Abstract:
:The presence of the sickle cell (Hb S) gene in Saudi Arabia was first reported by Lehmann et al. in 1963 [Nature 198, pp. 492-493]. Later, Hb S, alpha- and beta-thalassaemia, glucose-6-phosphate dehydrogenase deficiency and other enzymopathies were shown to occur at a variable prevalence in different regions of the country. Recent studies using restriction endonucleases have revealed alpha-globin gene arrangement and beta-globin gene polymorphism in the Saudi population. Interactions between abnormal genes are commonly encountered which often influence the clinical manifestations of sickle cell disease. In this paper, we present recent findings and discuss the status of haemoglobinopathies, thalassaemias and enzymopathies in Saudi Arabia.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
el-Hazmi MAdoi
10.1159/000205861subject
Has Abstractpub_date
1987-01-01 00:00:00pages
130-4issue
2-3eissn
0001-5792issn
1421-9662journal_volume
78pub_type
杂志文章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::In a retrospective study of 38 patients with histologically proven myelofibrosis, 5 parameters (recorded on the first admission) were investigated as to their usefulness to predict the course of the disease. In 9 patients the development of 'acute' myelofibrosis could be predicted by the finding of pancytopenia, low r...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207704
更新日期:1978-01-01 00:00:00
abstract::Of 34 patients with chronic inflammatory bowel disease (CIBD), 4 developed well-documented episodes of deep-venous thrombosis. All 4 patients had active disease at the time of thrombosis. This group was studied to determine if the tendency to deep-venous thrombosis in patients with CIBD was associated with reduced ant...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206688
更新日期:1983-01-01 00:00:00
abstract::The adaptation of cell physiological parameters of erythrocytes of the newborn to erythrocytes in later life was studied by cell separation in an isopycnic dextran density gradient. The cell characteristics during the first trimester of life were followed by repeated determinations. The mean cellular haemoglobin conce...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207034
更新日期:1982-01-01 00:00:00
abstract::Two family members (daughter and mother) with a bleeding disorder showed prolonged bleeding time and activated partial thromboplastin time associated with decreased plasma levels of factor VIII procoagulant activity, factor VIII-related antigen, and factor VIII-ristocetin cofactor activity. The ristocetin-induced plat...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206579
更新日期:1984-01-01 00:00:00
abstract::We describe a patient with recurrence of Hodgkin's disease and severe liver disease of unknown origin in whom autoimmune neutropenia developed. Because of possible seronegative viral hepatitis he was treated with high-dose intravenous immunoglobulin instead of steroids. He responded with a prompt but transitory increa...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203968
更新日期:1995-01-01 00:00:00
abstract::Multiple myeloma (MM) is a B cell malignancy characterized by accumulation of plasma cells (PCs) in the bone marrow. Traditional methods for the detection of minimal residual disease (MRD) measure the presence of monoclonal immunoglobulin protein secreted by the malignant PCs. However, changes in the level of MRD in M...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000077565
更新日期:2004-01-01 00:00:00
abstract::Phagocytic activity of leukaemic blasts in 20 adults and 16 children suffering from acute leukaemia was studied in vitro by the use of ferrioxidsaccharate. Most frequently phagocytosing blasts were encountered in myelomonocytic leukaemias. The morphological character of positive blasts resembled often that of monocyto...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208150
更新日期:1975-01-01 00:00:00
abstract::A subpopulation with alkaline phosphatase activity and neutrophilic granules was found in leukemic monocytes from a child with acute monocytic leukemia (M5B). Almost all leukemic cells were strongly positive for nonspecific esterase and phagocytized opsonized zymosans. These findings suggest that the subpopulation are...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206516
更新日期:1984-01-01 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 rare case of Hodgkin's disease which initially presented with a thyroid tumor in an 18-year-old-man is reported. The tumor involved most of the thyroid gland but was well demarcated, and the border between the tumor and the remnants of the thyroid gland was relatively clear, suggesting secondary Hodgkin's disease in...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203627
更新日期:1997-01-01 00:00:00
abstract::The establishment of mouse and human monoclonal anti-DNA antibodies is described. Common idiotypes were identified on these antibodies employing monoclonal (mouse) and polyclonal anti-idiotypic antibodies. The significance of the presence and titer of the common idiotypes as clinical activity marker was examined in pa...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206029
更新日期:1986-01-01 00:00:00
