bluuebunny/crossref_metadata_2025_split huggingface dataset:
Splenic sequestration associated with sickle cell trait and hereditary spherocytosis
Authors: Yang Yih‐Ming , Donnell Cynthia , Wilborn Walter , Goodman Steven R. , Files Bea , Moore R. Blaine , Mohandas Narla , Mankad Vipul N. 
Published: February 2007

AbstractCoexistence of sickle cell trait and hereditary spherocytosis (HS) is unusual, and only 16 cases have been reported in the literature. These patients have the same clinical and hematological features as individuals having HS alone. We report a serious complication, acute splenic sequestration crisis (ASSC), occurring in two patients with sickle cell trait and HS. One patient experienced four episodes of ASSC during an 11‐year span, while the other had two episodes of this complication during a 4‐year period. Red blood cell studies and membrane protein analysis confirmed the diagnosis of HS as a consequence of spectrin deficiency. Splenectomy resulted in marked clinical and hematological improvement in both patients. Histological examination of spleens following splenectomy confirmed that significant erythrostasis and sickling had indeed occurred. ASSC can occur in patients with coexistence of sickle cell trait and HS, and this potentially life‐threatening complication should be considered in this condition. © 1992 Wiley‐Liss, Inc.
A study of lymphoma of large granular lymphocytes with modern modalities: Report of two cases and review of the literature
Authors: Sun Tsieh , Schulman Philip , Kolitz Jonathan , Susin Myron , Brody Judith , Koduru Prasad , Muuse William , Hombal Shiril , Teichberg Saul , Broome John 
Published: February 2007

AbstractTwo cases of lymphoma of large granular lymphocytes are reported. The first case expressed natural killer (NK) cell, some T‐cell (CD 2, CD 5, CD 8), and HLA‐DR antigens, but was negative for other T‐cell (CD 3, CD 4, CD 7), T‐cell receptor (TCR), B‐cell, and myeloid antigens. Germline configuration was demonstrated for TCR, and immunoglobulin heavy and light chain genes. The second case expressed NK cell, some T‐cell (CD 3, CD 7, CD 8), and TCR antigens, but was negative for other T‐cell (CD 4, CD 5), B‐cell, myeloid, and HLA‐DR antigens. Rearrangement of TCR α and β chains were detected. Thus, the findings of case 1 were consistent with true NK cell lineage and case 2 with NK‐like T‐cell lineage. Our report underscores the heterogeneity of this newly recognized lymphoma, which nevertheless carries a consistently poor prognosis and is probably more prevalent in the Asian population. This study also provides information concerning immunopheno‐types of cellular infiltrates in internal organs and cytogenetic abnormalities in this lymphoma; neither has been reported frequently in the literature. The importance of detecting cytoplasmic granules in tissue imprints or electron micrographs for differentiating other T‐cell lymphomas is emphasized, and the classification of large granular lymphoproliferative disorders is discussed. © 1992 Wiley‐Liss, Inc.
Acquired von willebrand disease: Correction of hemostatic defect by high‐dose intravenous immunoglobulins
Authors: Delmer A. , Horellou M. H. , Bréchot J. M. , Prudent J. , Potevin F. , Lecrubier C. , Girard‐Longhini C. , Samama M. , Zittoun R. 
Published: February 2007

AbstractHigh‐dose intravenous immunoglobulins (ivIG) were used in a 57‐year‐old patient with acquired von Willebrand disease in order to correct a hemostatic defect before pneumonectomy for lung carcinoma. IvIG induced a rapid and complete correction of factor VIII (F VIII) and von Willebrand factor (vWF) and allowed surgery without additional factor coverage. F VIII and vWF returned to baseline values within 10 days after ivIG. © 1992 Wiley‐Liss, Inc.
Genetic epidemiology of β‐thalassemia in sicily: Do sequences 5′ to the Gγ gene and 5′ to the β gene interact to enhance HbF expression in β‐thalassemia?
Authors: Ragusa Angela , Lombardo Mario , Beldjord Cherif , Ruberto Concetta , Lombardo Turi , Elion Jacques , Nagel Ronald L. , Krishnamoorthy Rajagopal 
Published: February 2007

