Abstract:
:Two subjects, not previously reported in detail, had severe inherited deficiencies of erythrocyte pyrimidine nucleotidase. This was manifested hematologically by moderate hemolytic anemia with splenomegaly, morphologically by punctate basophilic stippling of Wright's stained erythrocytes, and biochemically by intraerythrocytic accumulation of pyrimidine nucleotides, elevated concentrations of reduced glutathione, and partial deficiencies of ribosephosphate pyrophosphokinase. All 5 of their children were asymptomatic and phenotypically normal except for intermediate reductions in activities of pyrimidine nucleotidase consistent with heterozygosity for an autosomal recessive defect.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Paglia DE,Fink K,Valentine WNdoi
10.1159/000207413subject
Has Abstractpub_date
1980-01-01 00:00:00pages
262-7issue
5eissn
0001-5792issn
1421-9662journal_volume
63pub_type
杂志文章abstract::A patient with regional enteritis had received iron dextran for treatment of iron deficiency. Subsequently he developed a large (3.1 g/100 ml) IgG-K serum spike which had precipitin activity against dextran sulfate but not a variety of other antigens. There has been no evidence of multiple myeloma and the spike gradua...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207755
更新日期:1978-01-01 00:00:00
abstract::Recombinant alpha-interferons are used as therapeutic agents in an increasing number of benign and malignant disorders. Long-term administration of recombinant alpha-interferon as a maintenance agent is associated with a small number of adverse side-effects which are responsible for patient intolerance of this drug. T...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204881
更新日期:1991-01-01 00:00:00
abstract::A 12-year-old male of Chinese ancestry had life-long hemolytic anemia attributed to alpha-thalassemia. Restriction endonuclease mapping of his DNA revealed that in reality, he had three alpha-globin loci, but he was homozygous for pyruvate kinase deficiency. The new pyruvate kinase variant carried by this patient was ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206830
更新日期:1983-01-01 00:00:00
abstract:BACKGROUND:Posterior reversible encephalopathy syndrome (PRES) is a rare but serious complication after allogeneic hematopoietic stem cell transplantation (alloHSCT). Among others, calcineurin inhibitors (CNI) for prophylaxis of graft-versus-host disease (GvHD) may promote the development of PRES, but the pathomechanis...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000430489
更新日期:2016-01-01 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::Hypercalcemia in malignancies is a frequent complication, mostly affecting patients with solid tumors or multiple myeloma. Calcium elevation is induced by direct bone infiltration of a tumor mass or through calcium liberation from the skeleton by a humoral mediator. The latter mechanism is referred to as humoral hyper...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000314646
更新日期:2010-01-01 00:00:00
abstract::Treating patients with hemophilia and inhibitors is often problematic. The presence of inhibitors negatively impacts the effectiveness of treatment to achieve hemostasis especially in patients with hemophilia B, owing mainly to allergic reactions to factor IX (FIX) concentrates and the low success rate of immune toler...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000508722
更新日期:2020-07-22 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::A study of E-rosette ultrathin sections has been undertaken in an attempt to analyze changes induced during the T-lymphocyte-sheep red blood cell interaction on the rosetted lymphocyte surface architecture. The formation of nonimmune rosettes implies different types of attachment between lymphocytes and sheep red bloo...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207570
更新日期:1979-01-01 00:00:00
abstract::Natural killer (NK) cell activity against K562 cell targets and the distribution of T cell subpopulations were investigated in the peripheral blood of 25 patients affected by beta thalassemia major, 18 clinically healthy heterozygotes, and 25 age-matched normal subjects. It was found that thalassemia major patients di...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206598
更新日期:1984-01-01 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::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:BACKGROUND:Splanchnic vein thrombosis (SVT) is a severe complication of essential thrombocythemia (ET). No clear explanation has been given for the occurrence of thrombosis in this unusual site in patients with ET, but the existence of a specific association between unexplained SVT and the JAK2 mutation has been report...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000221962
更新日期:2009-01-01 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::Platelet to leukocyte adhesion phenomena (PLAP) and the phagocytosis of platelets by neutrophils from the EDTA anticoagulated blood samples are described. PLAP was transferable to a normal blood specimen by patient's plasma or serum with or without complement. Further studies revealed in vitro evidence of hereditary p...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206966
更新日期:1982-01-01 00:00:00
