bluuebunny/crossref_metadata_2025_split huggingface dataset:
A clinicopathologic correlation of the idiopathic hypereosinophilic syndrome. I. Hematologic manifestations
Authors: Flaum MA , Schooley RT , Fauci AS , Gralnick HR 
Published: October 2019

Abstract
A retrospective blind study of 32 patients with the hypereosinophilic syndrome was undertaken utilizing a hematologic scoring system that was based on peripheral blood and bone marrow findings, cytogenetics B12 levels, and leukocyte alkaline phosphatase determinations. In addition to the grading system, which allowed formulation of a hematologic score, the date could also be normalized for individuals who did not have all tests performed by use of the hematologic quotient. This study clearly defined two groups of patients within the idiopathic hypereosinophilic syndrome. One group were those individuals with low hematologic scores and quotients who did not require therapy or who responded to prednisone therapy, while the second group of patients required cytotoxic therapy. These patients had significantly higher hematologic scores and quotients and a significant number of abnormalities similar to those seen in myeloproliferative syndromes, such as myelofibrosis and cytogenetic abnormalities. This type of hematologic scoring seems useful in predicting therapy and/or evaluating individuals or groups of patients with the hypereosinophilic syndrome.
Cytogenetic conversion following allogeneic bone marrow transplantation for advanced chronic myelogenous leukemia
Authors: McGlave PB , Miller WJ , Hurd DD , Arthur DC , Kim T 
Published: October 2019

Abstract
We performed a pilot study to test the effectiveness of allogeneic bone marrow transplantation in the treatment of chronic myelogenous leukemia. Five patients in the advanced stages of chronic myelogenous leukemia (four in blast crisis, one in accelerated phase) with abnormal chromosomes underwent matched-sibling allogeneic bone marrow transplantation after preparation with busulfan, vincristine, cyclophosphamide, and fractionated total body irradiation. Engraftment and conversion to normal chromosome patterns after transplantation occurred in all five patients. None of the patients reverted to an abnormal chromosome pattern of demonstrated clinical or hematologic evidence of recurrent disease during the course of this study; however, longest survival from transplant was 248 days. Allogeneic bone marrow transplantation can eradicate the abnormal clone even in far advanced chronic myelogenous leukemia and can provide normal hematopoiesis. We suggest that clinical complications of chemotherapeutic toxicity and infection were responsible for the short survival in this group of patients, and that these complications could be decreased by performing transplantation in the chronic phase or early accelerated phase of the disease.
Characterization of the phytohemagglutinin-induced proliferating lymphocyte subpopulations in chronic lymphocytic leukemia patients using a clonogenic agar technique and monoclonal antibodies
Authors: Davis S 
Published: October 2019

Abstract
Peripheral blood lymphocytes from normal donors and patients with chronic lymphocytic leukemia, B-cell type, were purified into T, helper T, and suppressor T lymphocytes by fluorescence-activated cell sorting using OKT3, OKT4, and OKT8 monoclonal antibodies. The maximum response of the purified subpopulations to stimulation by phytohemagglutinin (PHA) was determined by measuring the production of colonies when the stimulated cells were grown on agar. The helper T cells in normal and CLL patients were the most responsive to PHA stimulation, although the responsiveness of helper T cells to PHA was decreased in CLL. Purified CLL B cells responded minimally to PHA stimulation, but normal B lymphocytes did not. The abnormal response of CLL lymphocytes to PHA appears to be due abnormal helper T cells, and, to a smaller extent, to the ability of CLL B lymphocytes to respond.
Purine nucleoside phosphorylase (PNP) and adenosine deaminase (ADA) activities examined cytochemically in unfixed lymphocytes of patients with lymphoproliferative disorders
Authors: Maeda K , Ito K , Yamaguchi N 
Published: October 2019

