bluuebunny/crossref_metadata_2025_split huggingface dataset:
Monocyte-mediated antibody-dependent cellular cytotoxicity: a clinical test of monocyte function
Authors: Poplack DG , Bonnard GD , Holiman BJ , Blaese RM 
Published: October 2019

Abstract
The lack of a simple, rapid, and quantitative test of the functional activity of the monocyte has hampered studies of the contribution of this cell type to host defense and human disease. This report describes an assay of antibody-dependent cellular cytotoxicity, which depends exclusively upon the monocyte as the effector cell and therefore provides a convenient test of monocyte function. In this system, mononuclear leukocytes (MNL) obtained by Ficoll-Hypaque separation of whole blood are cytotoxic for 51Cr-labeled human erythrocyte targets coated with anti-blood group antibody. Removal of phagocytic monocytes from the MNL by iron ingestion, followed by exposure to a magnetic field, completely abolishes all cytotoxic activity from the remaining MNL population. Similarly, in severely mono-cytopenic patients with aplastic anemia, cytotoxic effector activity is absent. In normals and less severely monocytopenic aplastic anemia patients, cytotoxicity correlates significantly (p less than 0.001) with monocyte number. Application of this monocyte-mediated antibody-dependent cellular cytotoxicity assay to the study of patients with the Wiskott-Aldrich syndrome has revealed defective monocyte cytotoxic activity in spite of normal monocyte numbers, suggesting that this test may be useful for the assessment of monocyte function in a variety of clinical situations.
Stabilization of the shape of sickled cells by calcium and A23187
Authors: Clark MR , Greenquist AC , Shohet SB 
Published: October 2019

Abstract
Evaluation of the role of calcium in irreversible sickling has been approached by treating sickled cells with calcium and the ionophore A23187. A calcium-dependent stabilization of the sickled cell shape was observed after reoxygenation of cells in the presence of ionophore. At low calcium concentrations, this retention of sickled shape was maintained for periods up to 1 hr. However, the morphology of the oxygen-stable sickled cells was like that of deoxygenated sickle cells and significantly different from the characteristic morphology of native irreversibly sickled cells (ISCs). Because the stabilized cells did not fulfill the morphological criterion for ISCs, the shape- stabilizing effect of calcium in this system did not provide additional support for the hypothesis that calcium accumulation was the determining factor in ISC generation.
Bone marrow cellularity determination: comparison of the biopsy, aspirate, and buffy coat
Authors: Gruppo RA , Lampkin BC , Granger S 
Published: October 2019

Abstract
Bone marrow biopsies (244) performed with a Jamshidi needle were evaluated in 53 children with leukemia or aplastic anemia. Adequate specimens were obtained in 85%. Results of cellularity estimated by biopsy were compared to the cellularity of the aspirate versus volumetric determination of the myeloid-erythroid layer (buffy coat). A wide discrepancy was noted between marrow cellularity confirmed by biopsy versus the aspirate or buffy coat. The greatest variance was seen in the hypercellular or normocellular marrows, as estimated by biopsy, in which 39% were misinterpreted as moderately or severely hypocellular by aspirate. Volumetric measurement of buffy coat was least acceptable for estimating cellularity. Thus the biopsy has proved to be an important and reliable indicator of bone marrow cellularity.
Changes in hemopoiesis during the course of acute LCM virus infection in mice
Authors: Bro-Jorgensen K , Knudtzon S 
Published: October 2019

Abstract
Although severe hematologic and immunologic disorders occur in several viral infections, insight into the mechanisms by which viruses may affect hemopoietic tissues is poor. The previous demonstration of distinct immunohemopoietic lesions in mice with acute lymphocytic choriomeningitis (LCM) virus infection has led us to investigate the function of hemopoietic precursor cells in the course of this experimental infection. During the first week of infection, there was profound suppression of pluripotential stem cell (CFU) and in vitro colony-forming cell (CFU) compartments, and of 59Fe uptake into hemopoietic tissues. During the same period, we found enhanced activity of colony-stimulating factor, lack of responsiveness to erythropoietin, and appreciable titers of interferon in blood and spleen. After day 10 post infection, there was a striking increase in CFU and 59Fe uptake confined to spleen and blood. Restoration of bone marrow, however, was markedly delayed. With reference to recent studies on interferon, and the findings in mice with persistent LCM virus infection, we suggest that interferon may be the comprehensive suppressor of the hemopoietic precursor cells in the first stage of acute LCM virus infection, and that these cells in the recovery period are directed preferentially into erythropoiesis.
Relationship between the anticoagulant and antithrombotic effects of heparin in experimental venous thrombosis
Authors: Chiu HM , Hirsh J , Yung WL , Regoeczi E , Gent M 
Published: October 2019

