bluuebunny/crossref_metadata_2025_split huggingface dataset:
A new density gradient system for the separation of human red blood cells
Authors: Vettore Luciano , De Matteis Maria Concetta , Zampini Patrizia 
Published: February 2007

AbstractA new density system for the separation of human red blood cells by density‐gradient centrifugation is described.The gradient medium is made with colloidal silica particles coated with polyvinylpyrrolidone suspended in aqueous solution of meglamine diatrizoate.By this method, more than 10 red‐cell fractions can be separated. These show different ages (by creatine content and 59Fe in vivo labelling) and different characteristics (ie, potassium content). A 40‐fold enrichment in reticulocytes can be obtained in the top layers, with a great improvement of specific activity in labelling procedures of newly synthesized globin chains.The method is simple, rapid, inexpensive, reliable, nontoxic for erythrocytes, and is suitable for globin synthesis and other studies of erythrocyte metabolism.
Congenital neutropenia: Studies of pathogenesis
Authors: Chusid Michael J. , Pisciotta Anthony V. , Duquesnoy Rene J. , Camitta Bruce M. , Tomasulo Peter A. 
Published: February 2007

AbstractCongenital neutropenia (CN) was diagnosed in a five‐month‐old boy. A variety of studies was performed to define the pathogenesis of his disease. Opsonic antineutrophil antibodies were present in his serum. Transfused normal granulocytes circulated poorly. Incubation of the patient's serum with normal granulocytes failed to alter their metabolic or functional activity. The patient's marrow demonstrated increased numbers of colony‐forming units (CFUs) in vitro compared with control marrow. The patient's parents had low marrow CFU activity. The patient's serum and peripheral lymphocytes failed to inhibit normal marrow CFU activity. The patient's marrow did inhibit CFU growth of an HLA‐identical‐sibling's marrow in coculture. Histocompatibility studies demonstrated the HLA‐B12 antigen in this patient, a histocompatibility marker previously associated with CN. These studies suggest some cases of CN are associated with a genetically transmitted marrow factor capable of suppressing myelopoiesis in normal marrow.
Heterogeneity of hypogammaglobulinemia
Authors: Miller Michael E. 
Published: February 2007

AbstractIn 1952, Ogden Bruton described the first patient with recurrent infections and hypogammaglobulinemia [1]. For many years thereafter, hypogammaglobulinemia (or agammaglobulinemia) was believed to result from a deficiency (or absence) of the antibody‐producing cells, or “B” cell line of the lymphoid system. With increased recognition of functional subpopulations of the lymphoid system, however, it has become apparent that a variety of mechanisms may result in the phenotype of hypogammaglobulinemia. Since these entities may be clinically indistinguishable, it is important to understand the various categories of disease which may be involved. In this concise review, these areas are briefly summarized, More complete reviews of the subject (including acquired forms of immunodeficiency) are available [2, 3].
Bone marrow lipids in normal and anemic mice
Authors: Potter Jane E. R. , Wright Eric G. 
Published: February 2007

AbstractThe lipids in the bone marrow of six strains of normal mice, and mice with genetically determined anemias (W/Wv and S1/S1d) and experimentally induced anemia were analyzed. Cholesterol and phospholipid accounted for 60–75% of total lipid; the remainder was triglyceride and neutral glyceryl ether lipids. In mice with phenylhydrazine (PHZ)‐induced hemolytic anemia there was a threefold increase in neutral glyceryl ether lipids immediately preceding a twofold increase in recognizable erythroid precursors.Comparisons were made between the femoral bone marrow and the cells maintained in the adherent populations of long‐term bone marrow cultures. In cultures of B6D2F1 bone marrow (which support long‐term proliferation and differentiation of hemopoietic stem cells) the adherent layers contained the same lipids as freshly aspirated bone marrow. In W/Wv and S1/S1d cultures (which duplicate, respectively, the stem cell and environmental defect found in vivo) the lipid content of the adherent layers differed from bone marrow, particularly in the absence of ether‐containing lipids.The results are consistent with the hypothesis that marrow lipids have an active (as opposed to passive) role in murine hemopoiesis and indicate that long‐term bone marrow culture may prove useful in further biochemical studies of the hemopoietic inductive microenvironment.
Lymphopenic response of patients presenting with chronic lymphocytic leukemia associated with carcinoma of the prostate to diethylstilbestrol: Correlation of response to the in vitro synthesis of rna by patient lymphocytes and its relationship to transcortin
Authors: Narasimhan Parthasarathy , Amaral Leonard 
Published: February 2007

