Abstract:
:Polycythemias or erythrocytoses in childhood and adolescence are very rare. Systematic data on the clinical presentation and laboratory evaluations as well as on treatment regimens are sparse. The diagnostic program in absolute erythrocytosis includes extensive clinical, hematological, biochemical, and molecular biological examinations which should be applied following a stepwise algorithm. Absolute erythrocytoses are usually subdivided into primary and secondary forms. Primary erythrocytosis is a condition in which the erythropoietic compartment is expanding independently of extrinsic influences or by responding inadequately to them. Primary erythrocytoses include primary familial and congenital polycythemia (PFCP) due to mutations of the erythropoietin (Epo) receptor gene and the myeloproliferative disorder polycythemia vera. Secondary erythrocytoses are driven by hormonal factors (predominantly by Epo) extrinsic to the erythroid compartment. The increased Epo secretion may represent either a physiologic response to tissue hypoxia, an abnormal autonomous Epo production, or a dysregulation of the oxygen-dependent Epo synthesis. Congenital secondary erythrocytoses are caused, e.g., by hemoglobin variants with increased oxygen affinity, by 2,3-bisphosphoglycerate deficiency, or by mutations in the von Hippel-Lindau gene associated with a disturbed oxygen-dependent regulation of Epo synthesis.
journal_name
Ann Hematoljournal_title
Annals of hematologyauthors
Cario Hdoi
10.1007/s00277-004-0985-1subject
Has Abstractpub_date
2005-03-01 00:00:00pages
137-45issue
3eissn
0939-5555issn
1432-0584journal_volume
84pub_type
杂志文章,评审abstract::Several revisions of International Prognostic Index (IPI) have been proposed for patients with diffuse large B-cell lymphoma (DLBCL) after the introduction of rituximab. Expanding evidence suggests that baseline absolute lymphocyte count (ALC) is also an independent factor for outcome prediction. We investigated the o...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-013-1807-0
更新日期:2013-11-01 00:00:00
abstract::In this paper we report a rare association of a splenic marginal zone B-cell lymphoma with villous lymphocytes and a T-cell large granular lymphocytic leukemia coexpressing CD4 and CD8 as well as CD56 and CD57 natural killer-associated markers in an asymptomatic patient investigated because of an occasional finding of...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770100369
更新日期:2001-11-01 00:00:00
abstract::The possibility that human mesenchymal stromal cells (hMSC) may derive from the malignant clone in hematological malignancies (HM) is a controversial issue. In order to clarify hMSC origin and disclose possible cytogenetic heterogeneity in hMSC belonging to different patients, bone marrow (BM)-derived hMSC samples fro...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-012-1500-8
更新日期:2012-10-01 00:00:00
abstract::The purpose of this report is to provide long-term follow-up of 38 patients diagnosed of post-transplant lymphoproliferative disease (PTLD) included in a phase 2 clinical trial of first line therapy with rituximab and to evaluate the same therapy in a real world cohort of 21 consecutive patients treated once the trial...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-020-04056-9
更新日期:2020-05-04 00:00:00
abstract::In this retrospective study, we evaluated the clinical features and the effects of various treatment modalities on the clinical course in patients diagnosed with idiopathic thrombocytopenic purpura (ITP) at our center between 1984-2000. We retrospectively examined the medical records of 321 (229 females, 92 males) ITP...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-002-0488-x
更新日期:2002-08-01 00:00:00
abstract::An improvement in quality of life and survival occurred among thalassemia major (TM) patients: pregnancy in such patients has become a reality. Safe pregnancy and delivery require efforts to ensure the best outcomes. Between 2007 and 2016, 30 TM patients had 37 pregnancies. We analyzed the hematological parameters bef...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-017-2979-9
更新日期:2017-06-01 00:00:00
abstract::The anti-CD38 monoclonal antibody daratumumab is approved as a single agent for the treatment of patients with relapsed and refractory multiple myeloma (RRMM) who have received at least three prior lines of therapy, including a proteasome inhibitor (PI) and an immunomodulatory agent (IMID), or who are double refractor...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-019-03655-5
