Abstract:
:Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by isolated overproduction of platelets, thrombohemorrhagic complications, and a median age of 50-60. When it occurs in younger patients, the incidence of complications has been reported to be quite low, with a good long-term prognosis. We report a retrospective review of 13 patients with ET between the ages of 22 and 35 in which 11 were symptomatic at diagnosis, with only one remaining asymptomatic during follow-up. Three patients presented with potentially life-threatening complications (two myocardial infarctions, one stroke), although no deaths were observed. The majority of the nonlife-threatening complications were vaso-occlusive in nature, including erythromelalgia and transient neurologic symptoms. We conclude that ET in young adults is not always a benign disease and that potentially life-threatening complications are not rare. The optimum approach to treatment in this or any other age group remains uncertain.
journal_name
Am J Hematoljournal_title
American journal of hematologyauthors
Millard FE,Hunter CS,Anderson M,Edelman MJ,Kosty MP,Luiken GA,Marino GGdoi
10.1002/ajh.2830330106subject
Has Abstractpub_date
1990-01-01 00:00:00pages
27-31issue
1eissn
0361-8609issn
1096-8652journal_volume
33pub_type
杂志文章abstract::We present a case of chronic tonsillitis in a patient with chronic lymphocytic leukemia. Despite empiric radiation and antibiotic therapy, the patient's sore throat and tonsillar enlargement persisted. Excisional biopsy of the involved tonsil revealed the presence of Cryptococcus neoformans. Blood cultures also yielde...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830250413
更新日期:1987-08-01 00:00:00
abstract::In acute myeloid leukemia (AML), new prognostic tools are needed to assess the risk of relapse. Hematogones (HGs) are normal B-lymphocyte precursors that increase in hematological diseases and may influence remission duration in AML. HG detection was prospectively investigated in 262 AML patients to determine its prog...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24350
更新日期:2016-06-01 00:00:00
abstract::Paroxysmal nocturnal hemoglobinuria (PNH) is a rare clonal stem-cell disorder in which blood cells lack complement inhibiting membrane proteins, and become susceptible to complement-mediated injury, leading to chronic intravascular hemolysis and pancytopenia. Glucocorticoids have been a mainstay of therapy. For patien...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/(sici)1096-8652(199702)54:2<149::aid-ajh9>
更新日期:1997-02-01 00:00:00
abstract::Peptide-pulsed dendritic cells can stimulate T cells showing specific cytotoxicity in chronic myelogenous leukemia. We tried to induce a specific cytotoxic T-cell response stimulated by RNA-pulsed dendritic cells in acute myelogenous leukemia. The total RNA of WEHI-3BD+, a myelomonocytic leukemia cell line derived fro...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10471
更新日期:2004-03-01 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 hereditary persistence of fetal hemoglobin (HPFH)-6 is sporadically found in Thailand whereas the deletion-inversion type (G)gamma((A)gamma delta beta)(0)-thalassemia is described among Indians. We report a hitherto un-described case in which these two defects co-segregate. He was a 3-year-old Thai boy who had a f...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10202
更新日期:2002-10-01 00:00:00
abstract::A case of leukemia cutis (LC) of monocytic lineage in a patient with myelodysplastic syndrome (MDS) is presented. Cutaneous infiltrates were recognized concurrent with diagnosis of refractory anemia (RA) with monocytosis. Skin infiltrates subsequently spontaneously regressed although MDS progressed with increasing mon...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200102)66:2<120::AID-AJH1027>3.0
更新日期:2001-02-01 00:00:00
abstract::A 67-year-old woman was admitted to our hospital because of lymphadenopathy and lymphocytosis. Monoclonal integration of HTLV-I provirus DNA was detected, and a diagnosis of adult T-cell leukemia (ATL) was made. Flow cytometry revealed that the ATL cells expressed CD20 as well as T-cell-associated antigens, and expres...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/1096-8652(200101)66:1<39::AID-AJH1005>3.0.
