bluuebunny/crossref_metadata_2025_split huggingface dataset:
Cell surface antigens: prognostic implications in childhood acute lymphoblastic leukemia
Authors: Sallan SE , Ritz J , Pesando J , Gelber R , O'Brien C , Hitchcock S , Coral F , Schlossman SF 
Published: October 2019

Abstract
Lymphoblasts from 93 children with acute lymphoblastic leukemia (ALL) were characterized by immunologic cell surface markers. These patients were treated on a single protocol, featuring adriamycin therapy during remission, and have been followed from 2 to 6.5 yr (median 4 yr). Three classes of patients were defined serologically: HTA+ Ia- CALLA-, Ia+ CALLA+ HTA-, and Ia+ CALLA- HTA-. Disease-free survival and sites of relapse were assessed within immunologic subsets. Similar to the findings of others, T-cell (HTA+ Ia-) patients fared poorly as compared to non-T-cell (Ia+ HTA-) patients (median disease-free survival was 12 and 47 mo. respectively; p = 0.0004). The majority of relapses in the HTA+ patients occurred at extramedullary sites. Late testicular relapse was rare among Ia+ patients. In addition, the “common ALL antigen” (CALLA) may identify a relatively favorable subset within the Ia+ population. The prognostic value of the immunologic markers was compared with traditional clinical factors. There was much overlap between HTA+, older age, and elevated WBC. However, neither age nor WBC alone were of prognostic significance among the Ia+ patients. We conclude that surface markers define both biologic and prognostic characteristics. The course of childhood ALL must be viewed in the context of homogeneous subsets and within particular therapeutic programs.
The inhibition of high and low molecular weight urokinase in plasma
Authors: Murano G , Aronson D , Williams L , Brown L 
Published: October 2019

Abstract
The rates of inhibition of high molecular weight (HMW) and low molecular weight (LMW) urokinase (UK) incubated in plasma or with purified antithrombin III (AT-III) were compared. Using a fibrinolytic assay system to determine residual biologic activity, polyacrylamide gel electrophoresis to demonstrate the formation of complexes, and selective immunoprecipitation techniques to identify the plasma inhibitors participating in the neutralization process, it was established that: (A) HMW-UK is inhibited more rapidly than LMW-UK, both in plasma and with purified AT-III; (B) heparin (3--10 U/ml accelerates the neutralization process in both systems, but only slightly; and (C) in plasma, several inhibitors, alpha 2-macroglobulin, alpha 1-antitrypsin, and antithrombin III, neutralize the activity of HMW-UK and LMW-UK.
Mapping the alpha-globin genes in an Algerian HbH patient and his family
Authors: Whitelaw E , Pagnier J , Verdier G , Henni T , Godet J , Williamson R 
Published: October 2019

Abstract
The organization of alpha-globin genes in normal white European, normal Algerian, and alpha-thalassemic Algerian DNA was examined by restriction endonuclease mapping using HindIII, HpaI, Bamhi, EcoRI, BgIII, and PstI. The results for normal DNA confirm and add to the findings of Surrey et al. and Orkin; the two alpha-genes are approximately 3.0 kb apart. The restriction enzymes BgIII and HpaL cut between the two alpha-genes. Four PstL sites are located: two surrounding each alpha-gene. The physical maps for a number of Algerian controls (normal alpha- and beta-globin biosynthesis profiles) are identical to that of the European controls. The Algerian alpha- thalassemic presenting with HbH disease was found to be homozygous for a 3.5–3.7 kb deletion at the alpha-gene locus, leaving one alpha-gene per chromosome. The patient's mother and father are both found to be heterozygous for this deletion. An unaffected sibling carries both normal chromosomes. The deletion could be the result of a Lepore-like crossover fusion event between the two alpha-globin genes, or of a 3.7 kb deletion of either the entire 5′ alpha-gene or the entire 3′ alpha- gene. The Algerian case of HbH disease studied differs from Asian cases in both the mode of inheritance and the molecular pathology of the alpha-thalassemia mutation. If this type of deletion is the major cause of Algerian alpha-thalassemia, it would explain the apparent absence of Hydrops fetalis in this geographical area.
Glycerol-3-phosphate dehydrogenase activity in the red cells of patients with thalassemia
Authors: Fessas P , Anagnou NP , Loukopoulos D 
Published: October 2019

