bluuebunny/crossref_metadata_2025_split huggingface dataset:
Enzymologic classification of acute leukemias: nonspecific esterase markers distinguish myeloid and lymphoid varieties
Authors: Yourno J , Burkart P , Lizzi F , Tartaglia A 
Published: October 2019

Abstract
Nonspecific esterase zymograms of purified leukemic cells from a case of acute myelomonocytic leukemia (AMML) and from a case of acute non-B, non-T lymphocytic leukemia (ALL) were produced by isoelectric focusing and staining with alpha-naphthyl acetate (alpha NA) or alpha-naphthyl butyrate (alpha NB) substrate. A “myeloid” zymogram was found with AMML cells, which closely matched control monocyte and granulocyte zymograms. On the other hand, nonspecific esterase of ALL showed a striking departure from the zymogram pattern of control B lymphocytes and T lymphocytes. An intense reactivity with a very low isoelectric point accounted for most of the ALL nonspecific esterase activity. No corresponding reactivity or relatively small amounts thereof were seen in other zymograms. Conversely, few of the isoenzymes that were prominent in zymograms of control lymphocytes were apparent above trace levels in ALL zymograms. Thus, zymogram analysis of nonspecific esterases clearly differentiated the myeloid leukemia from the lymphoid leukemia and provided a potential marker for each. The AMML cells appeared well enough differentiated with respect to nonspecific esterases as to be similar to mature cells of like lineage. It is plausible that the ALL cells, however, were arrested at an earlier stage of esterase expression as reflected by the associated atypical species.
Correlation of occupation and karyotype in adults with acute nonlymphocytic leukemia
Authors: Golomb HM , Alimena G , Rowley JD , Vardiman JW , Testa JR , Sovik C 
Published: October 2019

Abstract
Seventy-four adult patients with acute nonlymphocytic leukemia (ANLL) were classified retrospectively as to whether or not they had had occupational exposure to insecticides, chemicals and solvents, or petroleum products. Fifty-eight patients were considered nonexposed and 16 were considered exposed. The chromosome banding pattern was abnormal in 37 of the 74 patients (50%). Twenty-five of the 58 (43%) nonexposed patients had a clonal chromosome abnormality, compared with 12 of the 16 (75%) exposed patients (p = 0.02). Only 2 of 23 (8.7%) females with an abnormal karyotype were exposed, whereas 10 of 14 (71%) males with an abnormal karyotype were exposed. Either a -5/5q- or a -7/7q- was present in 67% of the exposed patients with a chromosome abnormality, compared to 28% of the aneuploid nonexposed patients. The -7/7q- abnormality was present in 7 of the 12 (58.3%) exposed patients, versus 5 of the 25 (20%) nonexposed patients with abnormal karyotypes (p less than 0.05). The -5/5q- anomaly was observed in 4 of the 12 (33%) exposed patients and in 4 of the 25 (16%) nonexposed abnormal patients. Our study supports the observation that a subset of patients with ANLL de novo have a history of occupational exposure and a unique pattern of chromosome abnormalities.
Effects of neuraminidase on O2 consumption and release of O2 and H2O2 from phagocytosing human polymorphonuclear leukocytes
Authors: Suzuki H , Kurita T , Kakinuma K 
Published: October 2019

Abstract
Neuraminidase type X (NMD-type-X, Sigma Chemical Co., St. Louis, Mo.), which is obtained from a further purification of neuraminidase type V (NMD-type-V, Sigma), markedly enhanced the release of O2- and H2O2 from phagocytosing human polymorphonuclear leukocytes (PMN). In contrast, O2 consumption by NMD-type-X-treated PMN was identical to that of untreated PMN. Morphological observations suggested that the enhancement of O2- and H2O2 release was caused by excessive release of the oxygen metabolites into the extracellular medium from incompletely formed phagocytic vacuoles as was observed with cytochalasin-B-treated cells. Our observations are in contrast to the previous reports of Tsan et al. that showed complete inhibition of both O2- and H2O2 release from phagocytosing PMN by the treatment with NMD-type-V.
Evaluation of AMSA in previously treated patients with acute leukemia: results of therapy in 109 adults
Authors: Legha SS , Keating MJ , McCredie KB , Bodey GP , Freireich EJ 
Published: October 2019