Abstract
New techniques have been devised for the cytochemical demonstration of purine nucleoside phosphorylase (PNP) and adenosine deaminase (ADA) activities in unfixed human lymphocytes. A suspension of living lymphocytes is mixed with agarose sol containing the reagents for the detection of PNP or ADA activity on a glass slide. The mixture solidifies, is incubated, and then dried for lightmicroscopic observation. Reactive cells are recognized by the diffusely deposited granules of formazan, the end-product of the cytochemical reaction, and are divided into three groups of the cell with the low, middle, and high enzyme activity by the number of the granule. In healthy adults, the mean percentages of PNP- and ADA-positive cells were more than 90% in unfractionated lymphocytes, T-cell fractions, and complement- receptor cell fractions and cells with middle PNP and ADA activities were predominant. The PNP and ADA staining was observed in lymphoid cells of patients with lymphoproliferative disorders. A decrease in the percentage of PNP-positive cells concomitant with a relative increase of cells with the low enzyme activity was observed in the lymphocytes of nine patients with chronic lymphocytic leukemia (CLL). Similar findings were obtained in the ADA staining of the lymphocytes of five patients with B-cell CLL.
Evidence that essential thrombocythemia is a clonal disorder with origin in a multipotent stem cell
Authors: Fialkow PJ , Faguet GB , Jacobson RJ , Vaidya K , Murphy S 
Published: October 2019

Abstract
Essential thrombocythemia is characterized by proliferation of hematopoietic tissue predominantly involving megakaryocytes and resulting in marked thrombocytosis. The disorder has some clinical and laboratory features that resemble those seen in the clonal multipotent stem cell disorders chronic myelogenous leukemia, polycythemia vera, and agnogenic myeloid metaplasia. It has been argued that essential thrombocythemia should be classified together with those disorders as a myeloproliferative syndrome. However, without knowledge of the numbers and types of cells that are involved in essential thrombocythemia, this suggestion remains speculative. Three patients with thrombocytosis were studied. The diagnosis of essential thrombocythemia was considered to be firm in two patients and probable in the third one. The X-linked glucose-6-phosphate dehydrogenase locus was used as a cell marker. Whereas both A and B types of glucose-6-phosphate dehydrogenase were found in nonhematopoietic tissues, only a single-enzyme type was found in the granulocytes, red cells, and platelets from each patient. These data indicate that the disorders in these three patients are clonal and involve multipotent stem cells.
Combined factor V-VIII deficiency: a case report with studies of factor V and VIII activation by thrombin
Authors: Hultin MB , Eyster ME 
Published: October 2019

Abstract
A new case of combined factor V-VIII deficiency is reported with in vitro studies of factors V and VIII activation by thrombin. The normal activation of factors V and VIII demonstrated in the patient's plasma and the equivalent levels of factor VIII coagulant activity and coagulant antigen support the hypothesis that a quantitative rather than qualitative defect in factors V and VIII is present in this disorder.
Metabolic heterogeneity of eosinophils from normal and hypereosinophilic patients
Authors: Pincus SH , Schooley WR , DiNapoli AM , Broder S 
Published: October 2019

Abstract
Eosinophils, which may be associated with allergic, parasitic, or neoplastic disease, have a potent oxidative burst that may be activated by particulate or soluble stimuli. Eosinophils from normal persons and patients with hypereosinophilia were compared with respect to their ability to produce the active oxygen product, superoxide anion. Normal eosinophils produced large amounts of superoxide anion under resting conditions (0.53 +/- 0.15 nmoles cyto-c/10(5) eos/hr) and when stimulated by preopsonized zymosan (0.85 +/0 1.10 nmoles cyto-c/10(5) eos/hr) or phorbol myristate acetate (PMA) (2.38 +/- 0.46 nmoles cyto- c/10(5) eos/hr). Considerable variation was observed in superoxide production by eosinophils from patients with hypereosinophilia. Eosinophils from a group of four patients with hypereosinophilia associated with neoplastic disease produced less superoxide anion than normal eosinophils when stimulated by preopsonized zymosan or PMA (p less than or equal to 0.05). Eosinophils from a group of 5 patients with other causes of hypereosinophilia produced more superoxide anion than normal eosinophils when stimulated by PMA (p less than or equal to 0.01). These studies demonstrate metabolic heterogeneity of eosinophils from patients with hypereosinophilia, and further emphasize that normal eosinophils and eosinophils from hypereosinophilic patients are not functionally equivalent.
In vivo and in vitro activity of neutrophil alkaline phosphatase in acute myelocytic leukemia with 8;21 translocation
Authors: Kamada N , Dohy H , Okada K , Oguma N , Kuramoto A , Tanaka K , Uchino H 
Published: October 2019