AbstractThe present epidemiological study of the molecular characteristics of β‐thalassemia in Sicily was prompted by the disparate phenotypic expression (in clinical status and absolute HbF level) observed in two β‐thalassemic homozygotes who were also homozygous for the β‐like globin gene cluster haplotype III. We suspected that polymorphisms within haplotype III could be the cause for the discrepancy. Based on the association of particular conformations of the (AT)xTy motif (‐540 5′ to the β gene) with milder forms of thalassemia and sickle cell anemia, 38 homozygous β‐thalassemia patients were studied to define their haplotypes, the −158 site 5′ to the Gγ gene (linked to haplotype III) and the structure of the (AT)xTy motif. We found that the patient who was phenotypically mild and homozygous for β‐thalassemia, haplotype III, and the −158 C→T mutation was homozygous for the rare (AT)9T5 motif. In contrast, the patient homozygous for β‐thalassemia, haplotype III, and the —158 mutation, but exhibiting a severe clinical course, was homozygous for the (AT)7T7 configuration. Others have suggested that (AT)9T5 is a negative regulatory protein binding sequence, and it is a silent carrier state for β‐thalassemia. The usual configuration (AT)7T7, has considerably less affinity for regulatory protein binding, and it is the most common configuration in Sicilian β‐thalassemics (67 of the 78 chromosomes studied). Within the 38 patients studied, seven were informative because they had various combinations of the (AT)9T5 and (AT)7T7 motif, and the −158 C→T mutation. The results in these patients suggest that only the co‐presence of the (AT)9T5 configuration and a C→T change at −158.5′ to the Gγ gene is associated with high HbF expression and a mild clinical phenotype. We postulate that these two regions of the β‐like globin gene cluster interact, when endowed with the proper sequences, to enhance the expression of HbF secondary to anemia. © 1992 Wiley‐Liss, Inc.
Coagulation factor IX: Successful surgical experience with a purified factor IX concentrate
Authors: Goldsmith Jonathan C. , Kasper Carol K. , Blatt Philip M. , Gomperts Edward D. , Kessler Craig M. , Thompson Arthur R. , Herring Steve W. , Novak Patricia L. 
Published: February 2007

AbstractThe use of plasma‐derived coagulation factor concentrates has been marked by the transmission of viral agents. Infusions of factor IX complex concentrates have been additionally complicated by inappropriate thrombosis. Use of these concentrates in the neonate, in those with liver disease, and in surgical patients results in increased risk for this complication. Twenty patients have been infused with a purified coagulation factor IX concentrate for fall‐off and recovery studies. A two‐compartment model indicated an initial phase half‐life of 4.06 ± 2.86 hr and a β phase half‐life of 20.0 ± 3.8 hr following the administration of AlphaNine®, Coagulation Factor IX (Human). In vivo recovery was 62.7% ± 13.8%, with an average factor IX coagulant level of 73% ± 16% at 15 min after the infusion of a mean dose of 45 U/kg. Thirteen previously transfused patients with hemophilia B underwent major orthopedic or general or dental surgery using this purified factor IX. Operative outcomes were excellent in all patients. No excessive bleeding was noted. There was no laboratory or clinical evidence for a disseminated intravascular coagulopathy. The excellent surgical outcomes observed in this multitransfused group with biochemical evidence for active liver disease demonstrates the utility and safety of a purified coagulation factor IX concentrate. © 1992 Wiley‐Liss, Inc.
Effect of interleukin‐1, tumor necrosis factor‐α, and interferon‐α on the blast cells of acute myeloblastic leukemia
Authors: Carter Anna , Silvian‐Draxler Iiana , Tatarsky Iiana 
Published: February 2007

