Abstract:
:A 40-year-old patient with low-grade B-NHL developed a generalized macular-papular rash following the first cycle of fludarabine treatment which progressed to a complete epidermal necrolysis following the second cycle. Clinical symptoms and the results of the direct and indirect immunofluorescence were consistent with a mucocutaneous autoimmune syndrome (pemphigus). Immunohistochemical analysis demonstrated a dense epidermal infiltration of CD8+ lymphocytes associated with the histological features of single-cell necrosis of keratinocytes. Early and aggressive immunosuppressive treatment with steroids, cyclophosphamide, and high-dose immunoglobulins resulted in regression of symptoms and complete reconstitution of epidermal integrity. The malignant lymphoma has completely regressed. The findings suggest a fludarabine-induced defect in immunosurveillance--resulting in the uncontrolled activation of autoaggressive T-cell clones--as a pathogenetic mechanism of this life-threatening dermatological complication.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Braess J,Reich K,Willert S,Strutz F,Neumann C,Hiddemann W,Wörmann Bdoi
10.1007/s002770050347subject
Has Abstractpub_date
1997-11-01 00:00:00pages
227-30issue
5-6eissn
0939-5555issn
1432-0584journal_volume
75pub_type
杂志文章abstract::The practical usefulness of Helicobacter pylori eradication for immune thrombocytopenia (ITP) patients is still controversial. However, some ITP patients respond to H. pylori eradication. We conducted a multi-center, open label, prospective phase II study to define the efficacy and toxicities of H. pylori eradication ...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00277-014-2268-9
更新日期:2015-05-01 00:00:00
abstract::While much has been learned about the basic immunology and clinical characteristics of immune thrombocytopenia, many important questions remain with regard to pathogenesis, disease progression, identification of novel therapeutic targets and approaches, and clinical trials that rationalize and optimize use of existing...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-010-0917-1
更新日期:2010-07-01 00:00:00
abstract::Interstitial pneumonia (IP) is a lethal complication in lymphoma patients undergoing chemotherapy. A total of 2212 consecutive patients diagnosed with lymphoma between 2009 and 2014 were enrolled in the present study. IP was defined as diffuse pulmonary interstitial infiltrate found on computed tomography scans. IP wa...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-017-3157-9
更新日期:2018-01-01 00:00:00
abstract::Activation-induced cytidine deaminase (AID) is a mutator enzyme essential for somatic hypermutation (SHM) and class switch recombination (CSR) during effective adaptive immune responses. Its aberrant expression and activity have been detected in lymphomas, leukemias, and solid tumors. In chronic lymphocytic leukemia (...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-018-3520-5
更新日期:2019-02-01 00:00:00
abstract::The optimal treatment strategy for elderly patients with natural killer/T-cell lymphoma (NKTCL) remains to be established. A total of 63 elderly patients with newly diagnosed NKTCL were retrospectively reviewed. Among the patients with stage I-II disease, 58.3 % received radiotherapy (RT) ± chemotherapy, and 41.7 % re...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-015-2395-y
更新日期:2015-09-01 00:00:00
abstract::Indolent follicular lymphomas are diseases which are generally incurable with conventional therapy. Although patients can survive for prolonged periods, the median duration of first remissions is about 2.5 years, and subsequent remissions progressively shorten with time. High-dose therapy with hematopoietic stem cell ...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s002770050503
更新日期:1999-05-01 00:00:00
abstract::Approximately 10% of patients with polycythemia vera (PV) transform to acute leukemia (blast phase) at 10 years after initial diagnosis of PV. The bone marrow pathologic, cytogenetic, and molecular features of blast phase have not been well characterized. In this study, we reviewed 422 PV patients over a period of 11 ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-017-3211-7
更新日期:2018-03-01 00:00:00
abstract::The objective of this study was to evaluate retrospectively the clinical characteristics, treatments, and outcomes of patients with primary diffuse large B-cell lymphoma (DLBCL) of the female genital tract. The basic characteristics, treatments, and outcomes of six patients diagnosed with primary DLBCL of the female g...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-013-2003-y
