bluuebunny/crossref_metadata_2025_split huggingface dataset:
Partial trisomy of the long arm of chromosome 1 in myelofibrosis and polycythemia vera
Authors: Hsu Lillian Y. F. , Pinchiaroli Dona , Gilbert Harriet S. , Wittman Ruth , Hirschhorn Kurt 
Published: February 2007

AbstractWe have identified partial trisomy 1q in 2 patients with different hematologic disorders. The first patient was a 55‐year‐old female with myelosclerosis and myeloid metaplasia diagnosed at age 38 years presenting with anemia, fatigue, bruising, fever, and splenomegaly. At age 56, she had 50–95% myeloblast cells and 95–100 nucleated RBC precursors per 100 WBC. Chromosome analysis of unstimulated leukocytes with Q, G, and C banding showed 46,XX,‐6,+t(1;6) (q25;p22) in all metaphase cells. In vitro incorporation of Fe55 was demonstrated in 90% of metaphases by autoradiography. The second patient, a 49‐year‐old male, was diagnosed as having polycythemia vera at age 30 during a regular checkup. He since developed hepatosplenomegaly. Chromosome analysis from a direct bone marrow preparation at age 44 and 45 showed grossly normal karyotypes. At age 49, his marrow by Q and G banding showed almost 100% of cells with 46,XY,–13,+t(1;13) (q12;p12). Eleven cases of trisomy of 1q have been reported in various hematologic disorders. It is apparent that partial trisomy 1q represents another nonrandom chromosomal abnormality, in addition to the most common nonrandom chromosomal aberrations, such as the Philadelphia chromosome, trisomy 8, trisomy 9, and monosomy 7 in hematologic disorders.
Hemoglobin A1C separation by isoelectric focusing
Authors: Beccaria Luciano , Chiumello Giuseppe , Gianazza Elisabetta , Luppis Benedetta , Righetti Pier Giorgio 
Published: February 2007

AbstractA modified type of isoelectric focusing has been applied successfully to the separation of hemoglobin A1C (HbA1C) from HbA in normal and diabetic cell lysates. It consists of transforming a linear pH gradient into a nonlinear one, by the addition of an amphoteric substance (“separator” or “pH gradient modifier”) with an isoelectric point (pI) close to the pI's of the two hemoglobins. Among the “modifiers” tested, histidine, proline, threonine, β‐alanine, 6‐amino caproic acid, and 5‐amino valeric acid are not useful in the hemoglobin pI range (pH 6.9–7.0). The dipeptide histidyl‐glycine (pI = 6.8; pI −pK1 = 1) is very efficient in flattening the pH gradient, in the hemoglobin region, even when added in low concentrations (10–100 mM), thus affording full resolution of the two hemoglobin species.
Post‐transfusion alloimmunization in patients with sickle cell disease
Authors: Orlina Armando R. , Unger Phyllis J. , Koshy Mabel 
Published: February 2007

AbstractThe transfusion histories over a 33‐month period of 50 patients with sickle cell disease were reviewed to determine the frequency of alloimmunization to red cell antigens following transfusion in these patients. There were 30 females and 20 males, aged 19‐49 years. Eighteen (36%) were immunized of which thirteen were females. Five of the patients have formed only one antibody so far, while the other 13 have formed two or more. Thirty‐six antibodies were identified: 16 against various Rh antigens, 12 anti‐Lewis, 5 anti‐Kell and one each of anti‐Jka, ‐Fya and ‐M. The immunized patients received, on the average, more transfusions although there was a considerable degree of overlap between the immunized and nonimmunized groups. An approach to the hemotherapy of patients with sickle cell disease (SCD) is discussed.
Effect of chloramphenicol on colony formation from erythrocytic precursors
Authors: Hara Hiroshi , Kohsaki Masatoshi , Noguchi Kohji , Nagai Kyoyasu 
Published: February 2007

