Abstract:
:Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal condition with various underlying disorders in adult patients and is diagnosed based on the HLH-2004 criteria, which were established based on experience in pediatric patients. However, few studies have prospectively evaluated the treatment outcomes and diagnostic performance of HLH criteria in adult patients with secondary HLH. Thus, we performed a single-center, prospective cohort study of adult patients with suspected HLH, and we analyzed treatment outcomes of patients enrolled between 2017 and 2019 as an interim analysis ( ClinicalTrials.gov Identifier: NCT03117010). Of the 73 patients with suspected HLH, 70 patients completed the evaluation for ≥ 7 of the HLH-2004 criteria, and 55 patients were diagnosed with HLH (55/73, 75%). Although serum ferritin and fever had a sensitivity of more than 90%, both had exceptionally low specificity, whereas soluble CD25 had a sensitivity of more than 90% and specificity of 80%. Forty patients with malignancy-associated HLH had B cell (n = 19) or T- or NK-cell (n = 21) lymphoid malignancy, whereas 15 patients had non-malignant disorders. Non-malignancy-associated HLH had greater than 90% 1-year overall survival (OS) after diagnosis of HLH, whereas that for malignancy-associated HLH was less than 40%. In conclusion, our study showed promising treatment outcomes for patients enrolled in our prospective cohort study, and prospectively demonstrated the diagnostic performance of the HLH-2004 criteria in adult patients with suspected HLH. Given that lymphoma was the most common cause of HLH in adults, thorough evaluation for lymphoma should be performed in adults with suspected HLH.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Yoon SE,Eun Y,Huh K,Chung CR,Yoo IY,Cho J,Cho D,Ko YH,Park S,Kim WS,Kim SJdoi
10.1007/s00277-020-04083-6subject
Has Abstractpub_date
2020-09-01 00:00:00pages
2095-2104issue
9eissn
0939-5555issn
1432-0584pii
10.1007/s00277-020-04083-6journal_volume
99pub_type
杂志文章abstract::Rituximab was recently described also as first-line therapy of chronic graft-versus-host disease (cGvHD). We retrospectively analyzed the efficacy and safety of all patients receiving rituximab for treatment of cGvHD between 2005 and 2016 at the Regensburg University transplant center with a median follow-up after rit...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-019-03768-x
更新日期:2019-10-01 00:00:00
abstract::A 72-year-old-man with night sweats and a low-grade fever was found to have bilateral adrenal enlargement associated with incipient adrenal insufficiency. Without any intervention, these adrenal lesions regressed spontaneously, accompanied by disappearance of clinical symptoms. Seven months later, however, the lesions...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770100335
更新日期:2001-09-01 00:00:00
abstract::Early cytomegalovirus (CMV) replication (eCMV) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) has been suggested as an independent factor that reduces leukemia relapse risk. We retrospectively analyzed 74 patients with acute myeloid leukemia (AML) who underwent allo-HSCT between August 2006 and S...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-014-2190-1
更新日期:2015-02-01 00:00:00
abstract::In the present study, we questioned whether the cholesterol synthesis inhibitor lovastatin potentiates the cytotoxicity of chemotherapeutic agents in the primitive CD34(+) subpopulation of acute myeloid leukemia (AML) cells. AML mononuclear cells (n = 17) were sorted in CD34(+) and CD34(-) fractions and compared to no...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-008-0633-2
更新日期:2009-06-01 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::A 57-year-old man with acute myeloid leukemia (AML) French-American-British (FAB) 4 developed disseminated invasive cerebral and pulmonary aspergillosis during postinduction aplasia. According to international consensus, infection was categorized as probable (two host factors: deep neutropenia for >10 days and refract...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0792-0
更新日期:2004-06-01 00:00:00
abstract::Increasing the number of megakaryocytic cells in stem cell transplants by ex vivo expansion culture may provide an approach to accelerate platelet engraftment after high-dose chemotherapy. However, it is unknown if a relationship exists between the expansion potential of progenitor cells and the time to platelet engra...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-002-0443-x
更新日期:2002-04-01 00:00:00
abstract::Thirty six patients with acute promyelocytic leukemia were studied by reverse transcriptase-polymerase chain reaction (RT-PCR) and real-time PCR. There was concordance between the results achieved by both the methods except in one case, which was negative by RT-PCR but positive by real-time PCR. The prevalence of bcr3...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-005-1095-4
更新日期:2005-11-01 00:00:00
