Abstract:
:A 64-year-old woman with mild bilateral parotid gland swelling and bilateral lower extremity purpura was admitted for evaluation of xerostomia and pancytopenia. The patient had an increased erythrocyte sedimentation rate, pancytopenia, and positive tests for antibodies to nuclear antigen, SS-A, and SS-B. Impaired cell-mediated immunity was also present. Bone marrow aspiration showed a hypoplastic marrow with an increased percentage of lymphocytes. A positive Schirmer's test and keratoconjunctivitis were also noted. A diagnosis of primary Sjögren's syndrome was made by sialography and histological salivary gland findings. Therapy with prednisolone improved the pancytopenia. Addition of the patient's peripheral blood mononuclear cells to cultures of bone marrow mononuclear cells derived from a healthy volunteer dose dependently inhibited colony formation, including mixed hemopoietic colonies. On the other hand, addition of the patient's serum failed to inhibit colony formation by normal bone marrow mononuclear cells. These results suggested that one of the causes of pancytopenia in primary Sjögren's syndrome might be mediated by the inhibition of mononuclear cells to the hemopoietic progenitors.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Seki T,Kiyosawa K,Monno S,Imai Y,Furukawa K,Kumazawa S,Ota M,Saito H,Furuta S,Nakahata Tdoi
10.1002/ajh.2830430312subject
Has Abstractpub_date
1993-07-01 00:00:00pages
221-5issue
3eissn
0361-8609issn
1096-8652journal_volume
43pub_type
杂志文章abstract::Immunoglobulin (Ig) light chain amyloidosis is a clonal but nonproliferative plasma cell disorder in which fragments of an Ig light chain are deposited in tissues. The clinical features depend on the organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, and peripheral/autonom...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21934
更新日期:2011-02-01 00:00:00
abstract::Despite advances in chemotherapeutic agents, the prognosis for some cancers remains extremely poor, suggesting the need for other treatment modalities. Immunotherapy appears an ideal approach because the mechanisms of tumor cell killing induced by tumor vaccines are different from those from chemotherapy. Various inve...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20415
更新日期:2005-09-01 00:00:00
abstract::At sites of vascular injury, the platelet collagen receptor Glycoprotein Ia/IIa (GPIa/IIa) acts as an important mediator of platelet adhesion to fibrillar collagens. Two silent polymorphisms (807C/T and 873G/A) within the glycoprotein Ia gene have been implicated in increased risk of developing thrombosis and myocardi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10029
更新日期:2002-01-01 00:00:00
abstract::We describe 3 episodes of microangiopathic hemolytic anemia (MAHA) in 2 solid organ recipients under FK506 (tacrolimus) therapy. In both cases, discontinuation of FK506 and treatment with plasma exchange, fresh frozen plasma replacement, corticosteroids, aspirin, and dipyridamole led to resolution of MAHA. In one pati...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(SICI)1096-8652(199608)52:4<310::AID-AJH11
更新日期:1996-08-01 00:00:00
abstract::With the emerging success of treating CD19 expressing B cell malignancies with ex vivo modified, autologous T cells that express CD19-directed chimeric antigen receptors (CAR), there is intense interest in expanding this evolving technology to develop effective modalities to treat other malignancies including solid tu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24794
更新日期:2017-09-01 00:00:00
abstract::Although large vessel thrombi are occasionally reported in patients with homozygous sickle cell disease, the role of intravascular coagulation in typical pain crises is controversial. Therefore, we studied 24 sickle cell patients during and between episodes of pain crisis, using several sensitive tests of hemostasis. ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830230403
更新日期:1986-12-01 00:00:00
abstract::Among a cohort of 142 patients with Hodgkin disease (HD), pathologic stages (PS) IA through IVB, 84 remained in complete remission (CR) at least 3 years after the completion of initial protocol therapy. Eight of these patients subsequently developed a recurrence of HD and were defined as having a late relapse. The dis...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830100106
更新日期:1981-01-01 00:00:00
abstract::We report a patient with chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) who presented with rapid enlargement of a cervical lymph node due to localized herpes simplex lymphadenitis, which was clinically indistinguishable from large cell (Richter's) transformation. The diagnosis was made by excisional...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10006
更新日期:2001-12-01 00:00:00
abstract::AL amyloidosis, a systemic disorder characterized by widespread deposition of amyloid fibrils derived from monoclonal Ig light chains in organs and soft tissues, is typically caused by an underlying plasma cell dyscrasia. However, this disease can also be associated rarely with a B-cell lymphoproliferative disorder. I...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20635
更新日期:2006-09-01 00:00:00
abstract::The detection and identification of blood group antibodies in patients is crucial for successful allogeneic blood transfusions. Current methods are highly subjective and rely on red blood cells (RBCs), which simultaneously express many blood group antigens, have a short shelf-life, and carry potential biohazard risks....
