bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hematological evaluation of patients with various combinations of α‐thalassemia
Authors: Altay C. , Gurgey A. , Tuncbilek E. 
Published: February 2007

AbstractSix patients and their parents from five different families with Hb H have been evaluated clinically and hematologically. Previous studies using restriction endo‐nuclease mapping technique indicated that α‐thalassemia determinants in these cases are heterogeneous. Only one of the five cases have the usual genotype for Hb H, which is characterized by an α‐DNA‐specific fragment of 20 kb long by Eco RI digestion.Three cases from two different families have Hb H disease with α‐specific DNA fragments of 22.5 kb/2.6 kb long; and the other two have α‐specific DNA fragments of 20 kb/2.6 kb long, in Eco RI digestion of the cellular DNA. The hematological examination of the parents suggests that the α‐thalassemia condition associated with the Eco RI fragment of α‐specific cellular DNA of approximately 22.5 kb long produces an α‐thal‐2‐like clinical condition, while the other α‐thalassemia determinant associated with a fragment 2.6 kb long results in an α‐thal‐1‐like clinical condition. The clinical and hematological findings of the cases with 22.5 kb/2.6 kb fragment patterns were more severe than the case with the 20 kb/2.6 kb combination. This study suggests that variation in the clinical and hematological findings among patients with Hb H disease may well reflect a heterogeneity of the genotypic combination.
Priapism complicating chronic granulocytic leukemia
Authors: Suri R. , Goldman J. M. , Catovsky D. , Johnson S. A. , Wiltshaw Eve , Galton D. A. G. 
Published: February 2007

AbstractSince 1952 we have seen nine patients with priapism leading to a diagnosis of chronic granulocytic leukemia (CGL) and a tenth patient who gave a history of priapism when CGL was diagnosed as a result of other symptoms. Seven patients had had one or more transient episodes of prolonged erection before the diagnosis of CGL was established. All ten had high blood leukocyte counts (mean 380 × 109/liter, range 186‐782) in comparison with other newly diagnosed patients. We estimate the incidence of this complication at 1%–2% of all male patients presenting with CGL. Treatment of patients in this series varied greatly. Five patients were treated mainly by local measures with or without cytotoxic drugs at conventional dosage, three were treated by sapheno‐cavernous bypass operations and leukapheresis followed by cytotoxic drugs at high dosage, and two were treated initially by leukapheresis alone. In general, the prompt initiation of measures designed to reduce the leukocyte count seemed more valuable than the surgical procedures employed in these patients.
Characterization of lymphoid cells in the blood of healthy adults: Sequential immunological, cytochemical and cytokinetic studies
Authors: Hirt A. , Wagner H. P. 
Published: February 2007

AbstractWith a new method, sequential immunological, cytochemical and cytokinetic studies were done on lymphoid cells in the peripheral blood of 12 healthy adults. Every single lymphoid cell could therefore be characterized by the following markers: surface immunoglobulins (sIg); rosetting with sheep red blood cells (E); unspecific acid alpha‐naphthyl acetate esterase (ANAE); and 3HdT incorporation. Significantly more E+sIg− cells were ANAE+ than E−sIg+ cells (70% vs. 11%). About half of the E+sIg− cells were ANAE+. By combining esterase and surface marker analyses eight subpopulations of lymphoid cells could be distinguished. The most common subpopulations were E+sIg− ANAE+ and E+sIg−ANAE− cells (51% and 22% of all lymphoid cells, respectively). Of all ANAE+ cells 90% were E+, but 64% of all ANAE− cells were also E+. In all individuals a subpopulation of E+sIg+ cells was found. The esterase pattern of these cells was similar to that of E−sIg+ cells. The overall labeling index of the lymphoid cells examined was ≦ 0.2%.
Prolymphocytic leukemia: Flow microfluorometric, immunologic, and cytogenetic observations
Authors: Diamond Lawrence W. , Bearman Robert M. , Berry Paula K. , Mills Bonnie J. , Nathwani Bharat N. , Weisenburger Dennis D. , Winberg Carl D. , Teplitz Raymond L. , Rappaport Henry 
Published: February 2007

