bluuebunny/crossref_metadata_2025_split huggingface dataset:
The anemia of senescence
Authors: Lipschitz D. A. , Mitchell C. O. , Thompson C. 
Published: February 2007

AbstractA study of 196 healthy geriatric females and 26 males confirmed the high prevalence of anemia in this population. Forty‐two females (21.4%) had a hematocrit of less than 36% and nine (34%) of the males had a value of less than 40%. A careful evaluation, including a therapeutic trial of oral iron in some subjects, made a diagnosis of iron deficiency anemia in only three and the anemia of chronic disease could be diagnosed in two. No subject had folate or B12 defiency and hemolytic anemia was not present. Thus, in most of these subjects, the lower hematocrit was not due to commonly recognized causes. Further evaluation revealed a high prevalence of leukopenia in this population; 30% having counts less than 5 × 103/μd and 10% having values less than 4 × 10−3/ μl. Of most importance, leukocyte counts were significantly lower in anemic subjects in whom a high incidence of neutropenia was also demonstrated. This suggests that the mechanisms of the unexplained anemia and neutropenia is an overall reduction in hematopoietic cell numbers. This conclusion is consistent with the observation that many aged subjects have a decreased ability to mount an adequate leukocyte response to infection. These findings suggest that the presence of anemia marks an overall reduction in hematopoietic reserve and provides a clue to those aged subjects at greatest risk of an inadequate response to stress.
A simplified method for monitoring heparin therapy at the bedside: The activated whole blood clotting time
Authors: Forman Walter B. , Bayer Gerald 
Published: February 2007

AbstractTwelve patients receiving heparin during hemodialysis had coagulation parameters monitored using the whole blood clotting time (WBCT), activated partial thromboplastin time (APTT), plasma heparin levels and activated whole blood clotting time (AWBCT). The AWBCT, performed with a battery‐powered portable incubator/timer, correlated with both the corresponding WBCT and the plasma heparin levels. However, the correlation between the AWBCT and the APTT was quite poor. This assay is simply performed, inexpensive, and reproducible. It is recommended as an excellent method for monitoring heparin therapy.
Trace metals in hematopoiesis
Authors: Deur Charles J. , Stone Marvin J. , Frenkel Eugene P. 
Published: February 2007

AbstractThe trace metals represent an extremely small part of the total mass of the organism. In spite of this, these elements appear to play major roles in metabolism. At the level of the hematopoietic system, specific roles can be identified for iron, copper, zinc, and cobalt. Other trace metals appear to play less clearly defined roles in hematopoiesis but clearly are involved by virtue of a number of interactions at the level of absorption, intermediary metabolism, or modulation of toxicity. Still other trace metals, such as lithium, appear to have a therapeutic role in altering production of granulocytes, by an as yet unidentified mechanism.
Epinephrine potentiation of arachidonate‐induced aggregation of cyclooxygenase‐deficient platelets
Authors: Rao Gundu H. R. , White James G. 
Published: February 2007

AbstractEvaluation of platelet physiology, biochemistry, ultrastructure, and function in a young woman without history of hemorrhagic problems revealed that her platelets were deficient in cyclooxygenase activity. Her citrate platelet‐rich plasma (C‐PRP) responded monophasically when stirred with aggregating agents in the same manner as aspirin‐treated normal C‐PRP, but could be irreversibly aggregated by high concentrations of thrombin, collagen, and ADP. Her platelets did not aggregate when stirred with AA at concentrations as high as 2 mM. Ultrastructure and levels of serotonin and adenine nucleotides were normal. Amounts of 14C‐AA released after stirring with thrombin were similar to normal cells. However, evaluation of prostaglandin synthesis after stirring with 14C‐AA revealed no evidence of endoperoxide or thromboxane production, although products of the lipoxygenase pathway were produced in normal amounts. Aggregation in response to AA was completely corrected after mixing with 10% normal C‐PRP. However, equal volumes of her C‐PRP and normal aspirin‐treated C‐PRP did not respond to AA, whereas 10% normal platelets combined with aspirin‐treated cells corrected aggregation to AA. Since epinephrine pretreatment corrects the response of dog platelets that are not aggregated by AA, we evaluated the influence of epinephrine on her platelets. Preexposure to 5 μM epinephrine, a concentration that gave only primary waves of aggregation, resulted in normalization of her response to AA, even though correction was not associated with the generation of endoperoxides or thromboxanes. The results may explain why patients with platelet cyclooxygenase deficiency have mild or absent bleeding symptoms.
Factor‐X deficiency in amyloidosis: A critical review
Authors: Greipp Philip R. , Kyle Robert A. , Bowie E. J. Walter 
Published: February 2007

