bluuebunny/crossref_metadata_2025_split huggingface dataset:
Acute myeloblastic leukemia with hyperleukocytosis: Risk factors for early mortality in induction
Authors: Ventura Gerard J. , Hester Jeane P. , Smith Terry L. , Keating Michael J. 
Published: February 2007

AbstractEighty‐five patients with acute myeloblastic leukemia (AML) presenting with hyperleukocytosis (HL) were analyzed to assess morbidity and mortality in early induction. Patients who failed to achieve remission were older and more often had pulmonary leukostasis (62% vs 23%, p = .01) and hepatomegaly (54% vs 31%, p = .06) at presentation. Thirty‐seven patients (44%) did not achieve complete remission (CR); 17 (54%) died early in induction therapy, 11 directly as a result of pulmonary hemorrhage with respiratory failure, while 5 had both pulmonary hemorrhage with respiratory failure and CNS hemorrhage. Early death patients were older and more often had pulmonary leukostasis (88% vs 29%, p < .0001), hepatomegaly (71% vs 34%, p = .01), hyperbilirubinemia (60% vs 16%, p = .01) and hypofibrinogenemia (47% vs 12%, p < .01) at presentation. Primarily for technical reasons, preinduction leukapheresis was not employed as often in this very‐high‐risk group as in other patients (56% vs 82%, respectively). Thus, sufficient heterogeneity exists in patients presenting with HL to define a subset of patients at particularly high risk for early mortality. Preinduction leukapheresis applied in a prospective controlled fashion should be evaluated to assess if such treatment may decrease early mortality in this group.
Oxygen‐induced marrow red cell hypoplasia leading to transfusion in sickle painful crisis
Authors: Lane Philip K. , Embury Stephen H. , Toy Pearl T. C. Y. 
Published: February 2007

AbstractThe benefit of oxygen (O2) therapy in non‐hypoxic sickle cell patients in painful crisis is uncertain. We report a case of a non‐hypoxic sickle cell patient in painful crisis who developed marrow red cell hypoplasia requiring transfusion support after O2 therapy. The uncertain benefits of O2 use in such cases must be weighed against the serious and underrecognized risks of transfusion. In patients who develop O2‐induced marrow red cell hypoplasia, cessation of O2 therapy may reverse the anemia and obviate the need for transfusion.
Effects of purified human native granulocyte colony‐stimulating factor (G‐CSF) on proliferation of blast progenitors in acute myeloblastic leukemia
Authors: Nara Nobuo , Murohashi Ikuo , Suzuki Toshiya , Yamashita Yoko , Maruyama Yasuo , Aoki Nobuo , Tanikawa Shu , Onozawa Yasusuke 
Published: February 2007

AbstractBlast progenitors in acute myeloblastic leukemia (AML) grow in methylcellulose and suspension cultures. Blast colony formation in methylcellulose culture reflects the terminal divisions of blast progenitors, while secondary colony formation, by replating in methylcellulose and recovering clonogenic cells in suspension culture, reflects the self‐renewal of blast progenitors. To analyze the regulatory mechanisms of the proliferation of leukemic blast progenitors, the effects of highly purified native granulocyte colonystimulating factor (G‐CSF) obtained from human squamous cell carcinoma line (CHU‐2) on blast progenitors in AML patients were studied in methylcellulose and suspension cultures. Purified G‐CSF stimulated the growth of blast progenitors in both culture systems, although sensitivity to G‐CSF varied from patient to patient. No obvious maturation of leukemic blasts was noted in suspension culture in the presence of G‐CSF. The data suggest that a normal hemopoietic regulator may play a role in the growth of blast progenitors in AML patients.
Granulopoietic differentiation in long‐term bone marrow cultures from children with congenital neutropenia
Authors: Coulombel Laure , Morardet Nada , Veber Florence , Leroy Christine , Mielot Francoise , Fisher Alain , Teillet‐Thiebaud Françoise , Tchernia Gil , Griscelli Claude , Parmentier Claude 
Published: February 2007

AbstractThe capacity of granulopoietic precursor cells (CFU‐GM) to differentiate in vitro was evaluated in five children with congenital neutropenia using short‐term colony assays and long‐term marrow cultures. In all five children, methylcellulose assays revealed normal numbers of CFU‐GM, which displayed an appropriate response to various sources of GM‐CSF and differentiated up to the polymorphonuclear leukocyte state (PMN). In contrast, neutrophil PMN were not observed in long‐term bone marrow cultures from three patients, despite a normal production of CFU‐GM, myeloblasts, and promyelocytes during the 5‐6‐week culture period. Thus, in these patients, the characteristic “block” in granulocytic maturation observed in vivo was reproduced in vitro in long‐term cultures. Granulocytic differentiation proceeded normally in long‐term cultures from the two other patients, thus indicating heterogeneity in the expression of the defect. These results might indicate abnormal interactions between stromal and hematopoietic cells in long‐term marrow cultures from some patients with congenital neutropenia. Furthermore, our results showed some correlation between the granulocytic defect in vitro and the clinical outcome in vivo.
Prognostic implications of basophil differentiation in chronic myeloid leukemia
Authors: Denburg Judah A. , Browman George 
Published: February 2007