AbstractCells isolated from four patients with prolymphocytic leukemia were evaluated by surface markers, cytogenetics, and flow microfluorometric analysis of cell size and DNA content. All four patients had B‐cell markers with a high density of IgM, kappa type, and Ia‐like antigen. Less intense staining for surface IgD was also observed. In each patient studied, chromosomal modes were in the hypodiploid or near‐diploid range. Despite the karyotypic abnormalities, the cellular DNA content, as determined by flow microfluorometry, was within normal limits in all cases. This suggests that the variability in chromosome numbers seen in these patients may reflect an abnormality in DNA packaging rather than differences in total DNA content. The modal electronic cell size of the prolymphocytes, determined by light scatter, was readily distinguishable from that of normal peripheral blood lymphocytes and the lymphocytes of chronic lymphocytic leukemia. Fewer than 4% of the peripheral prolymphocytes had S‐phase DNA content, a finding consistent with the chronic nature of this leukemia.
Terminal‐deoxynucleotidyl‐transferase immunofluorescence on bone marrow smears: Serial studies on 28 patients
Authors: Miller William M. , Stass Sanford A. , Schumacher Harold R. , Bollum Frederick J. 
Published: February 2007

AbstractTerminal‐deoxynucleotidyl‐transferase (TdT) activity determinations, using the indirect immunofluorescence technique, were performed on 28 consecutive leukemia patients. Fifteen patients with acute lymphoblastic leukemia had 38 bone marrow smears examined. TdT‐positive cells were a good indicator of disease activity in acute lymphoblastic leukemia in that numbers were increased at the time of diagnosis, normal during remission, and increased during relapse. Ten patients with acute myeloid leukemias had 42 smears studied. The number of TdT‐positive cells did not reflect the activity of the disease, except for two patients with acute myelomonocytic leukemia in which TdT‐positive cells were mildly increased during relapse. Three patients with chronic myelogenous leukemia had eight smears studied. The number of positive cells seemed meaningful only in following the course of lymphoid blast crisis. It would therefore appear that TdT determinations seem to be of value in lymphoblastic leukemia, but of limited value in myeloid leukemias.
Hereditary pyruvate kinase abnormalities associated with erythrocytosis
Authors: Rosa Raymonde , Max‐Audit Isabelle , Izrael Victor , Beuzard Yves , Thillet Joélle , Rosa Jean 
Published: February 2007

AbstractA familial erythrocytosis is described. The propositus is a 52‐year‐old man who was found, on routine testing, to have an erythrocytosis with a Hb level of 19.8 g/dl. Physical examination and laboratory findings revealed an associated hemolysis. The P50 of the stripped hemolysate was normal. The 2,3‐DPG level was reduced to 15% of normal but that of ATP was increased more than twofold. Red cell diphosphoglycerate mutase activity was normal as were the levels of all red cell glycolytic and hexose monophosphate shunt enzymes with the exception of pyruvate kinase (PK). The level of the latter was elevated to 24.5 U/g(normal:6.3 ± 1.1). The electrophoretic pattern of hemolysate PK was abnormal, showing three additional bands; one of these migrated as the M2 isozyme. The findings were similar in the propositus's daughter and her daughter and in one of the two brothers of the propositus. The second brother and the son of the first brother also exhibited erythrocytosis but their red cell 2,3‐DPG levels were normal. In addition, the level of their RBC PK was reduced to between 50% and 60% of normal and the abnormal electrophoretic bands were absent. The erythrocytosis appears to be inherited as an autosomal‐dominant trait. The relationship between the PK abnormalities appears complex.
Platelet dysfunction in myeloproliferative syndromes
Authors: Phadke Kiran , Dean Shirley , Pitney W. R. 
Published: February 2007

AbstractThe most common abnormality of the platelets in 43 patients with a variety of myeloproliferative syndromes was impaired or absent aggregation when stimulated with collagen and adrenaline. Eight unselected cases studied in more detail showed a normal prostaglandin synthesis pathway as evidenced by normal aggregation with arachidonic acid and the production of normal amounts of malonyldialdehyde. Mixing experiments with aspirin‐tested platelets showed correction of the abnormal adrenaline and collagen responses and confirmed that the nature of the defect was different from that induced by aspirin. Stimulation of “myeloproliferative” platelets with thrombin after blocking the prostaglandin pathway with aspirin resulted in reduced aggregation, indicating either a deficiency of the storage pool of adenine nucleotides in the platelets or an abnormality of a membrane receptor for thrombin.
A microimmunofluorescent assay to detect human granulocyte antigens and antibodies
Authors: Zaroulis Charles G. , Jaramillo Sylvia 
Published: February 2007

