bluuebunny/crossref_metadata_2025_split huggingface dataset:
Fractionation of platelets according to size: Functional and biochemical characteristics
Authors: Carty D. J. , Gear A. R. L. 
Published: February 2007

AbstractThe functional and biochemical heterogeneity of platelets has been studied using graded differential centrifugation to fractionate human platelets according to size while maintaining their morphological and functional integrity as indicated by scanning electron microscopy and content of β‐thromboglobulin. Aggregation kinetics were studied by both optical and quenched‐flow methods involving single‐particle counting. Large platelets were significantly more sensitive to ADP, but aggregated less rapidly than small platelets. Thrombin exerted a similar influence. Large platelets were also enriched in surface sialic acid and sulfhydryl groups and in internal glycogen, ATP, ADP, calcium, cyclic AMP, malonaldehyde, and succinate cytochrome c reductase when compared to small platelets, even when normalized per unit volume. ADP caused a more rapid breakdown of cyclic AMP in small platelets.Potential aging relationships were tested by isotope studies in rats. 75Se‐selenomethionine was incorporated in vivo at a similar rate into all fractions. Large platelets labeled with 51Cr disappeared from circulation linearly and had a longer mean lifespan than small platelets, which disappeared exponentially. This behavior supports independent aging of platelet populations of differing size.The data suggest a distinct heterogeneity in platelet function and fate, which could derive from protection of large platelets against excessive activation by Ca2+‐regulated events.
Unbalanced 1;7 translocation and therapy induced hematologic disorders: A possible relationship
Authors: Morrison‐DeLap Sara J. , Kuffel Danlel G. , Dewald Gordon W. , Letendre Louis 
Published: February 2007

AbstractAn identical chromosome abnormality, which appears to be derived from a 1;7 translocation [ +der(1), t(1;7)(p11;p11)], was observed in the bone marrow of 12 patients with various hematologic disorders at the Mayo Clinic from 1980 to 1984. At the time of cytogenetic evaluation, nine of the patients had either a myeloproliferative disorder or a myelodysplastic syndrome, one had an acute leukemia, and two had treated multiple myeloma with no morphologic evidence of evolving myeloproliferative disease. Of the 11 patients for whom information about previous therapy was available, seven had a history of exposure to chemotherapy for a previous malignant disorder; we were unable to establish whether the remaining patient had had prior treatment. This study suggests a possible relationship between +der(1), t(1;7)(p11;p11) and some treatment‐induced hematologic disorders. Such chromosome abnormalities may be the result of a reciprocal chromatid translocation and adjacent I segregation of a quadriradial configuration in metaphase.
Acute myelocytic leukemia manifested by prominent generalized lymphadenopathy: Report of two cases with immunological, ultrastructural, and cytochemical studies
Authors: Davey Diane D. , Foucar Kathy , Burns C. Patrick , Goeken James A. 
Published: February 2007

AbstractAlthough it is well recognized that granulocytic sarcoma can cause localized lymphadenopathy, widespread nodal involvement by acute myelocytic leukemia (AML), clinically mimicking non‐Hodgkin's lymphoma, has only been previously described twice. We report the clinicopathological, immunological, and cytochemical features of two patients who had widespread, prominent lymphadenopathy secondary to AML as well as concurrent marrow leukemia (M1 and M2). For one patient the lymphadenopathy was the predominant abnormality prompting him to seek medical attention, while the second patient had symptoms of infection following a 9‐month history of myelodysplasia. The disease in both patients was aggressive; one patient survived only 1 week and the other survived only 5 weeks after diagnosis. In both cases the granulocytic sarcoma was confirmed by cytochemistry studies (naphthol ASD‐chloroacetate esterase on tissue sections and myeloperoxidase on imprint smears), and electron microscopy, including morphology (both cases) or ultrastructural localization of myeloperoxidase (case 2). Non‐specific esterase activity was not detected in either patient's blasts, although serum lysozyme was elevated in both cases. Immunological studies revealed reactivity of both patients' cells with panleukocyte, MY4, MY7, OKM‐1, and Leu‐M1 monoclonal antibodies and with alpha‐1‐antitrypsin and muramidase antibodies. The cells of one of these patients also reacted wtih anti‐S‐100 protein. Although the cytochemical studies indicated that both cases exhibited only myeloid differentiation, the immunological markers suggested that the tumor cells possessed some features of monocytes, perhaps explaining their propensity for widespread tumor formation. Morphological, immunological, cytochemical, and ultrastructural methods of diagnosing granulocytic sarcoma are presented.
The effect of intravascular neutrophil chemotactic factors on blood neutrophil and platelet kinetics
Authors: Issekutz Andrew C. , Ripley Murray 
Published: February 2007