Abstract
AMSA was evaluated in the treatment of 109 adults with previously treated acute leukemia. Of the 102 evaluable patients, 82 had AML, 17 ALL, and 3 CML in blastic phase. A number of different dose schedules of AMSA were explored, and we conclude that the optimum dose of AMSA for remission induction in acute leukemia is 120 mg/sq m/day for 5 days. Complete remissions were observed in 23 (28%) patients with AML and in 1 patient with ALL. Patients who achieved complete remission were maintained on AMSA using a dose of 30–40 mg/sq m/day for 5 days repeated at 4-wk intervals. The median duration of complete remission was 12 wk (3–59 wk), and the responders survived significantly longer than the failures (27 wk versus 8 wk, p = 0.002). The side effects associated with AMSA therapy included mild nausea and vomiting, stomatitis, diarrhea, phlebitis, alopecia, and myelosuppression-related infections. Our results indicate that AMSA is a useful new antileukemic agent for the treatment of relapsed acute leukemia and appears to have activity comparable to that of the currently available drugs, such as cytarabine and the anthracycline antibiotics.
Presence of mixed colony-forming cells in long-term hamster bone marrow suspension cultures: response to pokeweed spleen conditioned medium
Authors: Eastment CE , Ruscetti FW , Denholm E , Katznelson I , Arnold E , Ts'o PO 
Published: October 2019

Abstract
In contrast to the murine system, long-term hamster bone marrow suspension cultures maintain proliferation of both pluripotent and committed stem cells in the absence of an adherent layer and without addition of exogenous factors, such as hydrocortisone. Addition of pokeweed-mitogen-stimulated hamster spleen conditioned medium (SCM) to these long-term suspension cultures produces an increase in the number of mixed colonies assayed in soft-agar, These mixed colonies, which contained four cell lineages--granulocytic, erythroid, megakaryocytic, and macrophage--could be generated from cells grown in suspension for over 6 mo. Addition of SCM also induces an initial rapid expansion of the myeloid compartment, and this expansion results in 70% of the cells being terminally differentiated granulocytes. In contrast, addition of SCM to hamster bone marrow cultures containing both adherent cells and hematopoietic stem cells produced no change in the number of mixed colonies generated in the culture. This system allows the in vitro study of the process of stem cell proliferation and differentiation and also provides a means to examine the relationship of adherent and supernatant bone marrow populations.
Cyclosporin-A abrogates transfusion-induced sensitization and prevents marrow graft rejection in DLA-identical canine littermates
Authors: Storb R , Deeg HJ , Atkinson K , Weiden PL , Sale G , Colby R , Thomas ED 
Published: October 2019

Abstract
Previous studies had shown that marrow graft rejection generally did not occur in untransfused dogs given 900-rad total body irradiation and hemopoietic grafts from DLA identical littermates (only 1 of 59 rejected), but was seen in all instances after three preceding transfusions of whole blood from the marrow donor on days -24, -17, and -10 before transplantation (19 of 19 rejected). The present study was undertaken to investigate whether immunization by 3 preceding transfusions of whole blood from the DLA-identical littermate marrow donor could be abrogated by administration of the immunosuppressive agent cyclo-sporin-A, 20 mg/kg/day intramuscularly on days -5 to 0. Seven of 10 dogs showed sustained marrow engraftment. 2 failed to engraft, and 1 dog died to early to be evaluated. It was concluded that immunization to non-DLA antigens by preceding whole blood transfusions could be abrogated in most cases by a short-course of cyclosporin-A before total body irradiation and marrow transplantation, resulting in successful and sustained marrow engraftment.
Surface membrane vesicles from mononuclear cells stimulate erythroid stem cells to proliferate in culture
Authors: Dainiak N , Cohen CM 
Published: October 2019

Abstract
In order to examine the contribution of cell surface materials to erythroid burst-promoting activity (BPA), we separated media conditioned by a variety of human cell types into pellets and supernatants by centrifugation. When added to serum-restricted cultures of nonadherent human marrow cells, pellets contained about half of the total stimulatory activity. Freeze-fracture electron microscopy of the pellets revealed the presence of unilamellar membrane vesicles ranging from 0.10 to 0.40 microM in diameter. The amount of BPA in culture increased with added vesicle concentration in a saturable fashion. Preparation of leukocyte conditioned medium (LCM) from 125I-wheat germ agglutinin labeled cells and studies comparing the glycoprotein composition of vesicles with that of leukocyte plasma membranes suggest that LCM-derived vesicles are of plasma membrane origin. Moreover, partially purified leukocyte plasma membrane preparations also contained BPA. While disruption of vesicles by freezing/thawing and hypotonic lysis did not alter BPA, heat, trypsin, or pronase treatment removed greater than 65% of BPA, implying that vesicle surface rather than intravesicular molecules express BPA. Results of BPA assays performed in two-layer clots indicated that proximity to target cells is required for vesicle BPA expression. We conclude that membrane vesicles spontaneously shed from cell surfaces may be important regulators of erythroid burst proliferation in vitro.
The kinetics of iron uptake by isolated intestinal cells from normal mice and mice with sex-linked anemia
Authors: Peppriell JE , Edwards JA , Bannerman RM 
Published: October 2019