Abstract
L-alpha-Glycerol-3-phosphate dehydrogenase (EC 1.1.1.8) has been reported to be absent in the erythrocytes of normal adults, but can be found in those of cord blood and of thalassemia major. The aid of this study was to investigate whether there is any relation between GDH and gamma-chain synthesis. Erythrocyte GDH activity was determined on 118 different blood samples. It was undetectable in normal adult erythrocytes and definitely high in cord blood cells (23.6 UI/10(11) RBC). Considerable GDH activity was also noted in patients with thalassemia major (11.0 IU10(11) RBC) as well as in cases with pronounced reticulocytosis (11.4 IU/10(11) RBC). Red cells from beta- thalassemia heterozygotes exhibited moderate but distinct GDH activity (5.2 IU/10(11) RBC). After fractionation into young and old erythrocyte populations, clearly higher GDH activity was found in the younger cells; however, there was no significant correlation with the reticulocyte count. Presence of reticulocytes alone appears insufficient to explain the values obtained in cord blood and the thalassemias, especially heterozygous. Furthermore, no direct correlation between GDH and fetal hemoglobin (HbF) was obtained in cord and thalassemic erythrocytes.
Defective opsonization in multiple myeloma
Authors: Cheson BD , Plass RR , Rothstein G 
Published: October 2019

Abstract
The mechanisms responsible for the unusual susceptibility of multiple myeloma (MM) patients to infections are incompletely defined. Since MM is associated with decreased production of normal serum proteins, we investigated the possibility that the production of opsonins might also be impaired. The neutrophil chemiluminescence assay of opsonization was used to evaluate the ability of serum from patients with MM to opsonize zymosan. It was found that sera from 18 MM patients exerted only 50% +/- 2.5% (mean +/- SEM) of the opsonic activity found in 18 control sera (p less than 0.001). In mixture experiments, untreated normal serum completely restored the opsonic activity of MM serum, suggesting a deficiency of opsonic factors rather than an inhibitor. In other mixture experiments, heat-inactivated normal serum only partially corrected the opsonic defect in MM serum. Serum from three patients had low C3 levels, and treatment of particles with these resulted in a greater opsonic defect than the patient population as a whole (p less than 0.02). No correlation between the opsonic defect and infections was established over an 18-mo period. These data suggest that MM serum lacks both heat-stable and heat-labile opsonic activity, the direct clinical significance of which remains to be clarified. However, these studies support the concept that defective host resistance in MM may be multifactoral, combining opsonic abnormalities with other defects previously described.
Protein kinases in chronic lymphocytic leukemia
Authors: Samuel E , Chung C , Scher N , Rosenzweig B , Silber R 
Published: October 2019

Abstract
Experiments were performed to characterize the protein kinase activity in blood lymphocytes from patients with chronic lymphocytic leukemia (CLL). Using histone as a substrate, the average specific activity was 397 pmole/min/mg protein. The Km for ATP was 8 muM and for histone 0.3 mg/ml. The addition of optimal concentrations of cyclic adenosine monophosphate (cAMP) (1 muM) or cyclic guanosine monophosphate (cGMP) (10muM) resulted in a 2.2-fold stimulation in activity but had no effect on the Km for ATP or histone. Most of the properties of the CCL protein kinase were similar to those of the normal lymphocyte enzyme. These include the pH response, substrate affinity, as well as rates of phosphorylation and dephosphorylation. The phosphorylation pattern of endogenous proteins was determined using intact lymphocytes incubated with 32P and cell-free homogenates with AT32P. These results indicate that: (1) the cyclicnucleotide-protein kinase interactions are unimpaired in CLL lymphocytes; and (2) a sharply defined cyclic nucleotide concentration response occurs for CLL (as well as normal) lymphocytes, which may explain the reports of variable inhibitory (and stimulatory) effects on mitogenesis by these agents.
Correlation between fibrinogen binding to human platelets and platelet aggregability
Authors: Peerschke EI , Zucker MB , Grant RA , Egan JJ , Johnson MM 
Published: October 2019

Abstract
Fibrinogen is essential for aggregating platelets with adenosine diphosphate (ADP) and was recently shown to bind to platelets stimulated with ADP. The present work confirms the specific and saturable nature of the platelet-fibrinogen interaction. Binding of 125iodine-labeled fibrinogen to human gel-filtered platelts was maximal at 1 min, and the receptors were saturated when the fibrinogen concentration in the suspending medium approached 0.8 mg/ml. Assuming that one fibrinogen molecule interacts with a single receptor, experiments with 9 normal donors revealed the presence of 12,896 +/- 2456 receptors per platelet. Much of the bound material dissociated from platelets after incubation with apyrase or EDTA. Binding was markedly inhibited at pH 6.5, in the presence of EDTA, and with platelets from 3 thrombasthenic patients but not with those from a patient with the Bernard-Soulier syndrome. Fibrinogen binding was also virtually absent with platelets that had been incubated with EDTA for 8 min at 37 degrees C and pH 7.8. These platelets could not aggregate when mixed with ADP and adequate CaCl2 and fibrinogen, although they could still change their shape. Thus, ADP-induced binding of fibrinogen correlates with platelet aggregability.
Plasmapheresis with return of cryoglobulin-depleted autologous plasma (cryoglobulinpheresis) in cryoglobulinemia
Authors: McLeod BC , Sassetti RJ 
Published: October 2019

