paroxysmal nocturnal hemoglobinuria were studied taking 109 normal subjects of the Cuban population as control group. 26 HLA antigens corresponding to loci A and B were studied in both groups. Phenotypical frequency of both groups were compared. No statistically significant increase was found for any ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207369
更新日期:1980-01-01 00:00:00
abstract::Iron deficiency is the main cause of anemia in both sexes, with women being more commonly affected. Iron therapy is currently considered an effective and safe remedy to replenish the iron storages. Iron can be administrated both orally and intravenously. In particular, intravenous (IV) iron therapy is widely used when...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000503768
更新日期:2020-01-01 00:00:00
abstract::Malaria parasites growing inside human erythrocytes differ from mammalian cells in their mode of acquisition of bioavailable iron and in their susceptibility to the antiproliferative action of iron chelators. We have assessed here three major properties associated with these phenomena: (a) the stage-dependent nature o...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000203952
更新日期:1996-01-01 00:00:00
abstract::We present a case with the clinical and pathological impression of Ph1-positive chronic myelogenous leukemia in extramedullary blast crisis involving lymph nodes as demonstrated by morphological and cytogenetic studies. The hyperploid cell lines that were present in the lymph node were not present in the bone marrow. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206166
更新日期:1985-01-01 00:00:00
abstract::A case of angioimmunoblastic lymphadenopathy (AILD) with important autoimmune symptoms disappearing under Levamisole therapy is reported. Since Levamisole is thought to have no direct effect on B cells, it is supposed that in AILD it regulates B lymphocyte activity via the restoration of impaired T cell functions. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207551
更新日期:1979-01-01 00:00:00
abstract::A case of acquired haemophilia A presenting with extensive spontaneous bruising and anaemia is reported. The anaemia was due to myelodysplastic syndrome (FAB: refractory anaemia with ringed sideroblasts). A factor-VII:C-specific inhibitor was also found. Prednisone and pyridoxine were given, and the inhibitor became u...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204894
更新日期:1991-01-01 00:00:00
abstract::Recently, the use of novel targeted drugs has changed the treatment paradigms in chronic lymphocytic leukemia (CLL). Among the several drugs used for the management of relapsed/refractory (R/R) CLL, Bruton tyrosine kinase inhibitors (ibrutinib and acalabrutinib), phosphatidylinositol 3-kinase inhibitors (idelalisib an...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000510768
更新日期:2020-11-25 00:00:00
abstract:BACKGROUND:Argininemia is an autosomal recessive urea cycle disorder (UCD). Unlike other UCD, hyperammonemia is rarely seen. Patients usually present in childhood with neurological symptoms. Uncommon presentations like neonatal cholestasis or cirrhosis have been reported. Although transient elevations of liver transami...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000493678
更新日期:2018-01-01 00:00:00