Abstract:
:Treatment with all-trans retinoic acid (ATRA) improves the prognosis of patients with acute promyelocytic leukemia (APL), but ATRA syndrome may occur as a possible fatal side effect, especially in cases refractory to medication or involving pulmonary hemorrhage. We describe two patients with APL who suffered from intracranial hemorrhage. The first patient was a 16-yr-old girl who was treated with ATRA and then developed respiratory distress refractory to treatment with dexamethasone combined with anthracycline-cytarabine cytoreduction therapy. Treatment with Sivelestat, a small molecule inhibitor of neutrophil elastase, achieved rapid improvement in oxygenation and chest radiograph findings, and the patient has been in complete remission for 24 months. The second patient was a 10-yr-old boy in whom pulmonary hemorrhage developed following administration of ATRA, dexamethasone and cytoreduction therapy. Aspiration and administration of Sivelestat improved oxygenation and he remained stable. Hematological improvement was also achieved, but the patient died of brain dysfunction because of cerebral edema accompanied by intracranial bleeding. The two cases suggest that Sivelestat may be effective as an additional agent in the treatment of refractory ATRA syndrome, and, therefore, prospective randomized studies of treatment protocols are warranted.
journal_name
Eur J Haematoljournal_title
European journal of haematologyauthors
Kawasaki K,Akaike H,Miyauchi A,Ouchi Kdoi
10.1111/j.0902-4441.2006.t01-1-EJH2852.xsubject
Has Abstractpub_date
2006-11-01 00:00:00pages
448-52issue
5eissn
0902-4441issn
1600-0609pii
EJH743journal_volume
77pub_type
杂志文章abstract::A case of hemoglobin H disease in combination with hemoglobin Constant Spring and a beta-globin chain variant is reported in a 3-yr-old Thai girl. On routine cellulose acetate electrophoresis, one abnormal band in addition to the hemoglobins A, A2, H, Bart's and Constant Spring was detected. The amount of this abnorma...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2001.066005312.x
更新日期:2001-05-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章,多中心研究
doi:10.1111/j.1600-0609.2008.01122.x
更新日期:2008-11-01 00:00:00
abstract::Over the last two decades, the role of microRNAs has been extensively investigated, and it has become clear that these small non-coding RNAs play an essential role in several biological processes including erythropoiesis and that their dysregulation is associated with pathologies. Recent technical innovations have con...
journal_title:European journal of haematology
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abstract:INTRODUCTION:Health-related quality of life (HRQoL) is impaired in patients with hemophilia; however, the impact in mild/moderate hemophilia B and affected women is not well characterized. OBJECTIVE:To evaluate factors that affect HRQoL in adults with hemophilia B and caregivers of affected children. METHODS:US adult...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13055
更新日期:2018-06-01 00:00:00
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journal_title:European journal of haematology
pub_type: 临床试验,杂志文章
doi:10.1111/j.1600-0609.1995.tb00263.x
更新日期:1995-10-01 00:00:00
abstract::In a phase II study 28 patients with advanced multiple myeloma were treated with a five drug regimen consisting of vincristine, BCNU, adriamycin, melphalan and dexamethasone. 11 out of 13 patients without prior chemotherapy showed significant remissions (greater than 25% tumor cells mass reduction), 7 of them had more...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00831.x
更新日期:1988-03-01 00:00:00
abstract::A transgenic SCID (TG-SCID) mouse expressing the human cytokines interleukin-3 (IL-3) and granulocyte-macrophage colony-stimulating factor (GM-CSF) has been generated with the aim of making a model system allowing the in vivo proliferation of human hematopoietic cells. Using TG-SCID mice expressing high levels (30-35 ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1998.tb01067.x
更新日期:1998-08-01 00:00:00
abstract::Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired disorder of the hematopoietic stem cell that makes blood cells more sensitive to the action of complement. Patients experience intravascular hemolysis, smooth muscle dystonia, renal failure, arterial and pulmonary hypertension, recurrent infectious diseases and ...
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.12543
更新日期:2015-09-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12775
更新日期:2016-12-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1999.tb01112.x
更新日期:1999-01-01 00:00:00
abstract::Langerhans cells originate in bone marrow and probably belong to the monocyte-macrophage lineage. CD1 is a specific marker of Langerhans cells. By immunofluorescence and immunoelectron microscopy, CD1a antigen and myeloid markers (CD11, CD13, CD14, CD15, CD33, HLA-DR) were studied in 53 cases of acute myeloid leukemia...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1992.tb01789.x
更新日期:1992-01-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2007.00929.x
更新日期:2007-11-01 00:00:00
abstract::The molecular defects of the factor XIII A subunit gene were studied in a patient with factor XIII deficiency. Mutation analysis was performed on amplified DNA from each exon of this gene by single-strand conformation polymorphism (SSCP) and DNA sequencing techniques. A substitution of guanine by adenine at nucleotide...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1034/j.1600-0609.2000.065004279.x
更新日期:2000-10-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/ejh.12447
更新日期:2015-05-01 00:00:00
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pub_type: 杂志文章
doi:10.1111/ejh.13110
更新日期:2018-06-13 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1988.tb00798.x
更新日期:1988-01-01 00:00:00
abstract::The introduction of novel immunomodulatory drugs (IMiDs) has dramatically improved the survival of patients with multiple myeloma (MM). While it has been shown that patients with specific cytogenetic subtypes, namely t(4;14), have the best outcomes when treated with bortezomib-based regimens, the relationship between ...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.12192
更新日期:2013-12-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13459
更新日期:2020-10-01 00:00:00
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journal_title:European journal of haematology
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doi:10.1111/j.1600-0609.2009.01245.x
更新日期:2009-07-01 00:00:00
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pub_type: 杂志文章
doi:10.1046/j.0902-4441.2003.00204.x
更新日期:2004-04-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1990.tb00461.x
更新日期:1990-10-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2010.01454.x
更新日期:2010-08-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1997.tb00943.x
更新日期:1997-03-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1993.tb00069.x
更新日期:1993-01-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1034/j.1600-0609.2001.066006404.x
更新日期:2001-06-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.2009.01331.x
更新日期:2009-12-01 00:00:00
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journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1997.tb00981.x
更新日期:1997-10-01 00:00:00
abstract::Multiple mutations in the same gene within a haemophilia family are being increasingly reported and raise many issues with regard to the specificity of the mutations in causing the disease. In a proportion of families with multiple mutations, discordant phenotypic severity is often observed among the affected members....
journal_title:European journal of haematology
pub_type: 杂志文章,评审
doi:10.1111/j.1600-0609.2010.01564.x
更新日期:2011-03-01 00:00:00
abstract:BACKGROUND:Abnormal coagulation parameters have been reported in COVID-19-infected patients. Although the underlying mechanism of COVID-19 coagulopathy remains unknown, it has been suggested to be a form of disseminated intravascular coagulation (DIC). OBJECTIVES:The aim of our study was to analyze the coagulation par...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/ejh.13501
更新日期:2020-12-01 00:00:00
abstract::A number of small and lipophilic cations are able to reverse in vitro the resistance to anthracyclines and other natural products through their interaction with P-glycoprotein or P-gp. However, some modulators do not interact with P-gp. We have demonstrated in a previous a work, using confocal laser microspectrofluoro...
journal_title:European journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1600-0609.1998.tb01709.x
更新日期:1998-10-01 00:00:00