AbstractIn the present experiments, we compare the behavior of erythropoietic precursors and granulocytic precurosors exposed to chloramphenicol. Subcutaneous injection of 20 mg of chloramphenicol sodium succinate on five successive days induced a reduction in the number of erythrocytic colony‐forming units (CFU‐E) and erythropoietic burst‐forming units (BFU‐E) in murine marrow and spleen, while no change in number of granulocytic precursors (CFU‐C) in murine hemopoietic organs was observed.In vitro studies demonstrated concentration‐dependent inhibition of chloramphenicol or chloramphenicol sodium succinate in the colony formation of all these precursors from murine and human bone marrow. However, erythropoietic burst formation of murine and human BFU‐E was completely suppressed by the addition of chloramphenicol or chloramphenicol sodium succinate to the culture medium at therapeutic concentrations (20‐40 μ/ml).From the present results, it can be concluded that the reduced number of BFU‐E in mice after injection of chloramphenicol sodium succinate resulted from the selective toxicity of chloramphenicol to BFU‐E at therapeutic con centrations.
Sickle cell anemia and trait in Southern India: Further studies
Authors: Brittenham G. , Lozoff B. , Harris J. W. , Mayson S. M. , Miller A. , Huisman T. H. J. 
Published: February 2007

AbstractPopulation surveys and family studies among 568 members of nine ethnic groups in southern India identified 15 homozygotes for sickle hemoglobin (Hb S) who had mild clinical and hematological manifestations with high levels of fetal hemoglobin (mean = 20%, range 8–36%) in a heterogeneous red cell distribution. In one family, the heterozygous mother had a hemoglobin pattern consistent with a form of the heterocellular hereditary persistence of fetal hemoglobin. Sickle cell trait was found in 153 (27%) of those studied. Chromatographic quantitation of the hemoglobin fractions in these heterozygotes showed a trimodal distribution of the proportion of Hb S explicable by a genetic model postulating the presence of genotypes with two (−α/−α), three (−α/αα) and four (αα/αα) active α‐globin genes. Globin synthesis studies in four heterozygotes believed to have two active α‐globin genes demonstrated an α/non‐α total activity ratio (0.57) consistent with this model.
Acquired hemoglobin H disease in idiopathic myelofibrosis
Authors: Veer Ashabala , Kosciolek Barbara A. , Bauman Arthur W. , Rowley Peter T. 
Published: February 2007

AbstractA 68‐year‐old white male, diagnosed 1 year previously as having myelofibrosis, developed hemolysis, red cell inclusions, and 37% Hb H. The alpha/beta globin synthetic ratio for circulating reticulocytes, determined by 3H‐leucine incorporation and globin chain separation by carboxymethylcellulose chromatography in urea, was 0.049. When total RNA was purified from peripheral blood cells and translated in a wheat germ cell‐free translation system, the alpha/beta ratio of the translation products was 0.26, indicting mRNA as a major cause of the globin synthetic imbalance. This study demonstrates that myelofibrosis is one setting in which acquired Hb H disease occurs; that the synthetic imbalance may be extreme; and that it can be associated with an imbalance in the activities of specific globin mRNAs.
The decline in energetic metabolism with aging of the erythrocyte and its relationship to cell death
Authors: Seaman Carol , Wyss Sonja , Piomelli Sergio 
Published: February 2007

AbstractHuman erythrocytes were separated by buoyant density ultracentrifugation into fractions of progressively increasing mean cell age to measure the changes in glycolytic activity that occur during their 120‐day life‐span. The maximal activities of all glycolytic enzymes were shown to decline exponentially with cell age. Only three glycolytic enzymes exhibited a marked rate of decline with a t1/2 shorter than the cell life‐span: hexokinase, aldolase, and pyruvate kinase.Glucose utilization, when measured in steady‐state conditions (1 mM inorganic phosphate), showed a fourfold decrease through the erythrocyte life‐span; lactate production also declined, but at a slower rate. When incubating conditions were altered by the introduction of a metabolic stimulus (either high phosphate for glycolysis, or methylene blue for the pentose pathway) the youngest cell fractions responded with decidedly increased rates of glucose consumption and lactate production. However, this ability gradually declined with cell aging, and ultimately, the oldest cells had metabolic rates as low as if there were no stimulus present. The oldest erythrocytes appear to have lost the flexibility needed to respond to metabolic stress and are more vulnerable to events in the circulation that may require the ability to increase the basal rate. This defect is probably responsible for the disappearance of aged erythrocytes from the circulation.
Increased bone marrow delta‐aminolevulinic acid synthetase activity in the acute reversible sideroblastic anemia of alcoholics
Authors: Fraser M. B. , Schacter B. A. 
Published: February 2007