AbstractBasophilia has been reported to indicate an accelerated phase of chronic myeloid leukemia (CML), heralding a poor prognosis. We have studied 47 patients with chronic‐phase CML by basophil growth and differentiation assays in vitro, demonstrating an association between basophil growth index (BGI) and clinical time to blast crisis as well as overall survival. In addition to confirming an association between positive BGI and phase of CML in a larger group of patients, a positive BGI predicted death or blast crisis within 2 years' study of chronic phase CML (p<0.01), with a sensitivity of 78%, specificity, 81%; the positive predictive value of a positive test, 64%; and a negative predictive value of a negative test, 89%. The survival experience of the 22 evaluable patients with chronic‐phase CML and a positive BGI was significantly worse than the survival of the 19 patients with a negative BGI (p<0.0001). At 1, 2, and 3 years the proportion of surviving patients with a positive BGI was 0.64, 0.32, and 0.23, respectively, compared with 1.00, 0.90, and 0.79 for those with a negative BGI. The median survival of chronic phase CML patients with a positive test at diagnosis (n = 14) was 27 months versus 54 months for those with a negative diagnosis (n = 14) (p<0.05). These findings emphasize the prognostic utility of basophil growth assays in CML and suggest a molecular relationship between leukemic transformation and basophil lineage expression.
Metastatic extramedullary plasmacytoma: A case report and review of the literature of a rare pseudocarcinoma
Authors: Akiyama Hideki , Krigel Robert L. 
Published: February 2007

AbstractExtramedullary plasmacytomas are unusual plasma cell tumors arising outside the bone marrow. Most extramedullary plasmacytomas are solitary, but occasionally they disseminate, usually in conjunction with systemic myelomatosis. The authors report a case of disseminated extramedullary plasmacytoma with a picture suggestive of metastatic pancreatic carcinoma. This unusual presentation reconfirms the need for tissue diagnosis in all suspected cases of pancreatic carcinoma and demonstrates that extramedullary plasmacytoma can be an aggressive cancer without any sign of osseous or systemic disease.
Prolonged remission after life‐threatening gastrointestinal hemorrhage from coexistent angiodysplasia and acquired bleeding diathesis
Authors: Woodlock Timothy J. , Francis Charles W. , Rowe Jacob M. , Brown M. Julianne , Marder Victor J. 
Published: February 2007

AbstractA patient with angiodysplasia of the stomach and duodenum developed exceptionally severe and protracted gastrointestinal hemorrhage. Bleeding was intensified by a coexistent acquired hemostatic disorder characterized by decreased platelet aggregation with adenosine diphosphate, collagen, epinephrine and ristocetin, and a decrease in both plasma ristocetin cofactor activity and high‐molecular‐weight von Willebrand factor multimers. Cryoprecipitate infusion corrected the von Willebrand factor defect but did not improve platelet aggregation. Bleeding stopped after prolonged aggressive combined medical and surgical therapy, and the patient had no recurrence of bleeding while followed for 27 months. Hemorrhage from the vascular lesions themselves dominated the clinical picture of this patient and other reported patients with coexistent angiodysplasia and congenital or acquired hemostatic abnormalities.
Increased production of superoxide anion by neonatal polymorphonuclear leukocytes stimulated with a chemotactic peptide
Authors: Yamazaki Munehiro , Matsuoka Takafumi , Yasui Kozo , Komiyama Atsushi , Akabane Taro 
Published: February 2007