AbstractThirty cases of amyloidosis with factor‐X deficiency, including six of our own, were reviewed. Modest deficiency of factor X was often associated with severe bleeding. In many of the cases, clinical bleeding could not be accounted for by deficiency of factor X alone. Other hemostatic defects were found in these patients and probably contributed to the bleeding. Modes of treatment, including the empiric use of corticosteroids and splenectomy, were discussed in light of current knowledge of pathogenesis of this unusual blood clotting disorder. This involves the interaction of amyloid fibrils and blood clotting factors.
Idiopathic thrombocytopenic purpura and graves disease
Authors: Valenta Lubomir J. , Treadwell Thomas , Berry Richard , Elias Alan N. 
Published: February 2007

AbstractA 31‐year‐old black woman was studied who, at the age of 12 years, underwent splenectomy for a bleeding disorder due to idiopathic thrombocytopenic purpura (ITP). More than 20 years after the first signs of her bleeding disorder, the patient developed signs of Graves disease. This condition was treated with 131I with resulting hypothyroidism. The rare combination of ITP and Graves disease was considered to be a manifestation of two separate autoimmune diseases. For the second time in the literature, HLA typing was performed in a patient with such a disease combination, and it was found to be of the group A23, A28, and B17. This is different from B8, which is most frequently found in both isolated ITP and Graves disease. Possible racial factors may be involved. It is concluded that more cases of such combined disease need to be studied before possible genetic patterns can be established.
Nonsteroidal anti‐inflammatory drugs in the treatment of hemophilic arthropathy
Authors: Thomas Patricia , Hepburn Bonnie , Kim Hugh C. , Saidi Parvin 
Published: February 2007

AbstractThis study was undertaken to investigate the safety and efficacy of nonaspirin, nonsteroidal anti‐inflammatory drugs (NSAIDs) in controlling the pain and stiffness of hemophilic arthropathy. Data were collected from eight adult hemophilic patients in successive double‐blind controlled studies using choline magnesium trisalicylate (CMT) and ibuprofen (IPF). In seven patients, neither drug consistently affected bleeding times, platelet aggregation studies, frequency of joint hemorrhages, or frequency of factor infusions. In one patient, platelet aggregation appeared to be affected by CMT and bleeding time became markedly prolonged after IPF therapy. Three patients taking CMT and four patients taking IPF reported greater relief of pain and stiffness with the drugs than with placebos. Our results suggest that IPF and CMT can benefit the hemophiliac and that they can be used safely in most hemophilic patients under closely supervised conditions.
Coexistence of gaucher disease and philadelphia positive chronic granulocytic leukemia
Authors: Shinar E. , Leibovitz Gershon Z. ‐Ben , Leiserowitz R. , Matzner Y. , Yatziv S. , Polliack A. 
Published: February 2007

AbstractA patient with coexistent Gaucher disease and Philadelphia positive chronic granulocytic leukemia (CGL), who subsequently developed myeloblastic leukemia, is described. The diagnosis of CGL was established according to standard clinical, morphological, biochemical, and cytogenetic data, while the diagnosis of true Gaucher disease was based on biochemical data and the presence of Gaucher cells with typical ultrastructural features in the bone marrow and spleen. Enzyme studies showed low activity of ceramide‐β‐glucosidase in the patient's peripheral blood leukocytes, skin fibroblasts, and splenic tissue and the presence of increased amounts of ceramide‐β‐glucoside in the spleen.This case is reported in order to draw attention to the possible coexistence of these two diseases in the same patient, as opposed to the well‐recognized finding of “Gaucher‐like” cells in the bone marrow of patients with CGL. Enzyme studies enable distinction between these two situations.
Indications for platelet transfusion in children with acute leukemia
Authors: Murphy Scott , Litwin Samuel , Herring Leonard M. , Koch Penelope , Remischovsky Judith , Donaldson Milton H. , Evans Audrey E. , Gardner Frank H. 
Published: February 2007