Abstract
The relationship between the antithrombotic and anticoagulant effects of heparin was assessed using venous thrombi in rabbits. Accretion of 125I-fibrinogen onto jugular vein thrombi was used to assess the antithrombotic effect of heparin, and the protamine sulfate titration test (heparin activity) and the activated partial thromboplastin time (APTT) were used to measure its anticoagulant effect. The effect of heparin on jugular vein bleeding times was also measured in a separate group of animals. Fibrinogen accretion was significantly lower with continuous infusion than with intermittent injection. Heparin, given by continuous infusion, produced marked inhibition of fibrinogen accretion (to less than 10% of control accretion) at an APTT value of between 75 and 80 sec (control 34 sec) and at a level of heparin activity of 0.4- 0.5 U/ml. Infusion of cryoprecipitate reduced the effect of heparin on the APTT relative to its effect on heparin activity. In these cryoprecipitate-treated animals, marked inhibition of fibrinogen accretion occurred at a similar level of heparin activity (0.4–0.6 U/ml) but at a significantly lower APTT (35-50 sec) than in normal animals. On the other hand, there was a progressive increase in jugular vein bleeding time with both increasing APTT values and heparin levels in both groups of animals.
Induction of thrombocytopenia by thrombopheresis in man: patterns of recovery in normal subjects during ethanol ingestion and abstinence
Authors: Sullivan LW , Adams WH , Liu YK 
Published: October 2019

Abstract
Using the technique of thrombophoresis (TP), platelet and megakaryocyte dynamics following acute thrombocytopenia were studied in two normal subjects during periods of ethanol ingestion and abstinence. Thrombocytopenia was induced over a period of 12 hr. A logarithmic decline in platelet count during TP and the serial morphologic changes in megakaryocytes during recovery from thrombocytopenia are described. Although these parameters were not affected by ethanol ingestion, platelet counts after TP did not return to normal until ethanol was discontinued. 51Cr-labeled platelet survival was normal in one subject studied, and no evidence of increased platelet sequestration was found. It is concluded that heavy ethanol ingestion induces, augments, or sustains thrombocytopenia by impairing megakaryocytopoiesis in man. The mechanism by which ethanol induces thrombocytopenia may be due, in part, to “ineffective thrombopoiesis,” impairment of the differentiation of precursor cells into the megakaryocytic compartment, or a combination of these factors.
G-6-PD Long Prairie: a new glucose-6-phosphate dehydrogenase mutant exhibiting normal sensitivity to inhibition by NADPH and accompanied by nonspherocytic hemolytic anemia
Authors: Johnson GJ , Kaplan ME , Beutler E 
Published: October 2019

Abstract
The enzymatic properties of a new glucose-6-phosphate dehydrogenase (G- 6-PD) variant (G-6-PD Long Prairie) were studied in a white patient with chronic nonspherocytic hemolysis. The red cells were found to have 2.3%-7.7% normal enzymatic activity. The mutant enzyme exhibited marked heat instability, an increased pH optimum, a moderately decreased Km for G-6-P, and increased utilization of 2-deoxyglucose-6-phosphate and deamino NADP. The Km for NADP and Ki for NADPH were both normal. G-6-PD Long Prairie is an interesting new G-6-PD variant that demonstrates that chronic hemolysis can be associated with modestly decreased G-6-PD activity despite normal sensitivity to inhibition by NADPH. Although increased sensitivity to inhibition by NADPH has been postulated to decrease intracellular enzyme activity, resulting in enhanced susceptibility to hemolysis in certain G-6-PD variants with only moderately decreased enzymatic activity, an alternative mechanism of hemolysis, possibly enzyme thermolability, exists in G-6-PD Long Prairie.
Blood volume in monoclonal gammopathy
Authors: Alexanian R 
Published: October 2019