AbstractIn this study, we further established the role of interleukin‐1α (IL‐1α), interleukin‐1β, tumor necrosis factor‐α (TNF‐α), and interferon‐α (IFN‐α) as regulators of proliferation of acute myeloid leukemia (AML) cells. AML cells from 8 of 15 patients incorporated high levels of 3H‐thymidine (3H‐TdR) in the absence of exogenous growth factors. The spontaneous DNA synthesis could be abrogated with monospecific antibodies directed toward IL‐1α, IL‐1β, or TNF‐α, as well as with antigranulocyte‐macrophage colony‐stimulating factor (GM‐CSF). Human recombinant GM‐CSF reversed the inhibitory action of each of these antibodies and reinduced DNA synthesis in AML cells. Thus, in these cases, constitutively produced IL‐1 or TNF‐α had stimulated the synthesis of GM‐CSF, which resulted in GM‐CSF‐dependent proliferation of AML blasts. Exogenous IL‐1 up‐regulated the endogenous production of GM‐CSF, suggesting a positive regulation of autocrine growth factor production. We also present evidence that TNF‐α may exert both stimulative as well as inhibitory effects on DNA synthesis in AML cells. The enhancing effect of TNF‐α was mediated through the induction of GM‐CSF production, as stimulation of DNA synthesis in AML blasts could be abrogated with anti‐GM‐CSF antibody. A concentration‐dependent inhibitory effect of TNF‐α on 3H‐TdR incorporation into AML blasts was observed only when these cells were grown in the absence of GM‐CSF. Finally, we show that human recombinant IFN‐α is a potent inhibitor of AML cell proliferation in vitro. © 1992 Wiley‐Liss, Inc.
Regulation of transferrin receptors by iron in human erythroblasts
Authors: Abe Yasunobu , Muta Koichiro , Nishimura Junji , Nawata Hajime 
Published: February 2007

AbstractWe investigated the regulatory mechanism of human erythroblast transferrin receptors (Tf.R) under conditions of iron deprivation and iron loading. Treatment of erythroblasts with an iron chelator, desferrioxamine, induced an increase in surface Tf.R number associated with an elevation of biosynthetic rate and the mRNA level of Tf.R. Reduced cellular iron pool increased the Tf.R number by altering the level of mRNA, as in nonhemoglobinproducing cells. Although treatment of erythroblasts with hemin induced a decrease in the biosynthetic rate and in the level of mRNA, the number of surface Tf.R did not decrease. This phenomenon may explain the fact that a high level of serum iron has no influence on the surface Tf.R number in vivo, as we reported previously. We suggest the existence of a regulatory mechanism specific for hemoglobin‐producing cells that keeps surface Tf.R expression constant despite iron loading. © 1992 Wiley‐Liss, Inc.
Enhanced platelet reactivity and hypercoagulability in the steady state of sickle cell anaemia
Authors: Berney S. I. , Ridler C. D. , Stephens A. D. , Thomas A. E. , Kovacs I. B. 
Published: February 2007

AbstractA prospective controlled study was undertaken to investigate the haemostatic and coagulation status of 18 adult subjects in the steady state of sickle cell anaemia (SCA), using a relatively new in vitro technique. Shear induced haemostasis, whole blood dynamic coagulation, and spontaneous thrombolysis were measured using nonanticoagulated blood. As expected, the haemoglobin levels were significantly lower and platelet counts significantly higher in subjects with SCA compared with controls. Haemostasis and coagulation were significantly enhanced in SCA. No correlation was found between the raised platelet count and enhanced haemostasis or the reduced haemoglobin and hypercoagulation, respectively. Hyperactivity of the haemostatic system may have a pathogenic role in vaso‐occlusive microthrombotic events and in the leg ulcers, both of which occur frequently in SCA. © 1992 Wiley‐Liss, Inc.
Interleukin‐5 in eosinophilic gastroenteritis
Authors: Takahashi Takayuki , Nakamura Kishiko , Nishikawa Satoru , Tsuyuoka Reiko , Suzuki Akira , Murakami Masao , Amenomori Masahiro , Okuno Yoshiaki , Imura Hiroo 
Published: February 2007