Abstract
Three patients with cryoglobulinemia have been treated with automated plasma exchange procedures in which the replacement fluid was autologous plasma, obtained at a previous plasmapheresis and incubated in the cold to precipitate abnormal protein. All three responded with a reduction in cryoglobulin level and an improvement in clinical manifestations of cryoglobulinemia. The cost of treatment was less than that of conventional plasmapheresis, even though the quality of the replacement fluid was superior. The term “cryoglobulinpheresis” is suggested for this treatment process.
The influence of vitamin E quinone on platelet structure, function, and biochemistry
Authors: Cox AC , Rao GH , Gerrard JM , White JG 
Published: October 2019

Abstract
Although the effects of vitamin E on platelet function have been investigated in vivo and in vitro, vitamin E quinone, a natural metabolite of vitamin E, has been virtually overlooked. This oxidized form of vitamin E inhibits platelet aggregation and secretion induced by various aggregating agents more effectively than vitamin E by a magnitude of 5–10-fold. Vitamin E and vitamin E quinone do not alter platelet ultrastructure or cellular concentrations of serotonin and adenine nucleotides, including cAMP. Inhibition of aggregation by vitamin E quinone occurs in the absence of detectable reduction of vitamin E quinone or oxidation of vitamin E and is readily reversed by washing the platelet. Only vitamin E quinone prevents arachidonic acid release and slightly inhibits cyclooxygenase, whereas both agents partially prevent calcium release from a platelet subcellular organelle. Vitamin E quinone also inhibited synthesis of prostacyclin by endothelial cells with basal synthesis in the presence of external arachidonic acid being less affected than thrombin-stimulated PGI2 production. The greater potency of vitamin E quinone in suppressing platelet function compared to vitamin E suggests that this quinone metabolite may be the better antithrombotic agent and possibly responsible for in vivo effects previously attributed to vitamin E.
Hand-mirror lymphocytes in infectious mononucleosis
Authors: Thomas WJ , Yasaka K , Strong DM , Woodruff CM , Stass SA , Schumacher HR 
Published: October 2019

Abstract
The identification of a morphologically unique lymphocyte in the peripheral blood of patients with infectious mononucleosis (IM) led to additional studies. Examination of Wright-stained smears of EDTA anticoagulated blood obtained from 25 patients with IM at presentation revealed significantly increased (p less than 0.0001) percentages of hand-mirror lymphocytes (HML) (mean 9.2%) compared with normal controls (mean 2.7%) or controls with nonspecific viral syndromes (mean 2.2%). Follow-up blood samples obtained on 10 of these patients demonstrated a marked increase in the HML count (mean 12.1%) that coincided with the onset of recovery. E-rosette separation of Ficoll-Hypaque-derived peripheral blood lymphocytes from 5 patients with early recovery IM showed the HML to be present almost exclusively in the T-cell population, representing about 25% of the T cells. Identical procedures on 5 controls showed less than 5% HML in the T-cell sample. Cytochemistries supported a T-cell derivation for HML. Electron microscopic examination of HML in IM demonstrated that these cells have unique ultrastructural features that may be related to functions of cellular attachment and cytotoxicity.
The regulation of hemopoiesis in long-term bone marrow cultures. II. Stimulation and inhibition of stem cell proliferation
Authors: Toksoz D , Dexter TM , Lord BI , Wright EG , Lajtha LG 
Published: October 2019