更新日期:2001-01-01 00:00:00
abstract::The levels of adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), lactic dehydrogenase (LDH), and LDH isoenzyme patterns (LD1 to LD5) have been measured in lymphocyte extract from 28 patients with B-chronic lymphocytic leukemia (B-CLL). The activities of ADA, PNP, and LDH have been correlated with two mo...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830270302
更新日期:1988-03-01 00:00:00
abstract::Several studies provided evidence of a consistent antileukemic effect induced by cytomegalovirus (CMV) replication in acute myeloid leukemia (AML) patients receiving allogeneic hematopoietic stem cell transplantation (HSCT), however the use of antithymocyte globulin (ATG) as graft-versus-host disease prophylaxis, may ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.23998
更新日期:2015-06-01 00:00:00
abstract::Progressive multifocal leukoencephalopathy (PML), a demyelinating infectious disease caused by JC virus (JCV), occurs almost exclusively in immunocompromised patients usually with malignant diseases. We report here a Japanese female with follicular lymphoma who subsequently developed PML. In addition to JCV, human her...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.1108
更新日期:2001-07-01 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::Relapsed or refractory acute myeloid leukemia (R/R AML) has a poor prognosis and is best treated with salvage chemotherapy as a bridge to allogeneic stem cell transplant (alloSCT). However, the optimal salvage therapy remains unknown. Here we compared two salvage regimens; mitoxantrone, etoposide, and cytarabine (MEC)...
journal_title:American journal of hematology
pub_type: 杂志文章,多中心研究
doi:10.1002/ajh.25838
更新日期:2020-08-01 00:00:00
abstract::The trial CICL670AUS04 was a single-arm, open-label study of the cardiac efficacy of 18 months of deferasirox monotherapy [1]. Cardiac response in this study was related to the degree of liver siderosis. Patients with mild to moderate liver siderosis improved their cardiac T2* while more severely siderotic patients di...
journal_title:American journal of hematology
pub_type: 临床试验,信件
doi:10.1002/ajh.21830
更新日期:2010-10-01 00:00:00
abstract::National AIDS surveillance suggests that approximately 3.5% of all hemophilic patients in the United States have developed AIDS as of February 1988; however, the cumulative incidence of AIDS among seropositive patients at individual hemophilia treatment centers (HTCs) has been reported to be as high as 12%. The purpos...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830320304
更新日期:1989-11-01 00:00:00
abstract::The relationship between thrombocytopenia and the level of anticardiolipin antibodies (aCL) and/or the existence of lupus anticoagulant (LA) ware studied in 146 patients with systemic lupus erythematosus (SLE). These patients were divided into six groups: A, those LA positive with a high level of aCL (>10 U/ml) (10 ca...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199805)58:1<55::aid-ajh10>
更新日期:1998-05-01 00:00:00
abstract:OVERVIEW:Evidence suggests that even patients aged 70 or above benefit from specific AML therapy. The fundamental decision in AML then becomes whether to recommend standard or investigational treatment. This decision must rest on the likely outcome of standard treatment. Hence we review factors that predict treatment r...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.23834
更新日期:2014-11-01 00:00:00
abstract::Trimethoprim and sulfamethoxazole (Bactrim r) is a widely used antibiotic combination effective against a broad spectrum of microbial organisms. There are reports of neutropenia developing during even brief periods of oral therapy, particularly in individuals with either folate deficiency or increased folate requireme...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830230102
更新日期:1986-09-01 00:00:00
abstract::Extramedullary meningeal hematopoiesis (EMH) represents an uncommon finding after stem-cell transplantation. We describe the case of an allogeneic bone marrow transplantation (BMT) recipient who developed EMH 1 month after radiation myelitis had been diagnosed. A 39-year-old man with multiple myeloma underwent matched...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20341
更新日期:2005-08-01 00:00:00