AbstractDuring a 4‐year period a 28‐year‐old female had 4 episodes of eosinophilia of over 10,000/μ1; these episodes were associated with nausea, vomiting, diarrhea, and abdominal pain. On one occasion, she had ascites and pleural effusion which contained numerous mature eosinophils. On each occasion, these attacks disappeared within several weeks without any specific treatment. A diagnosis of eosinophilic gastroenteritis was made. A plasma sample obtained during the eosinophilia generated in vitro eosinophilic colonies when added to granulocyte/macrophage‐progenitor (CFU‐GM) cultures without exogeneous growth factors. Colony formation was inhibited by anti‐interleukin‐5 (IL‐5) antibody but not by antibodies toward IL‐3, granulocyte colony‐stimulating factor (G‐CSF) or GM‐CSF. A high plasma interleukin‐5 (IL‐5) level was noted when measured by enzyme‐linked immunosorbent assay, while IL‐3, G‐CSF, and GM‐CSF were undetectable. During remission the plasma gave negative results both for colony formation and IL‐5 level. These results indicate that the eosinophilia of this disease is mediated by IL‐5. © 1992 Wiley‐Liss, Inc.
Spanish Rhnull family caused by a silent Rh gene: Hematological, serological, and biochemical studies
Authors: Pérez‐Pérez Carmen , Taliano Victor , Mouro Isabelle , Huet Martine , Salat‐Marti Antonio , Martinez Antonio , Rouger Philippe , Cartron Jean‐Pierre 
Published: February 2007

AbstractAnother example of rare red cells that failed to react with all anti‐Rh and anti‐LW antibodies was discovered in a Spanish woman suffering from a severe hemolytic anemia typical of the Rhnull syndrome. Family study and Rh blood typings demonstrated clearly that the proposita was homozygous for a silent Rh gene complex (Rhnull of the amorph type) that she inherited from her parents who are first cousins. Western blot analysis carried out with glycosylation‐independent antibodies directed against the Rh polypeptide and the LW glycoprotein, respectively, confirmed that these protein components were absent from the red cells of the proposita. In addition, the patient was typed U‐positive, again in agreement with the presence on her red cells of 45–75 kDa glycoproteins detected with the murine monoclonal antibody 2D10. © 1992 Wiley‐Liss, Inc.
Myelodysplastic syndromes and malignant solid tumors: Analysis of 21 cases
Authors: Sans‐Sabrafen Jordi , Buxó‐Costa Josep , Woessner Soledad , Florensa Lourdes , Besses Carles , Malats NÚRia , Porta Miquel 
Published: February 2007

AbstractWe studied the association between myelodysplastic syndromes (MDS) and malignancies in a cohort of 155 patients with MDS, 21 of whom presented malignant solid tumors. Myelodysplasia was present after the diagnosis of cancer in eight patients (interval between the diagnosis of both conditions 18 months, median survival 49.5 months), simultaneously with diagnosis in 11 (median survival 8 months), and before malignancy in two patients (interval between the diagnosis of both conditions 47 and 7 months). One patient was given chemotherapy for lung cancer, and three patients received radiotherapy for adenocarcinoma of the kidney and cancer of the prostate. At the time of diagnosis of MDS, nine patients already presented metastatic spread. Fourteen patients died, ten as a result of tumor‐related complications and four because of transformation to acute nonlymphocytic leukemia. The analysis of the incidence of malignancy in patients with MDS was statistically significant for males, and the relative risk was significant in both sexes. The results of this study show that MDS patients present a higher incidence of malignant tumors than the general population, that MDS may be of real paraneoplastic significance, and that the occurrence of MDS in cancer patients may be considered to be related to the malignancy rather than an independent phenomenon. © 1992 Wiley‐Liss, Inc.
Circulating thrombomodulin as a novel endothelial cell marker: Comparison of its behavior with von willebrand factor and tissue‐type plasminogen activator
Authors: Takahashi Hoyu , Ito Seiki , Hanano Masaharu , Wada Ken , Niwano Hiroe , Seki Yoshinobu , Shibata Akira 
Published: February 2007