AbstractGranulocyte antibodies are important in the pathogenesis of neonatal granulocytopenia, in some varieties of idiopathic granulocytopenia, and in transfusion reactions, particularly since the development of leukocyte transfusion therapy for granulocytopenic patients. Previous assays for the detection of granulocytespecific antigens and antibodies were cumbersome, complex, and unreliable. Thus we have now described a new microimmunofluorescent method to assay human granulocyte antigens and their antibodies. This sensitive and reliable technique allows for the preservation of human granulocyte antigenicity for at least 24 hours. The granulocyte‐specific antigens of a Caucasian population, residing in metropolitan New York, were studied by this microimmunofluorescent method. We report the distribution of granulocyte‐specific antigens in this population, together with the corresponding gene frequency. This microimmunofluorescent test should improve our understanding of antigenic systems restricted to single cell populations.
Pure erythrocytosis: Reappraisal of a study of 51 cases
Authors: Najean Y. , Triebel F. , Dresch C. 
Published: February 2007

AbstractFifty‐one cases of pure, primary erythrocytosis were identified and followed at Hǒpital Saint‐Louis, Paris, and compared with 350 cases of polycythemia vera (PV) observed during the same period. At the initial evaluation, these cases did not differ from PV cases with respect to age, sex ratio, degree of red cell volume increase, and clinical symptoms. They did differ by the absence of splenomegaly, granulocytosis and thrombocytosis. At a late stage of evolution only a few cases developed classical criteria of PV. From this group of apparently homogeneous cases, two subgroups evolved. Sixty percent of the cases were highly responsive to myelosuppression with 32P. The median duration of the first remission was greater than five years, the mean yearly dose of 32P was very low, and there was a low incidence of complications. The other group (40% of cases) was relatively resistant to myelosuppressive agents. The development of better methods to investigate this disorder might help in discriminating these two groups from both an etiological and pathophysiological viewpoint. The thromboembolic risk of these diseases suggests that myelosuppressive therapy should be utilized in older patients with higher risk of vascular accidents, reserving phlebotomy for younger patients and those who are shown to be resistant to 32P therapy.
Increased T‐cell reactivity to leukemic B cells in chronic lymphocytic leukemia with change of stable disease to its progressive form
Authors: Fernandez Louis A. , MacSween J. Michael , Langley G. Ross 
Published: February 2007

AbstractSome patients with chronic lymphocytic leukemia (CLL) have a relatively stable clinical course without treatment, but many of these eventually develop progressive disease. We have followed 68 patients over 5½ years by employing conventional surface markers and lymphocyte reactivity, including that of separate enriched T cells to their own leukemic B cells, to phytohemagglutinin (PHA), and to normal allogeneic B cells; we have observed an unusual response in each of the seven patients who developed progressive disease over this period of time. During the stable phase of the disease the T cells from 27 leukemic subjects did not respond to their own leukemic B cells in culture. In the seven patients who developed progressive disease, a significant reactivity of their enriched T cells to their own leukemic B cells occurred. There was no consistent change in T‐cell reactivity to PHA or to allogeneic B cells, suggesting a change in the leukemic B‐cell populations that was not detected morphologically. The change in reactivity of T cells to leukemic B cells occurred prior to evidence of clinical or laboratory deterioration in one of the seven cases; there was increasing lymphocytosis and lymphadenopathy in six and two instances, respectively; and anemia and thrombocytopenia in the three and one cases, respectively.
Monocyte α‐naphtyl esterase deficiency in chronic granulomatous disease
Authors: Gougerot‐Pocidalo Marie‐Anne , Buriot Diego , Griscelli Claude , Hakim Jacques 
Published: February 2007

