DIAPH1 Mutation as a Novel Cause of Autosomal Dominant Macrothrombocytopenia and Hearing Loss.

abstract：OBJECTIVE:Adherence to tyrosine kinase inhibitor treatment is a significant factor in the achievement of a good clinical response in chronic myeloid leukemia (CML). The aim of this retrospective study is to investigate 1- and 2-year medication adherence to imatinib treatment, linking adherence rates with the clinical o...

journal_title：Acta haematologica

authors： Santoleri F,Lasala R,Ranucci E,La Barba G,Di Lorenzo R,Vetrò A,Di Bartolomeo P,Costantini A

更新日期：2016-01-01 00:00:00

abstract：:Among the 193 patients (82 female, 111 male) treated primarily for Hodgkin's disease at our clinic between 1990 and 2001 and followed up until 2003, 42 (22%) had mediastinal bulky tumours (MBTs) by the Cotswolds criteria. The rate of MBT diagnosis was significantly greater in the early stage of the disease, these pati...

journal_title：Acta haematologica

authors： Keresztes K,Lengyel Z,Devenyi K,Vadasz G,Miltenyi Z,Illes A

更新日期：2004-01-01 00:00:00

abstract：:Agranulocytosis developed in a 20-year-old Greek patient with beta-thalassaemia major, 11 weeks after commencing chelation with the oral iron chelator 1,2-dimethyl-3-hydroxypyrid-4-one (L1) and 6 weeks after receiving the drug at a total daily dose of 105 mg/kg. The patient presented with generalised weakness, low-gra...

journal_title：Acta haematologica

authors： al-Refaie FN,Veys PA,Wilkes S,Wonke B,Hoffbrand AV

更新日期：1993-01-01 00:00:00

abstract：:A diester lipase activity is described in human red blood cells (RBC). Diester lipase activity acts as a membrane-bound enzyme and is assayed using intact RBC as the enzyme source. An emulsion of di-[3H]-oleoylglycerol (0.6 mM) serves as the substrate. The optimum pH for the reaction is 7.8 at 37 degrees C. Lipolytic ...

journal_title：Acta haematologica

authors： Somma C,Arnaud J,Boyer J

更新日期：1983-01-01 00:00:00

abstract：BACKGROUND:Splanchnic vein thrombosis (SVT) is a severe complication of essential thrombocythemia (ET). No clear explanation has been given for the occurrence of thrombosis in this unusual site in patients with ET, but the existence of a specific association between unexplained SVT and the JAK2 mutation has been report...

journal_title：Acta haematologica

authors： Allegra A,Alonci A,Penna G,D'Angelo A,Rizzotti P,Granata A,Musolino C

更新日期：2009-01-01 00:00:00

abstract：:Two unrelated patients with Bernard-Soulier syndrome and their relatives were studied. The patients demonstrated severe bleeding diathesis, the relatives were asymptomatic. The propositi showed the characteristic abnormalities of the syndrome: thrombocytopenia, a percentage of giant platelets higher than 65%, prolonge...

journal_title：Acta haematologica

authors： De Marco L,Fabris F,Casonato A,Fabris P,Dal Ben MG,Barbato A,Girolami A

更新日期：1986-01-01 00:00:00

abstract：:The distribution of thymus-dependent (T cells) and bone marrow-derived lymphocytes (B cells) was studied in 74 patients with Hodgkin's disease and 33 normal controls. A T cell deficit was found in untreated patients as well as in long-term survivors in remission. Therapy slightly enhanced the T cell depletion in Hodgk...

journal_title：Acta haematologica

authors： Many A,Biniaminov M,Rosenthal E,Aghai E,Ramot B

更新日期：1976-01-01 00:00:00

abstract：:The increased longevity of patients with thalassemia and sickle cell disease (SCD) introduces new clinical challenges due to the accumulation of disease-related morbidity, psychosocial issues and health care adjustments. Patients with thalassemia major now live into adulthood without suffering heart failure but must c...

journal_title：Acta haematologica

authors： Yacobovich J,Tamary H

更新日期：2014-01-01 00:00:00

abstract：:Two subjects, not previously reported in detail, had severe inherited deficiencies of erythrocyte pyrimidine nucleotidase. This was manifested hematologically by moderate hemolytic anemia with splenomegaly, morphologically by punctate basophilic stippling of Wright's stained erythrocytes, and biochemically by intraery...

journal_title：Acta haematologica

authors： Paglia DE,Fink K,Valentine WN

更新日期：1980-01-01 00:00:00

abstract：:It is unusual to find microorganisms in peripheral blood smears, and their presence is frequently associated with overwhelming sepsis and consequently a poor prognosis. In this report, we demonstrate 4 cases with bacteria in blood smears. Two of them had a fatal outcome, but the other 2 were caused by a contamination ...