Abstract
The isolation of a DNA synthesis inhibitor (NBME fraction IV) and stimulator (RBME fraction III) specific for the hemopoietic stem cell (CFU-s) from freshly isolated normal adult and regenerating murine bone marrow, respectively, has been well documented. We have utilized long- term liquid bone marrow cultures in a further analysis of the role of these factors in the regulation of CFU-s proliferation. Our results show that shortly after feeding, at a time when the cultured CFU-s are actively proliferating, high levels of the hemopoietic stem cell proliferation stimulator fraction III can be isolated from the culture medium. In contrast, the presence of essentially noncycling CFU-s found in cultures fed 8–10 days previously correlates with high levels of the hemopoietic stem cell inhibitor fraction IV. These results suggest that a certain balance between these factors determines CFU-s proliferation in the long-term cultures. In support of this, DNA synthesis in actively cycling CFU-s in the long-term cultures is inhibited for at least 3 days by the addition of excess NBME fraction IV (inhibitor). Furthermore, DNA synthesis in noncycling cultured CFU-s is stimulated for at least 5 days by the addition of RBME fraction III (stimulator).
Effect of peripheral blood mononuclear cells from aplastic anemia patients on the granulocyte-macrophage and erythroid colony formation in samples from normal human bone marrow in vitro--a cooperative work
Authors: Takaku F , Suda T , Mizoguchi H , Miura Y , Uchino H , Nagai K , Kariyone S , Shibata A , Akabane T , Nomura T , Maekawa T 
Published: October 2019

Abstract
Thirty-six patients with aplastic anemia and three with aplastic anemia- PNH syndrome in eight institutes were studied for the presence of peripheral blood mononuclear cells having a suppressive effect on granulocyte-macrophage (G-M) and erythroid colony formation using a uniform protocol. In 11 cases of 29 (38%) and 9 cases of 29 (31%), the presence of mononuclear cells with a suppressive effect on G-M and erythroid colony formation, respectively, was demonstrated. However, the presence of mononuclear cells suppressive both to G-M and erythroid colony formation was demonstrated only in 3 of 19 cases (16%).
Histiocytic lymphoma fifteen years following remission of acute lymphoblastic leukemia
Authors: Dosik H , Anon R , Lee SL , Allen AC , Krishnamurthy M 
Published: October 2019

Abstract
A 19-yr-old female presented with diffuse histiocytic lymphoma after a 15-yr remission of acute lymphoblastic leukemia. All treatment had been discontinued 7 yr before the onset of the lymphoma. The possible relationships of these two neoplasms are discussed.
A plaque assay to enumerate circulating Ig-secreting cells of each type of the different Ig classes
Authors: Shimizu K , Hirano A , Kunii A 
Published: October 2019

Abstract
A new method to quantitate circulating cells secreting immunoglobulin (Ig) of each type of the different classes by hemolytic plaque technique is reported. The ratio of kappa (k)-secreting cells and lambda(lambda)-secreting cells of the different lg classes in normal individuals is almost equal to that of kappa:lambda light chains in corresponding serum lg class, whereas the ratio in plasma cell myeloma patients is extraordinarily high or low, indicative of the type and class specificity of this assay system as well as the monoclonal nature of circulating Ig-secreting cells in such patients.
Cryopreservation of human granulocytes: study of granulocyte function and ultrastructure
Authors: Boonlayangoor P , Telischi M , Boonlayangoor S , Sinclair TF , Millhouse EW 
Published: October 2019

Abstract
Human granulocytes can be cryopreserved with dimethyl sulfoxide (DMSO) at--80 degrees C. However, the percent recovery of functional cells has been unsatisfactory to date. Throughout this study we have been able to prepare relatively pure granulocytes approximately equal to 85%), cryopreserve them with 5%--10% DMSO with and without serum, and store them at--80 degrees C for up to 4 mo. The parameters studied were absolute cell counts and viability determination, myeloperoxidase activity, phagocytosis, candidacidal activity, bactericidal activity, nitroblue tetrazolium reduction, chemiluminescence, and cell morphology by transmission and scanning electron microscopy. Based on our investigation, granulocytes cryopreserved without serum showed an intact membrane of superior integrity as compared with those preserved with serum. At least 50% of the cells recovered were functional after 2 mo of storage, but there was a progressive loss of viability and function on prolonged storage. The property of phagocytosis was the best preserved after storage for 4 mo, whereas myeloperoxidase activity, killing activity, nitroblue tetrazolium reduction, and chemiluminescence were maintained less efficiently. Morphological studies of cryopreserved granulocytes revealed that the nuclear, cytoplasmic, and cell surface architectures were altered by storage. Depletion of nuclear and cytoplasmic material, as well as changes in configuration, were also noted.
Chronic myelodysplastic syndrome (preleukemia) with the Philadelphia chromosome
Authors: Roth DG , Richman CM , Rowley JD 
Published: October 2019

