Abstract:
:Hemochromatosis has generally been considered to be a genetic disease in which progressive iron accumulation over many years can lead to cirrhosis of the liver, hepatocellular carcinoma, diabetes, cardiomyopathy, and arthropathy. Iron depletion by phlebotomy has been the recommended therapy although a randomized trial of phlebotomy versus no treatment has never been reported. Since the discovery of the HFE gene in 1996, it has been possible to predict the risk of developing iron overload by a simple blood test to detect C282Y homozygotes of the HFE gene. The application of the hemochromatosis genetic test in large population studies often initiated to investigate other diseases has provided a fascinating glimpse into the natural history of untreated C282Y homozygotes followed for over 20 years without phlebotomy treatment. These observations are summarized in this review article which raises questions about the need for phlebotomy in all C282Y homozygous patients.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Adams PCdoi
10.1159/000243797subject
Has Abstractpub_date
2009-01-01 00:00:00pages
134-9issue
2-3eissn
0001-5792issn
1421-9662pii
000243797journal_volume
122pub_type
杂志文章abstract::Basic fibroblast growth factor (b-FGF) mediates a variety of biological responses such as angiogenesis and hematopoiesis. We examined the effect of b-FGF on human neutrophil functions in vitro. The surface expression of effector cell molecules on neutrophils was determined by flow cytometry and monoclonal antibodies. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000041024
更新日期:2000-01-01 00:00:00
abstract::In order to study the abnormal differentiation and proliferation of precursor T cells in homosexual men, a study was made of T-cell colony formation in 15 healthy homosexual men without anti-human immunodeficiency virus (HIV) antibody. The colony forming method used was the one-step monolayer technique using phytohema...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205374
更新日期:1989-01-01 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::Aspergillus vegetative endocarditis developing in a patient with acute lymphocytic leukemia during the phase of hematological remission has led to a fatal outcome, complicated with severe hemolytic anemia with red cell fragmentation. Systemic aspergillosis may involve heart valves with underlying disorders, but seldom...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206021
更新日期:1986-01-01 00:00:00
abstract::Increased levels of hemoglobin A(2) (HbA(2)) are present in most beta-thalassemia carriers. The mechanism of this effect is not understood, although the increase may result from transcriptional and posttranscriptional changes. In the present study, we quantitate delta-globin mRNA levels in peripheral-blood-enriched re...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040959
更新日期:1999-01-01 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::Pyrimidine 5'-nucleotidase (P5'N) partial deficiency has been described in several hematological disorders and also in the beta-thalassemic trait. To check if the P5'N deficiency in thalassemia was acquired we used thalassemic red cells (from either homo- or heterozygous subjects), whose P5'N activity was lower than i...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205286
更新日期:1989-01-01 00:00:00
abstract::To characterize intracellular signaling in peripheral blood (PB) cells of acute myeloid leukemia (AML) patients undergoing pretransplant conditioning with CXCR4 inhibitor plerixafor, granulocyte colony-stimulating factor (G-CSF), and busulfan plus fludarabine (Bu+Flu) chemotherapy, we profiled 153 proteins in 33 funct...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000495456
更新日期:2019-01-01 00:00:00
abstract::This report describes a case of hairy cell leukemia (HCL) occurring with autoimmune chronic active hepatitis (CAH). A 74-year-old woman presented with typical clinical and histologic features of HCL for which splenectomy was performed. 2 years later she developed abnormal liver function tests due to auto-immune CAH. T...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206291
更新日期:1985-01-01 00:00:00
abstract::Richter syndrome (RS) describes the development of high-grade non-Hodgkin's lymphoma (NHL) from low-grade NHL. RS isolated to the brain is very rare and has a poor prognosis. We describe the cases of high-grade large B-cell diffuse NHL in a 56-year-old male with chronic lymphocytic leukemia and in a 71-year-old female...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000334068
更新日期:2012-01-01 00:00:00
abstract::Acute megakaryoblastic leukemia is uncommon and comprises about 5% of acute nonlymphoid leukemias in the French-American-British classification. Cell lines from such leukemias are relatively rare with only about 8 reported in the literature. We established a cell line from a case of acute megakaryoblastic leukemia ari...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203557
更新日期:1997-01-01 00:00:00
abstract::Electron-microscopic examination of peripheral blood from a patient with 'hairy cell' leukemia revealed classical 'hairy cells', atypical lymphoid cells and numerous pathological plasma cells. Osmiophilic granular material coated the cell surface of 'hairy cells' and lymphoid cells but not the plasmalemma of the plasm...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208089
更新日期:1975-01-01 00:00:00
abstract::The level of granulocyte-macrophage colony-stimulating factor (GM-CSF) in the cerebrospinal fluid from 14 infants and children with meningitis and 6 patients who suffered other diseases besides meningitis was measured by our sensitive enzyme linked immunosorbent assay for GM-CSF. The minimal detection level of GM-CSF ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204796