Abstract
Cytogenetic studies were made on 160 patients with acute nonlymphocytic leukemia (ANLL) between 1963 and 1979, of whom 115 had acute myelocytic leukemia with 67 patients showing aneuploidy (58.3%). Among these, 24 patients were found to have similar chromosome alterations that appeared to involve specifically chromosomes 8 and 21. Banding studies on at least 7 of these patients confirmed the presence of a translocation between these two chromosomes. Of 160 ANLL patients, 142 were scored for neutrophil alkaline phosphatase (neutrophil AP) at the time of diagnosis. Fifty-nine patients showed a low neutrophil AP score, 42 a normal value, and 41 a high value. All patients with 8;21 (or C/G) translocation had a low neutrophil AP score and leukemic cells with maturation (M2 of FAB classification) in the bone marrow. In vitro liquid culture for 2 wk of 8;21 translocated leukemic cells revealed no increase of neutrophil AP activity nor increase of mature granulocytes, whereas 9;22 translocated chronic myelocytic leukemia cells with a low neutrophil AP score did so. Neutrophil AP score at the time of diagnosis in acute myelocytic leukemia is very useful for detecting 8;21 translocation AML and for studying the pathophysiology and genetic alterations of the characteristic subgroup of AML with 8′21 translocation.
Inhibitors of cell division reversibly modify hemoglobin concentration in human erythroleukemia K562 cells
Authors: Erard F , Dean A , Schechter AN 
Published: October 2019

Abstract
The human leukemia K562 cell line can be induced by 20 micro M hemin to reversibly accumulate embryonic and fetal hemoglobins without any change in the rate of cell division. When we reduced the rate of cell division by glutamine starvation or addition of hydroxyurea, the cells increased by tenfold the basal hemoglobin level of 0.3–0.5 pg Hb/cell. The combined effects of hemin and inhibitors of cell division permitted K562 cells to attain levels of hemoglobin (26–34 pg Hb/cell) close to that found in normal red cells. This superinduction was reversible and cells could be recycled indefinitely. Furthermore, electrofocusing experiments show that the three primary hemoglobin species produced by these cells (Hb Gower 1, Hb Portland, and fetal Hb), were induced, or reinduced, synchronously by inhibitors of cell division but asynchronously by hemin. Differing effects of hemin and inhibitors of cell division were observed in the absence of irreversible differentiation and suggest different molecular mechanisms controlling globin gene expression.
Mode of action of the IgG inhibitor of erythropoiesis in transient erythroblastopenia of children
Authors: Dessypris EN , Krantz SB , Roloff JS , Lukens JN 
Published: October 2019

Abstract
Twelve cases of transient erythroblastopenia of childhood (TEC) have been studied to evaluate their marrow cell erythropoiesis in vitro and the effect on it of their serum or IgG. The number of colony-forming units-erythroid (CFU-E) and burst-forming units-erythroid (BFU-E) in the bone marrow of nine cases was extremely variable and did not allow any conclusion regarding the pathogenesis of this anemia. An IgG inhibitor of growth of erythroid colonies or bursts was detected in 8/12 cases. This IgG inhibitor had no effect on the growth of granulocyte-macrophage colonies. Further studies on its mode of action indicated that the IgG did not have antierythropoietin antibody properties and did not affect the mature erythroblasts, as shown by a lack of inhibition of their responses to erythropoietin and by the lack of a cytotoxic effect on 59Fe-labeled erythroblasts. In four cases, preincubation studies demonstrated a direct effect of the IgG on the CFU-E, which was complement-mediated in three cases and complement- independent in one case. In two other cases, the IgG suppressed the growth of normal BFU-E only without affecting the growth of CFU-E. The IgG inhibitor was no longer present after the erythroblastopenia had remitted. These studies demonstrate that in the majority of cases of TEC, an IgG suppressor of erythropoiesis in vitro is present. Its mode of action is heterogeneous regarding its requirement for complement. Its target cells are the earlier or later erythroid progenitors, BFU-E or CFU-E, but not the differentiated erythroblasts.
Abnormal autologous mixed lymphocyte reaction in autoimmune thrombocytopenic purpura
Authors: Zinberg M , Francus T , Weksler ME , Siskind GW , Karpatkin S 
Published: October 2019