Abstract
No significant differences of Km or Vmax could be demonstrated for the initial rate (0–3 min) of iron uptake by preparations of isolated duodenal mucosal epithelial cells from normal or sex-linked anemic (sla) mice. Isolated cells from sla mice do, however, take up significantly more iron than normal cells after 20-min incubation. Upon reincubation with various types of mobilizing agents, the amount of iron released from, and the amount of iron remaining in, cells from sla mice was significantly elevated compared to control values. The percentage of iron released was the same for both types of cells, for all mobilization media employed. The current data, in conjunction with previous studies that showed deficient mucosal to serosal transport of iron in everted duodenal sacs from sla mice, suggest that the lesion in these mice may be located between the mucosal cell and the vascular compartment rather than in the mucosal cell.
T-cell lymphoma and the Chediak-Higashi syndrome
Authors: Argyle JC , Kjeldsberg CR , Marty J , Shigeoka AO , Hill HR 
Published: October 2019

Abstract
The majority of patients with Chediak-Higashi syndrome (CHS) develop a lymphoproliferative disorder during the accelerated phase of the disease. Controversy exists regarding the benign versus malignant nature of this cellular proliferation. For the first time, we have characterized the immunologic cell markers on the cellular infiltrate in a lymph node from a patient with CHS. The infiltrate was composed almost entirely of T cells, with histopathologic features consistent with a non-Hodgkin's T-cell lymphoma.
Association between HLA-D region antigens and disease-free survival in childhood non-T, non-B acute lymphocytic leukemia
Authors: Casper JT , Marrari M , Piaskowski V , Lauer SJ , Duquesnoy RJ 
Published: October 2019

Abstract
The frequency of three serologically defined HLA-D region antigens--DR, MB, and MT--was determined in a group of 74 children with non-T, non-B acute lymphocytic leukemia (ALL). Statistically, there were no significant differences in the frequency of any antigen in these ALL patients as compared with a panel of 85 normal controls. However, significant differences in HLA-DR frequencies were observed between patients who relapsed or who remained disease-free during a 30-mo period of chemotherapy. An increased incidence of relapse was associated with DR5, while disease-free remission during chemotherapy was associated with DR7. Life table analysis also demonstrated that DR5 was significantly associated with a decrease in disease-free survival in these patients. These data suggest that HLA-associated genetic factors may influence the responses of ALL patients to chemotherapy.
Peripheral blood myeloid progenitor cell cultures in patients with hypereosinophilic syndrome (CFU-eos in hypereosinophilic syndrome)
Authors: Bjornson BH , Harley JB , Andre-Schwartz J , Fauci AS , Desforges JF 
Published: October 2019

Abstract
Myeloid progenitor cell cultures (CFU-C) were established in a double- layer agar system with peripheral blood mononuclear cells from 13 patients with the hypereosinophilic syndrome (HES). Normal controls produced 49% +/- 3.5% eosinophil colonies; results in 7 of the 13 HES patients were within the normal range, while in 5, the proportion of eosinophil colonies was greater than 3 standard deviations above the normal mean, and in 1 patient there was a low proportion of eosinophil colonies. The production of an increased proportion of eosinophil colonies correlated with more aggressive disease. Experiments in which normal progenitor cells were cultured over feeder layers of mononuclear cells demonstrated that cells of 3 of the 5 patients had an excess production of eosinophil colony-stimulating activity. When HES patients progenitor cells were cultured over normal feeder layers, 2 of the 5 patient samples continued to produce an increased proportion of eosinophil colonies, suggesting that these patients have an excess proportion of progenitor cells committed to eosinophil differentiation. Thus, the results demonstrated heterogeneity of growth characteristics for the HES patients. None, however, had the colony growth characteristic of acute or chronic myelogenous leukemia.
Activation of factor VIII by factor IXa
Authors: Rick ME 
Published: October 2019