AbstractThree patients with chronic lymphocytic leukemia (CLL) and carcinoma of the prostate were treated for the latter condition with diethylstilbestrol (DES). All three patients responded with rapid reductions in their peripheral white counts. The previously observed lymphocytic infiltration of the bone marrow of one patient was not evident after three months of DES treatment. The response of this patient to DES correlates well with the enhanced level of plasma transcortin subsequent to treatment. Treatment of this patient with DES alters the ability of peripheral lymphocytes to incorporate radioactive uridine into RNA as well as the in vitro response to either cortisol or β‐estradiol.
The first stable variant of erythrocyte glucose‐phosphate isomerase associated with severe hemolytic anemia
Authors: Zanella A. , Izzo C. , Rebulla P. , Perroni L. , Mariani M. , Canestri G. , Sansone G. , Sirchia G. 
Published: February 2007

AbstractA new variant of glucosephosphate isomerase (GPI) associated with hemolytic anemia, mental retardation, and muscular hypotonia is described. The defective enzyme showed increased affinity for fructose‐6‐phosphate (F‐6‐P), decreased affinity for glucose‐6‐phosphate (G‐6‐P), altered electrophoretic and isoelectrofocusing patterns, and shift to the left of the precipitin curve. The enzyme was stable under all the conditions tested (heat, urea, guanidine‐HCl, and storage). Optimum pH, Ki for 6‐phosphogluconic acid (6‐PGA) and for erythrose‐4‐phosphate (E‐4‐P), molecular weight, GPI‐related antigen concentration, immunodiffusion pattern, and immunoinactivation were in the normal range. This is the first example of the association of a stable mutant GPI with severe hemolytic anemia. Enzyme instability has been present in all previously reported cases.
Platelet interaction with bacteria. VI. Contrasting the role of fibrinogen and fibronectin
Authors: Clawson C. C. , White James G. , Herzberg Mark C. 
Published: February 2007

AbstractThe roles of fibrinogen and fibronectin were contasted in the responses of human platelets to Staphylococcus aureus and collagen. Congenital afibrinogenemic (CA) platelets and washed normal platelets had delayed aggregation due to a prolonged latent phase in response to contact with the bacteria when fibrinogen was absent from the suspending media. When a physiologic level of human fibrinogen was added to the CA platelets in autologous plasma or to the washed platelets in balanced saline, the times to aggregation were significantly shortened (P < 0.05) and restored to rates comparable to those of normal platelets in whole plasma. In contrast, the presence or absence of fibrinogen in the media had no significant effect on the initial adherence and shape‐change response of CA or washed platelets to collagen. Platelets with a demonstrated aberration of fibronectin responded normally to all ratios of S aureus, but they responded only to doses of collagen that were several times the threshold levels for normal platelets. These results demonstrate an important role for fibrinogen as a cofactor in platelet interactions with S aureus and indicate that fibronectin is not a major factor in the responses of human platelets to this microorganism.
31P NMR spectroscopy of erythrocytes in congenital hemolytic anemias: Detection of heterogeneous erythrocyte populations and quantification of intracellular 2,3‐diphosphoglycerate
Authors: Labotka Richard J. , Honig George R. 
Published: February 2007

AbstractPhosphorus‐31 nuclear magnetic resonance (NMR) spectra were determined from intact erythrocytes of a patient with homozygous β‐thalassemia and from three patients with pyruvate kinase (PK) deficiency.The intracellular 2,3‐diphosphoglycerate‐(2,3‐DPG) were mildly elevated in the thalassemia patient and in PK heterozygotes, and were markedly elevated in the PK‐deficient homozygotes. The 2,3‐DPG chemical shifts in the patients' NMR spectra were consistently positioned at a higher magnetic field than normal. When the patients with thalassemia or PK deficiency were transfused with blood from a normal donor, or when a patient's erythrocytes were mixed with normal erythrocytes, two distinct sets of 2,3‐DPG resonances appeared in the resulting spectra, allowing the simultaneous quantification of the 2,3‐DPG levels in each erythrocyte population. The ability of NMR to detect heterogeneous cell populations may be useful for the diagnosis of congenital hemolytic anemias in patients who have been transfused.
Infectious mononucleosis and acute lymphoblastic leukemia—hand mirror cells: A qualitative and quantitative ultrastructural study
Authors: Schumacher H. R. , Thomas W. J. , Creegan W. J. , Pitts L. L. 
Published: February 2007