AbstractCirculating thrombomodulin is a novel endothelial cell marker, which may reflect the endothelial injury. Plasma levels of thrombomodulin were quantitated by an enzymelinked immunosorbent assay (ELISA) in patients with hematological malignancies, liver disease, diabetes mellitus, collagen disease, thrombotic disease, and disseminated intravascular coagulation (DIC), and the thrombomodulin values were compared with those of von Willebrand factor antigen (vWf:Ag) and tissue‐type plasminogen activator (t‐PA) which are released from stimulated or damaged endothelial cells. The mean plasma concentrations of thrombomodulin in these disease states were elevated as compared with healthy subjects. A relatively high mean thrombomodulin level was observed in DIC, liver disease, and collagen disease. Abnormally high thrombomodulin values (> normal mean value + 3 SD) were found in 32.3% of patients with hematological malignancies, 57.7% of patients with liver disease, 39.3% of patients with diabetes mellitus, 30.0% of patients with collagen disease, 23.1% of patients with thrombotic disease, and 69.0% of patients with DIC. Plasma concentrations of both vWf:Ag and t‐PA were also elevated in these patients. On the whole, the plasma thrombomodulin concentration was positively correlated with vWf:Ag (r = 0.441, P < 0.001) and t‐PA (r = 0.398, P < 0.001). These findings indicate that the elevation of plasma thrombomodulin is frequently seen in a variety of diseases and circulating thrombomodulin is possibly useful for evaluating the endothelial damage in selected disease states. © 1992 Wiley‐Liss, Inc.
Human interleukin‐9 supports formation of a subpopulation of erythroid bursts that are responsive to interleukin‐3
Authors: Sonoda Yoshiaki , Maekawa Taira , Kuzuyama Yuko , Abe Tatsuo , Clark Steven C. 
Published: February 2007

AbstractWe have investigated the biological activities of recombinant human interleukin‐9 (IL‐9) on enriched hematopoietic progenitors, alone or in combination with other cytokines, including Epo, G‐CSF, IL‐3, and GM‐CSF, under serum‐containing and serum‐free cultures. IL‐9 alone did not support colony formation. However, IL‐9 plus Epo induced erythroid burst (BFU‐E) formation derived from peripheral blood (PB) progenitors. Delayed addition experiments demonstrated that a part of bone marrow (BM) derived BFU‐E, which seems to be immature, only responded to IL‐9 and formed erythroid bursts. The burst‐promoting activity (BPA) of IL‐9 was confirmed using neutralizing aIL‐3, aGM‐CSF, and aIL‐9 antisera and serum‐free culture. IL‐9 supported a part of BFU‐E population that respond to IL‐3, which was almost identical to the number of BFU‐E supported by GM‐CSF. IL‐9 had no additive effect on erythroid and mixed colony formation supported by IL‐3. In contrast, IL‐9 showed an additive effect on erythroid burst formation supported by GM‐CSF in serum‐free culture. These data suggest that IL‐9 and GM‐CSF act on distinct IL‐3‐responsive BFU‐E population. In addition, delayed addition experiment clearly demonstrated that IL‐9 supports survival and the early stage of proliferation of BFU‐E. These results led us to propose that IL‐9 possibly acts as a BPA and selectively supports a subpopulation of early class of BFU‐E that respond to IL‐3. © 1992 Wiley‐Liss, Inc.
Black β‐thalassemia homozygotes with specific sequence variations in the 5′ hypersensitive site‐2 of the locus control region have high levels of fetal hemoglobin
Authors: Beris Ph. , Kitundu M. N. , Baysal E. , Öner C. , Lanclos K. D. , Dimovski A. J. , Kutlar F. , Huisman T. H. J. 
Published: February 2007