abstract::Cerebral involvement is an unusual complication in multiple myeloma: herein four patients who presented myelomatous meningitis with multiple intraparenchymal lesions or a localized cerebral plasmacytoma are described. Two of these patients relapsed with meningeal involvement and a very limited disease outside the cent...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-001-0387-6
更新日期:2001-12-01 00:00:00
abstract::Follicular lymphoma (FL) is generally considered an indolent disorder. With modern day treatments, long remissions are often achieved both in the front-line and relapsed setting. However, a subset of patients has a more aggressive course and a worse outcome. Their identification is the main purpose of modern day progn...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-017-3154-z
更新日期:2018-02-01 00:00:00
abstract::Children with sickle cell disease (SCD) benefit from newborn screening, because life-threatening complications can be prevented by pre-symptomatic diagnosis. In Germany, the immigration of people from endemic countries is steadily growing. Comprehensive data about the epidemiology and prevalence of SCD in Germany are ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-015-2573-y
更新日期:2016-02-01 00:00:00
abstract::The evidence that leukocytes may contribute to the pathogenesis of thrombosis in Chronic Myeloproliferative Neoplasms is increasing but not definitive. To further enforces whether an increased leukocyte count is associated with thrombosis and whether this effect can be modulated by cytoreductive therapy, we analyzed t...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00277-009-0706-x
更新日期:2009-10-01 00:00:00
abstract::Autosomal recessive "malignant" osteopetrosis is a rare congenital disorder relating to bone resorption abnormalities. It is believed to arise due to the failure of osteoclasts to resorb immature bone. This leads to abnormal bone marrow cavity formation and, clinically, to the signs and symptoms of bone marrow failure...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-002-0578-9
更新日期:2003-01-01 00:00:00
abstract::The B cell surface antigen CD19 is a target for treating B cell malignancies, such as B cell precursor acute lymphoblastic leukemia and B cell non-Hodgkin lymphoma. The BiTE® immuno-oncology platform includes blinatumomab, which is approved for relapsed/refractory B cell precursor acute lymphoblastic leukemia and B ce...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-020-04221-0
更新日期:2020-10-01 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::The aim of the study was to investigate the association of leptin with hematological parameters in beta-thalassemia patients in Greece. We measured plasma levels of soluble transferrin receptor (sTfR) and leptin by enzyme-linked immunosorbent assay (ELISA) in 40 beta-thalassemia patients (21 transfusion dependent and ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-002-0499-7
更新日期:2002-09-01 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 objective of this study is to compare and evaluate the diagnostic value of hereditary spherocytosis (HS) by three screening tests, comparing mean spherical corpuscular volume (MSCV) to mean corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), and flow cytometric osmotic fragility test. Perip...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-014-2270-2
更新日期:2015-05-01 00:00:00
abstract::Single-agent bortezomib, a potent, selective, and reversible inhibitor of the 26S proteasome, has demonstrated clinical efficacy in relapsed and refractory mantle cell lymphoma (MCL). Objective response is achieved in up to 45% of the MCL patients; however, complete remission rates are low and duration of response pro...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-011-1377-y
更新日期:2012-06-01 00:00:00
abstract::Eight patients who were carriers of beta-thalassemia induced by the cd121 (G-->T) mutation are described in four nonrelated Dutch families. This mutant, which is considered rare and inherited in a dominant manner, is expressed in a different way among each of the four families and even among carriers of the same famil...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050453
更新日期:1998-12-01 00:00:00
abstract::Bernard-Soulier syndrome (BSS) is a rare autosomal recessive genetic disorder characterized by thrombocytopenia, circulating giant platelets, and prolonged bleeding time. BSS is explained by a defect in primary hemostasis owing to quantitative or qualitative defect in the GPIb-IX-V complex, composed of four subunits: ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-008-0611-8
更新日期:2009-05-01 00:00:00
abstract::Azacitidine and decitabine, two hypomethylating agents, are known to be effective in the treatment of high-risk myelodysplastic syndromes (MDS) and acute myeloid leukemia (AML) patients who cannot endure intensive cytotoxic chemotherapy or are not eligible for transplantation. However, the treatment response rate is l...