AbstractIn order to assess the functional property of neonatal polymorphonuclear leukocytes (PMNs), we studied their chemoattractant‐stimulated superoxide anion (O2−) production using a soluble chemotactic peptide N‐formyl‐methionyl‐leucyl‐phenylalanine (FMLP) as a PMN stimulus. O2− production by neonatal PMNs was increased: when stimulated with 10−7M FMLP, O2− values (nanomoles; mean ± SE) produced by 106 PMNs were 16.5 ± 1.3 for neonatal PMNs and 12.6 ± 0.6 for adult PMNs (p < .02). When studied under various concentrations (10−9 to 10−6M) of FMLP, neonatal PMNs produced more O2− than adult cells under the stimulation of a lower concentration (10−8M) as well as a higher concentration (10−7M) of FMLP. The increased O2− production by neonatal PMNs was also observed under their stimulation by phorbol myristate acetate. Neonatal and adult PMNs produced equal amounts of O2− when stimulated with a particulate stimulus of opsonized zymosan. To analyze further the mechanism for the increase in FMLP‐induced O2− production by neonatal PMNs, we next studied whether there was some abnormality in their FMLP receptors. Neonatal PMNs had normal numbers of FMLP receptors; the receptor numbers (mean) per cell were 54,000 in neonatal PMNs and 56,000 in adult cells. Additionally, the FMLP receptors had normal affinity for the peptide. These results demonstrate that the increased production of O2− by FMLP‐stimulated neonatal PMNs is not due to the abnormality of its binding to the receptors but is due to that of the subsequent events.
Abnormal platelet von willebrand factor interaction in patients with TTP
Authors: Rock G. , Tittley P. , Taylor J. R. 
Published: February 2007

AbstractThrombotic thrombocytopenic purpura (TTP) is associated with abnormal platelet function and disturbances in coagulation; however, a specific causative factor is not defined. Plasma infusion or plasma exchange (PE) are thought to be of benefit in replacing a deficient plasma component or removing some toxic compound. In three patients with TTP, samples taken prior to initiation of PE showed high levels of vWF:Ag in the plasma (208, 264, and 321 U/dl), whereas the VIII:C levels were normal. The vWF:Ag multimer patterns of the plasma demonstrated a decrease in the amount of high molecular weight (HMW) forms. Analysis of the platelets from one patient also showed an increase in the HMW multimers. Platelets from all three patients showed a decreased ability to absorb vWF:Ag, with little or no absorption of the HMW forms. Following extensive PE and resolution of disease, the platelets regained their ability to absorb vWF:Ag in the one patient examined.
Hemolytic uremic syndrome in a patient with small cell lung cancer
Authors: Avvento Louis , Gordon Sandra , Silberberg Jeffrey M. , Zarrabi M. Hosein , Zucker Stanley 
Published: February 2007

AbstractHemolytic uremic syndrome is a rare entity in patients with carcinoma and presents with a triad of renal insufficiency, microangiopathic hemolytic anemia, and thrombocytopenia. We report this syndrome for the first time in a patient with small cell lung carcinoma. Spontaneous platelet aggregation of washed normal platelets was demonstrated using patient plasma. Circulating immune complex levels were not elevated. The entity completely resolved after treatment with plasma, vincristine, aspirin, and dipyridamole.
Occurrence of spontaneous malignant lymphoma in the course of idiopathic myelofibrosis
Authors: Bogliolo Giuseppe V. , Leza Roberto A. , Ruggieri Corrado , Pannacciulli Ivo M. 
Published: February 2007

AbstractThe spontaneous development of malignant non‐Hodgkin lymphoma in a patient with idiophatic myelofibrosis is described. The tumor appeared 3 years after clinical diagnosis of the latter. Implications of the association between myeloproliferative and lymphoproliferative disorders at a pathogenetic level are briefly discussed.
Restriction fragment length polymorphism in the interzeta hypervariable region for prenatal diagnosis of non‐deletion α thalassemia
Authors: Chan Vivian , Chan T. K. , Wong A. C. K. , Chan T. P. T. , Ghosh A. , Todd D. 
Published: February 2007

AbstractA Bam HI restriction fragment length polymorphism in the interzeta hypervariable region (IZ HVR) of the ζ‐α gene cluster was used for the prenatal diagnosis of a pregnancy at risk for Hb H hydrops fetalis. The parents had ζ‐α thalassemia 1 and non‐deletion α thalassemia, respectively, and a previous hydrops was missed using the conventional method of gene detection. In this prenatal diagnosis, linkage to IZ HVR was used to exclude non‐deletion α thalassemia, and the numbers of ζ and α genes in the fetus were quantitated to predict the exact genotype. Confirmation was made by analysis of cord blood at delivery.
Altered platelet deformability in patients with type IIa and type IV hyperlipoproteinemia
Authors: Smith Clark M. , Burris Steven M. , Hunninghake Donald B. , White James G. 
Published: February 2007