AbstractIn an attempt to determine the indications for platelet transfusion in thrombocytopenic patients, we randomized 56 children with acute leukemia to one of two regimens of platelet transfusion. The prophylactic group received platelets when the platelet count fell below 20,000 per mm3 irrespective of clinical events. The therapeutic group was transfused only when significant bleeding occurred and not for thrombocytopenia alone. The time to first bleeding episode was significantly longer and the number of bleeding episodes were significantly reduced in the prophylactic group. The survival curves of the two groups could not be distinguished from each other. Prior to the last month of life, the total number of days on which bleeding was present was significantly reduced by prophylactic therapy. However, in the terminal phase (last month of life), the duration of bleeding episodes was significantly longer in the prophylactic group. This may have been due to a higher incidence of immunologic refractoriness to platelet transfusion. Because of this terminal bleeding, comparison of the two groups for total number of days on which bleeding was present did not show a significant difference over the entire study period.
Osmotic error in erythrocyte volume determinations
Authors: Beautyman William , Bills Terry 
Published: February 2007

AbstractBecause of osmotic effects erythrocytes suspended in their native plasma do not have the same volume as the same erythrocytes suspended in Isoton. The discrepancy varies depending upon the osmolality and composition of the native plasma and the length of time the cells have been suspended in Isoton. Consequently, the MCV recorded in an electronic particle counter (Coulter in this case) may differ markedly from the true in vivo MCV. A similar error affects the Coulter hematocrit, which is calculated from the MCV and the erythrocyte count. This matrix effect should be taken into account in any laboratory quality assurance program.
Mixed myeloid—lymphoid colonies in a patient with polycythemia vera
Authors: Beckman Barbara , Anderson William F. , Beltran G. , Chen I. , Fisher James W. 
Published: February 2007

AbstractPeripheral blood mononuclear cells from a patient with polycythemia vera were cultured in a methylcellulose system employing human serum. Electron microscopy documented the appearance of mixed colonies containing lymphocytes, granulocytes, megakaryocytes, and erythrocytes. In vitro culture characteristics were similar to those seen for other patients with polycythemia vera, ie, colonies grew in the absence of added erythropoietin or other pathway‐specific regulators. Plating efficiency was linearly related to the number of cells plated, which supports the concept that each colony arose from a single cell. The appearance of mixed myeloid—lymphoid colonies points to the existence of a primitive stem cell capable of giving rise to multiple hematopoietic cell lines.
Simultaneous detection of two mechanisms of immune destruction of penicillin‐treated human red blood cells
Authors: Yust Israel , Frisch Bertha , Goldsher Naomi 
Published: February 2007

AbstractTwo separate processes of putative red‐cell destruction in penicillin‐induced immune hemolysis were measured simultaneously by a rapid (3 hour) assay utilizing 51Cr‐labelled red blood cells (RBC). Antibody‐dependent, cell‐mediated cytotoxicity (ADCC) was estimated by release of 51Cr; and antibody‐dependent phagocytosis (ADPh) by quantitation of 51Cr uptake into mononuclear phagocytes as well as by counts of engulfed RBC. Attacking cells were obtained by Ficoll‐Hypaque separation of peripheral blood from normal donors. Phagocytosis as well as lysis were proportional to anti‐penicillin antiserum concentration, to incubation time, and to the concentration of the attacking cells. Enrichment of mononuclear phagocytes in the attacking cell population by albumin gradient separation led to an increase in phagocytosis as well as in cytotoxicity. Depletion of mononuclear phagocytes resulted in a decline in both processes. Dilution of antiserum abolished ADCC but affected ADPh only slightly. Iodoacetate as well as colchicine inhibited both activities. These results indicate that both processes may be operative in the immune destruction of RBC in vivo.
The generation of antibody diversity
Authors: Rose David R. 
Published: February 2007

AbstractBy their nature, antibody molecules exhibit a wide range of binding specificities. The antigen‐binding properties of the antibody reside entirely in the amino‐terminal portion of the molecule, termed the variable domain. Structurally, the combining site specificity is determined by the amino‐acid residues within 6 short lengths, 3 each in the heavy and light chains, of unusually variable sequence. The hypervariability of 2 of these lengths arises from the somatic recombination of short gene segments into a single stretch of mRNA which encodes the entire variable region of 1 polypeptide chain. For example, a V gene segment that codes for most of the variable portion of a light chain, can combine with one of a number of much shorter J gene segments to create the complete variable region gene. In heavy chain genes, a third element, the D gene segment, increases the potential for diversity even further. A mechanism has been proposed by which variability occurs at the point where 2 gene segments join. Thus, a large part of the generation of antibody diversity occurs in the somatic recombination of small genetic elements.
Kinetic alterations of the red cell membrane phosphatase in α‐ and β‐Thalassemia
Authors: Morlé L. , Dorléac E. , Alloisio N. , Jaccoud P. , Colonna P. , Bachir D. , Delaunay J. 
Published: February 2007