AbstractIntravenous infusion of an analogue (f‐met‐leu‐phe [FMLP]) of a bacterial‐derived polymorphonuclear leukocyte (PMNL) chemotactic factor, or of the complementderived chemotactic stimulus, zymosan‐activated plasma (ZAP, containing C5ades Arg) into rabbits induces acute PMNL margination in the pulmonary vasculature. This process also occurs during hemodialysis and the adult respiratory distress syndrome. The pulmonary PMNL sequestration is accompanied by thrombocytopenia. Because of the role platelets and PMNLs play in hemostasis and defense against infection, we studied the fate of these blood elements following sequestration induced by chemotactic factors. By employing 111In‐labelled platelets and external radioisotope scanning, platelets were found to sequester in the pulmonary vasculature during FMLP infusion. Simultaneous 51Cr PMNL and 111 In‐platelet studies showed that following sequestration, PMNLs returned to the circulation and disappeared with a normal half‐life (T1/2) whereas the T1/2 of the platelets was markedly shortened (T1/2 of control = 49 ± 3.0 hr; FMLP or ZAP infused T1/2 = 27 ± 2.7 hr). Infusion of platelet‐activating factor (PAF) induced PMN and platelet sequestration with similar abnormalities in platelet kineties. Studies with 51Cr‐ and 14C‐serotonin‐labelled platelets showed that platelets did not release serotonin during FMLP, ZAP, or low dose PAF‐induced sequestration. In contrast to platelet survival, platelet size, platelet aggregation responses, and platelet glycoprotcins were not affected by transient sequestration. These results indicate that during PMNL margination induced by relatively „pure”︁ PMNL stimuli such as FMLP, platelets may reversibly marginate and subsequently be cleared at an accelerated rate. The reason for accelerated platelet clearance is not a result of circulating platelet aggregates or detectable proteolytic modification of membrane glycoproteins. Such altered platelet kinetics may contribute to thrombocytopenia during sepsis, the adult respiratory distress syndrome, and other states in which excess PMNL margination occurs.
T‐lymphocyte subpopulation changes during hemolysis in glucose‐6‐phosphate dehydrogenase (G6PD)‐deficient children
Authors: Schilirò G. , Minniti C. , Sciotto A. , Bellino A. , Russo A. 
Published: February 2007

AbstractLymphocyte subpopulations were studied by means of sheep red blood cell (SRC)‐rosetting technique and OKT11, OKT3, OKT4, and OKT8 monoclonal antibodies in ten Sicilian glucose‐6‐phosphate dehydrogenase‐deficient children during the hemolytic crisis due to fava bean ingestion. The number of SRC‐rosetting lymphocytes was significantly reduced, while the number of OKT3‐positive cells was normal. An inversion of the OKT4/OKT8 ratio was observed, due to a decrease of OKT4‐positive cells and an increase of OKT8‐positive cells. All these abnormalities reverted to normal in the four children studied after recovery.
Porcine factor VIII and plasmapheresis in the management of hemophiliac patients with inhibitors
Authors: Bona Robert D. , Pasquale Dominick N. , Kalish Robert I. , Witter Barbara A. 
Published: February 2007

AbstractManagement of hemophiliac patients with inhibitors can be a difficult problem. We report here a patient with severe hemophilia A, a high titer inhibitor, and an abdominal abscess requiring surgery. Despite significant cross reactivity of the inhibitor with porcine FVIII, he was successfully managed with plasmapheresis and porcine FVIII infusions. Other approaches to the management of such patients are briefly reviewed.
Fibers, crystals, and other forms of HbS polymers in deoxygenated sickle erythrocytes
Authors: Kaperonis Athanasia A. , Handley Dean A. , Chien Shu 
Published: February 2007

AbstractWe have examined by electron microscopy the formation of fibers and crystals from sickle hemoglobin within sickle erythrocytes following deoxygenation during capillary storage from 1 to 132 days. Intracellular fibers were found on the first day and throughout the period of study. The fibers exhibited a diameter (mean ± SD) of 17.4 ± 0.62 nm and were aligned in the cell with a fiber‐to‐fiber spacing of 18.6 nm (x‐axis) by 22.7 nm (y‐ axis). Between 65 and 132 days, extracellular hemoglobin crystals developed, with a lattice periodicity of 9.63 ± 0.6 nm. Fibers and crystals coexist as separate structures. These results suggest that crystal formation upon storage of packed deoxygenated sickle erythrocytes may proceed via a phase of fiber dissolution followed by hemoglobin reassembly into extracellular crystals, rather than by a progressive alignment and direct fusion of existing fibers.
The effect of Hb F and α‐thalassemia on the red cell indices in sickle cell anemia
Authors: Milner Paul F. , Garbutt George J. , Nolan‐Davis L. V. , Jonah Felix , Wilson Lois B. , Wilson John T. 
Published: February 2007