Abstract
The plasma volume, red cell volume, or both were measured in 170 normal, anemic, or polycythemic subjects. For anemic subjects without a serum protein abnormality or splenomegaly, the relationship between hematocrit and red cell volume was linear and predictable. In patients with a serum monoclonal globulin on electrophoresis, the plasma voluem was significantly increased for the hematocrit in 30%, and the total blood volume was increased in 45%. The frequency of an elevated plasma volume was higher in patients with a markedly increased level of monoclonal protein. Reductions of abnormal proteins with chemotherapy were associated with declines in plasma volume. For a specific concentration, the serum viscosity was highest in patients with IgM proteins and lowest in patients with IgG globulins. Marked elevations in viscosity were noted only in sera with macroglobulinemia or with more than 5 g/dl of IgG or IgA globulins.
Changing erythrocyte populations in juvenile chronic myelocytic leukemia: evidence for disordered regulation
Authors: Dover GJ , Boyer SH , Zinkham WH , Kazazian HH Jr , Pinney DJ , Sigler A 
Published: October 2019

Abstract
Fetal and adult erythrocyte characteristics were studied serially in a 30-mo-old female with juvenile chronic myelocytic leukemia. On presentation the erythrocytes exhibited predominantly fetal characteristics as indicated by 69% hemoglobin F (HbF), 1.1% hemoglobin A2 (HbA2), absent I antigen, and fetal levels of the erythrocyte enzymes, carbonic anhydrase I and II, glucose-6-phosphate dehydrogenase, hexokinase, pyruvate kinase, and lactate dehydrogenase; 100% of the erythrocytes present contained HbF. However, Orskov-Jacobs- Stewart hemolysis demonstrated that at least one adult characteristic was present. Seven months later HbF was 17%; I antigen and carbonic anhydrase I had increased to adult levels. The number of cells containing HbF had decreased to 30%. Further studies indicated that at least three new populations of red cells were present after 7 mo which had not previously been detected. Two of these populations exhibited a mixture of both fetal and adult characteristics. Such findings suggested that an ongoing disturbance of regulatory mechanisms was responsible for the variable expression of fetal versus adult erythrocyte characteristics.
Multiple myeloma: quantitative staging and assessment of response with a programmable pocket calculator
Authors: Salmon SE , Wampler SB 
Published: October 2019

Abstract
Studies of M-component synthesis and metabolism, as well as clinical staging of multiple myeloma, have provided the basis for calculation of the total body tumor cell number and quantitation of response to treatment. Many of the calculations involved have been complex and have previously required the availability of a computer. The advent of programmable pocket calculators has permitted simplification of these programs for general clinical and investigative applications in studies of myeloma and related monoclonal gammopathies. In this paper we have included the background information and a clinical example of the use of the programmable calculator, as well as the logic steps and the calculator programs themselves, for several of the most useful programs for clinical staging and quantitation of response to therapy in IgG myeloma.
Proliferation of diffusion chamber colony-forming units (CFUD) in cultures of normal human bone marrow in diffusion chambers in mice
Authors: Jacobsen N 
Published: October 2019

Abstract
Normal human bone marrow contains cells capable of forming colonies of hemopoietic cells in fibrin clots in diffusion chambers implanted intraperitoneally (i.p.) into irradiated mice. The present paper describes the proliferation of such colony-forming units (CFUD) in cultures in vivo. Cells harvested from diffusion chambers after 1–14 days of culture in 450-R irradiated mice contained CFUD, which formed neutrophilic, eosinophilic, or megakaryocytic colonies when tested by secondary culture in fibrin clot chambers. When bone marrow was precultured in irradiated mice at a concentration of 10(6) cells per chamber, an initial fall in the number of neutrophilic CFUD was observed. This decrease was followed by an increase to a maximum at day 2, and then a secondary decrease. The number of neutrophilic CFUD recovered after 2 days of preculture in irradiated mice varied between 60% and 250% of the number present before preculture. Preculture in nonirradiated mice resulted in a significantly lower recovery of neutrophilic CFUD. In vitro treatment of bone marrow cells with hydroxyurea (OHU) after 2 days of preculture in irradiated mice resulted in a 68% +/- 5% reduction in the number of neutrophilic CFUD. In contrast, OHU had no similar effect on precultures from nonirradiated mice. Both the recovery and sensitivity to OHU of eosinophilic CFUD were independent of host irradiation. Similarly, no effect of host irradiation on the recovery or the 3H-thymidine (3HTdR) labeling index of morphologically recognizable granulocytic cells was observed at day 2. The data suggest an effect of humoral host factor(s) on the proliferation of early precursor cells, which are or become committed to differentiate into the neutrophilic pathway in diffusion chambers.
Intraerythrocytic adaptation (2,3 DPG,P50) in thalassemia minor
Authors: Pearson HA , Motoyama E , Genel M , Kramer M , Zigas CJ 
Published: October 2019