AbstractWe studied the red cell membrane neutral phosphatase, which is part of the Na+K+ ATPase, in several types of oxidative hemolytic anemias. We used an artificial substrate, the p‐nitrophenylphosphate. In controls and in patients heterozygous for various unstable hemoglobins (Hb Hope, Hb Köln, or Hb Hammersmith), the kinetics were of the Michaelis‐Menten type. On the contrary, in nearly all patients with α‐ or β‐thalassemia, the kinetics displayed an abnormally biphasic character. The apparent Michaelis constant (KMapp) was significantly decreased. The biphasic character correlated with the imbalance of globin chain synthesis. The β‐mercaptoethanol markedly increased Vmax in controls, but had little effect on the biphasic kinetics. Omission of K+abolished the biphasic kinetics. The abnormal kinetics failed to appear with another artificial substrate, the 4‐methylum‐belliferylphosphate, nor did it appear with ATP, the natural substrate. In vitro, H2O2 treatment of normal and thalassemic red cells was unable to induce or exaggerate, respectively, the biphasic kinetics, but generated alterations of a different nature. We suggest that the various kinetic alterations of the phosphatase in thalassemic syndromes originate from the imbalance of globin chain synthesis. However, the involvement of an oxidative process remains to be demonstrated.
Emperipolesis of neutrophils by dysmorphic megakaryocytes
Authors: Parmley R. T. , Kim T. H. , Austin R. L. , Alvarado C. S. , Ragab A. H. 
Published: February 2007

AbstractNeutrophil engulfment by megakaryocytes was observed within 20 to 30% of megakaryocytes from two children: one with metastatic rhabdomyosarcoma, the other with fever of unknown origin. Other cell types and neutrophil precursors were not observed within megakaryocytes. Only late megakaryocytes were involved in the process, and often these cells appeared vacuolated or degenerating at the light and electron microscope level. Ultrastructurally the engulfed neutrophils were intact and were within the open canalicular system of the megakaryocyte cytoplasm. No evidence of neutrophil granule exocytosis could be demonstrated in ultrastructural morphologic and peroxidase preparations; however, many neutrophils appeared to be endocytosing portions of the megakaryocyte cytoplasm. The phenomenon could not be transferred to normal marrow incubated with patient serum or plasma. Thus, our patients differ from previous observations of emperipolesis in: 1) the extreme frequency of the observation; 2) the selective involvement of neutrophils; and 3) the association of the anomaly with dysmorphic and/or disrupted megakaryocytes. These observations are consistent with a neutrophil response to altered and/or injured megakaryocytes.
Successful treatment of thrombotic thrombocytopenia purpura by vincristine
Authors: Schreeder Marshall T. , Prchal Josef T. 
Published: February 2007

AbstractTwo patients with thrombotic thrombocytopenia purpura (TTP) are described in whom vincristine administration appeared to play a role in successful outcomes. The first patient with chronic TTP responded partially to plasma exchange followed by plasma infusion and transiently to splenectomy, but developed a complete long lasting remission after a single injection of intravenous vincristine. The second patient developed a complete remission following vincristine administration and plasma exchange. Although vincristine's mechanism of action in TTP is obscure, we suggest that vincristine is effective in the treatment of this infrequent and often fatal disease.
Paroxysmal nocturnal hemoglobinuria terminating in TdT‐positive acute leukemia
Authors: Katahira Jun'Ichi , Aoyama Masako , Oshimi Kazuo , Mizoguchi Hideaki , Okada Michiko 
Published: February 2007

AbstractA case of paroxysmal nocturnal hemoglobinuria (PNH) which developed terminal transfer‐ase (TdT)‐positive leukemia 5 years after the diagnosis of PNH was studied. Most of the leu‐kemic cells were suggestively lymphoid by cytochemistry and electron microscopy, and TdT‐positive by immunofluorescence studies. The development of acute lymphoblastic leukemia during the course of PNH suggests that in PNH the clonal abnormality may involve lymphoid cells as well as myeloid cells, thus raising the possibility of the disease being a disorder of the pluripotential stem cell.
Transcobalamin II deficiency associated with unusual bone marrow findings and chromosomal abnormalities
Authors: Rana Sohail R. , Colman Neville , Goh Kong‐Oo , Herbert Victor , Klemperer Martin R. 
Published: February 2007