更新日期:1991-01-01 00:00:00
abstract::Upper-extremity deep venous thrombosis (UEDVT) is an increasingly important clinical problem in children. These events are classified as primary or secondary, with the latter being the most common and usually associated with the presence of a central venous line. Among primary UEDVT, the so-called Paget-Schroetter syn...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000090939
更新日期:2006-01-01 00:00:00
abstract::Sera from 154 hemophiliacs, including 132 with hemophilia A and 22 with hemophilia B, were examined for antibodies against human T cell lymphotropic virus type III (HTLV-III) and type I by strip radioimmunoassay based on the Western blotting technique. Sixty-two patients lived in Kyushu, a known endemic area of HTLV-I...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205944
更新日期:1987-01-01 00:00:00
abstract::Recent molecular biological investigations have shown that genetic information on the short arm of chromosome 17 is frequently deleted or inactivated in acute myeloid leukaemias and a number of solid tumours. In earlier studies we have found clonal occurrence of a 17p deletion (17p-) in patients with refractory anaemi...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205033
更新日期:1990-01-01 00:00:00
abstract::Allogeneic stem cell transplantation (SCT) is the treatment of choice for selected patients with chronic myeloid leukemia (CML). However, it is associated with a high risk of treatment-related mortality (TRM) and morbidity. To assist in decision making about transplantation, a simple scoring system to assess the risk ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000069283
更新日期:2003-01-01 00:00:00
abstract::A family with hereditary ovalocytosis (HO) is described. The probands, 2 brothers, had splenic rupture after modest trauma as preenting symptoms. 7 members of the family had HO. The sister of the pobands had a moderately enlarged spleen. The other members proved normal on routine clinical examination. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207420
更新日期:1980-01-01 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::Micromegakaryocytes (MMK) were defined morphologically by the cell area, nucleus form and cytoplasmic structure. Bone marrow smears of 7,156 patients were retrospectively analyzed. MMK were found most frequently and abundantly in acute non-lymphatic leukaemia, chronic myeloid leukaemia and pre-leukaemia. The presence ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207213
更新日期:1980-01-01 00:00:00
abstract::We report the identification of an Hb S homozygosity in a 29-year-old multiple-transfused patient with severe kidney disease using a new reverse-transcription polymerase chain reaction (RT-PCR) technique with mRNA as starting template. The procedure requires considerably less time than sequencing of genomic DNA and is...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204121
更新日期:1995-01-01 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::We describe an unusual case of invasive pulmonary aspergillosis (IPA) complicated by subclavian artery occlusion in a 32-year-old man with severe aplastic anemia, who underwent allogeneic stem cell transplantation. He was severely neutropenic after the conditioning for transplantation, but he had no history of fungal ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000203614
更新日期:1997-01-01 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::A rare case of acute promyelcytic leukemia (APL) is reported in a 7-year-old boy. The patient displayed the typical features of APL including impaction of the marrow with promyelocytes, marked elevation of the serum vitamin B12 and transcobalamin I levels and a hemorrhagic diathesis. The bleeding diathesis in the case...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207589
更新日期:1979-01-01 00:00:00
abstract::The increased longevity of patients with thalassemia and sickle cell disease (SCD) introduces new clinical challenges due to the accumulation of disease-related morbidity, psychosocial issues and health care adjustments. Patients with thalassemia major now live into adulthood without suffering heart failure but must c...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000360235
更新日期:2014-01-01 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::A 28-year-old woman with Bence-Jones multiple myeloma (MM) presented with several osteolytic lesions and a massive bone marrow infiltration with mature plasmocytes. After 6 cycles of chemotherapy with melphalan and prednisone, the patient, in apparent clinical remission, underwent allogeneic bone marrow transplantatio...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205969
更新日期:1987-01-01 00:00:00
abstract::Using enzyme-linked immunosorbent assay (ELISA) and confirmatory immunoprecipitation tests, sera from 640 Nigerians from Lagos and Cross River States were examined for antibodies against HIV. These comprised 570 blood donors and their family members, 56 patients with various haematological conditions and 14 patients w...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205728
更新日期:1988-01-01 00:00:00
abstract::The last few years have seen an enormous increase in our knowledge on the haematopoietic growth factor erythropoietin (Epo), firstly with its purification and determination of its primary amino acid sequence, and more recently with the isolation of the Epo gene and its expression in mammalian cell lines. This review a...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000204826
更新日期:1991-01-01 00:00:00