abstract::An ultrastructural study of the aspirated bone marrow of a patient with congenital dyserythropoietic anemia type I is presented. Both in transmission electron microscopy (TEM) and in scanning electron microscopy (SEM) ultrastructural abnormalities of the bone marrow erythroid precursors were seen. These abnormalities ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206735
更新日期:1983-01-01 00:00:00
abstract::To test whether anti-idiotypic immunoregulation of factor VIII(FVIII)-inhibiting antibodies could be feasible in hemophiliacs, we assayed the minimal number and range of immunogenic, functional FVIII epitopes to which a series of murine anti-FVIII monoclonal antibodies (MAb) were directed. Rabbit anti-idiotypic sera t...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205171
更新日期:1990-01-01 00:00:00
abstract::In the myelodysplastic syndromes (MDS) clonogenic marrow cell culture studies have demonstrated intrinsic hemopoietic stem cell and progenitor cell abnormalities consistent with these disorders representing clonal hemopathies. Abnormal responsiveness of these cells to stimulatory and inhibitory growth factors indicate...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000205911
更新日期:1987-01-01 00:00:00
abstract::We evaluated the results of high-dose therapy (HDT) and autologous hematopoietic stem cell transplantation (ASCT) in patients with relapsed or primary refractory Hodgkin's disease (HD), using a previously reported prognostic model based on the presence of three poor prognostic factors at the start of salvage therapy/p...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000074220
更新日期:2003-01-01 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::95 of 1,019 (9.3%) sera with monoclonal immunoglobulins (MIg) were found to have cold-reacting lymphocytotoxins (LCT). There was no difference in the prevalence of LCT in multiple myeloma, macroglobulinemia, cancer, lymphoma or benign monoclonal gammopathy. Prevalence of LCT was similar in various classes and types of...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206328
更新日期:1985-01-01 00:00:00
abstract::350 patients with idiopathic thrombocytopenic purpura (ITP) aged 2/12-15 years (mean 6.3 +/- 2.7) were followed up during the period January 1st, 1975 to March 31, 1992. They constituted 40% of cases with hemorrhagic diathesis attending the Hematology/Oncology Clinic, Children's Hospital, Ain Shams University (relativ...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000204392
更新日期:1993-01-01 00:00:00
abstract::There have been many reports of patients with ampulla cardiomyopathy described as takotsubo-shaped cardiomyopathy in the cardiovascular field. This unique cardiomyopathy is characterized by transient apical ballooning and hypokinesis of the left ventricle. We describe 2 cases of ampulla cardiomyopathy associated with ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000098461
更新日期:2007-01-01 00:00:00
abstract::2 fatal cases of graft-versus-host disease (GvHD) occurred following blood product transfusions given to patients receiving standard chemotherapy for Hodgkin's disease. GvHD was established by HLA typing, clinical course, and compatible skin biopsy. 23 cases of GvHD following transfusion of blood products from normal ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206599
更新日期:1984-01-01 00:00:00
abstract::Gene therapy targeting hematopoietic cells has arrived at a new stage of potency. While the potential for curing inherited disorders of the immune system has been demonstrated in clinical trials, we were also confronted with the first serious adverse events related to random insertion of foreign DNA into cellular chro...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000072459
更新日期:2003-01-01 00:00:00
abstract::A case of Philadelphia-positive (Ph) acute lymphoblastic leukaemia (ALL) in a 40-year-old male is presented. At diagnosis, 80% of bone marrow cells were Ph. Remission with normal blood counts was achieved but the marrow became hypercellular, indicating conversion to chronic granulocytic leukaemia (GCL). The Ph clone p...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204497
更新日期:1993-01-01 00:00:00
abstract::Coagulation factor VIII inhibitor arising in a patient with autoimmune disease was immunologically analyzed. A 63-year-old man who had been diagnosed as suffering from polyarteritis nodosa was treated with prednisolone for 10 years. Severe bleeding tendency developed and coagulation studies demonstrated a high titer o...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204879
更新日期:1991-01-01 00:00:00
abstract::The in vitro immune functions of peripheral blood lymphocytes have been studied in 12 children with beta-thalassaemia major and hypersplenism. The study was performed prior to splenectomy and on the 2nd, 6th, 15th and 30th day after splenectomy. It was found that before splenectomy, patients had low numbers of blood l...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205886
更新日期:1987-01-01 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::Eltrombopag is a thrombopoietin receptor agonist frequently used to manage immune thrombocytopenia and aplastic anemia. At the high doses used for aplastic anemia, but not the doses used for immune thrombocytopenia, eltrombopag can cause reddish-brown discoloration of plasma, which can interfere with bilirubin measure...
journal_title:Acta haematologica
pub_type:
doi:10.1159/000508599
更新日期:2020-07-22 00:00:00