AbstractIn an effort to clarify the biochemical pathogenesis of the acute reversible siderblastic anemia of alcoholics, bone marrow delta‐aminolevulinic acid (ALA) synthetase activity was measured in anemic alcoholic patients with and without pathological ring sideroblasts in their marrows and compared with the activity of this enzyme in normal subjects and in patients with idiopathic sideroblastic anemia. Mean ALA‐synthetase activity measured (following addition of exogenous pyridoxal phosphate in three patients with the ring sideroblast morphologic abnormality) was 502 ± 147 (SE) pmoles ALA per 106 erythroblasts per 30 minutes, as compared with 201 ± 69 in the seven alcoholic patients without pathologic sideroblastic marrow abnormalities, 169 ± 32 in the six normal subjects, and 52 ± 4 pmoles ALA per 106 erythroblasts per 30 minutes in two patients with idiopathic sideroblastic anemia. Comparison of enzyme activity in the presence and absence of pyridoxal phosphate revealed that addition of this enzyme cofactor accentuated ALA‐synthetase activity by an average of only 24% in the alcoholic sideroblastic group, as compared with 42% accentuation in the normal group and 8% accentuation in the idiopathic sideroblastic group. These results suggest that abnormalities in pyridoxal phosphate metabolism in alcoholics may not be of importance in the inhibition of heme biosynthesis at the level of ALA synthetase but, rather, that an enzymatic or metabolic block should be sought at a more distal site in the heme biosynthetic pathway.
Serum ferritin in hematologic malignancies
Authors: Matzner Y. , Konijn A. M. , Hershko C. 
Published: February 2007

AbstractSerum ferritin was measured in a variety of hematologic malignancies at presentation, in remission following therapy, and in relapse. Ferritin was strikingly increased in all acute leukemias at presentation and in relapse, in the blastic crisis of CML, and in smouldering leukemia. Remission in both ALL and ANLL was associated with a reduction of serum ferritin, and this normalization was a function of remission duration. In the malignant lymphomas serum ferritin was related to tumor histology. Highest levels were found in Hodgkin disease and histiocytic lymphoma, normal levels in lymphocytic lymphoma, and intermediate levels in mixed histiocytic‐lymphocytic lymphoma. In all cases, remission was associated with normalization of serum ferritin. These correlations suggest that serum ferritin measurements may be of clinical usefulness in the initial evaluation and in the assessment of response to therapy in patients with acute leukemia and malignant lymphoma.
Hexokinase “New Hyde Park”: A low activity erythrocyte isozyme in a chinese kindred
Authors: Paglia Donald E. , Shende Ashok , Lanzkowsky Philip , Valentine William N. 
Published: February 2007

AbstractChronic hemolytic anemia in a 7‐year‐old Chinese boy was found to be associated with decreased activity of erythrocyte hexokinase (approximately 20% of appropriate control values). Alterations in kinetics, stability, pH optimum, or electrophoretic patterns could not be demonstrated with proband hexokinase, but subtle differences between maternal and paternal hexokinase characteristics suggested that two separate mutant isozymes were involved and that the proband was heterozygous for each. Comparisons with previously reported cases of hexokinase deficiency demonstrate the broad genetic heterogeneity that characterizes this disorder.
Effect of zinc supplementation on serum testosterone level in adult male sickle cell anemia subjects
Authors: Prasad Ananda S. , Abbasi Ali A. , Rabbani Parviz , Dumouchelle Elizabeth 
Published: February 2007

AbstractPreviously, we have documented primary testicular failure in adult male subjects with sickle cell anemia. We have also reported the occurrence of zinc deficiency and suggested that androgen deficiency may be related to zinc deficiency in such patients. In this study, we present data with respect to the effect of oral zinc supplementation on serum testosterone levels in adult male patients with sickle cell anemia. An increase in serum testosterone, neutrophil zinc, and neutrophil alkaline phosphatase activity was observed in the zinc‐supplemented group in comparison with the group on placebo. Additionally, body weight increased and serum lactic dehydrogenase activity decreased in response to zinc supplementation. We conclude that androgen deficiency in adult male subjects with sickle cell anemia is correctable with zinc supplementation and that the determination of neutrophil zinc and alkaline phosphatase activity in the neutrophils may be utilized as good indicators of body zinc status in such subjects.
The aggregation of human platelets by ascitic fluid: A possible mechanism for disseminated intravascular coagulation complicating LeVeen shunts
Authors: Salem Hatem H. , Koutts Jerry , Handley Chris , Van Der Weyden Martin B. , Dudley Frank J. , Firkin Barry G. 
Published: February 2007