abstract::The 2013 discovery of calreticulin (CALR) mutations in myeloproliferative neoplasms was attended by their association with longer survival in primary myelofibrosis (PMF). Subsequent studies have suggested prognostic distinction between type 1/like and type 2/like CALR mutations and detrimental effect from triple-negat...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.24978
更新日期:2018-03-01 00:00:00
abstract::Fifty-nine HIV-1 antibody positive and 58 antibody negative hemophiliacs were evaluated over a 2 year study period to gain insight into the natural history and prognosis of HIV-1 disease in members of this risk group. Mean CD4 (Leu 3+) cell counts calculated at 6 month intervals decreased gradually in seropositive pat...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830320406
更新日期:1989-12-01 00:00:00
abstract::Pancytopenia is a rare complication of the thionamide therapy reported secondary to aplastic anemia, the bone marrow being invariably hypocellular. We present a case of a 16-year-old female with Graves' disease who presented with massive bone marrow plasmocytosis mimicking multiple myeloma. The patient had already bee...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.1127
更新日期:2001-08-01 00:00:00
abstract::Skin fibroblasts from patients with a variety of hematologic disorders were infected with SV40 virus in vitro in attempts to discover the reason for increased susceptibility of Fanconi anemia cells to this transforming virus. The proportion of skin fibroblasts expressing SV40 T-antigen by immunofluorescent methods was...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830080407
更新日期:1980-01-01 00:00:00
abstract::By using Dexter-type long-term marrow cultures (D-LTMC), it has been shown previously that hematopoietic progenitor cells (HPC) from patients with aplastic anemia (AA) have a deficient proliferation in vitro. The studies reported to date, however, have focused exclusively on granulomonocytic progenitors and no informa...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/(sici)1096-8652(199810)59:2<149::aid-ajh8>
更新日期:1998-10-01 00:00:00
abstract::Variation in bleeding in the perioperative period is a complex and multifactorial event associated with immediate and delayed consequences for the patient and health care resources. Little is known about the complex genetic influences on perioperative bleeding. With the discovery of multiple variations in the human ge...
journal_title:American journal of hematology
pub_type: 杂志文章,评审
doi:10.1002/ajh.21205
更新日期:2008-09-01 00:00:00
abstract::A specialized serum ferritin assay has been developed for the detection of iron deficiency in epidemiologic studies. An enzyme immunoassay (EIA) was employed to eliminate the need for radioisotopes. The problem of low sensitivity inherent with the EIA for serum ferritin was eliminated by the use of monoclonal immunolo...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830230209
更新日期:1986-10-01 00:00:00
abstract::FIP1L1-PDGFRA-positive myeloid neoplasm with eosinophilia (F/P+ MN-eo) is a rare disease: robust epidemiological data are lacking and reported issues are scarce, of low sample-size and limited follow-up. Imatinib mesylate (IM) is highly efficient but no predictive factor of relapse after discontinuation has yet been i...
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.25945
更新日期:2020-11-01 00:00:00
abstract::Two patients underwent allogeneic hematopoietic stem cell transplantation (HSCT) for acute myeloid leukemia. Chronic graft-versus-host disease (GVHD) developed, with persistent symptomatic oral lesions. At 2 and 6 years post-HSCT, both patients developed squamous cell carcinoma (SCC) of the tongue in areas previously ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.20160
更新日期:2004-10-01 00:00:00
abstract::Neutrophil elastase (NE) is a serine protease stored in the primary granules of neutrophils that proteolytically cleaves multiple cytokines and cell surface proteins on release from activated neutrophils. Recent reports of mutations in the gene encoding this enzyme in some patients with neutropenic syndromes prompted ...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.10434
更新日期:2003-11-01 00:00:00
abstract::The human spleen normally retains about one-third of the body's platelets in an exchangeable pool which can be released into the circulation by alpha-adrenergic stimulation. Some previous investigators concluded that the splenic platelet population was enriched in a subpopulation of large, young, dense platelets (mega...
journal_title:American journal of hematology
pub_type: 杂志文章
doi:10.1002/ajh.2830340302
更新日期:1990-07-01 00:00:00