AbstractThis study examines the effect of different levels of fetal hemoglobin (Hb F) and the presence or absence of genes for α‐thalassemia on the red cell indices and degree of anemia among 102 patients with homozygous sickle cell disease (S/S) between the ages of 15 and 62 years. Patients were divided into those with an average Hb F of < 10 gm/L („low”︁ Hb F group) and those with > 10 gm/L („high”︁ Hb F group). α‐Thalassemia was assessed by restriction enzyme analysis of DNA by the Southern blotting technique. Homozygosity for the βs gene was confirmed by restriction enzyme analysis of DNA using the enzyme Mst II.There were 51 patients with four α‐globin genes, 28 of whom had „high”︁ and 23 „low”︁ Hb F levels. Fifty‐one patients had α‐thalassemia, 38 of whom were heterozygous and 13 homozygous for the 3. 7 kb α‐thalassemia deletion. Nine had „high”︁ and 31 had „low”︁ Hb F. Irrespective of α‐globin genotype, patients in the high Hb F group had a higher mean Hb, Hct, MCV, and MCH than those in the low HB F group. In patients without α‐thalassemia Hb F was positively correlated with MCV and MCH (p < 0.001), patients with high Hb F levels having macrocytosis confirmed by microhematocrit studies. Patients with α‐thalassemia had a lower MCHC than patients with four α‐globin genes and this was not significantly affected by the level of Hb F. The combination of α‐thalassemia and high levels of Hb F appears to result in a distinctive S/S phenotype that is similar to the type of S/S disease described in Southern India.
Congenital thrombotic disorders
Authors: Rodgers George M. , Shuman Marc A. 
Published: February 2007

AbstractThe investigation of kindreds with recurrent thrombotic disease has advanced the understanding of the mechanisms of coagulation and fibrinolysis. In those cases where an etiology has been established, congenital thrombotic disorders are associated either with deficiencies or qualitative abnormalities in inhibitors of activated coagulation factors, qualitative abnormalities of fibrinogen, fibrinolytic defects that impair clot lysis, or an inborn error of metabolism, homocystinuria. The etiologies of congenital thrombotic disorders, their clinical features, and an approach to their laboratory diagnosis are summarized in this review.
In vivo study of lactoferrin and murine rebound myelopoiesis
Authors: Poppas Athena , Faith Michael R. , Bierman Howard R. 
Published: February 2007

AbstractLactoferrin has been reported to inhibit the production of colony‐stimulating factor (CSF); thus we sought to study its possible effects on myelopoiesis in vivo.The characteristics of rebound myelopoiesis in C57BL mice injected with a sublethal dose of cyclophosphamide (CY) were used to test lactoferrin for any granulopoietic activity. An experimental group received daily injections of 50 μg of human lactoferrin beginning 24 hr after CY injections. By measuring the total nucleated cellularity of the femoral marrow, the peripheral blood count, and the incorporation of tritiated thymidine by the marrow in six replicate experiments, no statistically significant difference was noted between the lactoferrin injected groups and the control groups. Neither the route of lactoferrin administration (i.v. or i.p.) nor the sex of the animal altered the myelopoietic recovery.Lactoferrin had no stimulatory or inhibitory effect on murine rebound myelopoiesis in vivo contrary to the reported in vitro results.
Cyclic neutropenia as a premalignant manifestation of acute lymphoblastic leukemia
Authors: Lensink Daniel B. , Barton Ann , Appelbaum Frederick R. , Hammond William P. 
Published: February 2007

AbstractA 20‐year‐old female from the Philippines developed anemia and granulocytopenia. With androgen therapy, her anemia improved but she continued to show a pattern of fluctuating neutropenia consistent with human cyclic neutropenia: Blood neutrophil oscillation was regular with a periodicity of 21 days. She developed recurrent pharyngitis and apthous stomatitis but there was no cycling of other blood elements. Bone marrow aspiration and biopsy showed normal developing myeloid cells, a clonal chromosomal abnormality, and myelofibrosis. During the fourth documented cycle, blasts appeared and complete lymphoblastic transformation ensued. Blast cells were CALLA positive, Ia positive, and contained intranuclear TdT; they were negative for E, EAC, and EA rosettes. She was treated for non‐T, non‐B CALLA‐positive ALL and within 6 weeks was in a remission without evidence of cycling neutrophil counts. This young woman's case suggests that cyclic neutropenia may represent a previously unrecognized premalignant state associated with acute lymphoblastic leukemia.
Association of HLA and autoantibody in transfused sickle cell disease patients
Authors: Ofosu Mildred D. , Saunders David A. , Dunston Georgia M. , Castro Oswaldo , Alarif Lamya 
Published: February 2007

