Abstract:
:This paper presents data on the occurrence and pattern of inherited bleeding syndromes (IBS) in Jordan, a hitherto unexplored problem. In 1978, during the first 12 months of a prospective study at a major medical center, 91 patients from 51 families were diagnosed as having IBS. All patients were referred because of moderate-to-severe bleeding diatheses; they included 52 hemophiliacs, 27 patients with von Willebrand's disease, 4 with hemophilia B (IX-deficit), 2 with afibrinogenemia, 1 with prothrombin deficiency, and 4 were thought to have platelet dysfunction. The clinical and laboratory features of the patients observed in Jordan do not seem to be significantly different from those of patients in Western Europe or North America.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Alsabti EA,Hammadi Mdoi
10.1159/000207625subject
Has Abstractpub_date
1979-01-01 00:00:00pages
47-51issue
1eissn
0001-5792issn
1421-9662journal_volume
61pub_type
杂志文章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
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::Bone marrow fragments from 10 patients with a megaloblastic anaemia due to vitamin B12 or folate deficiency were studied by electron microscopy and electron microscope autoradiography. A proportion of the erythroblasts showed ultrastructural abnormalities. Some of the cells containing autophagic vacuoles, large sidero...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207854
更新日期:1977-01-01 00:00:00
abstract::The goal of the study was to investigate changes in expression of selected growth factors tentatively involved in regeneration of haematopoietic tissues (bone marrow and spleen) following cyclophosphamide (CY) damage in the mouse. The bone marrow (BM) and spleen were examined separately, since the regenerating pattern...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000068490
更新日期:2003-01-01 00:00:00
abstract::Cardiac scintigraphy has been performed in 60 beta-thalassaemia major patients aged 8-35 years who received regular blood transfusions and subcutaneous desferrioxamine (DFX) chelation. Fifty-seven showed no clinical, radiological or electrocardiographic evidence of heart disease and 3 had clinically apparent cardiac f...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205046
更新日期:1990-01-01 00:00:00
abstract::A 59-year-old man presented with lymphocytosis with huge splenomegaly. The abnormal lymphocytes had a high nucleoplasm:cytoplasm ratio, a prominent nucleolus and hairy cytoplasmic projections. Immunophenotyping revealed B-cell leukemia with negative reactions to CD5 and CD25. Cytogenetic study showed 46,XY,der(5)t(5;6...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203984
更新日期:1995-01-01 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::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 patient with acute monoblastic leukemia developed a coagulopathy during chemotherapy. Coagulation studies showed thrombocytopenia, hypofibrinogenemia, high levels of fibrinogen degradation products, a negative protemine test but a normal antithrombin III level. These observations suggest that coagulation abnormaliti...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207433
更新日期:1980-01-01 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::To clarify the role of thrombopoietin (c-Mpl ligand, TPO) in 'hypersplenic' thrombocytopenia, we used an enzyme-linked immunosorbent assay to examine changes in serum TPO levels accompanied with splenectomy in 6 patients with liver cirrhosis, 4 patients with gastric cancer, and 2 patients with lymphoid malignancies. W...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040888
更新日期:1998-12-01 00:00:00
abstract::The management of severe late-onset hemorrhagic cystitis (LO-HC) after allogeneic hematopoietic stem cell transplantation (HSCT) is still challenging. Because mesenchymal stromal cells (MSCs) possess anti-inflammatory and tissue repair-promoting properties, we retrospectively analyzed the efficacy and safety of MSC in...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000362530
更新日期:2015-01-01 00:00:00
abstract::Fibroblast-like cells were grown from the bone marrow of acute lymphoblastic leukemia (ALL) patients before, during and off therapy. A diminished growth capacity was observed in cells from patients before and during therapy. In the three groups studied, differences were also observed in the effect of hydrocortisone on...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206284
更新日期:1985-01-01 00:00:00
abstract::The case history of a 71-year-old woman with three episodes of a microangiopathic hemolytic anemia over a 22-year span is detailed. During the last episode a possible response of her thrombotic thrombocytopenic purpura (TTP)-like syndrome to the administration of intravenous immunoglobulin is documented. In retrospect...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000205377
更新日期:1989-01-01 00:00:00
abstract::A 26-year-old female with Ki-1-positive large-cell anaplastic lymphoma is reported. The neoplastic cells were phenotypically and genotypically of T cell origin. Initially, neoplastic cells invaded the skin and lymph nodes, and then invaded the sternal and vertebral bones, ribs and the iliopsoas muscle. Central nervous...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204672