Abstract
Thrombin causes an increase in factor VIII coagulant (VIII:C) activity, which is followed by a decay of VIII:C activity to below baseline levels. It has been suggested that a similar interaction of trace amounts of thrombin and factor VIII is a necessary prerequisite before factor VIII can participate in the coagulation cascade. In the current study, factor IXa, a serine protease with structural similarities to thrombin, is shown to cause an increase and subsequent fall in VIII:C in a manner qualitatively similar to the reaction with thrombin. The reaction is inhibited by a human inhibitor to factor IX and the interaction appears to involve only VIII:C, since factor-VIII-related protein (VIII:RP) is not changed on polyacrylamide gel electrophoresis (PAGE) or radioimmunoassay during the reaction. Phospholipid increases the activation of factor VIII by factor IXa, and high concentrations of diisopropylfluorophosphate and hirudin inhibit the reaction. The physiologic significance of the interaction of factor IXa with factor VIII is not entirely clear since the concentration of factor IXa needed for activation is much greater than the concentration of thrombin required for similar activation of factor VIII. Factor IXa is most likely to play a role in the intrinsic cascade acting as an initial activator of factor VIII, since factor IXa precedes thrombin in this clotting sequence. In addition, factor IXa may be important wherever relatively high local concentrations of factor IXa and factor VIII occur, particularly in the presence of phospholipid, which may serve to localize the coagulation factors.
Platelet-type von Willebrand's disease: characterization of a new bleeding disorder
Authors: Miller JL , Castella A 
Published: October 2019

Abstract
An autosomally transmitted bleeding diathesis sharing some, but not all, features previously described in von Willebrand's disease (vWd) was studied in five patients representing three generations of a single family. Bleeding times in the upper normal range in conjunction with low-normal platelet counts, normal factor VIII coagulant activity and VIII-related antigen, decreased VIII-ristocetin cofactor activity, selective decrease of the higher molecule weight factor VIII/von Willebrand factor (VIII/vWF) multimers, and increased ristocetin- induced platelet agglutination at low ristocetin concentrations were characteristic. Binding of patient VIII/vWF to washed normal platelets was within normal limits, whereas binding of normal VIII/vWF to patient platelets was significantly increased (p less than 0.001 at 0.6 mg/ml ristocetin). This disorder accordingly appears to involve an intrinsic platelet abnormality affecting platelet-VIII/vWF interactions. It is proposed that the concept of vWD be broadened to include patients with this abnormality, which may appropriately be called “Platelet-type von Willebrand's disease.”
Allogeneic bone marrow transplantation for chronic myelogenous leukemia in chronic or accelerated phase
Authors: Champlin R , Ho W , Arenson E , Gale RP 
Published: October 2019

Abstract
Eight patients with Ph1-positive chronic myelogenous leukemia (CML) in chronic or accelerated phase received high-dose cyclophosphamide, total body irradiation, and bone marrow transplantation from an HLA-identical sibling donor. All patients had prompt engraftment and achieved complete hematologic remission. Six patients remain alive and in continuous remission with a normal bone marrow karyotype 3–20+ mo posttransplant. One patient died from cytomegalovirus interstitial pneumonitis. Only one patient who was transplanted in accelerated phase relapsed 6.5 mo posttransplant and died in blast crisis. High-dose combined modality therapy is capable of producing sustained complete remissions in patients with CML treated during chronic or accelerated phase.
Cumulative experience with a simplified solid-phase radioimmunoassay for the detection of bound antiplatelet IgG, serum auto-, allo-, and drug-dependent antibodies
Authors: Faig D , Karpatkin S 
Published: October 2019

Abstract
A simplified, sensitive, solid-phase radioimmunoassay employing 125I- staphylococcal protein A has been developed that is capable of detecting bound antiplatelet IgG as well as serum auto-, allo-, and drug-dependent antiplatelet antibodies. The simplified assay employs a ratio of test over control platelet counts per minute (cpm) for detection of positive results. All reagents are commercially available. The assay can be performed with as little as 10(6) washed platelets (10 microliters of whole blood) that have been stored for as long as 8 wk at 4 degrees C in microtiter plates. The assay time, employing stored platelets, is 4 hr. Bound platelet IgG is positive in 93% of 46 thrombocytopenic patients with autoimmune disease and correlates inversely with their platelet count, r = -0.65, p less than 0.001. The ability of this assay to detect serum antibody was studied with a rabbit anti-human platelet antibody capable of giving optimal immunoprecipitation with solubilized platelet membranes at a tier of 1:10. The present assay increases the sensitivity of antibody detection 256-fold to a titer of 1:2560. Human serum antiplatelet membrane antibody was positive in 2 of 2 patients with anti-PLA-1 antibody (titers of 1:256 and greater than 1:64); 7 of 12 multiply transfused patients who were refractory to platelet transfusion (2 had titers of greater than 1:256 and greater than 1:32); 5 of 19 patients with autoimmune thrombocytopenic purpura (2 had titers of 1:64 and 1:32); and 10 of 14 patients with clinical histories of drug-dependent antiplatelet antibody (2 had titers of 1:1280 for quinidine and 1:384 for phenazopyridine).
Myeloma protein kinetics following chemotherapy
Authors: McLaughlin P , Alexanian R 
Published: October 2019