AbstractA female infant presented at seven weeks of age with failure to thrive, progressively severe pancytopenia, hypogammaglobulinemia and, mucosal ulceration. Bone marrow morphology showed severed megaloblastic changes in the myeloid series with a shift to the left and an increased number of blasts with abnormal morphology. Erythroid precursors and megakaryocytes were markedly decreased. Cytogenetic studies showed marked aneuploidy and increased chromosomal breakage. Treatment with high doses of vitamin B12 resulted in a dramatic clinical response with hematological values becoming normal. The patient's serum showed absence of transcobalamin II, and very little TC I and TC III binding. The patient's parents had only half the lower limits of normal transcobalamin II. QUSO G‐32 was used for separation of transcobalamins, and the results were confirmed by Sephacryl S‐300. This case illustrates the usefulness of QUSO in the rapid diagnosis of transcobalamin II deficiency.
Alpha chain and gamma chain abnormal hemoglobins in newborn babies: Structural and genetic aspects
Authors: Nakatsuji T. , Carver J. , Wilson J. B. , Lam H. , Reese A. L. , Nagle S. , Miwa S. , Huisman T. H. J. 
Published: February 2007

AbstractStructural studies and quantitative analyses were conducted on the hemoglobin of 55 newborn babies. Seven α chain variants (G‐Philadelphia, Montgomery, Inkster, I‐Philadelphia, Matsue‐Oki, Winnipeg, and O‐Indonesia) were present in 26 heterozygous new‐borns (17 black, eight Caucasian, and one Indonesian). The relative amount of the αx containing abnormal Hb F of the Hb G‐Philadelphia and Hb Winnipeg babies was less than observed in heterozygous adults, which may indicate a decreased rate of assembly of the αx‐γ dimer over that of the αx‐β dimer. Of the 29 newborns with γ chain variants, 16 were Caucasian babies; of these 15 had a Hb Aγ F‐Hull heterozygosity and one a Hb GγF‐Marietta heterozygosity. Six black babies were heterozygous for Hb AγF‐Texas‐I and six for Hb GγF‐Port Royal. One Japanese baby had a heterozygosity for AγF‐Iwata and a second was heterozygous for ATF‐Yamaguchi. Quantitative analyses of the isolated normal Hb F0 as well as an evaluation of the relative amounts of the Hb Fx in the red cell lysates gave data useful for a speculation of the genetic condition in each of these babies. It was concluded that the babies with the Hbs F‐Texas‐I, F‐Iwata, F‐Hull, and F‐Marietta were simple heterozygotes with either the Gγ,Aγ/Gγ,AγN or the Gγ,Aγ/Gγx,Aγ genic arrangement. The babies with Hb F‐Port Royal had a Gγ,GγxGγ,Aγ arrangement, which may result from a (to be determined) gene conversion. The newborn baby with Hb F‐Yamaguchi has the Gγ,Aγ/Aγ/TN.—. genic arrangement, suggesting the presence of three distinctly different γ chain genes of which one, the AγT‐X gene, produces an Aγ chain (with threonyl at position γ75 and an Asn at position γ80) at a level usually seen for Gγ rather than Aγ chains. These studies were greatly facilitated by the use of high pressure liquid chromatographic methods.
Pericardial tamponade associated with myeloid metaplasia
Authors: Bubley Glenn , Come Patricia , MacDougall Don , Thurer Robert , Goldberg Joan 
Published: February 2007

AbstractA 32‐year‐old white male with agnogenic myeloid metaplasia developed pericardial tamponade shortly after elective splenectomy. Tamponade was relieved by pericardiocentesis but recurred, necessitating partial pericardiectomy. Following surgery and a course of radiation therapy to the heart, there was no further significant reaccumulation of fluid. Pathologic evaluation demonstrated foci of extramedullary hematopoiesis in the pericardium. To our knowledge, pericardial tamponade has not previously been reported as a biopsy‐proven complication of myeloid metaplasia.
Essential thrombocythemia: A clonal disorder of hematopoietic stem cell
Authors: Singal Usha , Prasad Ananda S. , Halton David M. , Bishop Carter 
Published: February 2007

AbstractWe studied 5 patients with essential thrombocythemia utilizing glucose‐6‐phosphate dehydrogenase (G‐6‐PD) enzyme as a cell marker for determining clonality. One of the patients was found to be heterozygous for isoenzymes B and A in the nonhaemopoietic tissues such as fibroblasts, but manifested only isoenzyme type B in the erythrocytes, neutrophils, and platelets. Our studies support the concept that essential thrombocythemia is a clonal disorder arising in a multipotent stem cell.