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(200003)63:3<114::aid-ajh2>
更新日期:2000-03-01 00:00:00
abstract::The prevention of Rhesus D alloimmunization through Rh immune globulin (RhIg) administration is the major indication for the accurate detection and quantification of fetomaternal hemorrhage (FMH). In the setting of D incompatibility, D-positive fetal cells can sensitize the D-negative mother, resulting in maternal ant...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.22255
更新日期:2012-04-01 00:00:00
abstract::Reduced ferrochelatase activity in erythropoietic protoporphyria (EPP) causes the accumulation of protoporphyrin IX (PPIX) leading to acute cutaneous photosensitivity and liver injury. Many EPP patients also have a mild hypochromic, microcytic anemia and iron deficiency. Iron deficiency can lead to decreased PPIX accu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.25743
更新日期:2020-05-01 00:00:00
abstract::An infectious episode is the presenting manifestation of hairy cell leukemia (HCL) in approximately 30% of cases. Most often this is bacterial and only rare cases of opportunistic fungal infection are described. We report a patient who presented with sporotrichal involvement of multiple cutaneous sites and lymph nodes...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460214
更新日期:1994-06-01 00:00:00
abstract::In May 2005 at the 8th International Symposium on Myelodysplastic Syndromes (MDS), a consensus meeting was held on iron overload in MDS (Seymour, Hematol Oncol Clin 2005; Suppl 1:18-25). The recommendations of the 2005 consensus meeting were discussed in the context of currently available evidence at the 9th Internati...
journal_title:American journal of hematology
pub_type: 杂志文章,实务指引
doi:10.1002/ajh.21269
更新日期:2008-11-01 00:00:00
abstract::Four factor XI (F XI)-deficient patients are described, all of whom formed circulating anticoagulants against F X1. In the three most severely affected patients (F XI 0%-6% activity), the anticoagulant appeared to have been stimulated by plasma infusion. However, in the milder case (25% F XI activity), no infusion had...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830260405
更新日期:1987-12-01 00:00:00
abstract::Defective fibrinolysis due to decreased tissue-type plasminogen activator (t-PA) activity is a well-established finding in patients with systemic lupus erythematosus (SLE). The possibility that this decrease in t-PA activity may be related to the presence of autoantibodies directed against t-PA, and the possible role ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830490203
更新日期:1995-06-01 00:00:00
abstract:DISEASE OVERVIEW:Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS), now classified under myelodysplastic syndromes with RS (MDS-RS) and RARS with thromboc...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.24637
更新日期:2017-03-01 00:00:00
abstract::A single point mutation of the factor V (FV) gene, leading to the substitution Arg506Gln in the FV molecule (FV-Leiden) and hence resistance to its breakdown by activated protein C (APC), is the most prevalent risk factor for venous thrombosis in the Caucasians. A ratio determined by activated partial thromboplastin t...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199703)54:3<214::aid-ajh7>
更新日期:1997-03-01 00:00:00
abstract::The objective of this case-matched study was to compare the efficacy and toxicity of the addition of clarithromycin (Biaxin) to lenalidomide/low-dose dexamethasone (BiRd) vs. lenalidomide/low-dose dexamethasone (Rd) for newly diagnosed myeloma. Data from 72 patients treated at the New York Presbyterian Hospital-Cornel...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1002/ajh.21777
更新日期:2010-09-01 00:00:00
abstract::Immunologic studies were performed on mononuclear cells from ten chronically transfused children with sickle cell disease, and the results were compared with those from five other groups: 21 sickle cell patients who were not receiving regular transfusions, 6 chronically transfused children with other forms of refracto...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830200106
更新日期:1985-09-01 00:00:00