AbstractThis study evaluates autoantibody production in sickle cell disease patients and determines whether genes in the major histocompatibility complex are associated with autoantibody responses. Rheumatoid factor was significantly increased for both male and female patients and was less prevalent in highly transfused patients. Significant increases were also detected in the incidences of antinuclear antibody for females and antismooth muscle antibody for males. Low incidence of antinuclear antibody was significantly associated with HLA‐DR3. Significant associations were also found between the incidence of antinuclear antibody and both HLA‐A28 and B15.
Influence of HbS levels upon the hematological and clinical characteristics of sickle cell trait
Authors: Kennedy Alan P. , Walsh David A. , Nicholson Rosie , Adams Junius G. , Steinberg Martin H. 
Published: February 2007

AbstractThe variable concentration of HbS in individuals with sickle cell trait led us to study the relationship between HbS level and selected vascular events in 355 hospitalized black men with sickle cell trait. There were significant differences in hemoglobin concentration and mean corpuscular volume found in four groups divided by their HbS level, the lowest proportion of HbS (<30%) being associated with the lowest hemoglobin concentration (12.6 g/dl) and MCV (77 fl). The percent HbS did not influence the incidence of pulmonary embolism, thrombophlebitis, myocardial infarction, cerebrovascular accident, or idiopathic hematuria.Our results suggest that HbS level does not influence vascular disease, and while certain hematological alterations occur, they are very unlikely to have any clinical significance. Regardless of the proportion of HbS, sickle cell trait in black men is benign.
Trisomy 1q in polycythemia vera and its relation to disease transition
Authors: Swolin B. , Weinfeld A. , Westin J. 
Published: February 2007

AbstractClinical and cytogenetic details of 12 patients with polycythemia vera and complete or partial trisomy of the long arm of chromosome 1 are reported. All patients had trisomy for at least the segments 1q22 to 1qter. The 1q or material from 1q was translocated to another chromosome in eight patients. This was chromosome 9 in four patients, and those cases all had trisomy also for 9p. The trisomy 1q was found at the time of diagnosis in three patients, later during the polycythemic phase in five, and in four patients when they were first examined during a late stage of the disease. Acute leukemia or a myelodysplastic syndrome developed in eight of the 12 patients. Signs of advanced disease, eg, myeloid metaplasia or myelofibrosis, preceded the leukemia in four cases and was noted in one more patient.Trisomy 1q was the most frequent structural chromosome abnormality in patients with polycythemia vera. It is thus one of several nonrandom abnormalities that can appear at any stage of the disease. It seems to occur with higher frequency in patients with myelofibrosis and/or leukemia, but it is not a specific characteristic of these complications.
Primary amyloidosis (AL) in families
Authors: Gertz Morie A. , Garton John P. , Kyle Robert A. 
Published: February 2007

AbstractWe report the occurrence of immunoglobulin‐related amyloidosis (AL) in three separate families, each family having two members affected. None of the six patients had evidence to suggest the presence of familial amyloidosis (AF). Previously, immunoglobulin‐related amyloidosis (AL) was considered to be a sporadic disease process. Because of the occasional familial occurrence of other monoclonal gammopathies such as monoclonal gammopathy of undetermined significance, multiple myeloma, and macroglobulinemia of Waldenström, amyloidosis (AL) should be added to the list of immunopathies with a familial predisposition.
Philadelphia‐positive metaphases in the marrow after bone marrow transplantation for chronic granulocytic leukemia
Authors: Apperley Jane F. , Rassool Feyruz , Parreira Antonio , Geary Colin G. , Harrison Christine , Stansfield Derek , Goldman John M. 
Published: February 2007