Abstract
P50 and 2,3 DPG content of erythrocytes were determined in 25 patients with heterozygous beta thalassemia minor to assess the adaptive mechanisms to anemia. 2,3 DPG levels were appropriately elevated for the degree of anemia. However, P50 values were not proportionately increased. No correlations were noted between hemoglobin level, 2,3 DPG, or P50 and the presence of symptomatic complaints of fatigue or weakness in these heterozygous patients.
Effects of ascorbic acid on glucose-6-phosphate dehydrogenase-deficient erythrocytes: studies in an animal model
Authors: Udomratn T , Steinberg MH , Campbell GD Jr , Oelshlegel FJ Jr 
Published: October 2019

Abstract
There is little information available concerning the possible effects of ascorbic acid on glucose-6-phosphate dehydrogenase (G-6-PD) - deficient erythrocytes. We therefore studied the survival of G-6-PD- deficient cells, incubated with ascorbic acid, in rats with partial reticuloendothelial and complement blockade. Animals were pretreated with ethyl palmitate and cobra venom factor. They were then transfused with 51Cr-labeled erythrocytes which had been incubated in the presence or absence of ascorbic acid. G-6-PD-deficient cells, incubated with ascorbic acid, had impaired survival when compared to that of controls. G-6-PD-deficient cells, in the absence of ascorbic acid, had survival times equal to those of normal control erythrocytes. At the concentrations used, ascorbic acid did not appear to alter the survival of normal cells. Ascorbic acid causes premature loss of G-6-PD- deficient human erythrocytes in this rat model. On the basis of previous clinical and experimental observations, as well as this study, caution should be used when administering ascorbic acid in large doses to individuals who might be G-6-PD deficient.
Techniques for demonstration of the specificity of circulating anticoagulants against antihemophilic factor (factor VIII), with studies of two cases possibly related to diphenylhydantoin therapy
Authors: Poon MC , Saito H , Ratnoff OD , Forman WB , Wisnieski J 
Published: October 2019

Abstract
Circulating anticoagulants against antihemophilic factor (AHF, factor VIII) sometimes seem to inactivate other clotting factors as well. Measurements of the concentration of clotting factors in highly diluted plasma, or after neutralization of the anticoagulant with purified AHF, have demonstrated the specific nature of the anticoagulant in a patient under treatment with diphenylhydantoin. A second case in a patient treated with this agent, and with penicillin, an agent previously associated with the evolution of circulating anticoagulants, is also described.
Blood viscosity in Waldenstrom macroglobulinemia
Authors: MacKenzie MR , Lee TK 
Published: October 2019

Abstract
Patients with Waldenstrom macroglobulinemia were studied for the presence or absence of the hyperviscosity syndrome, the relative serum viscosity value, and the calculated whole blood viscosity to identify a level at which symptoms occurred. The majority of symptomatic patients had whole blood viscosity values above 8.0 centipoises. There was a direct correlation between whole blood viscosity and relative serum viscosity, r = 0.75. One patient with central nervous system abnormalities was identified as having a high whole blood viscosity but a low serum viscosity. It was concluded that the vast majority of patients with the hyperviscosity syndrome will be identified by measuring the relative serum viscosity. In patients with central nervous system findings and a low serum viscosity, the whole blood viscosity should be determined either by direct measurement or by calculation.
One hundred patients with acute leukemia treated by chemotherapy, total body irradiation, and allogeneic marrow transplantation
Authors: Thomas ED , Buckner CD , Banaji M , Clift RA , Fefer A , Flournoy N , Goodell BW , Hickman RO , Lerner KG , Neiman PE , Sale GE , Sanders JE , Singer J , Stevens M , Storb R , Weiden PL 
Published: October 2019

