Abstract:
:We describe in this paper a female patient affected by chronic granulomatous disease with all the features of the classic X-linked form of the disease and presenting a mild form of the disease, the major clinical manifestation being a granulomatous cheilitis. The capability of the patient's phagocytes to undergo a respiratory burst in response to different stimuli was markedly depressed and only 10% of the patient's neutrophils were able to reduce nitroblue tetrazolium when stimulated with phorbol myristate acetate to an extent similar to normal cells. With this test, the neutrophils of the patient's mother showed a clear mosaicism, only 40% being able to reduce the dye. Activation of NADPH oxidase in cell-free systems showed that the phagocyte defect was at the level of a membrane component. Difference in spectra revealed that the observed membrane defect was due to a lack of cytochrome b558, the terminal component of NADPH oxidase. Incubation for 2 or 24 h of the patient's neutrophils with human recombinant interferon-gamma and granulocyte macrophage colony-stimulating factor did not correct their defective capability to undergo a respiratory burst However, cultivation of the patient's monocytes with interferon-gamma for prolonged times substantially enhanced their capability to produce hydrogen peroxide.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Dusi S,Poli G,Berton G,Catalano P,Fornasa CV,Peserico Adoi
10.1159/000205028subject
Has Abstractpub_date
1990-01-01 00:00:00pages
49-56issue
1eissn
0001-5792issn
1421-9662journal_volume
84pub_type
杂志文章abstract::A 69-year-old man with a rare case of lymphoma-type alpha-chain disease was admitted to the hospital with marked cervical and inguinal lymph node swelling. Lymph node biopsy showed marked infiltration of plasma cells, plasmacytoid cells and immunoblastoid cells, alone or in combination. Immunoelectrophoresis and immun...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204814
更新日期:1991-01-01 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: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::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::The plasma cortisol levels of 108 children aged 1-16 years with various Hb genotypes (AA, AS and SS) were measured. The mean plasma cortisol levels of both the AS and SS groups (during steady state) fell within the normal range but the mean of the SS group is significantly lower than the AA group. In fact, 9 of the si...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205689
更新日期:1988-01-01 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::With the widespread use of genetic diagnostic technologies, many novel mutations have been identified in hereditary spherocytosis (HS)-related genes, including SPTA1, SPTB, ANK1, SLC4A1, and EPB42. However, mutations in HS-related genes are dispersed and nonspecific in the diagnosis of some HS patients, indicating sig...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000486229
更新日期:2018-01-01 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::Serum IgG, IgM and IgA were determined in 25 patients with homozygous beta thalassemia and 7 with the trait. The levels were increased in homozygous patients and increased further after splenectomy. Serum opsonic activity against Salmonella typhi and staphylococci was impaired in homozygous patients. Splenectomy cause...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206875
更新日期:1983-01-01 00:00:00
abstract::Fourteen patients with hematologic neoplasia (11 acute myeloid leukemias, 2 non-Hodgkin's lymphomas and 1 blast crisis of chronic myeloid leukemia) who underwent high-dose cytosine arabinoside (HIDARAC) therapy with or without sequential asparaginase (ASNase) were investigated in order to evaluate liver toxicity and a...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000206094
更新日期:1986-01-01 00:00:00
abstract::Monocytoid B cell lymphoma (MBCL) is an immunologically and morphologically well-defined low-grade lymphoma with a predilection for lymph nodes of the parotid region. We describe an association of MBCL with anti-myelin-associated glycoprotein (MAG) polyneuropathy in a 53-year-old male. The diagnosis of stage IV MBCL w...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000046603
更新日期:2001-01-01 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::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
abstract::B-cell chronic lymphocytic leukaemia (B-CLL) is characterized by a high frequency of infections, including those of viral aetiology. Previous reports have demonstrated a specific immunologic response to influenza virus vaccine in B-CLL patients with normal IgG levels. In this study, we have evaluated different immunop...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040709
更新日期:1998-01-01 00:00:00
abstract::The dilution in the circulating blood of lymphocytes lablled in vitro with 3H-cytidine was examined after autotransfusion in 9 patients with Hodgkin's disease (HD) stage II A-IV B, 5 of whom were untreated; in 2 untreated patients with carcinoma, and in 1 treated patient with scleroderma. The blood transit time of exc...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208185
更新日期:1975-01-01 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::A 56-year-old male presented with inguinal lymphadenopathy and leucocytosis (WBC 98 x 10(9)/l). Bone marrow morphology showed myeloid hyperplasia, with eosinophilia. Cytogenetic analysis showed no evidence of the Philadelphia chromosome, and fluorescence in situ hybridisation studies for the BCR/ABL fusion were negati...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000046638
更新日期:2002-01-01 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::In 34 patients with chronic lymphatic leukaemia (CLL) the lymphocytes have been separated and sized using a C1000 Channelyzer. The modal volume and the volume range of the populations have been obtained and related to clinical stage and mouse red blood cell (MRBC) rosetting capacity. Over 1 year's observation with sev...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206714
更新日期:1983-01-01 00:00:00
abstract::We report on a patient with chronic myeloid leukemia (CML) with a rapidly growing left cervical tumor 5 months after the initial diagnosis of CML. This tumor was diagnosed as a very early manifestation of extramedullary myeloblastoma by a minimally invasive method. A fine needle aspirate (26-gauge needle) was obtained...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040873
更新日期:1998-01-01 00:00:00
abstract::Slow intravenous infusion of Na laurate (NaL) into guinea pigs caused a rapid appearance of platelet aggregates in the arterial blood and a precipitous fall in platelet counts. During the infusion of Na arachidonate (NaA) thrombocytopenia developed slowly, and few and smaller platelet aggregates appeared in the arteri...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207864
更新日期:1977-01-01 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::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::All-trans-retinoic acid (ATRA) combined with anthracyclines is currently the standard treatment for acute promyelocytic leukemia (APL). In elderly patients the presence of comorbidities, such as cardiomyopathy or different organ failures, often represents an absolute contraindication to standard chemotherapy. In this ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000097880
更新日期:2007-01-01 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::Treatment of chronic myeloid leukemia (CML) and Philadelphia chromosome-positive acute leukemia (Ph+ ALL) has been revolutionized with the advent of tyrosine kinase inhibitors (TKIs). Most patients with CML achieve long-term survival similar to individuals without CML due to treatment with TKIs not only in frontline b...
journal_title:Acta haematologica
pub_type: 共识发展会议,杂志文章,评审
doi:10.1159/000501927
更新日期:2020-01-01 00:00:00
abstract::RAS mutations are found in about 25% of acute myeloid leukemia (AML) cases. The importance of these changes is unknown. If RAS mutations confer growth advantage to leukemia subclones in which they emerge, substantially more nonconservative than conservative mutations should be found. The incidence of conservative muta...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204200
更新日期:1994-01-01 00:00:00
abstract::A 38-year-old Japanese man had suffered from trichophyton infection for several years. The white blood cell count was 20,300/mm3, including 54% abnormal lymphocytes with irregularly convoluted nuclei. Adult T-cell leukemia (ATL) was diagnosed based on proliferation of CD4-positive lymphocytes, positive anti-HTLV-I ant...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204727
更新日期:1992-01-01 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::We examined the in vivo effect of granulocyte colony-stimulating factor (G-CSF) on the surface expression of putative counterligands for endothelial selectins on neutrophils in healthy volunteers. G-CSF (50 microg/m2/day) was administered subcutaneously to 5 healthy volunteers for 4 days. The expression of surface ant...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000040901
更新日期:1998-01-01 00:00:00