AbstractTo identifiy causative factors responsible for the disseminated intravascular coagulation complicating peritoneovenous (LeVeen) shunts, the ascitic fluid from 12 patients with alcoholic liver disease or peritoneal malignancy was examined for its effects on human platelets. In all patients, concentrated ascitic fluid caused irreversible platelet aggregation. Properties of the aggregating factor suggested that it is collagen, and subsequently, the presence of collagen in ascitic fluid was confirmed.This finding, together with the known effects of collagen on platelets and contact clotting factors, would be sufficient to explain the development of disseminated intravascular coagulation following this procedure. Aspirin by inhibiting collagen‐induced aggregation may have a therapeutic role in the management of this problem.
Hematopoiesis in the human spleen
Authors: Freedman Melvin H. , Saunders E. Fred 
Published: February 2007

AbstractTo assess the capacity of the human spleen to function as a hematopoietic organ, spleen mononuclear cells from patients undergoing splenectomy were cultured to assay CFU‐E, BFU‐E, and CFU‐C. Although the peripheral blood from cases of hereditary spherocytosis, Gaucher disease, Thalassemia major, and idiopathic thrombocytopenic purpura yielded 3–20 BFU‐E/5 × 105 cells plated and 1–7 CFU‐C/5 × 105, suspensions of spleen cells failed to grow colonies. Histologically these spleens did not show extramedullary hematopoiesis. In contrast, peripheral blood from a patient with osteopetrosis (marble bone disease) yielded 130 BFU‐E/5 × 105, 40 CFU‐E/5 × 105, and 9 CFU‐C/5 × 105. The spleen had extensive extramedullary hematopoiesis on microscopy and grew 180 BFU‐E/105, 210 CFU‐E/105, and 25 CFU‐C/105. We conclude that spleens from patients without extramedullary hematopoiesis do not contain committed hematopoietic progenitors in spite of normal precursors in the blood. In osteopetrosis the spleen contained stem cells in a concentration comparable to bone marrow, and the embryonic role of the spleen in blood formation appeared to continue in postnatal life.
The myeloproliferative reaction in a child with down syndrome: Cytological and chromosomal evidence for a transient leukemia
Authors: Lazarus Kenneth H. , Heerema Nyla A. , Palmer Catherine G. , Baehner Robert L. 
Published: February 2007

AbstractInfants with Down syndrome occasionally develop marked myeloproliferative reactions (MPR) indistinguishable from acute myelocytic leukemia but which spontaneously regress. Most authors have attributed MPR to immature or defective control on bone marrow development. In this report serial cytomorphologic and chromosomal findings in the bone marrow and peripheral blood of a child with the MPR suggests that MPR may be due to a spontaneously regressing clone of malignant cells. At the height of the myeloproliferative response, chromosomal studies demonstrated a clone of blast‐like cells with a 21‐22 translocation consistent with Down Syndrome and an X‐8 translocation which was present only in the initial peripheral blood analysis harvested at 24 hours but was not present in the 72 hours samples. This X‐8 translocation was not demonstrated in bone marrow or peripheral blood samples obtained at random times throughout the one year followup. During this period there was complete resolution of the MPR. In addition the chromosomes from one cell demonstrated several double minutes at the time of the MPR. Double minutes have previously been reported only in malignant diseases, especially tumors of neural origin and leukemia. These findings raise questions about the benign origin of the MPR and support the idea the MPR may be the result of a spontaneously regressing clone of malignant cells.
Coexistence of paroxysmal nocturnal hemoglobinuria and chronic lymphocytic leukemia
Authors: Omura George A. 
Published: February 2007

AbstractA man with paroxysmal nocturnal hemoglobinuria and a lymphoproliferative disorder is described. The latter condition evolved into a typical picture of chronic lymphocytic leukemia. The significance of this association is obscure, but treatment of one or the other disease was clearly not an etiologic factor in this concurrence.
Surface immunoglobulins on hairy cells of 55 patients with hairy cell leukemia
Authors: Golomb Harvey M. , Davis Susan , Wilson Carol , Vardiman James 
Published: February 2007