AbstractMonocytes from five unrelated children (four boys and a girl) with chronic granulomatous disease were studied for their ability to reduce nitroblue tetrazolium dye after stimulation with zymosan, and for their α‐naphtyl butyrate esterase activity. As expected, monocytes ingested zymosan particles but failed to reduce nitroblue tetrazolium dye. However, monocytes from two boys out of the five patients were α‐naphtyl butyrate esterase‐negative, whereas both their neutrophils and monocytes were positive for granular naphtol AS‐D esterase activity.
The modification of human erythrocyte membrane structure by membrane stabilizers: An electron spin resonance study
Authors: Janoff Andrew S. , Mazorow D. L. , Coughlin R. T. , Bowdler A. J. , Haug A. , McGroarty E. J. 
Published: February 2007

AbstractMembrane structure in intact human erythrocytes was analyzed by electron‐spin‐resonance (ESR) spectroscopy. The spin probes 5‐doxyl stearate and 5‐doxyl stearate methyl ester revealed thermally‐induced structural transitions in the membrane at 37°C and 15°C. The addition of propranolol, diazepam, chlorpromazine, or Pluronic F68 all caused a decrease in the temperature of the upper transition, but did not markedly alter the temperature of the lower transition. In addition, diazepam caused a significant decrease in the ordering or packing of the membrane‐lipid acyl chains. It is proposed here that the protection from hypotonic hemolysis that has been reported in the presence of these drugs is mediated by a structural rearrangement in the erythrocyte membrane involving a change in protein‐lipid interactions.
Chronic myelomonocytic leukemia in childhood
Authors: Thomas William J. , North Robert B. , Poplack David G. , Slease Robert B. , Duval‐Arnould Bertrand 
Published: February 2007

AbstractA four‐year‐old child with recurrent infections and increasing hepatosplenomegaly over a three‐year period was evaluated. Increased numbers of myeloid precursors packed the bone marrow and infiltrated the peripheral blood. A diagnosis of chronic myelogenous leukemia (CML) was considered but could not be confirmed by laboratory studies appropriate for the types of CML usually observed in childhood. Examination of the patient's peripheral blood smears revealed many atypical monocytoid cells with unipolar hairy projections. Scanning electron microscopy showed these to be leukemic monoblasts with characteristic broad‐based ruffles on the cell surface. A population of myeloid precursors possessing narrow ridge‐like profiles was also observed. Progressive infiltration of the spleen caused hypersplenism which necessitated splenectomy. Subsequently, massive liver and bone marrow involvement led to the patient's death. Terminally, the proliferating blast cells were demonstrated to be leukemic monoblasts by analysis of cytochemical staining patterns, surface immunoglobulins, serum lysozyme levels, and monocyte‐mediated antibody‐dependent cellular cytotoxicity studies. The findings in this case are most compatible with a diagnosis of chronic myelomonocytic leukemia (CMML), a condition not previously described in childhood. Several myeloproliferative disorders with prolonged survival have been reported in children, but special studies were not performed to determine which cell lines were abnormally proliferating. The similarities between these children and our patient with CMML suggest that monocyte studies may be useful in the diagnosis of these unusual disorders, provide insights into their pathogenesis, and aid in the selection of appropriate therapy.
Factors affecting iron balance
Authors: Conrad Marcel E. , Barton James C. 
Published: February 2007

AbstractThe availability of radioisotopes of iron led to the performance of physiologic studies of the absorption, excretion, and kinetics of iron. The effects of dietary constituents and intestinal secretions upon iron absorption have been thoroughly explored and have permitted the development of tools for nutritional studies in various geographic areas of the world. The basic mechanisms for regulation of iron absorption are not fully understood. However, the isolation and characterization of newer iron‐binding proteins, the existence of mucosal receptors in which there is competition for iron‐binding sites with other metals, and the identification of a number of animal models with genetic abnormalities of iron absorption provide promise for development in the foreseeable future. The means by which the body informs the intestine to increase or decrease iron absorption is poorly understood, and the abnormality in hemochromatosis and iron overloading disorders remains unexplained. Much has been learned in recent years regarding the structure of iron‐containing proteins and the variations that exist in different species. Similar problems remain to be solved with regard to the intermediary metabolism of iron in red blood cell precursors and other cells in the body. It is postulated that the combined usage of physiologic, biochemical, and immunologic investigations will provide the basic information required.
Thirteen cases of pyruvate kinase deficiency found in Japan
Authors: Ishida Yoji , Miwa Shiro , Fujii Hisaichi , Fujinami Noboru , Takegawa Susumu , Yamato Kunio 
Published: February 2007

