Abstract:
:In most patients with large granular lymphocyte (LGL) leukemia, the disease appears to progress slowly if at all, and no therapy is generally required. We present a patient with CD3+ CD8+ CD16+ LGL leukemia, who showed a benign clinical course for more than 7 months without therapy, but subsequently developed aggressive disease. A feature of considerable interest was the transformation into an acute or aggressive form in this patient affected preferentially the liver and spleen, and was not associated with the hematologic features of the circulating leukemic cells, as assessed by morphologic, phenotypic and molecular analyses. The patient was treated with combination chemotherapy; this resulted in clinical remission with persistence of the abnormal clone in the peripheral blood. The mechanism responsible for the preferential organ involvement and the process of progression from the chronic to the acute form in this case are discussed from the viewpoint of a lymphoproliferative disorder.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Matsubara A,Matsumoto M,Takada K,Hato T,Hasegawa H,Tamai T,Yasukawa M,Fujita Sdoi
10.1159/000204336subject
Has Abstractpub_date
1994-01-01 00:00:00pages
206-10issue
4eissn
0001-5792issn
1421-9662journal_volume
91pub_type
杂志文章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::The course and management of avascular necrosis of the femoral head (AVNFH) in six hips of 5 sickle cell syndrome patients (3 with Hb SS, 1 with Hb SC and 1 with Hb S/beta+-thalassaemia) are described. Two patients (aged 13 and 17 years) presented with Perthes- and osteochondritis dessicans-type lesions. These hips pr...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205873
更新日期:1987-01-01 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::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::Afibrinogenaemia in a female Saudi child is reported. Two siblings died of bleeding. The parents, who are first cousins, a brother and two sisters are asymptomatic heterozygotes with fibrinogen levels of less than 1.5 g/l. The presentation was described and compared to other cases reported. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206624
更新日期:1984-01-01 00:00:00
abstract::The effect of 3 different detergents (Triton X-100, Lubrol WX and Nonidet) on brain thromboplastin was examined and compared with the solubilisation due to deoxycholate. The effect of deoxycholate is superior over that of the other examined detergents. Deoxycholate extracts thromboplastic activity from brain tissue to...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208068
更新日期:1975-01-01 00:00:00
abstract::A patient developed a drug rash and neutropenia while receiving tobramycin, ticarcillin and flucloxacillin intravenously for osteomyelitis. Incorporation of these antibiotics into in vitro cultures of bone marrow granulocyte macrophage precursors (CFU-C) showed no inhibition of the patient's marrow or normal marrow by...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206794
更新日期:1983-01-01 00:00:00
abstract::Treatment options are limited for patients with advanced forms of myeloproliferative neoplasms (MPN) including blast-phase disease (MPN-BP). Decitabine has frequently been deployed but its efficacy and safety profile are not well described in this population. We retrospectively reviewed 42 patients treated with decita...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000506146
更新日期:2021-01-01 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::Three consecutive patients considered to have end-stage acquired aplastic anaemia were given 100-160 mg/kg antilymphocyte globulin (ALG) i.v. followed by an infusion of 2-3.8 x 10(8) nucleated marrow cells/kg i.v. from HL-A one haplotype-identical, MLC-positive family donors. All patients showed autologous marrow reco...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208005
更新日期:1976-01-01 00:00:00
abstract::Two patients with paraneoplastic cerebellar degeneration accompanying Hodgkin's disease were treated with plasma exchange in combination with chemotherapy or radiation therapy, respectively. Significant improvement in neurologic symptoms was obtained in 1 of them. ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204012
更新日期:1995-01-01 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
abstract::Agranulocytosis is a rare side effect of phenytoin treatment. We describe the case of an elderly man who developed agranulocytosis 2 weeks following initiation of phenytoin treatment, on no cytotoxic drugs or any other medications except decadron. The white blood cell count was 300/mm3 with absent granulocytes. The li...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204838
更新日期:1991-01-01 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::Platelet aggregation induced in vitro with ADP, adrenalin and ristocetin was tested in 7 patients with megakaryoblastic leukemia (MKL). All patients had normal or high platelet counts and presented with hemorrhagic diathesis including purpura ecchymosis and epistaxis. Platelet morphology was grossly abnormal and elect...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206366