AbstractSurface immunoglobulins (SIg) were determined on peripheral blood samples from 55 patients with hairy cell leukemia (HCL) and on hairy cells from spleen preparations of 14 of these 55 patients. The patterns of SIg for HCL was compared to the patterns on peripheral blood leukemic cells from 39 patients with chronic lymphocytic leukemia (CLL) and 15 patients with poorly differentiated lymphocytic (PDL) lymphoma. Of the 55 HCL patients, 42 could be scored for individual heavy and light chains; 16 had only IgG, 14 had two or three heavy chains, 7 had only IgD, and 5 cases had no SIg and were E‐rosette negative. This pattern was different from the B‐cell pattern in CLL and PDL where there were few cases of IgG alone (5%) and many cases of IgM alone (50%). Surface marker profile did not correlate with survival in any of the sub‐groups tested. HCL appears to be a B‐cell lymphoproliferative disease in greater than 90% of cases; many combinations of heavy chains with only a single light chain can be demonstrated.
Increased adsorption of cytoplasmic proteins to the erythrocyte membrane in ATP‐depleted normal and pyruvate kinase‐deficient mature cells and reticulocytes
Authors: Allen David W. , Groat Jerome D. , Finkel Barbara , Rank Brian H. , Wood Patricia A. , Eaton John W. 
Published: February 2007

AbstractHow the metabolic defect of pyruvate kinase deficiency (PK(–)) accelerates red blood cell (RBC) destruction is not established, but may be related to RBC membrane abnormalities associated with altered cellular metabolism. Furthermore, it has been shown that PK(–) reticulocytes are especially sensitive to metabolic depletion. Therefore, we compared the membranes of reticulocyte‐rich PK(–) RBC, both fresh and ATP depleted, with membranes of fresh and ATP depleted normal mature RBC and reticulocytes. There was no difference between the specific gravity (SG) of the membranes of normal mature RBC (SG 1.152 ± 0.004) and membranes of reticulocyte‐rich RBC from several anemias (SG 1.150 ± 0.002). However, membranes from fresh, reticulocyte‐rich PK(–) RBC were dense with SG of 1.165 ± 0.004 which correlated with a corresponding increase of protein to lipid phosphorus ratio of 66 ± 8 μg protein/μg lipid phosphorus (normal 52 ± 6 μg/μg). The membrane density of PK(–) RBC was further increased when the PK(–) RBC ATP was depleted by anaerobic incubation (SG 1.188 ± 0.004) or cyanide inhibition (SG 1.182 ± 0.001). When ATP was depleted in normal RBC and in non‐PK(–) reticulocytes, corresponding increases in membrane SG occurred. A distinctive 50,000 MW peptide is adsorbed from the cytoplasm to the membranes of reticulocytes (both normal and PK(–) when these cells were depleted of ATP. The increased membrane adsorption of cytoplasmic proteins by PK(–) RBC was not associated with increased RBC calcium uptake, sulfhydryl oxidation, or altered membrane protein phosphorylation. All the observed abnormalities of PK(–) RBC membranes could by reproduced by ATP depletion of reticulocyte‐enriched non‐PK(–) RBC.
Monocytes with binding capacity to sheep red blood cells in a case of acute undifferentiated leukemia
Authors: Liso Vincenzo , Tursi Alfredo , Pavone Vincenzo , Specchia Giorgina , Loria Maria Paola , Bonomo Lorenzo 
Published: February 2007

AbstractMonocytes with binding capacity to sheep red blood cells (SRBC) are described in a case of acute undifferentiated leukemia with blast cells showing no cytochemical or immunological markers. The monocyte nature of the rosetting cells was confirmed by intense reactions of naphthol AS esterase and α‐naphthyl esterase and sometimes by peroxidase positivity.
Severe thrombocytopenia following oral cholecystography with iocetamic acid
Authors: Insausti Carmen L. G. , Lechin Fuad , van der Dijs Bertha 
Published: February 2007

AbstractWe report the first case of severe thrombocytopenia occurring after ingestion of a widely used cholecystographic medium, iocetamic acid. The patient had not been given any treatment before he received the gallbladder contrast medium. Onset of symptoms was acute, and the clinical course was benign with complete recovery after one week. Bone marrow aspiration showed increased numbers of megakaryocytes, suggesting that platelets were rapidly removed from circulation. In vitro tests for antiplatelet antibodies were not performed because iocetamic acid is insoluble in aqueous solutions. Since only a few cases of thrombocytopenia following ingestion of iodine‐compounds have been reported, it seems highly unlikely that purpura will be, in the future, a major hazard in cholecystography. However, it would be wise to question patients as to any prior sensitivity to iodine before proceeding with oral cholecystography.