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 thrombocytosis (RARS-T); now called myelodysplastic/myeloproliferative neoplasm with RS and thrombocytosis (MDS/MPN-RS-T). DIAGNOSIS:MDS-RS is a lower risk MDS, with single or multilineage dysplasia (SLD/MLD), <5% bone marrow (BM) blasts and ≥15% BM RS (≥5% in the presence of SF3B1 mutations). MDS/MPN-RS-T, now a formal entity in the MDS/MPN overlap syndromes, has diagnostic features of MDS-RS-SLD, along with a platelet count ≥ 450 × 10(9)/L and large atypical megakaryocytes (similar to BCR-ABL1 negative MPN). MUTATIONS AND KARYOTYPE:Mutations in SF3B1 are seen in ≥80% of patients with MDS-RS-SLD and MDS/MPN-RS-T, and strongly correlate with the presence of BM RS; MDS/MPN-RS-T patients also demonstrate JAK2V617F, ASXL1, DNMT3A, SETBP1, and TET2 mutations; with ASXL1/SETBP1 mutations adversely impacting survival. Cytogenetic abnormalities are uncommon in both diseases. RISK STRATIFICATION:Most patients with MDS-RS-SLD are stratified into lower risk groups by the revised-International Prognostic Scoring System (R-IPSS). Disease outcome in MDS/MPN-RS-T is better than that of MDS-RS-SLD, but worse than that of essential thrombocythemia. Both diseases have a low risk of leukemic TREATMENT: Anemia and iron overload are complications seen in both and are managed similar to lower risk MDS and MPN. Aspirin therapy is reasonable in MDS/MPN-RS-T, especially in the presence of JAK2V617F, but the value of platelet-lowering drugs is uncertain.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Patnaik MM,Tefferi Adoi
10.1002/ajh.24637subject
Has Abstractpub_date
2017-03-01 00:00:00pages
297-310issue
3eissn
0361-8609issn
1096-8652journal_volume
92pub_type
杂志文章,评审abstract::Hemostatic changes were evaluated in ten patients with acute lymphoblastic leukemia and lymphoma who received chemotherapy with L-asparaginase, vincristine, and prednisolone for 1 week. Following treatment, prothrombin time and activated partial thromboplastin time were significantly prolonged, while a marked decrease...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830320105
更新日期:1989-09-01 00:00:00
abstract::Cyclooxygenases (COX) are key enzymes in the conversion of arachidonic acid to prostaglandins. Several studies have shown a relation between angiogenesis and COX-2 expression. Elevated expression of cyclooxygenase-2 (COX-2), however, has not been reported in multiple myeloma (MM) in the literature. The aim of this stu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20460
更新日期:2005-11-01 00:00:00
abstract::The efficacy of the three common intra- and extragenic polymorphic sites of the factor VIII and IX genes has been examined in the Indian population, with an aim to develop a strategy that would be accurate and informative, yet economical. The approach for hemophilia A carrier detection includes tests for BclI, XbaI, a...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199704)54:4<271::aid-ajh2>
更新日期:1997-04-01 00:00:00
abstract::Karyotype is frequently used to predict response and outcome in leukemia. This post hoc exploratory analysis evaluated the relationship between baseline cytogenetics and outcome in patients with relapsed/refractory acute lymphoblastic leukemia (R/R ALL) treated with inotuzumab ozogamicin (InO), a humanized CD22 antibo...
journal_title:American journal of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ajh.25394
更新日期:2019-04-01 00:00:00
abstract::Congenital malformations of the inferior vena cava (IVC) are uncommon and may be associated with an increased risk of venous thrombosis. We report the case of a man with congenital absence of the IVC and remote history of deep venous thrombosis who now presents with severe abdominal wall superficial thrombophlebitis. ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21089
更新日期:2008-03-01 00:00:00
abstract::Seventeen adults received the antifibrinolytic drug tranexamic acid during cardiac surgery utilizing extracorporeal circulation (ECC). In 8 patients, drug administration began prior to skin incision (pre-ECC); infusions commenced after ECC and protamine administration in another 9 patients (post-ECC). Compared with th...