更新日期:1984-01-01 00:00:00
abstract::The transcription factors GATA-1 and GATA-2 play key roles in gene regulation during erythropoiesis. Gene ablation studies in mouse revealed that GATA-2 is crucial for the maintenance and proliferation of immature hematopoietic progenitors, whereas GATA-1 is essential for the survival of erythroid progenitors as well ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000065660
更新日期:2002-01-01 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 studied neutrophil chemotaxis and surface membrane glycoproteins in 12 patients suffering from myeloid disorders with abnormal karyotype using in vitro techniques in all 12. Chromosome studies were also carried out virtually simultaneously. We chose to study only patients showing a deficit of chemotaxis (p less tha...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205543
更新日期:1989-01-01 00:00:00
abstract::Human urinary erythropoietin has been purified to homogeneity. The seven-step procedure yielded a preparation with a potency of 225,000 U/mg protein. SDS-polyacrylamide gel electrophoretic analysis of the purified hormone revealed a single protein band with a molecular weight of about 35,000 that migrated with the bio...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205057
更新日期:1990-01-01 00:00:00
abstract::We report a patient with myelodysplastic syndrome (MDS), refractory anaemia with excess blasts in transformation, in whom complete remission (CR) was achieved with the administration of granulocyte colony-stimulating factor (G-CSF). The 76-year-old patient was admitted to our hospital with a fever and a productive cou...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000057637
更新日期:2002-01-01 00:00:00
abstract::A 66-year-old Japanese woman was referred to us because of severe anemia and fever and presented at our hospital. She was eventually diagnosed as having acute myeloblastic leukemia (AML; M0) with non-Hodgkin lymphoma (NHL). We investigated the therapeutic efficacy of L-asparaginase (L-Asp), vincristine and prednisolon...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000243725
更新日期:2009-01-01 00:00:00
abstract::Premature neonates require blood transfusions, and biological parents may wish to be directed donors. Biological mothers pose a potential danger because their plasma may contain antibodies that will react with blood cell antigens inherited by the infant from the father. We studied 25 healthy, pregnant women at the tim...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204889
更新日期:1991-01-01 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::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::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::Serum folate levels and unsaturated folate-binding capacity (UFBC) in a mixed population of outpatients and inpatients were analyzed with respect to race, sex, and age. The results supported the relatively high prevalence of subnormal serum folates in patient populations, did not find lower serum folates in the elderl...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206909
更新日期:1983-01-01 00:00:00
abstract:BACKGROUND AND OBJECTIVES:Umbilical cord blood (UCB) contains haematopoietic stem cells and can be used as an alternative to bone marrow transplantation in certain cases. Engraftment was dependent upon the haematopoietic progenitor cell content of the cord blood units. This study was designed to investigate the influen...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000084677
更新日期:2005-01-01 00:00:00
abstract::Patients with sickle cell disease have an increased tendency to develop frequent and severe pyogenic infections. Lymphocyte subpopulations were studied in 14 patients with homozygous sickle cell anaemia (SCA) using the OKT monoclonal antibody. The mean absolute lymphocyte counts observed in patients with SCA were simi...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206155
更新日期:1985-01-01 00:00:00
abstract::Interferon-alpha has been used as standard therapy for patients with Philadelphia-positive chronic myeloid leukemia (CML) for more than 20 years. Recently randomized trials have shown a superiority of the tyrosine kinase inhibitor imatinib in respect to its efficacy to induce complete hematological and cytogenetic rem...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000089476
更新日期:2006-01-01 00:00:00
abstract::In a 42-year-old Caucasian female, acute myelocytic leukemia (M2) presented as occlusion of the distal aorta and right iliac artery. She died after 10 days of respiratory failure. At postmortem examination the aortic wall contained multiple thrombi composed of fibrin and leukemic cells. There was no evidence of diffus...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206100
更新日期:1986-01-01 00:00:00