AbstractPlatelets from subjects with hyperlipoproteinemia (HLP) differ from normal platelets in lipid composition and function depending upon the phenotypic classification of the HLP. The present study has evaluated the deformability of platelets from human subjects with type Ila and type IV HLP. Platelets suspended in autologous plasma diluted 30‐fold with buffer were aspirated into micropipettes 0.7‐0.8 microns in diameter by step‐wise increment in tension, and the resulting extension lengths were recorded. Platelets from type IIa subjects could not be aspirated as far into the micropipettes as normal platelets. However, less tension was required to reach maximum cell extension than with normal platelets, and the initial extension lengths and slopes of the stress responses were the same as the control. In contrast, platelets from subjects with type IV HLP showed a generalized increase in deformability. The initial cell extensions aspirated from type IV platelets were longer than normal, and larger maximum cell extensions were achieved at lower tensions than control platelets. The type IV platelets were also mechanically fragile and fragmented at lower tensions than control or type IIa platelets. The variance in platelet deformability between subjects of the same phenotype was not directly correlated to plasma lipid or lipoprotein concentrations. This study confirms alterations in the structural organization of platelets from subjects with type IIa and type IV HLP.
Asymptomatic association of hemoglobin Dunn (α6[A4]Asp→Asn) and hemoglobin O‐Arab (β121[GH4]Glu→Lys) in a Moroccan man
Authors: Baklouti Faouzi , Francina Alain , Dorléac Evelyne , Baudin‐Chich Véronique , Gombaud‐Saintonge Germaine , Plauchu Henri , Wajcman Henri , Delaunay Jean , Godet Jacqueline 
Published: February 2007

AbstractWe report on the association of Hb Dunn (α6[A4]Asp→Asn) and Hb O‐Arab (β121[GH4]Glu→Lys) in a healthy Moroccan man. Hb Dunn had the same electrophoretic properties as Hb G‐Philadelphia, but its percentage was lower. Its identification was based on sequence determination of the αT1 peptide. Bgl II and Eco RI mapping showed the presence of four α‐genes. Hb O‐Arab was easily recognized through its electrophoretic properties and was confirmed by the suppression of the Eco RI site located in exon 3 of the β‐gene. The percentages of the various hemoglobins showed that the doubly mutated hemoglobin Dunn/O‐Arab has a normal stability and suggested that the Dunn mutation is carried by the α1‐gene. In cord blood [propositus's son], the output of the αDunn gene was found equivalent to that existing in the adult.
Simultaneous study of karyotype and cell morphology in childhood erythroleukemia
Authors: Stamberg Judith , Zaslav Ann , Shende Ashok , Festa Robert 
Published: February 2007

AbstractWe followed a 2‐year‐old girl with erythroleukemia (EL) for 7 months, from the time of her initial diagnosis until her death. Immunophenotyping of bone marrow was negative for markers of myeloid and lymphoid lineages. Chromosome study of marrow at diagnosis revealed abnormalities in all mitotic cells, with a clonal karyotype of 48,XX,t(2;12)(p11.2; p13), + 6, + 21. Subsequent studies showed that the clone rapidly evolved and accumulated additional structural and numerical abnormalities, in spite of intensive chemotherapy during the final months.Simultaneous study of the karyotype and cell morphology of dividing bone marrow cells after 24 hours of in vitro culture, using a technique that preserves cell structure, showed that cells with the chromosome abnormalities were PAS‐negative. The combined immunological, cytogenetic, and morphological data indicate that the original malignant cell type was neither myeloblast nor erythroblast but an early progenitor type that rapidly proliferated.
Factor XI deficiency: Genetic and clinical studies of a single kindred
Authors: Litz Craig E. , Swaim William R. , Dalmasso Agustin P. 
Published: February 2007

AbstractA four‐generation 25‐member kindred with Factor XI:C deficiency is reported. Factor XI:C levels in heterorygotes varied from 15 to 58%, suggesting that Factor XI:C values for homozygote determination should be less than 15%. The frequency of bleeding was not correlated with Factor XI:C levels in this range. Individuals with joint pain had significantly lower Factor XI:C levels than members without joint pain and pain occurred more frequently in frequent bleeders. Lod scores showed no close genetic linkage of Factor XI:C deficiency with blood group MNSs (chromosome 4), complement components Bf and C4B (chromosome 6), or blood group P.
Erythrocytes in Hb SC disease are microcytic and hyperchromic
Authors: Ballas Samir K. , Kocher William 
Published: February 2007

AbstractWe have used the new Technicon H.1 Hematology Analyzer to determine the indices of erythrocytes obtained from 18 nonthalassemic (αα/αα genotype) adult patients with Hb SC disease. Controls were 14 normal black adults and 29 white adults. The data showed that SC erythrocytes are significantly smaller than normal RBC (P < .001) with a significantly higher MCHC value (P < .001) than controls. These features of SC RBC could not be reproduced by an Ortho ELT‐8 electonic counter. Hb SC erythrocytes have unique indices which are best demonstrated either by semimanual methods or by the H.1 system.
Acute promyelocytic leukemia presenting as sudden blindness and sinus vein thrombosis
Authors: Hazani Anna , Weidenfeld Yaakov , Tatarsky Llana , Bental Efraim 
Published: February 2007