journal_title:American journal of hematology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ajh.2830380208
更新日期:1991-10-01 00:00:00
abstract::Iron studies were compared in 434 patients from 80 hemochromatosis families classified as putative homozygotes, heterozygotes, and normal by HLA typing. There were 28 of 255 (11%) heterozygotes with an elevated serum ferritin and 22 of 255 (8.6%) with an elevated transferrin saturation. Serum ferritin (140 +/- 10.2 mi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830450210
更新日期:1994-02-01 00:00:00
abstract::Agnogenic myeloid metaplasia (AMM) is a chronic myeloproliferative disorder arising from a single hematopoietic cell. Approximately 5% of reported cases of AMM have terminated in leukemic crisis; however, the precise characteristics of the leukemic cells have rarely been reported. We report a case of AMM that occurred...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830440111
更新日期:1993-09-01 00:00:00
abstract::Conventional treatment of mantle cell lymphoma (MCL) yields modest responses and short remissions. We report 30 hematopoietic stem cell transplants (HSCT) for MCL: 13 autologous, 10 allogeneic myeloablative, and 7 nonablative. After a median 1.2 years from diagnosis (range 0.5 to 4.7) and a median of 2 pre-HSCT chemot...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20646
更新日期:2006-07-01 00:00:00
abstract::Polycythemia vera (PV) is associated with a high incidence of thrombosis. The association of apparent and secondary polycythemia with thrombosis is not clear. It was suggested that activation of the coagulation system contributes to thrombus formation in PV. However, the mechanism of activation is unknown. Monocytes g...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199709)56:1<5::aid-ajh2>3.
更新日期:1997-09-01 00:00:00
abstract:DISEASE OVERVIEW:Hodgkin lymphoma (HL) is an uncommon B-cell lymphoid malignancy affecting 8500 new patients annually and representing approximately 10.2% of all lymphomas in the United States. DIAGNOSIS:HL is composed of two distinct disease entities: classical HL and nodular lymphocyte predominant HL. Nodular sclero...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25071
更新日期:2018-05-01 00:00:00
abstract::Sixty-two previously untreated patients with B-cell chronic lymphocytic leukaemia were analysed to study the prognostic value of both the immunologic phenotype and the clinicobiologic characteristics. Univariate studies showed that none of the immunological markers analysed, sheep-rosette, mouse-rosette, slg, and HLA/...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830310105
更新日期:1989-05-01 00:00:00
abstract::Primary iron overload encompasses a variety of genetic iron overload syndromes, dominated in frequency by HFE-related, or Type 1 hemochromatosis, for which French diagnostic and therapeutic guidelines have been recently proposed. Differential diagnosis of Type 1 hemochromatosis can be made from both clinical data and ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.21071
更新日期:2007-12-01 00:00:00
abstract::Familial Pseudohyperkalemia (FP) is a dominant red cell trait characterized by increased serum [K(+)] in whole blood stored at or below room temperature, without additional hematological abnormalities. Functional gene mapping and sequencing analysis of the candidate genes within the 2q35-q36 critical interval identifi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23357
更新日期:2013-01-01 00:00:00
abstract::Total body irradiation (TBI) has been thought to promote donor cell engraftment in allogeneic hematopoietic cell transplantation (HCT) from alternative donors. However, recent progress in HCT strategies may affect the clinical significance of TBI on neutrophil engraftment. With the use of a Japanese transplant registr...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24613
更新日期:2017-02-01 00:00:00
abstract::CD19+CD5+ lymphocytes constitute a minority of peripheral blood B cells. In view of the importance of these cells in the pathogenesis of the immunoregulation of myeloma, their incidence in another lymphoid organ was determined. CD5+ B cells were studied in 9 spleens from patients with multiple myeloma and in 10 spleen...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830370306
更新日期:1991-07-01 00:00:00
abstract::An adolescent male patient developed functional asplenia in the course of graft versus host disease (GVHD) after successful allogeneic bone marrow transplantation (BMT) for aplastic anemia. Coincident with the onset of the asplenia, amelioration of the ongoing GVHD was observed. Unexpectedly, after 34 months of functi...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830120112
更新日期:1982-02-01 00:00:00
abstract::Intravenous anti-D immune globulin (anti-D IGIV) is indicated for the treatment of immune thrombocytopenic purpura (ITP) in nonsplenectomized patients who are Rh(D)-positive. Recent reports have described episodes of intravascular hemolysis (IVH) and acute renal failure (ARF) after anti-D IGIV. We report the first adu...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10432
更新日期:2003-12-01 00:00:00
abstract::Aberrant promoter DNA methylation is a well-described mechanism of leukemogenesis within hematologic malignancies, including acute lymphoblastic leukemia (ALL). However, the importance of methylation patterns among the adolescent and young adult (AYA) ALL population has not been well established. DNA methylation of 18...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23511
更新日期:2013-09-01 00:00:00
abstract::Lentiviral modification combined with ex vivo erythroid differentiation was used to stably inhibit RhAG expression, a critical component of the Rh(rhesus) membrane complex defective in the Rh(null) syndrome. The cultured red cells generated recapitulate the major alterations of native Rh(null) cells regarding antigen ...