journal_title：Acta haematologica

authors： van der Meer W,Verwiel JM,Gidding CE,de Metz M,de Keijzer MH

更新日期：2002-01-01 00:00:00

abstract：:One of the major determinants of erythrocyte survival is membrane deformability, and an important intrinsic parameter of membrane deformability is the shear elastic modulus (mu) with higher mu values corresponding to increased membrane rigidity. Using a micropipette technique, we determined the shear elastic modulus o...

journal_title：Acta haematologica

authors： Athanassiou G,Symeonidis A,Kourakli A,Missirlis YF,Zoumbos NC

更新日期：1992-01-01 00:00:00

abstract：:Platelet functions were evaluated in 20 healthy pregnant women in the last months of pregnancy, during labor, and 5-12 weeks after labor. The results showed an increased platelet count, bleeding time and nitroblue tetrazolium (NBT) test, as well as an increased plasma calcium level postlabor. ADP- and epinephrine-indu...

journal_title：Acta haematologica

authors： Sagi A,Creter D,Goldman J,Djaldetti M

更新日期：1981-01-01 00:00:00

abstract：:Although multiple myeloma (MM) remains an incurable disease, its treatment has improved over the past decade. This improvement has been at least in part due to the introduction of novel antimyeloma agents with new mechanisms of action, including those that target both myeloma cells and the tumor microenvironment, with...

journal_title：Acta haematologica

authors： Cibeira MT,Mercadal S,Arenillas L,Muntañola A,Salamero O,Bladé J

更新日期：2006-01-01 00:00:00

abstract：:We describe in this paper a female patient affected by chronic granulomatous disease with all the features of the classic X-linked form of the disease and presenting a mild form of the disease, the major clinical manifestation being a granulomatous cheilitis. The capability of the patient's phagocytes to undergo a res...

journal_title：Acta haematologica

authors： Dusi S,Poli G,Berton G,Catalano P,Fornasa CV,Peserico A

更新日期：1990-01-01 00:00:00

abstract：:Recently, a factor was discovered in the serum of hepatectomized animals which was capable of augmenting the hepatic erythropoietin response to hypoxia when injected into normal rats. This substance was localized in the liver via an in situ perfusion technique and was termed the hepatic erythropoietic factor (HEF). Pa...

journal_title：Acta haematologica

authors： Naughton BA,Liu P,Naughton GK,Gordon AS

更新日期：1983-01-01 00:00:00

abstract：OBJECTIVE:We aimed to determine the effects of intravenous iron therapy on blood parameters in pediatric patients who do not tolerate oral iron therapy for any reason. PATIENTS AND METHODS:The patient group consisted of candidates for elective operations requiring blood transfusions in order to raise hemoglobin (Hb) c...

journal_title：Acta haematologica

authors： Akarsu S,Taskin E,Yilmaz E,Yilmaz H,Kilic M,Aygun AD

更新日期：2006-01-01 00:00:00

abstract：:Automated cytochemistry by the Hemalog D carries out leukocyte differential counts in a continuous flow mode on whole blood EDTA-collected samples, through the optical measurement of enzyme activity and cell size. This operative principle thus parallels the basis of the FAB classification of acute leukemias. Although ...

journal_title：Acta haematologica

authors： d'Onofrio G,Mango G

更新日期：1984-01-01 00:00:00

abstract：:We describe a B cell chronic lymphocytic leukemia/lymphoma (B-CLL) patient with an adrenocortical adenoma. He was treated initially with oral cyclophosphamide and prednisolone for 2 months, followed by low-dose prednisolone for an additional 2 years, which resulted in the prompt disappearance of CLL cells. After a per...

journal_title：Acta haematologica

authors： Adachi Y,Adachi M,Hinoda Y,Fukushima H,Takahashi T,Imai K

更新日期：1997-01-01 00:00:00

abstract：:Low-risk patients with myelodysplastic syndromes (MDS) are inclined to long-term accumulation of iron in the organs due mostly to red blood cell transfusion and ineffective erythropoiesis. The effect of free toxic iron species in the liver and heart sites is well known, but recent knowledge assumes that oxidant-mediat...

journal_title：Acta haematologica

authors： Pilo F,Caocci G,Mele G,La Nasa G

更新日期：2020-10-02 00:00:00

abstract：:Two human stromal cell lines were established previously from bone marrow-derived primary long-term cultures by immortalization using the SV40 large T antigen and cellular cloning. After irradiation, the fibroblast-like cell lines L87/4 and L88/5 support hematopoietic differentiation of allogeneic cord blood cells in ...