更新日期:2019-06-01 00:00:00
abstract::In contrast to adult medicine, specific scoring systems predicting the treatment response for an individual pediatric patient (pt) with chronic myeloid leukemia (CML) have not yet been defined. We evaluated to what extend prognostic scores as described for adults (e.g., Sokal, Hasford, EUTOS score) resulted in compara...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-015-2367-2
更新日期:2015-08-01 00:00:00
abstract::Recently, it was shown that glycoproteins with N-glycans close to the NH(2) terminus can directly enter the calnexin/calreticulin cycle and bypass BiP binding. This should allow efficient secretion of glycoproteins such as factor VIII (FVIII) whose secretion is negatively affected by BiP interaction. Examination of th...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-007-0380-9
更新日期:2008-02-01 00:00:00
abstract::Post-transplantation cyclophosphamide (PTCy) demonstrated effectiveness to prevent GVHD after haploidentical hematopoietic cell transplantation (HCT). Reducing toxicities with a maximized efficacy is still challenging in HCT. In this retrospective study, we analyzed the safety and efficacy of transplantation from a 1-...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-019-03673-3
更新日期:2019-06-01 00:00:00
abstract::The incidence of multiple myeloma (MM) has increased in the last 20 years, particularly in middle and low-middle income countries. Access to diagnostic and prognostic tests and the availability of effective care is highly variable globally. Latin America represents 10% of the world population, distributed in countries...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s00277-020-03983-x
更新日期:2020-05-01 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::We assessed the origin of peripheral blood cells and bone marrow cells of 92 samples obtained from 19 patients after allo BMT by two-step polymerase chain reaction (PCR) amplification of MCT118, one of the variable number of tandem repeat regions (VNTR) which has a different length in 19 of 32 sibling pairs examined, ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/BF01834365
更新日期:1994-04-01 00:00:00
abstract::Wilms' tumor gene 1 (WT1) is gaining increasing attention as a therapeutic target molecule due to its common expression in acute leukemias and its involvement in cell proliferation. Here, we reported on WT1 messenger RNA expression levels at diagnosis in a series of 238 adult acute lymphoblastic leukemia (ALL) samples...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-009-0746-2
更新日期:2009-12-01 00:00:00
abstract::Although cytopenia is a common manifestation of myelodysplastic syndrome (MDS), isolated thrombocytopenia is rare. The term "refractory thrombocytopenia" (RTC) has been proposed as a counterpart of refractory anemia. We describe here a case of RTC associated with chromosome abnormality on 11q23. A 59-year-old man was ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050209
更新日期:1996-08-01 00:00:00
abstract::To investigate whether cytokine genetic polymorphisms influence the outcome of diffuse large B cell lymphoma (DLBCL), we tested 337 consecutive DLBCL treated with CHOP or rituximab-CHOP (R-CHOP) from interleukin 10 (IL10), Bcl-2, and tumor necrosis factor (TNF)-α polymorphisms. Patients who carried the IL10 rs1800871 ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-016-2857-x
更新日期:2017-02-01 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::A patient with painful peripheral neuropathy is presented, whose symptoms were thought to result from an infection with Borrelia burgdorferi sensu lato. Investigations of the cerebrospinal fluid for signs of inflammation and borrelial antibodies were negative, and the patient did not benefit from repeated antibiotic t...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770000267
更新日期:2001-04-01 00:00:00
abstract::The frequency of apoptotic cells in bone marrow trephine biopsies and cytospins of immunomagnetically isolated myeloid progenitor cells was determined in 39 patients with chronic idiopathic neutropenia (CIN) and 12 hematologically normal individuals using the in situ end-labeling (ISEL) apoptosis detection method. We ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0709-y
更新日期:2003-10-01 00:00:00