Abstract
The effects of chemotherapy were evaluated in 43 multiple myeloma patients with high monoclonal globulin levels in both serum and urine. In responding patients, Bence Jones protein excretion declined more rapidly and markedly than the serum myeloma protein. Bence Jones protein excretion was reduced by 50% within 2 mo in responders, remained unchanged in nonresponders, and declined with a slow halving time of 2–7 mo in patients with partial disease control. Three patients with clinical resistance to treatment showed atypical protein changes and progressively more Bence Jones protein excretion relative to their serum monoclonal component. Serial measurements of Bence Jones protein excretion provided an early and reliable index of tumor mass change in patients with multiple myeloma.
Nonrandom chromosome abnormalities in angioimmunoblastic lymphadenopathy
Authors: Kaneko Y , Larson RA , Variakojis D , Haren JM , Rowley JD 
Published: October 2019

Abstract
Cytogenetic and pathologic studies were performed on six patients with angioimmunoblastic lymphadenopathy (AILD). All six had diffuse lymphadenopathy; five had fever, four had weight loss, and four had a diffuse erythematous rash. All patients except one had a polyclonal elevation of immunoglobulin. All patients had diagnostic findings in lymph node (LN) and bone marrow (BM) biopsies. Two patients died of progressive AILD; one patient died after transformation of AILC to immunoblastic sarcoma (IBS); one patient died of gastrointestinal bleeding of unknown cause. The remaining two patients, who have achieved complete remission with intensive chemotherapy, are alive 20 and 8 mo after the diagnosis; one of these had AILD and the other, both AILD and IBS. Despite diagnostic BM biopsy findings, none of the patients had chromosome abnormalities in their BM cells. In studying LN cels of 5 patients, however, we found chromosome abnormalities in each; clonal abnormalities were detected in two, both clonal and nonclonal abnormalities in two, and only nonclonal single-cell abnormalities in one. An extra chromosome 3, seen in four patients, was clonal in two and nonclonal in the two others. Cells with +5, +15, +19, +21, +22 were seen in two patients. All patients had 50% or more normal dividing cells in their LN. The mosaicism of unrelated abnormal cells in their LN. The mosaicism of unrelated abnormal karyotypes that was seen in four patients suggests that this malignant tumor is not necessarily monoclonal in its early stages, but that one clone may be selected and predominate in the late stage. Because nonrandom acquired clonal chromosome abnormalities are a consistent feature of malignancies, our data suggest that AILD may be a malignant disease despite its original description as a benign proliferative process. Therefore, it may require aggressive chemotherapy.
Characteristic abnormality of deoxyribonucleoside triphosphate metabolism in megaloblastic anemia
Authors: Iwata N , Omine M , Yamauchi H , Maekawa T 
Published: October 2019

Abstract
To elucidate the biochemical basis of megaloblastic hematopoiesis, the cellular content and metabolism of deoxyribonucleoside triphosphates (dNTPs) were investigated using the bone marrow cells from nine patients with untreated vitamin B12 deficiency and one with folic acid deficiency. The marked imbalance among four dNTPs was noted in all patients. dTTP was invariably elevated rather than depressed. The most striking abnormality, however, was the excessive accumulation of dCTP, which represented the consistent feature exclusive for megaloblastic anemia. Purine nucleotides were also involved to a lesser extent. The apparent turnover pattern of the dTTP pool of megaloblastic anemia marrow cells, in the presence or absence of hydroxyurea, did not differ significantly from that of normoblastic hematopoiesis. The megaloblastic cells assimilated exogenous thymidine into dTTP pool in vitro with enhanced efficiency. It was suggested that the excessive accumulation of dCTP may be related more closely to the pathogenesis of megaloblastic hematopoiesis than to the presumed but not proved deficiency of dTTP.