AbstractThirteen cases of pyruvate kinase (PK) deficiency, considered to be heterozygous for different PK mutants because of no consanguinities in their parents, were characterized by the International Committee for Standardization in Haematology (ICSH) recommended methods. These deficiency cases are named PK “Kagoshima,” PK “Kyoto,” PK “Takamatsu,” PK “Abeno,” PK “Kobe,” PK “Marugame,” PK “Hoenzaka,” PK “Osaka,” PK “Motomachi,” PK “Gifu,” PK “Hiroshima” PK “Matsumoto,” and PK “Tama.”The characteristics of mutant PK enzymes suggest that the cause of chronic hemolysis depends mainly on decreased affinity for phosphoenolpyruvate, thermolability, increased inhibition by adenosine triphosphate, and low activation by fructose‐1, 6‐diphosphate.
Antigranulocyte antibodies and deranged immune function associated with phenytoin‐induced serum sickness
Authors: Menitove Jay E. , Rassiga Anne L. , McLaren Gordon D. , Daniel Thomas M. , Mahmoud Adel A. F. 
Published: February 2007

AbstractA phenytoin‐associated serum‐sickness‐like syndrome was followed by severe pancytopenia in a 17‐year‐old female. Serum obtained during the acute phase of her illness contained antibodies against homologous eosinophils and neutrophils, antibodies against autologous neutrophils, and antibodies to phenytoin. The serum also inhibited bone marrow granulocyte colony formation in vitro. Lymphocyte transformation by pokeweed mitogen was impaired and, after recovery, the patient's lymphocytes were transformed in vitro by the addition of phenytoin. These data demonstrate that profound derangements of immunity can be associated with a phenytoin‐induced serum‐sickness‐like illness.
Freeze preservation of sickle erythrocytes
Authors: Castro Oswaldo , Hardy Kathryn Phillips , Winter William P. , Hornblower Marne , Meryman Harold T. 
Published: February 2007

AbstractThe effect of short‐term cryopreservation on metabolic, functional, and survival characteristics of erythrocytes from patients with sickle cell disease was examined. Post‐thaw hemolysis of glycerolized sickle (SS) erythrocytes was greater (mean 12.9%) than in hemoglobin‐AA cells (mean 4.7%). Freeze preservation had no apparent effect on red cell morphology, percent irreversibly sickled cells, and fetal hemoglobin content. There were modest reductions in ATP and 2,3‐diphosphoglycerate in thawed, washed sickle erythrocytes (12.7% and 29.7%, respectively). However, the autologous survival of 51 Cr‐labelled SS red cells was not shortened by cryopreservation. The safety and efficacy of autotransfusion of cryopreserved red cells in alloimmunized sickle cell disease patients with anemic episodes unrelated to sickling need to be determined.
Labile anticoagulant in a patient with lymphoma
Authors: Goldsmith G. H. , Saito H. , Muir W. A. 
Published: February 2007

AbstractA patient with diffuse lymphocytic lymphoma and monoclonal IgM kappa immunoglobulin was found to have an unstable circulating anticoagulant. The anticoagulant inhibited phospholipid‐dependent plasma coagulation reactions. Unlike previously described anticoagulants of this type, this plasma inhibitor was neutralized in vitro by products of platelet lysis but not by exogenous addition of other phospholipids or by intact platelets. Inhibitory activity seemed dependent on the monoclonal immunoglobulin, but isolated immunoglobulin fractions lacked anticoagulant activity, suggesting that the inhibitory function was dependent on an easily disrupted macromolecular aggregate. Recognition and characterization of other similar anticoagulants may provide a means of studying the role of phospholipids in normal hemostasis.
Freeze‐drying of hemoglobin solutions without adjuvant and in presence of glucose, tris, and β‐alanine: A study by electron spin resonance of the oxidized compounds produced
Authors: Chaillot B. , Labrude P. , Vigneron C. , Simatos D. 
Published: February 2007

AbstractHemoglobin cannot be freeze‐dried without the presence of protective compounds. Carbohydrates are a well‐known example of such compounds, but we have shown that some amine buffers and amino acids are also very effective. The mechanism of action of all these molecules is unknown. We report here experimental data showing that the protective effect is not the result of a direct bond between iron and the protective compound added.