AbstractA 28‐year‐old man with Ph‐positive chronic granulocytic leukemia (CGL) was treated by high‐dose chemoradiotherapy and transplantation of marrow cells harvested from his HLA‐identical brother. One year after bone marrow transplantation (BMT) examination of his marrow showed a minority population of Ph‐positive cells; their proportion subsequently fell such that 2 years after transplant analysis of marrow cells showed only cytogenetically normal cells. The patient remains clinically normal with a persisting mild lymphocytosis but without hematological evidence of leukemia. We cannot in this patient distinguish between persisting leukemia that later could no longer be recognized and relapse of leukemia that is now suppressed, perhaps only temporarily. This case emphasizes the need for caution in interpreting chromosomal finding after BMT for CGL.
Leukapheresis for control of chronic myelogenous leukemia during pregnancy
Authors: Fitzgerald Denis , Rowe Jacob M. , Heal Joanna 
Published: February 2007

AbstractLeukapheresis was used as the sole modality of treatment of a patient with chronic myelogenous leukemia (CML) during her pregnancy. The specific requirements for safe leukapheresis during pregnancy are discussed, and the literature describing the management of CML during pregnancy is reviewed. Leukapheresis may be the treatment of choice in selected pregnancy patients with CML since it avoids the potential teratogenic effects of chemotherapy or radiation therapy.
Frequency of α‐thalassemia in Greece
Authors: Kanavakis E. , Tzotzos S. , Liapaki A. , Metaxotou‐Mavromati A. , Kattamis C. 
Published: February 2007

AbstractUsing hematological and gene mapping techniques, a cord blood survey was carried out to estimate the frequency of α‐thalassemia in the Greek population. Out of 227 newborns studied, 16 (7.05%) were found by gene mapping to be α‐thalassemia 2 heterozygotes (‐α/αα), and of these only two had increased levels of hemoglobin Bart's in the cord blood (1.2 and 2.0%). Similarly, one heterozygote for the common Mediterranean α‐thalassemia 1 haplotype (–/αα) and one for the 20.5‐kb deletion type (‐α)20.5/αα) were found, showing increased levels of Bart's of 4.8 and 6.6%, respectively. Four (1.76%) heterozygotes for the triple α gene arrangement (ααα/αα) were found. One individual with a level of Bart's in the cord blood of 8% was found to be a double heterozygote for α‐thalassemia 2 and a dysfunctional α gene arrangement (‐α/‐(α)?). These results give an overall incidence for α‐thalassemia in the Greek population of 8.4%.
Antenatal diagnosis of β‐thalassemia by isoelectric focusing in immobilized pH gradients
Authors: Manca Mario , Cossu Gianfranco , Angioni Giuliano , Gigliotti Bruno , Bosisio Adriana Bianchi , Gianazza Elisabetta , Righetti Pier Giorgio 
Published: February 2007

AbstractA new method for antenatal diagnosis of thalassemias is reported based on the analysis of the major Hb components of fetal cord blood, sampled at week 18 of pregnancy under ultrasonic guidance, by isoelectric focusing in immobilized pH gradients (IPG). In an IPG gel encompassing a pH 6.7–7.6 span, HbA and HbFac are separated by a distance nine times greater than in a conventional carrier ampholyte pH 6–8 gel and three times greater than in an ampholine gel with separators (an equimolar mixture of β‐alanine and 6‐amino caproic acid). Band evenness (in terms of uniform protein concentration within a zone) and straightness (in terms of parallel alignment of the bands to the electrodes), because of insensitivity of IPG gels to salt distortions, allows for accurate and reproducible quantitation of HbF, ‐A, and ‐Fac levels. The possibility of greatly overloading IPG matrices in total Hbs increases the sensitivity of the technique to the detection of only 0.5% HbA in the total Hb mixture, the lower limit of conventional IEF being only 2.5% HbA. Of 15 fetuses from couples at risk analyzed in the region of Ozieri, three were found to be homozygous β‐thalassemic, eight heterozygous, and four normal with no false‐positives or ‐negatives.
Busulphan versus combination chemotherapy for initial treatment of chronic granulocytic leukaemia
Authors: Jacobs Peter 
Published: February 2007

AbstractIn a pilot study, 21 consecutive and previously untreated adults with chronic granulocytic leukaemia received either busulphan (group 1: n = 11) or a combination of the epipodophyllotoxin VP16‐213 with cytosine arabinoside and doxorubicin (group 2: n = 10). Using a standardised protocol, objective response, defined as a granulocyte count consistently less than 10 × 109/1 with no clinical evidence of the disease, was achieved in 45 and 40% of the patients, respectively, and median survivals were 89 and 107 weeks (p > 0.05). Although there was no difference in morbidity, the study was discontinued because the combination chemotherapy was more complex to administer, was associated with greater patient inconvenience, and offered no advantage over single‐agent therapy in managing the stable phase of chronic granulocytic leukaemia.