abstract::Cryoglobulinemia is uncommon in Southern Chinese in Hong Kong, with tropical climates and low incidence of hepatitis C virus (HCV) infection. Eight positive cases were detected among 481 patients screened for cryoglobulins over a 10-year period. Three HCV carriers (38%) ran benign courses. The others included two carr...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-004-0955-7
更新日期:2005-02-01 00:00:00
abstract::Imatinib is a new promising therapeutic option for chronic myeloid leukemia (CML) with efficacy even in the blast phase of the disease. However, most patients treated with imatinib in the blast phase develop progressive disease rapidly. Thus, treatment with imatinib has to be followed by other treatment strategies. Th...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0643-z
更新日期:2003-05-01 00:00:00
abstract::The hypomethylating agents azacytidine and decitabine are unaffordable for many patients with MDS. The combination of the DNA methyltransferase inhibitor hydralazine and the histone deacetylase inhibitor valproate has shown preliminary efficacy in MDS. The aim of this study is to evaluate the clinical efficacy and saf...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-017-3103-x
更新日期:2017-11-01 00:00:00
abstract::Iron deficiency anemia is among the most frequent causes of disability. Intravenous iron is the quickest way to correct iron deficiency, bypassing the bottleneck of iron intestinal absorption, the only true mechanism of iron balance regulation in human body. Intravenous iron administration is suggested in patients who...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-020-04361-3
更新日期:2020-12-02 00:00:00
abstract::In 1989, a prospective randomized multicenter study was initiated in order to determine the safety and efficacy of oral clodronate in myeloma patients. The primary objective of this long-term trial is to evaluate whether supportive clodronate is able to prevent or retard the progression of bone disease and reduce the ...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1007/BF01697625
更新日期:1993-03-01 00:00:00
abstract::The laboratory diagnosis of hereditary spherocytosis (HS) is based on several screening and confirmatory tests; our algorithm includes clinical features, red blood cell morphology analysis and cryohaemolysis test, and, in case of positive screening, sodium dodecyl sulphate polyacrylamide gel electrophoresis as a diagn...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-014-2127-8
更新日期:2014-11-01 00:00:00
abstract::Primary mediastinal large B cell lymphomas (MLCL) differ from other diffuse large cell lymphomas, leading to a description as a separate entity in the current World Health Organization classification. Dose intensification improves long-term results, but no standard therapy has been established so far. We investigated ...
journal_title:Annals of hematology
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1007/s00277-008-0625-2
更新日期:2009-05-01 00:00:00
abstract::Arsenic trioxide (As2O3) is a highly effective agent in the treatment of acute promyelocytic leukemia (APL), whereas other hematopoietic tumors are less responsive to this agent and mechanisms underlying As2O3,-resistance are poorly understood. To better understand the complex network of GSH-related pathways in As2O3 ...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-006-0139-8
更新日期:2006-10-01 00:00:00
abstract::Acute megakaryoblastic leukaemia (AML-M7) is an uncommon disease, composing 0.5-1.2% of newly diagnosed adult acute myeloid leukaemias (AML). It is characterised by higher incidence and complexity of cytogenetic abnormalities. We report a rare case of Philadelphia (Ph) chromosome-positive AML-M7, presenting with extre...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s00277-003-0783-1
更新日期:2004-06-01 00:00:00
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...
journal_title:Annals of hematology
pub_type: 杂志文章
doi:10.1007/s002770050347
更新日期:1997-11-01 00:00:00
abstract::In the context of discussions on the reproducibility of clinical studies, we reanalyzed a prospective randomized study on the role of splenic irradiation as adjunct to the conditioning for hematopoietic stem cell transplantation (HSCT) for chronic myeloid leukemia (CML). Between 1986 and 1989, a total of 229 patients ...
journal_title:Annals of hematology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00277-016-2638-6
更新日期:2016-05-01 00:00:00