journal_title：Acta haematologica

authors： Thalmeier K,Meissner P,Moosmann S,Sagebiel S,Wiest I,Huss R

更新日期：2001-01-01 00:00:00

abstract：:The management of severe late-onset hemorrhagic cystitis (LO-HC) after allogeneic hematopoietic stem cell transplantation (HSCT) is still challenging. Because mesenchymal stromal cells (MSCs) possess anti-inflammatory and tissue repair-promoting properties, we retrospectively analyzed the efficacy and safety of MSC in...

journal_title：Acta haematologica

authors： Wang Y,Chen F,Gu B,Chen G,Chang H,Wu D

更新日期：2015-01-01 00:00:00

abstract：:A case of acute leukaemia with t(4;11) chromosomal abnormality in a 28-year-old woman is reported. At diagnosis, two blast cell populations were seen: 60% of the cells were small cells with lymphoid morphology, 40% were large cells with monocytic morphology. Cytochemical examination was consistent with acute myeloid l...

journal_title：Acta haematologica

authors： Campos L,Espinouse D,Felman P,Charrin C,Gentilhomme O,Germain D

更新日期：1986-01-01 00:00:00

abstract：:The last few years have seen an enormous increase in our knowledge on the haematopoietic growth factor erythropoietin (Epo), firstly with its purification and determination of its primary amino acid sequence, and more recently with the isolation of the Epo gene and its expression in mammalian cell lines. This review a...

journal_title：Acta haematologica

authors： Foa P

更新日期：1991-01-01 00:00:00

abstract：:A study of E-rosette ultrathin sections has been undertaken in an attempt to analyze changes induced during the T-lymphocyte-sheep red blood cell interaction on the rosetted lymphocyte surface architecture. The formation of nonimmune rosettes implies different types of attachment between lymphocytes and sheep red bloo...

journal_title：Acta haematologica

authors： Renau-Piqueras J,Cervera J

更新日期：1979-01-01 00:00:00

abstract：:To assess the effect of prophylactic treatment with antithymocyte globulin (ATG) on graft-versus-host disease (GvHD) in myeloablative transplant patients, we performed a meta-analysis of randomized and cohort studies. Medline, Embase, the Cochrane Controlled Trial Register and the Science Citation Index were searched ...

journal_title：Acta haematologica

authors： Sheng Z,Ma H,Pang W,Niu S,Xu J

更新日期：2013-01-01 00:00:00

abstract：:Platelet aggregation in citrated and heparinized plasma by ionophore A 23187 and Ristocetin was studied in normal subjects and in patients with von Willebrand's disease and congenital afibrinogenemia. Aggregation by ionophore was normal in all groups both in citrated and heparinized plasma. Aggregation by Ristocetin i...

journal_title：Acta haematologica

authors： Girolami A,Fabris F,Marco L,Peruffo R

更新日期：1976-01-01 00:00:00

abstract：:The expression of the enzyme marker terminal deoxynucleotidyl transferase (TdT) was examined by immunofluorescence assay in the cells from 333 cases with various types and subtypes of leukemia or lymphoma. More than 90% of cALL and T-ALL, 70% of Null-ALL and 80% of pre-B-ALL were TdT-positive. One case in the commonly...

journal_title：Acta haematologica

authors： Drexler HG,Menon M,Minowada J

更新日期：1986-01-01 00:00:00

abstract：:Marginal-zone B cells of the mucosa-associated lymphoid tissue (MALT) are the normal counterpart of the neoplastic cells in MALT lymphoma. In both cases these lymphocytes express surface immunoglobulins, but are negative when stained for B cell associated antigens like CD10 and CD23. Furthermore, the B cell gene rearr...

journal_title：Acta haematologica

authors： Fritscher-Ravens A,Petrasch S,Tiemann M,Wacker H,Dörr T,Kemmeries G,Hilgers W,Reinacher-Schick A,Kunstmann E,Schmiegel W

更新日期：1999-01-01 00:00:00

abstract：:Plasma samples from patients with disseminated intravascular coagulation (DIC) associated with acute promyelocytic leukemia (APL) exhibited higher levels of the D-fragment of fibrin and fibrinogen degradation products [FDP(D)], with relatively lower levels of cross-linked fibrin degradation products (XDP), than sample...

journal_title：Acta haematologica

authors： Okajima K,Koga S,Okabe H,Inoue M,Takatsuki K

更新日期：1989-01-01 00:00:00

abstract：:Upper-extremity deep venous thrombosis (UEDVT) is an increasingly important clinical problem in children. These events are classified as primary or secondary, with the latter being the most common and usually associated with the presence of a central venous line. Among primary UEDVT, the so-called Paget-Schroetter syn...

journal_title：Acta haematologica

authors： Brandão LR,Williams S,Kahr WH,Ryan C,Temple M,Chan AK

更新日期：2006-01-01 00:00:00