更新日期:2014-06-01 00:00:00
abstract::Diagnosis of thrombotic thrombocytopenic purpura (TTP) is usually based upon the clinical features, and does not always involve histopathological evidence. We recently had experience with a patient who developed the five signs characteristic for TTP. He had been treated for liver cirrhosis associated with chronic hepa...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770100323
更新日期:2001-08-01 00:00:00
abstract::Bortezomib synergizes with melphalan in preclinical and early clinical studies. Updated data from our phase 1/2 study assessing the safety and efficacy of bortezomib plus melphalan in relapsed/refractory multiple myeloma (MM) are presented. Bortezomib (0.7, 1.0, or 1.3 mg/m(2)) on days 1, 4, 8, and 11 and oral melphal...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-008-0501-0
更新日期:2008-08-01 00:00:00
abstract::The clinical impact of KIT mutations in core binding factor acute myeloid leukemia (CBF-AML) is still unclear. In the present study, we analyzed the prognostic significance of each KIT mutation (D816, N822K, and other mutations) in Japanese patients with CBF-AML. We retrospectively analyzed 136 cases of CBF-AML that h...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-017-3074-y
更新日期:2017-10-01 00:00:00
abstract::An additional affiliation for the first author was not indicated. Hyewon Lee is also affiliated with: Department of Internal Medicine, Yonsei University College of Medicine, Gangnam Severance Hospital, Seoul, South Korea. ...
journal_title:Annals of hematology
pub_type: 杂志文章,已发布勘误
doi:10.1007/s00277-019-03868-8
更新日期:2020-01-01 00:00:00
abstract::Cytokines and adhesion molecules play an important role in the pathophysiology of vaso-occlusion in sickle cell disease (SCD), and their in vivo profiles are potential tools for assessing SCD severity. We compared steady-state soluble vascular cell adhesion molecule-1 (sVCAM-1) serum levels to clinical (painful crisis...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0609-1
更新日期:2003-02-01 00:00:00
abstract::Infections and especially blood stream infections (BSI) with gram-negative bacteria (GNB) represent a major threat for patients with hematological diseases undergoing chemotherapy and mainly contribute to morbidity and mortality. In this retrospective single-center study, we analyzed the impact of BSI with different g...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-018-3423-5
更新日期:2018-11-01 00:00:00
abstract::Acute leukemia (AL) patients may experience more than one episode of bloodstream infection (BSI) caused by the same pathogen during the entire chemotherapy program. In order to identify factors influencing BSI recurrence (R-BSI) during subsequent phases of treatment, we analyzed all BSIs occurring to consecutively tre...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-013-1965-0
更新日期:2014-05-01 00:00:00
abstract::We investigated the efficacy of an antithymocyte globulin/cyclophosphamide preparative regimen prior to allogeneic stem cell transplantation from HLA-identical siblings in patients with severe aplastic anemia. Since 1990, 21 patients, 6 males and 15 females, with a median age of 25 years (range: 7-43) have been enroll...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-002-0566-0
更新日期:2002-11-01 00:00:00
abstract::Multiresistant bacterial infections are a potentially life-threatening condition in acute leukaemia (AL) patients. We aimed to better define the very recent epidemiology and outcome of bloodstream infections (BSIs) in a real-life setting. We prospectively collected all consecutive febrile/infectious episodes occurring...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00277-016-2815-7
更新日期:2016-12-01 00:00:00
abstract::Venous thromboembolism (VTE), including deep venous thrombosis (DVT) and pulmonary embolism (PE), occurs secondary to a number of hereditary and acquired disorders of hemostasis. A recently recognized polymorphism in Factor V (FV) gene H1299R (also named HR2) has been reported to be a possible risk factor for the deve...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-008-0543-3
更新日期:2008-12-01 00:00:00
abstract::Due to their homing properties, extranodal marginal zone B-cell lymphoma (MZBL) of mucosa-associated lymphoid tissue (MALT) type remain localized for long periods of time, and therefore have an excellent prognosis. However, if generalization and/or transformation into a diffuse large-cell lymphoma occurs, the prognosi...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770000203
更新日期:2000-12-01 00:00:00