AbstractThe hand mirror cell (HMC) in infectious mononucleosis (IM) is a T cell which appears at the time of improvement and apparently has an important role in immune surveillance. Fifty electron micrographs of HMC from an unusual untreated patient with ALL‐HMC variant were compared to 50 electron micrographs of HMC from five patients beginning to recover from IM. Quantitative differences between the IM‐HMC and ALL‐HMC were observed in multivariate analysis of variance (p < 0.001), nuclear perimeter (p < 0.0001), nuclear length (p < 0.0001), cytoplasmic length (p < 0.0001), total cell area (p < 0.0001), cytoplasmic area (p < 0.0001) and nuclear cytoplasmic ratio (p < 0.004), and numbers of mitochondria (p < 0.001). The qualitative differences included greater nuclear irregularity in the IM‐HMC, and prominent nucleolonema in some of the ALL‐HMC. In addition, the IM‐HMC showed more polyribosomes, parallel tubular arrays and microtubules, but much less glycogen. Since the IM‐HMC apparently enter effectively into immune surveillance, and some cases of ALL have HMC, the ultrastructural similarities and differences above contribute to a better understanding of the importance of this unique cell configuration in benign and malignant disorders.
Dysgranulopoietic neutropenia and abnormal monocytes in childhood vitamin B12 deficiency
Authors: Crist William M. , Parmley Richard T. , Holbrook C. Tate , Castleberry Robert P. , Denys Francis R. , Malluh Ahmad 
Published: February 2007

AbstractNeutropenia and/or leukopenia (associated with elevated serum lysozyme levels) in three children with vitamin B12 deficiency were evaluated using soft agar culture and ultrastructural and cytochemical techniques. In two patients a marked increase in peripheral blood myeloid colony forming cells (CFC) was observed; whereas a marginal increase in CFC was present in the third, less symptomatic, patient. Marrow CFC was normal or slightly increased. Serum colony stimulating activity (CSA) was normal but elaboration of CSA by white blood cells was low. Normal maturation of the progenitors was present in vitro and serum inhibitors of myelopoiesis were absent.Megaloblastic neutrophils and monocytes with nuclear‐cytoplasmic asynchrony were observed ultrastructurally in directly sampled marrow specimens. These cells contained autophagic and/or heterophagic vacuoles and an increase in cytoplasmic granules. Both monocytes and neutrophils also contained enlarged‐disrupted centrioles. Many marrow macrophages contained phagocytic vacuoles, which enclosed disrupted neutrophils and cellular debris.We conclude that although a large number of dysmorphic myeloid cells degenerate in the marrow, sufficient marrow CFC are present and peripheral blood CFC are elevated, suggesting abnormal release and/or maturation failure. All of these abnormalities are corrected by administration of vitamin B12.
A patient with apparent idiopathic A‐megakaryocytic thrombocytopenia
Authors: Kim Hyoung D. , Boggs Dane R. 
Published: February 2007

AbstractA 71‐year‐old woman developed life‐threatening thrombocytopenia which resolved (probably spontaneously), recurred, and then again resolved. The thrombocytopenia was characterized by selective aplasia of megakaryocytes, near‐normal platelet survival, and very slow recovery. No underlying disorder nor any drug could be incriminated, so we must assume this patient suffered from an idiopathic and very rare form of thrombocytopenia.
Diurnal changes in circulating myeloid progenitor cells in man
Authors: Verma Dharmvir S. , Fisher Richard , Spitzer Gary , Zander Axel R. , McCredie Kenneth B. , Dicke Karel A. 
Published: February 2007

AbstractTo examine the diurnal changes in circulating myeloid progenitor cells (CFU‐Cs), 15 normal human volunteers were studied. Total and differential peripheral blood leukocyte counts and CFU‐C levels, using the in vitro agar culture method, were obtained at 8 AM, 11 AM, 3 PM, and 8 AM the following morning (in nine subjects) or only at 8 AM and 3 PM (in six subjects). On the average, an approximate twofold rise in circulating CFU‐C levels (per ml of blood) was found at 3 PM as compared to the 8 AM levels (P 0.002), and a smaller rise at 11 AM was found than at 8 AM levels (P 0.03). The ratios of CFU‐C per ml of blood to mononuclear cells per ml of blood and neutrophils per ml of blood, respectively, were significantly higher at 3 PM than at 8 AM (P 0.002 and 0.004, respectively). The rise in CFU‐C levels is usually significantly higher when compared to that in peripheral leukocyte counts.
Chronic idiopathic neutropenia: Granulopoietic assessment by both marrow culture and granulocyte kinetics
Authors: Rickard K. A. , Brown R. D. , Yuen E. , Kronenberg H. 
Published: February 2007