journal_title:American journal of hematology
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doi:10.1002/ajh.23414
更新日期:2013-05-01 00:00:00
abstract::The intravascular pool of human polymorphonuclear leukocytes (PMN) is composed of one compartment which is circulating and another that is marginated to the vascular endothelium. Administration of B-adrenergic agonists leads to a rapid demargination with an increase in the circulating PMN pool. The marginating PMN has...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830240107
更新日期:1987-01-01 00:00:00
abstract::We have developed a rapid approach to detect the two most common alpha-thalassemia-2 (alpha-thal-2) determinants by the polymerase chain reaction (PCR) technique, which takes a few hours to complete. Specific oligonucleotides selectively amplify appropriate segments of the chromosome with the deletion and the normal c...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830460309
更新日期:1994-07-01 00:00:00
abstract::Plasma cell dyscrasias are a group of clinically and biochemically diverse disorders of unknown etiology, characterized by the disproportionate proliferation of one or more clones of B cells, and the presence of a structurally and electrophoretically homogeneous (monoclonal) immunoglobulin or polypeptide subunit in se...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.20736
更新日期:2006-12-01 00:00:00
abstract::Disseminated intravascular coagulation (DIC) is a complex acquired coagulopathy resulting from excessive thrombin formation. Abnormal tissue factor (TF) expression is a major mechanism initiating DIC in many disorders, including obstetrical complications, sepsis, cancer, and trauma. Numerous laboratory tests are avail...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/(sici)1096-8652(199809)59:1<65::aid-ajh13>
更新日期:1998-09-01 00:00:00
abstract::The goal of the present study was to search for criteria that allow one to distinguish between normal individuals and heterozygotes as well as compound heterozygotes for pyruvate kinase (PK) deficiency. As the residual activity of PK with heterozygotes was between 35% and 110% of the normal activity, it was necessary ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830310402
更新日期:1989-08-01 00:00:00
abstract::The aim of this study was to evaluate the clinical efficacy and safety of the ultrasound-guided fine needle biopsy (UG-FNB) of the spleen in a large population of patients. We collected retrospectively the findings concerning the application of UG-FNB of the spleen from eight Italian clinical centers that utilized thi...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.1085
更新日期:2001-06-01 00:00:00
abstract::Individuals with more than one defect in natural coagulant/anticoagulant systems have been postulated to be at an increased risk for thrombotic events. We report a case of combined protein S and C deficiency in a young woman, which resulted in fatal arterial mesenteric thrombosis. The role of coagulation defects in ar...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199807)58:3<246::aid-ajh17
更新日期:1998-07-01 00:00:00
abstract::The objective of the study was to explore the risks and benefits of splenectomy in advanced agnogenic myeloid metaplasia (AMM). We searched the literature (Medline, 1970-1987) for studies of postoperative survival, operative mortality and effects of splenectomy on painful splenomegaly, and portal hypertension or trans...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.2830330210
更新日期:1990-02-01 00:00:00
abstract::The presence of teardrop-shaped red cells in peripheral blood has traditionally been felt to reflect altered marrow architecture, namely myelofibrosis. We evaluated two patients with splenomegaly, moderately severe hemolytic anemia due to warm-reactive IgG anti-red cell autoantibody, and bone marrow erythroid hyperpla...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830210410
更新日期:1986-04-01 00:00:00
abstract:DISEASE OVERVIEW:Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include heart failure with preserved ejection fraction, nephrotic syndrome, h...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.25819
更新日期:2020-07-01 00:00:00