abstract::Umbilical cord blood is capable of hematopoietic stem cell reconstitution in children. However, the major limitation of cord blood is a relatively low content of pluripotent progenitor cells. Thus, safe engraftment for adolescents and for adults is still not predictable and a technology for ex vivo expansion of umbili...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050530
更新日期:1999-08-01 00:00:00
abstract::While inherited X-linked (XL) isolated thrombocytopenia is a mild condition, the Wiskott-Aldrich syndrome (WAS) associates severe thrombocytopenia with an immunodeficiency component and has a poor prognosis. Whether these conditions correspond to separate genetic entities or to different mutations of the same gene(s) ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01707282
更新日期:1991-08-01 00:00:00
abstract::Parainfluenza virus (PIV) infection is a significant cause of morbidity and mortality, especially in hematologic malignancy patients including hematopoietic stem cell transplantation (HCT) recipients. However, limited information is available for risk stratification in PIV-infected patients with hematologic malignancy...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-020-03996-6
更新日期:2020-06-01 00:00:00
abstract::High-dose immunosuppressive therapy (HDIT) with autologous hematopoietic stem cell transplantation (AHSCT) is a promising approach to treatment of multiple sclerosis (MS) patients. In this paper, we present the long-term outcomes of a prospective single-center study with the analysis of the safety and efficacy of HDIT...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-015-2337-8
更新日期:2015-07-01 00:00:00
abstract::Along with their immunogenic role, dendritic cells (DCs) are also critical in maintaining tolerance to self-antigens by inducing regulatory T cells (Tregs) via the expression of the immunomodulatory enzyme indoleamine 2,3-dioxygenase 1 (IDO1). In turn, Tregs modulate the maturation and/or function of DCs. In immune th...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-012-1556-5
更新日期:2013-01-01 00:00:00
abstract::The cytogenetic and molecular data is recognized as the most valuable prognostic factor in acute myeloid leukemia (AML). Our aim was to systemically analyze the cytogenetics of Korean AML patients and to compare the cytogenetic profiles of various races to identify possible geographic heterogeneity. We retrospectively...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00277-016-2691-1
更新日期:2016-08-01 00:00:00
abstract::Two male patients with severe and recurrent bleeding episodes under phenprocoumon therapy are reported. Both patients exhibited a strong decrease of their factor IX activities below 1% of normal, whereas the activities of the vitamin K-dependent factors prothrombin, VII, and X were found to be within or above the expe...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050297
更新日期:1997-06-01 00:00:00
abstract::Nodal peripheral T cell lymphomas (nPTCL) present aggressive clinical course, and its heterogeneous nature and poor prognosis with current therapeutic strategies make it a target for the development of new prognostic markers. Thus, we investigated tumor-associated macrophages (TAM) according to the number of cells exp...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章
doi:10.1007/s00277-019-03731-w
更新日期:2019-09-01 00:00:00
abstract::Platelet function disorders (PFD) and Von Willebrand disease (VWD) are among the uncommon causes of bleeding in haematological practice. The inherited variety of PFD includes defects in platelet adhesion, aggregation, secretion and platelet procoagulant activities. VWD is classified into three major categories-type 1 ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-006-0244-8
更新日期:2007-06-01 00:00:00
abstract::The most common causes of morbidity and mortality in myeloproliferative neoplasms (MPN) are thrombotic and hemorrhagic complications. The JAK2V617F mutation, commonly found in MPN, correlates with several clinical and laboratory characteristics even if the relevance of JAK2V617F allele burden in the natural history of...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-017-3040-8
更新日期:2017-08-01 00:00:00
abstract::We report on a patient who was diagnosed as having B-cell chronic lymphocytic leukemia (CLL) with atypical morphology. Flow cytometry disclosed CD5, CD19, and CD23 positivity, an immunophenotype seen mostly in B-CLL. Histology of the spleen and bone marrow suggested a diagnosis of small lymphocytic lymphoma. Upon blas...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050582
更新日期:2000-04-01 00:00:00