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND:The treatment of relapsed/refractory (R/R) peripheral T cell lymphoma (PTCL) is limited to a few agents. Romidepsin, a histone deacetylase inhibitor, was approved for PTCL treatment as a single agent in the R/R setting, yet with partial efficacy. Several attempts to combine romidepsin with other chemotherapy...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000498905
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND:The relation with SNF5 mutation and chromosome 22 abnormalities is not clear in hematological neoplasms. METHODS:To elucidate the relevance of the SNF5 gene on 22q11.2, karyotypes were reviewed in 283 hematological neoplasms. Loss of heterozygosity (LOH) on 22q was analyzed in 21 plasma cell myelomas withou...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000324932
更新日期:2011-01-01 00:00:00
abstract:BACKGROUND:Not all patients with diffuse large B-cell lymphoma (DLBCL) are candidates for aggressive regimens. (90)Y ibritumomab tiuxetan ((90)Y-IT), an anti-CD20 radionuclide-conjugated antibody, has demonstrated clinical efficacy in DLBCL with a favorable toxicity profile. METHODS:This phase II trial investigated th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000368291
更新日期:2015-01-01 00:00:00
abstract::An unusual complication of chronic lymphocytic leukemia (CLL) is reported. The patient, a 79-year-old man, had a long standing history of CLL, that had been complicated by the development of a Guillain-Barré-like syndrome and a peripheral biclonal gammopathy. The biclonal immunoglobulins identified in the serum were I...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204593
更新日期:1992-01-01 00:00:00
abstract::Plasma samples from patients with disseminated intravascular coagulation (DIC) associated with acute promyelocytic leukemia (APL) exhibited higher levels of the D-fragment of fibrin and fibrinogen degradation products [FDP(D)], with relatively lower levels of cross-linked fibrin degradation products (XDP), than sample...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205392
更新日期:1989-01-01 00:00:00
abstract::The activity of gamma-glutamyl transpeptidase was investigated in normal and leukemia leukocytes. Enzyme activity was positively correlated with the proportion of mature neutrophils and monocytes. In isolated leukocytes from patients with acute myeloid leukemia low values were obtained compared to controls. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207384
更新日期:1980-01-01 00:00:00
abstract::The fibrinolytic response to surgical trauma was studied longitudinally in 7 pigs by measuring the fibrinolytic activity in the blood and vein wall simultaneously. The results indicated a marked suppression in fibrinolytic activity in the blood which was closely correlated with the activity in the vein wall. This corr...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207397
更新日期:1980-01-01 00:00:00
abstract::The Philadelphia chromosome-negative myeloproliferative disorders (MPDs) polycythemia vera (PV), essential thrombocytosis (ET) and primary myelofibrosis (PMF) are characterized by increased proliferation of terminally differentiated myeloid cells. Although these disorders were recognized as clonal hematopoietic stem c...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000140634
更新日期:2008-01-01 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::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::Patients with HIV-associated lymphocyte-depleted Hodgkin lymphoma (HIV-HL) often present with advanced, extranodal disease and aggressive clinical features, limiting definitive therapeutic intervention. Here we report two patients with HIV-HL who presented with multi-organ dysfunction as an initial manifestation of th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000500222
更新日期:2020-01-01 00:00:00
abstract::A 69-year-old Japanese female was admitted to our hospital due to a 2-month history of vomiting after eating. Examination of the small intestinal tract revealed a tumor with calcification in the inner portion, from the horizontal portion to the ascending portion of the duodenum, and jejunojejunostomy was performed. Th...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000041012
更新日期:1999-01-01 00:00:00
abstract::Thalidomide acts on the microenvironment of myelodysplastic syndromes (MDS) by influencing cytokine networks, and growing evidence supports thalidomide's usefulness in the management of haematological malignancies, such as MDS. The European Collaboration Group on Myelofibrosis with Myeloid Metaplasia reviewed patients...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000087042
更新日期:2005-01-01 00:00:00
abstract::Platelet functions were evaluated in 20 healthy pregnant women in the last months of pregnancy, during labor, and 5-12 weeks after labor. The results showed an increased platelet count, bleeding time and nitroblue tetrazolium (NBT) test, as well as an increased plasma calcium level postlabor. ADP- and epinephrine-indu...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207151
更新日期:1981-01-01 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