Abstract
The autologous mixed lymphocyte reaction (auto-MLR) measures the ability of non-T cells to stimulate autologous T cells to proliferate in tissue culture. The auto-MLR was studied in 11 patients with autoimmune thrombocytopenic purpura (ATP). Seven patients had decreased auto-MLR, which averaged 4440 +/- 3364 cpm (SEM) compared to 15,360 +/- 6905 cpm for simultaneously studied controls. The average of the ratios of cpm incorporated by patients/cpm incorporated by control subjects was 0.20 +/- 0.06 (p less than 0.01). Serum from all 7 patients with low auto-MLR decreased the auto-MLR of normal subjects by an average of 56% +/- 8.5 (p less than 0.001). Preliminary results indicate that the inhibitory effect was mediated by a component of the IgG immunoglobulin fraction of serum. Sera from normal persons and from ATP patients with normal or high auto-MLR did not affect the auto-MLR of normal subjects. It was further shown that non-T cells from 3 of 5 patients with decreased auto-MLR failed to stimulate allogeneic T cells normally. It is concluded that many patients with ATP have decreased auto-MLR apparently due to the presence of a serum blocking factor and, perhaps, a defective stimulatory capacity of non-T-cells.
A new case of deficiency of the R binder for cobalamin, with observations on minor cobalamin-binding proteins in serum and saliva
Authors: Carmel R 
Published: October 2019

Abstract
A patient presented at the age of 77 yr with a low serum cobalamin level. Subsequent study showed that he had persistently very low R binder (TC I) cobalamin-binding capacity in serum (less than 5 ng/liter versus 213 +/- 171 ng/liter in normal controls), and that almost all of his endogenous serum cobalamin was carried by TC II instead of TC I. His saliva also demonstrated virtually undetectable R binder (binding capacity of 31–38 ng/liter versus 41,690 +/- 23,820 ng/liter for control subjects). Unlike previous cases of R binder deficiency, he seemed to maintain normal serum cobalamin levels while receiving monthly cyanocobalamin injections. This and his normal serum unsaturated binding capacity were due to elevated TC II levels. TC II carried 72%-98% of his endogenous cobalamin, the rest being attached to minor binders. As incidental findings, the patient had a serum component of molecular weight of approximately 70,000 that carried 7%- 8% of his endogenous cobalamin and also had small quantities of TC II demonstrable in his saliva. Both these heretofore unappreciated minor peaks were identifiable because of the lack of R binder. The patient's clinical presentation supports the conclusion that R binder deficiency is a benign disorder. Whether his mild hypersegmentation of neutrophils and neuropathy were related to the R binder deficiency or, more likely, arose from coexisting folate deficiency and alcohol abuse, the overall picture contrasts dramatically with the severe clinical sequelae of TC II deficiency.
Pseudothrombocytopenia: an immunologic study on platelet antibodies dependent on ethylene diamine tetra-acetate
Authors: Pegels JG , Bruynes EC , Engelfriet CP , von dem Borne AE 
Published: October 2019

Abstract
Antibodies specifically reacting with platelets only in the presence of EDTA, by the platelet immunofluorescence test, were found in the serum of 20 patients with pseudothrombocytopenia due to in vitro EDTA- dependent platelet agglutination. These antibodies reacted optimally at 0–4 degree C. In 19 patients, IgG antibodies were detected; in 8 patients, IgM or IgA antibodies were also found. In one patient, only IgM antibodies were found. In 14 patients, the IgG antibodies were IgG1, but IgG2, IgG3, and IgG4 antibodies were also seen in 7 patients. The reaction of platelets with the antibodies was detectable in the presence of Na2EDTA, the K, Ca, and Mg salts of EDTA, and K2EGTA. F(ab')2 or F(ab') fragments of the IgG antibodies reached as strongly as the intact antibodies, indicating that the reaction is dependent on the antibody-combining site. The EDTA-dependent antibodies did not show platelet-group specificity. However, platelets from patients with Glanzmann disease did not react with the antibodies.
Release of platelet-activating factor from HL-60 human leukemic cells following macrophage-like differentiation
Authors: Camussi G , Bussolino F , Ghezzo F , Pegoraro L 
Published: October 2019

Abstract
Platelet-activating factors (PAF), a phospholipid mediator of anaphylaxis, is also known to be released in vitro from both phagocytic polymorphonuclear neutrophils (PMN) and monocytes in response to a variety of stimuli. The fact that human myeloid cells of the HL-60 line can be made to differentiate in vitro into macrophage-like cells by 12- O-tetradodecanoylphorbol-13-acetate (TPA) prompted us to investigate the generation and release of PAF during this transformation. Both passive release of PAF at pH 9.5, and active release, following phagocytosis of C3b- and C3d-opsonized yeast spores, and stimulation with C5a anaphylatoxin from untreated and TPA-treated HL-60 cells, PMN, and plastic-adherent normal human monocytes were studied. It was found that after 3 days of TPA treatment, HL-60 cells released PAF following phagocytosis of C3b- and C3d-opsonized yeast spores. Inhibition of PAF release by a selective inhibitor of phospholipase A2 and labeling of PAF with sodium 14C-acetate indicated that PAF generation is a two-step process: (1) release of PAF precursor from cell membranes and (2) its acetylation. A model for the in vivo study of mechanisms and metabolic events involved in PAF generation and release could perhaps be built on these findings.
Ability of thrombocytes to acquire HLA specificity from plasma
Authors: Lalezari P , Driscoll AM 
Published: October 2019