AbstractGranulopoietic assessment was made in 14 patients with chronic idiopathic neutropenia (CIN) whose neutrophils were consistently less than 1.5 × 109/ liter and in whom there was absence of splenomegaly and recent drug ingestion. Granulopoiesis was studied using a combination of bone marrow culture in nutrient agar and granulocyte kinetics. Agar colony growth assessed bone marrow concentration of granulocyte progenitor cells (GPC) and the proportion of GPC in DNA synthesis by in vitro 3HTdR suicide. Granulocyte kinetics with in vitro DF32P labelling of patient granulocytes measured granulocyte half‐life (T1/2), turnover rate (GTR), and the circulating, marginated, and total blood granulocyte pools. The results indicated that either GPC concentration or the proportion of GPC in DNA synthesis was outside the normal range in all but one patient and decreased in ten out of 14 patients. CIN was also characterized by reduced total, circulating, and marginated blood granulocyte pools, reduced GTR, and normal granulocyte half‐life. The neutropenia appeared to be due to a variety of intra‐marrow causes, including either a reduction in the GPC compartment, a reduction in GPC proliferation, a maturation arrest, or a reduced amplication during granulopoiesis. Increased granulocyte utilization, intra‐vascular destruction or excessive margination could be excluded as possible causes of CIN in this series. Although GPC parameters tended to be reduced, suggesting a production defect, there were signs in nine patients that the bone marrow was attempting to compensate for the peripheral neutropenia. It is suggested that for a complete assessment of granulopoiesis in man, granulocyte kinetic studies need to be combined with quantitative studies of the bone marrow granulocyte progenitor compartment using the agar colony system.
Successful doxorubicin therapy of primary macroglobulinemia resistant to alkylating agents
Authors: Clamon Gerald H. , Corder Michael P. , Burns C. Patrick 
Published: February 2007

AbstractTwo patients with macroglobulinemia refractory to alkylating agent therapy did have an objective response to therapy with doxorubicin. For patients refractory to alkylating agents, doxorubicin is an alternative to other alkylating agents or procarbazine.
Recent advances in the quantitation of human fetal hemoglobins with different gamma chains
Authors: Huisman T. H. J. , Wilson J. B. 
Published: February 2007

AbstractThree types of γ chain of human hemoglobin (Hb) F have been discovered. The γ and Aγ chains differ at position 136 (glycine in Gγ; alanine in Aγ) and are the products of nonallelic genes. The Tγ chain is found in rather high frequency in certain world populations, and is characterized by a threonyl residue at position 75 replacing the commonly occurring isoleucyl residue. Convincing evidence is present that the Tγ chain is the product of an allele of the Aγ chain gene, and should be renamed AγT chain. Thus, the three γ chains are GγI, AγI, and AγT.Until recently, the presence and the quantity of these chains were determined by chemical analyses of the tryptic peptides of the isolated Hb F. These procedures involve chromatographic separations or fingerprinting methods. The introduction of high pressure liquid chromatography (HPLC) has greatly facilitated the separation of these peptides. Since minute quantities are required, the latter method is ideally suited for the identification of the Hb F produced in in vitro cell colonies.Electrophoretic and chromatographic (HPLC) methods have been developed which make it possible to separate the Gγ and Aγ chains in small amounts of Hb F (less than 0.5 mg). These procedures allow accurate quantitation of the Gγ/Aγ ratio in the Hb F and may also be suitable for in vitro biosynthetic analyses. One of the HPLC procedures has been modified so that the three types of γ chain can be quantitated in one single chromatographic experiment. Examples of separations are provided, details of some of the methods are discussed, and their applicability illustrated.
Fibrinogen houston: A dysfibrinogen exhibiting defective fibrin monomer aggregation and α‐chain cross‐linkages
Authors: Weinger Ronald S. , Rudy Christine , Moake Joel L. , Conlon Charles L. , Cimo Philip L. 
Published: February 2007