AbstractAn 11‐year‐old boy was hospitalized with sudden blindness and sagittal sinus vein thrombosis, which were the presenting signs of acute promyelocytic leukemia (APL). The association of disseminated intravascular clotting (DIC) with APL is well‐known, and DIC usually affects the smaller blood vessels. The obliteration of a large blood vessel, as in our case, is an uncommon manifestation of DIC. This presentation of acute childhood leukemia is unusual and, to the best of our knowledge, has not been previously reported.
Regional and temporal variation in oscillatory blood flow in sickle cell disease
Authors: Kennedy Alan P. , Williams Barbara , Meydrech Edward F. , Steinberg Martin H. 
Published: February 2007

AbstractWe examined 55 patients with sickle cell anemia (HbSS) and 16 with hemoglobin SC disease (HbSC) to ascertain the presence of periodic microcirculatory flow (PMF) using laser‐Doppler velocimetry. Forty‐nine percent of patients with HbSS and 12.5% of HbSC patients had PMF on at least one occasion and in at least one site. The presence of PMF could be correlated with an increase in packed cell volume (PCV) and hemoglobin in individuals with HbSS and an increased white blood cell and platelet count in patients with HbSC. PMF may result from complex interactions among perfusion pressure, capillary tone, and the numbers and intrinsic properties of formed blood elements.
Simultaneous expression of lymphoid and myeloid phenotypes in acute leukemia arising from myelodysplastic syndrome
Authors: Komatsu Norio , Yoshida Minoru , Eguchi Mitsuoki , Akashi Makoto , Sasaki Ryuhei , Sakamoto Shinobu , Miura Yasusada 
Published: February 2007

AbstractA 57‐year‐old female developed myelodysplastic syndrome (MDS) that terminated as a biphenotypic leukemia after exposure to chemoradiotherapy. Double staining of blast cells, using monoclonal antibodies specific for myeloid and lymphoid lineage, demonstrated that one‐third of the leukemic cells simultaneously expressed the E rosette‐associated antigen (OKT11) and myeloid‐associated antigen (MY7). This finding suggests the possibility that some cases of MDS are clonal disorders that arise in a pluripotent stem cell that can also differentiate to T cell lineage.
Plasma cell leukemia with iga paraproteinemia and hyperviscosity
Authors: Kosmo Michael A. , Gale Robert Peter 
Published: February 2007

AbstractPrimary IgA plasma cell leukemia is a rare disorder in which hyperviscosity has not been reported. We studied a patient with plasma cell leukemia and IgA lambda monoclonal gammopathy with an elevated relative serum viscosity and congestive heart failure. Plasmapheresis and chemotherapy were successful in controlling symptoms. We discuss this unusual association and review the literature.
Genetic analysis of adenosine deaminase expression in adult T‐cell leukemia
Authors: Morisaki Takayuki , Horiuchi Naomi , Tsutsumi Hisashi , Ogura Hiromi , Kanno Hitoshi , Tani Kenzaburo , Fujii Hisaichi , Miwa Shiro 
Published: February 2007

AbstractWe investigated the genetic basis for the increased adenosine deaminase (ADA) expression in adult T‐cell leukemia (ATL). We found a correlation between the levels of ADA‐specific mRNA and ADA immunoreactive protein in ATL. Southern blot analysis revealed no gene amplification or rearrangement of the ADA gene. These findings indicate that increased ADA expression in ATL cells reflects increased transcriptional activity for the ADA gene or increased stability of ADA mRNA.
Use of differentiation inducing agents in the myelodysplastic syndrome and acute non‐lymphocytic leukemia
Authors: Hoffman Stephen J. , Robinson William A. 
Published: February 2007

AbstractThe use of chemical agents that induce differentiation of malignant cells to normal cells has held great promise as an adjunct to standard chemotherapy. In vitro data has shown that 13‐cis‐retinoic acid can differentiate certain leukemia cell lines (e.g., HL‐60) into stable granulocyte cells. In this study, oral 13‐cis‐retinoic acid was administered to four patients with the myelodysplastic syndrome (MDS) and to four patients with acute nonlymphocytic leukemia (ANLL). None of the MDS patients showed an hematologic response to the drug, while three of four ANLL patients responded with normalized peripheral blood counts. The side effects of the drug at 80‐120 mg/d (dry skin, cheilitis, epistaxis) were self limiting.