Abstract:
:Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is a rare lymphoproliferative disorder characterized by diffuse lymphadenopathy, fever, hepatosplenomegaly, hemolytic anemia, and polyclonal hypergammaglobulinemia. Morphologically, the involved lymph nodes demonstrate complete effacement of the normal architecture, prominent neovascularization and infiltration by immunoblasts and plasma cells. Other terms that have been used to describe this entity include diffuse plasmacytic sarcomatosis, immunoblastic lymphadenopathy, lymphogranulomatosis X, and immunologic aberrations in idiopathic reticulosis. Initially, AILD was thought to be a disease of B-cell origin that represented reactive immune response to unknown stimulus and high potential for malignant transformation. It is now evident that AILD in 80% of cases follows an aggressive course with short median survival, especially, if complete response with chemotherapy is not achieved. Immunologic and molecular studies have demonstrated that the majority of AILD cases are T-cell clonal disorders. Despite the numerous reports on the role of Epstein-Barr virus in this disorder, it is unknown whether the presence of this virus is associated with the immune defect that accompanies AILD, or whether it is a pathogenetic factor. In contrast to non-Hodgkin's lymphomas, a stage is not usually assigned to the patient since the disease is systemic in nature, subsequently, parameters such as extent of disease and tumor bulk used to identify high-risk patients with non-Hodgkin's lymphomas, do not appear to correlate with disease activity or prognosis in AILD. Treatment of AILD has been unsatisfactory, with approximately 25% of patients achieving complete and sustained remission when combined chemotherapy agents are used. This article is devoted to a discussion of the different manifestations, suggested pathogenesis, and treatment of AILD.
journal_name
Acta Haematoljournal_title
Acta haematologicaauthors
Sallah S,Gagnon GAdoi
10.1159/000040811subject
Has Abstractpub_date
1998-01-01 00:00:00pages
57-64issue
2eissn
0001-5792issn
1421-9662pii
40811journal_volume
99pub_type
杂志文章,评审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::In a dose titration study we tested the efficacy and tolerance of recombinant human erythropoietin (rhEPO) in 10 patients with myelodysplasia (MDS) and 2 patients with idiopathic myelofibrosis. Patients with a haemoglobin level < 100 g/l were treated as out-patients for 12 weeks with daily doses ranging from 30 U/kg b...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204378
更新日期:1993-01-01 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::34 patients with myocardial infarction were studied with daily measurements of beta-thromboglobulin (BTG) and 125I fibrinogen scanning in order to detect deep-venous thrombosis (DVT). Serial levels of BTG were unhelpful in the early detection of this condition, which occurred in 7 of the patients studied. 5 of the pat...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000207238
更新日期:1980-01-01 00:00:00
abstract::Aspirates from bone marrow, spleen and liver were morphologically analysed in 15 untreated patients wich chronic myeloid leukaemia. Megaloblastic changes of the erythroblasts were found to be more common in the spleen and liver than in the bone marrow. A significant increase of 'erythroblastic islands', i.e. erythrobl...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208026
更新日期:1976-01-01 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::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::A new case of IgE myeloma is described. A 77-year-old woman presented with bone pain and fatigue. Serum protein analysis revealed a paraprotein of the IgE kappa type; bone marrow aspirate and immunofluorescence confirmed the diagnosis; ultrastructural examination showed immature plasma cells. Treatment with prednisone...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000204851
更新日期:1991-01-01 00:00:00
abstract::Essential thrombocythemia (ET), one of the chronic myeloproliferative disorders, is a clonal disorder of multipotent stem cells. Although most patients with ET have a prolonged benign course, a minority of patients may develop a blastic crisis similar to chronic myelogenous leukemia (CML). A case of ET terminating in ...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000204260
更新日期:1994-01-01 00:00:00
abstract::Non-transfusion-dependent thalassemia (NTDT) is associated with various forms of thalassemia and genetic modifiers. We report the molecular basis of NTDT in hemoglobin (Hb) E-β-thalassemia disease. This study was done in 73 adult patients encountered at the prenatal diagnosis center of Khon Kaen University, Northeast ...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000449120
更新日期:2016-01-01 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::The effect of methylprednisolone on fresh cells from patients with chronic lymphocytic leukaemia (CLL) has been studied using the differential staining cytotoxicity (DiSC) assay resulting in LC90s of < or = 0.2 to 2,000 micrograms/ml. Cells from previously treated patients were, on average, significantly more sensitiv...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章
doi:10.1159/000204115
更新日期:1995-01-01 00:00:00