Abstract
Thrombocytes from HLA-A1 and HLA-A2 negative donors were incubated with plasma obtained from respective HLA-positive donors. The plasma-treated thrombocytes were shown to acquire HLA specificity of the plasma as demonstrated by their ability to absorb lymphocytotoxic activity of monospecific anti-HLA sera. The data are consistent with the hypothesis that the expression of HLA antigens in cells of hematopoietic origin diminishes in the course of differentiation and maturation and that the expression of HLA antigens on thrombocytes is primarily due to absorption of these antigens from plasma.
Cytologic characterization and significance of normal karyotypes in t(8;21) acute myeloblastic leukemia
Authors: Berger R , Bernheim A , Daniel MT , Valensi F , Sigaux F , Flandrin G 
Published: October 2019

Abstract
A cytologic and cytogenetic study of 10 cases of acute myeloblastic leukemia with maturation and t(8;21) translocation is reported. Despite a certain polymorphic appearance, the characteristic cytologic picture, consisting essentially of large myeloblasts with an abundant cytoplasma containing a large Auer rod, allowed the presence of the chromosome anomaly to be predicted. t(8;21) translocation was attended by the loss of a sex chromosome in 7 of 10 cases. The comparative study of mitoses using cytologic and cytogenetic techniques showed that cells exhibiting normal karyotypes were essentially erythroblasts. This finding suggests that the chromosome anomaly does not affect all the bone marrow cell lines. After short-term culture, the percentage of normal karyotype mitoses diminished, as did the number of mitoses in erythroblasts.
Expression of Ia-like and HLA-A,B antigens on human multipotential hematopoietic progenitor cells
Authors: Fitchen JH , Le Fevre C , Ferrone S , Cline MJ 
Published: October 2019

Abstract
Human multipotential hematopoietic progenitor cells can be assayed by their ability to form colonies of mixed cell lineages in vitro. These cells display la-like antigens and HLA-A,B,C antigens as evidenced by inhibition of colony formation by specific monoclonal antibodies and complement.
Human platelet surface binding of endogenous secreted factor VIII-von Willebrand factor and platelet factor 4
Authors: George JN , Onofre AR 
Published: October 2019

Abstract
Washed human platelets in buffers containing either 2 mM Ca++ or 4 mM EDTA were stimulated by human alpha-thrombin to induce secretion. The binding of two endogenous secreted proteins, factor-VIII-related protein (VIII-R) (von Willebrand factor) and platelet factor 4, was measured by reacting thrombin-treated and control platelets with specific antibodies to these proteins, then quantifying antibody binding with 125I-staphylococcal protein A. Both of these granule proteins were associated with the platelet membrane surface by a calcium-dependent mechanism after thrombin-induced secretion. This ability to bind endogenous secreted proteins to the plasma membrane surface may provide a mechanism by which the platelet can concentrate and organize its secreted proteins for subsequent physiologic reactions.
Gamma (immune) interferon production by leukocytes from a patient with a TG cell proliferative disease
Authors: Hooks JJ , Haynes BF , Detrick-Hooks B , Diehl LF , Gerrard TL , Fauci AS 
Published: October 2019

Abstract
We report a patient with a disease characterized by proliferation of T cells with Fc receptors for IgG (TG). However, unlike lymphoid cells from normal individuals or from patients with other lymphoid malignancies, the patient's lymphocytes spontaneously produced gamma interferon (IFN-gamma) in vitro. The peripheral lymphocytes consisted of 95% TG cells, which exhibited the morphological characteristics of T- cell chronic lymphocytic leukemia (CLL) and were normal on cytochemical and chromosome analysis. The majority of TG cells were OKT3+, OKT8+, and OKT4-, 3A1-. These cells failed to express suppressor cell activity and displayed depressed levels of natural killer activity, but mediated antibody-dependent cell-mediated cytotoxicity. The spontaneous production of IFN-gamma by human peripheral lymphoid cells as demonstrated in this study may serve as a probe for studying the relationship between IFN-gamma and the proliferation of human T-cell subsets.