abstract::A rare association of congenital afibrinogenemia and hereditary protein C deficiency is described in a 37-year-old female who suffered from ischemic necrosis in the left first toe. The diagnosis of afibrinogenemia was assessed by the absence of fibrinogen in clotting and immunological assays. The diagnosis of heredita...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830410111
更新日期:1992-09-01 00:00:00
abstract::It has been suggested that alterations of cell cycle genes probably contribute to the pathogenesis of endemic Burkitt's lymphoma (BL) in addition to c-MYC translocation. Mutations disrupting the normal nuclear localization signal of the retinoblastoma-related gene Rb2/p130 have been documented in BL cell lines and pri...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20749
更新日期:2007-03-01 00:00:00
abstract::Iron deposition in combination with inflammatory and immunogenetic factors is involved in the pathophysiology of cardiac dysfunction in β-thalassemia major. We investigated the mechanical and endocrine function of the left atrium and ventricle to identify early signs of dysfunction. We studied 90 patients (mean age: 2...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23581
更新日期:2014-01-01 00:00:00
abstract::Argentine hemorrhagic fever (AHF) is a viral disease caused by Junin virus and characterized by hematologic and neurological involvement. The main hematologic features are leukopenia, thrombocytopenia, and bone marrow hypoplasia. Hematopoietic growth factors serum levels were measured by ELISA technique in forty-eight...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(200005)64:1<1::aid-ajh1>3.
更新日期:2000-05-01 00:00:00
abstract::The arachidonic acid metabolites produced by human peripheral blood monocytes were studied to determine which metabolites could have a role in thrombogenesis. Monocytes were found to be free of platelets by scanning electron microscopy and by measurement of 12-HETE. Human peripheral blood monocytes produce thromboxane...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830310302
更新日期:1989-07-01 00:00:00
abstract::A 75-year-old female patient with agranulocytosis caused by ticlopidine is reported. She took the drug at 200 mg/day for 30 days to prevent recurrence of cerebral infarction. The leukocyte count at the nadir was 500/microliters on the 34th day since she started to take the drug. Complete recovery of her peripheral leu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370405
更新日期:1991-08-01 00:00:00
abstract::Thrombocytopenia is a poor prognostic indicator in the myelodysplastic syndromes (MDS). Treatment options for patients with symptomatic thrombocytopenia are limited. Danazol, an attenuated androgen, may have some efficacy in increasing the platelet count of patients with MDS. We retrospectively reviewed 33 patients wi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10209
更新日期:2002-11-01 00:00:00
abstract::A 59-year-old woman, after complete recovery from an episode of drug-induced agranulocytosis, was found to sustain a chronic absence of recognizable mature and immature eosinophils in blood and bone marrow during a follow-up period of 8 years. Her bone marrow and peripheral blood cells cultured in vitro were able to p...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120211
更新日期:1982-04-01 00:00:00
abstract::Although infectious complications represent a relevant cause of morbidity and mortality in patients with myelofibrosis (MF), little is known about their incidence, outcome and risk factors. We retrospectively evaluated a cohort of 507 MF patients, diagnosed between 1980 and 2014 in five Italian hematology centers, to ...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.24572
更新日期:2017-01-01 00:00:00
abstract::Autologous stem cell transplantation (ASCT) is feasible and effective in selected older patients with Multiple Myeloma, but specific criteria for evaluating ASCT eligibility in elderly patients are lacking. We evaluated 131 patients aged 65-75 considered for ASCT at our center: The Charlson Comorbidity Index (CCI), He...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章
doi:10.1002/ajh.25797
更新日期:2020-07-01 00:00:00