Abstract
A patient with severe anemia, reticulocytopenia, and erythroid hyperplasia of the bone marrow developed fatal acute nonlymphocytic leukemia after 3 yr. A Philadelphia chromosome with the typical 9/22 translocation t(9q +;22q-) was identified by banding techniques in a small number of bone marrow cells throughout the preleukemic phase of the illness (14%--38% of metaphases) and during the acute transformation (50%). Granulocytic colony formation in vitro was abnormal in the preleukemic phase. The diagnosis of chronic granulocytic leukemia was excluded on the basis of clinical and laboratory findings. The identification of the Ph1 chromosome in this form of chronic myelodysplastic syndrome (preleukemia) provides a new example of a hematologic disorder predisposing to acute leukemia in which this chromosomal abnormality occurs.
Acute lymphoblastic leukemia with Burkitt cell morphology and cytoplasmic immunoglobulin
Authors: Ganick DJ , Finlay JL 
Published: October 2019

Abstract
A case of acute lymphoblastic leukemia with morphological characteristics of Burkitt's leukemia (L3 morphology) is presented. This patient's lymphoblasts were lacking in surface immunoglobulin, but were found to contain cytoplasmic IgM. This is the first report of a morphologically B-cell leukemia showing pre-B-cell characteristics immunologically.
Serum lactic dehydrogenase (LDH) levels in acute leukemia: marked elevations in lymphoblastic leukemia
Authors: Kornberg A , Polliack A 
Published: October 2019

Abstract
Serum total lactic dehydrogenase (LDH) levels were examined in 42 patients with acute leukemia, 9 patients with chronic myeloid leukemia, 6 of them in blastic crisis, and 53 patients with lymphoma and other lymphoproliferative disorders. The mean range of serum LDH leveles in Hodgkin's and non-Hodgkin's lymphoma was 402 +/- 210 IU/liter and 313 +/- 113 IU/liter, while that of patients with nonmalignant disorders was 308 +/- 74 IU/liter. In acute nonlymphoblastic leukemia (ANLL), the range was 126–684 IU/liter (mean value 413 +/- 146 IU/liter). In 6 of the patients (11.3%) with lymphoma and in 6 cases (26.8%) with ANLL, the LDH levels were above 500 IU/liter. None of these patients had levels over 900 IU/liter. Patients with acute lymphoblastic leukemia (ALL) had a range of 402–3582 IU/liter (mean value of 1669 + 1038 IU/liter). In 15 of the 19 patients (78.9%) with ALL, serum LDH values were above 900 IU/liter. In addition, 3 patients with chronic myeloid leukemia (CLM) in blastic crisis had levels of 970–1940 IU/liter. One of these 3 patients had lymphoblastic crisis, while the second case responded clinically to vincristine and prednisone, but was not regarded as ALL. The differences in serum LDH levels between ALL and ANLL are statisticaly significant (p < 0.001). It appears that markedly elevated serum LDH levels in acute leukemia are suggestive of ALL, and that in individual patients, the LDH levels were correlated with the number of blasts during remission and relapse.
Purine nucleoside phosphorylase activity in acute lymphoblastic leukemia
Authors: Blatt J , Reaman GH , Levin N , Poplack DG 
Published: October 2019

Abstract
Purine nucleoside phosphorylase (PNP) activity has been measured in the lymphoblasts of 22 patients with acute lymphoblastic leukemia (ALL) and correlated with routine immunologic cell surface markers. Fourteen of the 22 patients were considered to have non-T, non-B-cell ALL; 8 patients had T-cell disease. The median PNP activity in 21 control samples of normal peripheral blood mononuclear cells was 83 U. The median PNP activity of the non-T, non-B lymphoblasts was 79 U. No statistical difference in PNP activity between these two groups could be discerned (p < 0.37). In contrast, T-cell lymphoblasts demonstrated diminished PNP activity with a median of 38 U. The differences in activity between T lymphoblasts and both non-T, non-B leukemic cells and normal peripheral blood mononuclear cells were significant (p < 0.001 and p < 0.003, respectively). Evaluation of PNP activity provides further evidence of biochemical heterogeneity among immunologic subclasses of ALL.
Cytotoxicity as an indicator for transport mechanism: evidence that murine bone marrow progenitor cells lack a high-affinity leucine carrier that transports melphalan in murine L1210 leukemia cells
Authors: Vistica DT 
Published: October 2019

Abstract
Cytotoxicity of melphalan to murine L1210 leukemia cells was reduced to a limiting maximum value of 50% by 2-aminobicyclo[2,2,1]heptane-2- carboxylic acid (BCH), indicating that cytotoxicity is partially dependent on drug uptake by system L. L-Leucine, but not alpha- aminoisobutyric acid (AIB), completely reduced the remaining 50% of drug cytotoxicity. These results contrast with those obtained with a sensitive host tissue, the bone marow progenitor cells of the white cell series (CFU-C), in that a high-affinity leucine transport system corresponding to system L was not identified.