abstract::We describe a case of hemolytic anemia with subacute bacterial endocarditis due to Streptococcus sanguis. The major hematological features of the patient were a normocytic anemia with reticulocytosis, an elevation of serum lactate dehydrogenase, and a marked reduction of serum haptoglobin. All these signs of hemolytic...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203981
更新日期:1995-01-01 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::In the present study, we investigated the inhibitory natural killer cell receptor (NKR) expression of CD94/NKG2A on PBMC after allogeneic bone marrow transplantation (BMT). The proportion of CD94 expression on PBMC was higher in patients without chronic graft-versus-host disease (cGVHD) and also in cGVHD patients with...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000046540
更新日期:2001-01-01 00:00:00
abstract::The ability of human bone marrow particles to produce a microenvironment conducive to granulocytopoiesis was tested by culturing them in vitro without an exogenous source of colony stimulating activity (CSA). Granulocytopoiesis in this system was confirmed by the following observations: (1) presence of mitotic figures...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207809
更新日期:1977-01-01 00:00:00
abstract::Janus kinases are critical components of signaling pathways that regulate hematopoiesis. Mutations of the non-receptor tyrosine kinase JAK2 are found in many BCR-ABL-negative myeloproliferative neoplasms. Preclinical results support that JAK2 inhibitors could show efficacy in treating chronic myeloproliferative neopla...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000356784
更新日期:2014-01-01 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::Fetal hemoglobin (HbF) concentrations were measured by a radial immunodiffusion assay in 233 patients with various malignancies. In 96 of these, alpha-fetoprotein (AFP) was also measured by radioimmunoassay. The concentration of HbF exceeded 2 SDs above the normal mean in 39 of 233 patients, most notably in patients w...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207839
更新日期:1977-01-01 00:00:00
abstract::Inferferon alfa-2b (IFN) plays a major role in the current management of previously untreated patients with chronic myelogenous leukemia (CML) as well as patients with CML who have relapsed after bone marrow transplantation. Hydroxyurea (HU) is the best conventional drug for treatment of CML in the chronic phase. Ten ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203859
更新日期:1996-01-01 00:00:00
abstract::We studied 14 adult patients presenting with fever and cytopenia of the peripheral blood and histiocytic hyperplasia with hemophagocytosis (HHH) in the bone marrow regarding an association of cytomegalovirus (CMV) and Epstein-Barr virus (EBV) by using in situ hybridization (ISH) and also evaluated the clinical and lab...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203746
更新日期:1996-01-01 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::Monosomy 7 myelodysplasia is a rare hematological entity and is associated with morphological abnormalities in bone marrow and peripheral smear, and poor prognosis in children. We describe 2 children with infantile monosomy 7 myelodysplasia which evolved to leukemia. One of them died after 1 month, and the other is st...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204135
更新日期:1994-01-01 00:00:00
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::This paper reports the bone marrow histological picture in 7 patients with hairy cell leukemia treated with recombinant interferon. Bone marrow biopsies were performed at diagnosis, after 1, 3 and 7 months from the beginning of treatment and at 12 months at treatment suspension. The main modifications observed were a ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205996
更新日期:1987-01-01 00:00:00
abstract::Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and highly aggressive hematological malignancy derived from precursors of plasmacytoid dendritic cells. This disease typically presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and periphe...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000334703
更新日期:2012-01-01 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::Neurologic complications of allogeneic hematopoietic cell transplantation (allo-HCT) include infections, cerebrovascular events, therapy-induced neurotoxicity, recurrent malignancies, and neurologic manifestations of graft-versus-host disease (GVHD). Anti-glutamic acid decarboxylase (GAD) antibody-associated cerebella...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000494423
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND/AIMS:The aim of this work was to investigate the efficacy and predictive factors of CLAG treatment in refractory or relapsed (R/R) acute myeloid leukemia (AML) patients. METHODS:Sixty-seven R/R AML patients were enrolled in this prospective cohort study and treated by a CLAG regimen: 5 mg/m2/day cladribine ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000493250
更新日期:2019-01-01 00:00:00
abstract::Granulocytic sarcoma (GS) is a rare extramedullary tumor composed of immature myeloid cells. It is usually associated with leukemia or other myeloproliferative disorders, but can also occur without overt hematologic disease, i.e. in patients with a normal bone marrow and no history of acute myelogenous leukemia. This ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000041067
更新日期:2000-01-01 00:00:00