AbstractA 38‐year‐old male patient with a life‐long history of easy bruising and mild bleeding had a prolonged activated partial thromboplastin time (APTT), prothrombin time (PT), and thrombin time (TT). Reptilase (Bathrops atrox) clotting time was normal. His undiluted and diluted plasma prolonged the APTT, PT, and TT of normal plasma. Fibrin produced from patient plasma was insoluble in 5 M urea. Plasma fibrinogen level was increased when measured as clottable protein and by Laurell electroimmunoassay. Specific assays of plasma factors II, V, VII, X, VIII, IX, XI, and XII were normal. A circulating antithrombin in patient plasma was excluded by demonstrating normal thrombin‐induced platelet aggregation of gel‐separated platelets in the presence of patient plasma. Purified patient fibrinogen reproduced the anticoagulant effect of patient plasma. Patient fibrinogen antigen was similar to normal fibrinogen antigen by immunodiffusion, immunoelectrophoresis (pH 5.2 and 8.6), and crossed immunoelectrophoresis. His unreduced purified fibrinogen had normal migration on polyacrylamide slab gels. Also, the migration in gel slabs of Aα, Bβ and γ‐polypeptide chains, produced by mercaptoethanol reduction of purified patient fibrinogen, was similar to reduced normal fibrinogen. Thrombin‐induced total fibrinopeptide release was normal. However, fibrin monomers produced from patient fibrinogen by thrombin (devoid of fibrinopeptides A and B) reaggregated abnormally; fibrin monomers produced by reptilase (devoid of only fibrinopeptides A) reaggregated normally. Fibrin generated from patient plasma in the presence of factor XIII and calcium, was defective in the formation of covalently bonded α‐α polymers and demonstrated an increased susceptibility to the lytic effects of plasmin (generated in vitro by the addition of streptokinase).
The pennsylvania hemophilia program 1973–1978
Authors: Eyster M. Elaine , Lewis Jessica H. , Shapiro Sandor S. , Gill Frances , Kajani Mehdi , Prager David , Djerassi Isaac , Rice Samuel , Lusch Charles , Keller Anne 
Published: February 2007

AbstractIn Pennsylvania, the prevalence of hemophilia is one per 10,000 males. Factor VIII deficiency is five times more frequent than Factor IX deficiency, and 34% of the patients have no relatives affected with the disease. The mean age is 23 years old, and 50% of the patients are less than 20 years old. Approximately one‐third of the patients with Factor VIII deficiency and one fourth of the patients with Factor IX deficiency have levels of < 0.01 μ/ml. By clinical criteria, 55% of those with Factor VIII deficiency are severe compared to 45% of those with Factor IX deficiency. Factor VIII‐deficient patients are treated an average of 18 times per year compared to ten times per year for patients with Factor IX deficiency. Hemarthroses account for 70% of the hemorrhages treated and for 40% of the concentrate usage. Home therapy patients use an average of 45,950 Factor VIII units per year at a cost of $4,170 per patient and their use accounts for 60% of the total Factor VIII usage of 1.7 million units. Less than five days per patient per year are lost from school or work because of bleeding, and patients are hospitalized for bleeding an average of only two to three days per patient per year. Adverse immediate reactions to therapy are infrequent. Five percent of patients have persistence of the hepatitis B virus, and 7.5% have inhibitors. The mortality rate is 0.04% per year, with half of the deaths being hemophilia‐related.
Terminal deoxynucleotidyl transferase in the blastic phase of chronic myelogenous leukemia: An indicator of response to vincristine and prednisone therapy
Authors: Tanaka Masao , Kaneda Tsuguhiro , Hirota Yutaka , Yoshida Shonen , Kitajima Koichi 
Published: February 2007

AbstractTerminal deoxynucleotidyl transferase (TdT) activity was assayed in leukemic cells of 34 patients in blastic phase of Philadelphia‐positive chronic myelogenous leukemia (CML). The TdT levels were correlated with the response to vincristine and prednisone therapy. None of 20 TdT‐negative patients, but 10 of 14 TdT‐positive patients responded to vincristine and prednisone. There was poor correlation between blast cell morphology and response to this therapy. This study confirms that TdT levels in the majority of patients predict effectiveness of vincristine and prednisone therapy in blastic phase of CML.