AbstractWe have sequenced the 5′ hypersensitive‐2 (5′HS‐2) site of the locus control region (LCR) and the promoters of the two γ‐globin genes located on chromosome 11 of a black patient with mild β‐thalassemia (β‐thal) major due to a homozygosity for the C → T mutation at position – 88 of the β promoter and with a high Hb F level. Sequence variations in the 5′HS‐2 were the same as observed for the βs chromosome with haplotype number 3, while most of the Gγ promoter and the Aγ promoter had sequences similar to that of the βs chromosome with haplotype number 19. This atypical haplotype (number 19A) is apparently associated with an increased γ chain production which is particularly evident during periods of severe hematopoietic stress. Additional studies on relatives of the proband and on 10 unrelated black β‐thal homozygotes with either the C → T mutation at – 88 or the A → G mutation at –29, confirm the possible importance of the sequence differences in the 5′HS‐2, and also suggest that at least two additional factors, namely a C → T mutation at position –158 of the Gγ promoter and a relative deficiency in α chain synthesis play a (perhaps less important) role in the increased Hb F synthesis in these patients. © 1992 Wiley‐Liss, Inc.
Sequential maturation stages of monoclonal B lineage cells from blood, spleen, lymph node, and bone marrow from a terminal myeloma patient
Authors: Jensen Gitte S. , Mant Michael J. , Pilarski Linda M. 
Published: February 2007

AbstractIn order to fully understand the complexity of the monoclonal B lineage cells in multiple myeloma, it is necessary to evaluate the extent to which these cells are resident in solid lymphoid tissues and the phenotypic differences and similarities as compared to the circulating or bone marrow derived B lineage cells. Peripheral blood mononuclear cells from a patient with multiple myeloma were obtained 8 and 3 days prior to death, and mononuclear cells from lymph nodes, spleen, and bone marrow were obtained at autopsy. Rapid changes in the stage of differentiation of blood late‐stage B lineage cells towards mature end‐stage plasma cells were observed during the last week prior to death. Lymphoid cells within the blood comprised very few T cells, sub‐normal numbers of monocytes, and 80% of B lineage cells which were at a late stage of differentiation. Shortly before death, plasma cells were found in the peripheral blood, indicating progression to plasma cell leukemia. At autopsy, the monoclonal B lineage cells in lymph node, spleen, and bone marrow represented different stages of terminal B cell differentiation. In each tissue, the B lineage cells were at an earlier differentiation stage, as defined phenotypically, than the circulating B lineage cells found in blood 3 days prior to death. Analysis of B cell markers and CD45 was used to define the differentiation stage of the relevant B cell populations, revealing a series of differentiation stages. The least mature B lineage cells (CD45hi) were found in lymph node. However, the CD45 isoform expressed was CD45R0, unlike most normal lymph node B cells. More differentiated B lineage cells (CD45mod) were found in the bone marrow, and three sequential stages of pre‐plasma cells were found in the spleen (CD45bright, CD45moderate and CD45low‐neg), all of which were CD45R0+. The B cells in normal spleen and bone marrow are CD45RA. The presence of monoclonal B lineage cells in spleen was confirmed by Southern blotting. The B lineage cells from peripheral blood 3 days prior to death were approaching an end‐stage plasma cell stage (CD45low/). On B lineage cells from the various myeloma tissues, a concomitant loss of CD11b and increasing density of CD29 were observed as a function of progression to terminally differentiated stages. © 1992 Wiley‐Liss, Inc.