Abstract
One hundred patients, 54 with acute myelogenous leukemia (AML) and 46 with acute lymphoblastic leukemia (ALL), considered to be in the end stages of their disease, after combination chemotherapy were treated by marrow transplantation. All patients were given a marrow graft from an HLA-identical sibling after receiving 1000-rad total body irradiation (TBI). One group of 43 patients was given cyclophosphamide (CY), 60 mg/kg on each of 2 days, 5 and 4 days before TBI. In a second group of 31 patients, additional chemotherapy was given before CY and TBI. In a third group of 19 patients, BCNU was given before CY and TBI. A fourth group of 7 patients received other chemotherapy regimens before TBI. Six patients died 3–17 days after marrow infusion without evidence of engraftment. Ninety-four patients were engrafted and only one patient rejected the graft. Thirteen patients are alive with a marrow graft, on no maintenance antileukemic therapy, and without recurrent leukemia 1–4 1/2 yr after transplantation. Three have chronic graft-versus-host disease (GVHD). Four patients are alive 1 1/2 - 3 1/2 yr after grafting but have had a relapse of their leukemia. Of 93 evaluable patients, 19 did not develop GVHD and 24 developed very mild GVHD. Fifty patients developed moderate to severe GVHD, and 40 of these were treated with antithymocyte globulin. Interstitial pneumonia occurred in 54 patients and was the primary cause of death in 34. Interstitial pneumonia often occurred in association with GVHD and the most common etiologic agent was cytomegalovirus. A total of 31 patients have had a relapse of leukemia. There was no definite correlation between relapse of leukemia and the presence or absence of GVHD. The relapse rate appeared to be relatively constant over the first 2 yr and was extremely low after that time. Neither survival nor leukemic relapse appeared to be influenced by the type of leukemia nor by the preparative chemotherapy regimen given before TBI. Patients in fair clinical condition at the time of transplantation showed significantly longer survival times than patients in poor condition (p = 0.001). This observation, coupled with the observation that some patients may be cured of their disease, indicates that marrow transplantation should now be undertaken earlier in the management of patients with acute leukemia who have an HLA- matched sibling marrow donor.
Ph1-positive childhood leukemias: spectrum of lymphoid-myeloid expressions
Authors: Forman EN , Padre-Mendoza T , Smith PS , Barker BE , Farnes P 
Published: October 2019

Abstract
Two children presented with Ph1-positive leukemia, confirmed by Giemsa banding as 22q-. One child showed an initial presentation characteristic of acute lymphoblastic leukemia, followed by development of chronic myelocytic leukemia 2 yr later. A second child presented in blast crisis. Both patients showed blast cells possessing both lymphoid and myeloid characteristics, as demonstrated by histochemical, biochemical, or surface receptor properties of each cell series. The evidence provided supports the assumption of mixed lymphoid-myeloid properties of blast cells in chronic myelocytic leukemia in children. Detailed study of the leukemic cells may aid in the understanding of complex stem cell relationships and suggest more effective therapeutic approaches.
Systemic mastocytosis in a patient with polycythemia vera treated with radioactive phosphorus
Authors: Eagan JW Jr , Baughman KL , Miller S , Conley CL , Eggleston JC 
Published: October 2019

Abstract
Systemic mastocytosis occurred as a fatal event in a patient with long- standing polycythemia vera. The patient had been treated over the course of 21 yr with radioactive phosphorus. Possible relationships between mastocytosis and polycythemia vera, and also between mastocytosis and treatment with ionizing radiation, are discussed. Histopathologic and electron microscopic findings are illustrated. Difficulties in establishing the diagnosis of mast cell disease in this setting are also described.
Evidence for differentiation of human leukemic blood cells in diffusion chamber culture
Authors: Hoelzer D , Kurrle E , Schmucker H , Harriss EB 
Published: October 2019

Abstract
Peripheral blood cells of 21 patients with different forms of acute leukemia were cultured in diffusion chambers (5 x 10(5) cells/chamber) implanted intraperitoneally in 650 R preirradiated host mice over a period of up to 21 days. In patients with acute myeloid leukemia (AML), acute erythroleukemia (AEL), or acute myelomonocytic leukemia (AMMoL), the total number of cells which developed during this culture period exceeded the implanted value and also the values for normal peripheral blood cells from ten controls. In acute undifferentiated leukemia (AUL), two out of six patients showed considerable growth whereas the others, and also two patients with acute lymphoid leukemia (ALL), had poor growth. Differential counts revealed that the rise in total cells was due mainly to proliferation of blast cells and formation of granulopoietic cells. The latter exceeded the numbers from normal peripheral blood cells in 9 out of 13 patients with AML, AEL, or AMMoL and in 2 out of 6 patients with ALL. The production of granulopoiesis was not restricted to proliferating cells, but included mature cells which were of abnormal morphology in some cases. From the amount of granulopoiesis and the time of its development it was assumed that they were at least partly derived from leukemic blast cells. Chromosome analyses to decide whether the granulopoietic cells were of leukemic or normal cell origin are in progress.