abstract::A 69-year-old woman with typical thrombotic thrombocytopenic purpura is reported, who was treated successfully by plasma exchange. An initial chromosomal analysis of peripheral blood lymphocytes revealed abnormal patterns of 45, XO/46, XX/47, XXX; a second analysis showed 45, XO/46, XX/47, XXX/47, XX + 21, and a third...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205068
更新日期:1990-01-01 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::The increased incidence of parallel tubular structures in lymphocytes of patients with Hodgkin's disease was investigated for a correlation with either impairment of cellular immunity (measured by DNCB-skin test and PHA-induced lymphocyte stimulation in vitro) or an increase of antibodies against cytomegalovirus or Ep...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000208056
更新日期:1975-01-01 00:00:00
abstract::Herein, we describe a case of a female patient in whom B cell chronic lymphocytic leukemia (CLL) and mantle cell lymphoma (MCL) were diagnosed simultaneously. She presented with anemia, thrombocytopenia and splenomegaly. Flow cytometry demonstrated two immunophenotypically distinct CD5-positive monoclonal B cell popul...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000324193
更新日期:2011-01-01 00:00:00
abstract::A 3-year-old child developed severe aplastic anaemia following hepatitis A. Since no HLA-compatible donor was available, he was treated with oxymetholone, antithymocytic globulin and methylprednisolone, but no haematologic recovery was observed and he consequently died of pneumonia. Although the association of aplasti...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000206061
更新日期:1986-01-01 00:00:00
abstract:BACKGROUND:Our aim is to determine comorbidities associated with pulmonary hypertension (PHT) in clinically stable sickle-cell disease (SCD) patients and to evaluate left ventricular (LV) and right ventricular (RV) function in those patients. METHODS:Echocardiography was performed in 87 SCD patients that were divided ...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000102588
更新日期:2007-01-01 00:00:00
abstract::An improved plasma clot culture method for CFU megakaryocytes (MK) has been developed with a higher plating efficiency and easier identification and enumeration of MK colonies by an indirect immunoperoxidase staining using a monoclonal antibody specific to the platelet glycoprotein IIb/IIIa complex. This technique has...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205837
更新日期:1987-01-01 00:00:00
abstract::We report a patient with lung cancer who developed CD56-positive acute lymphoblastic leukemia. He was referred to our hospital for thrombocytopenia. Atypical cells were found in the blood and the bone marrow. These cells were immunophenotypically positive for CD3epsilon, CD56, and terminal deoxynucleotidyl transferase...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000087891
更新日期:2005-01-01 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 variety of adhesion receptors are expressed on the blast cells in patients with acute myelogenous leukemia. The panel of receptors expressed demonstrates heterogeneity just as there is morphologic, histochemical, cytogenetic, and molecular genetic variation between various cases of acute myelogenous leukemia. The ad...
journal_title:Acta haematologica
pub_type: 杂志文章,评审
doi:10.1159/000203659
更新日期:1997-01-01 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::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::Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is an interferon (IFN)-induced molecule with apoptotic activity. We examined gene mutations in the death domains of TRAIL receptor 1 (TRAIL-R1) and TRAIL receptor 2 (TRAIL-R2), and in the TRAIL gene promoter in 46 chronic myelogenous leukemia (CML) patien...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000083449
更新日期:2005-01-01 00:00:00
abstract::A patient with common acute lymphoblastic leukaemia (ALL), hypereosinophilic syndrome and t(5;14) (q31.1;q32.3) translocation is described. Even with intensive treatment only short periods of complete remission were achieved. Recurrence of the leukaemia was always accompanied by the appearance of eosinophilic granuloc...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000205289
更新日期:1989-01-01 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::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::Twenty-four infants and children suffering from glucose-6-phosphate dehydrogenase (G6PD) deficiency during hemolytic crisis were included in this study. Their ages ranged between 3 and 36 months with a median of 10 months. 22 were males and 2 were females. Fourteen out of them received a single bolus dose of desferrio...
journal_title:Acta haematologica
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000205320
更新日期:1989-01-01 00:00:00
abstract::Downregulation of apoptosis has been proposed as a mechanism of clonal expansion in low-grade B cell neoplasms. We have previously described an unusual case of CD5+ B cell lymphoma characterized by cycles of leukemic phase alternating with spontaneous remission. In the present study, we examined the involvement of apo...
journal_title:Acta haematologica
pub_type: 杂志文章
doi:10.1159/000041046
更新日期:2000-01-01 00:00:00