journal_title:Annals of hematology
pub_type: 杂志文章,评审
doi:10.1007/s00277-018-3464-9
更新日期:2018-11-01 00:00:00
abstract::The combination of sickle cell disease crisis and thrombotic thrombocytopenic purpura has been described only a few times. Here we present the case of a patient with a hemolytic crisis due to sickle cell disease complicated by thrombotic thrombocytopenic purpura. We also review the cases previously reported and compar...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050558
更新日期:1999-12-01 00:00:00
abstract::The role of 18FDG-PET/CT during follow-up of patients affected by Hodgkin's lymphoma (HL) in complete remission after treatment is not fully elucidated, since a wide use of 18F fluorodeoxyglucose positron emission tomography/computed tomography (18FDG-PET/CT) in this setting could be limited by a relative high rate of...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-009-0752-4
更新日期:2009-12-01 00:00:00
abstract::Fanconi anemia (FA) is a DNA repair disorder resulting from mutations in genes encoding for FA DNA repair complex components and is characterized by variable congenital abnormalities, bone marrow failure (BMF), and high incidences of malignancies. FA mosaicism arises from reversion or other compensatory mutations in h...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-020-03954-2
更新日期:2020-05-01 00:00:00
abstract::The hemoglobinopathies are a group of disorders passed down through families (inherited) in which there is abnormal production or structure of the hemoglobin molecule. They are among the most common inherited diseases around the world. Those that produce abnormal hemoglobin are called structural hemoglobinopathies whi...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-019-03696-w
更新日期:2019-07-01 00:00:00
abstract::The general impact of blood donation on iron status was studied in a population survey comprising 1359 nonpregnant Danish women in age cohorts of 30, 40, 50, and 60 years; 809 were premenopausal and 550 postmenopausal; 180 (13%) were blood donors. Iron stores were assessed by serum (S)-ferritin and hemoglobin (Hb). Hb...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01714957
更新日期:1991-07-01 00:00:00
abstract::It has been suggested that Asian and Western myelodysplastic syndrome (MDS) patients have different cytogenetic and prognostic features. In this study, we retrospectively analyzed clinical and cytogenetic data from 435 Chinese adult primary MDS patients. In addition, we evaluated the prognostic value of the World Heal...
journal_title:Annals of hematology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00277-009-0861-0
更新日期:2010-06-01 00:00:00
abstract::The Wilms' tumor 1 (WT1) expression has been recognized in a substantial number of acute myeloid leukemia (AML) patients. Some studies indicated the association of diagnosed WT1 higher expression (WT1(H)) and poor outcome in the AML patients, while other studies had different opinions. Therefore, we performed a meta-a...
journal_title:Annals of hematology
pub_type: 杂志文章,meta分析
doi:10.1007/s00277-014-2295-6
更新日期:2015-06-01 00:00:00
abstract::Parenteral human immunoglobulin (IVIG) administration is widely used in low birth weight (LBW) infants for prevention and therapy of neonatal infection. In previous studies, IVIG preparations containing IgG and low IgM concentrations were commonly used. In this study we compare immunoglobulin serum levels in two group...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1007/BF01703445
更新日期:1991-10-01 00:00:00