bluuebunny/crossref_metadata_2025_split huggingface dataset:
Kinetics and sites of destruction of 111indium‐oxine‐labeled platelets in idiopathic thrombocytopenic purpura: A quantitative study
Authors: Heyns A. Dup. , Lötter M. G. , Badenhorst P. N. , De Kock Felicity , Pieters H. , Herbst C. , van Reenen O. R. , Kotze H. , Minnaar P. C. 
Published: February 2007

AbstractKinetics andw quantification of the sites of destruction of 111‐Indium‐oxine‐labeled autologous platelets were investigated in eight patients with idiopathic thrombocytopenic purpura. The mean platelet count was 17 ± 9 × 109/liter; platelets were separated by differential centrifugation and labeled with 5.6 ± 2.5 MBq 111In. Whole body and organ 111In‐platelet distribution was quantitated with a scintillation camera and a computer‐assisted imaging system acquisition matrix. Areas of interest were selected with the computer and organ 111In‐radioactivity expressed as a percentage of whole body activity. Mean platelet survival was 49.5 ± 29.6 hr and the survival curves were exponential. Equilibrium percentage organ 111In‐radioactivity was (normal values in parentheses): spleen 33.7 ± 8.8(31.1 ± 10.2); liver 16.1 ± 9.5(13.1 ± 1.3); thorax 22.8 ± 3.7(28.2 ± 5.6). Percentage organ 111In‐activity at the time when labeled platelets had disappeared from the circulation was: spleen 44.5 ± 16.4 (40 ± 16); liver 16.0 ± 11.5 (32.4 ± 7.2); thorax 19.7 ± 6.0 (17.7 ± 10.3). Thorax activity corresponds to bone marrow radioactivity. Three patterns of platelet sequestration were evident. Three patients had mainly splenic sequestration, two mainly hepatic sequestration, and three diffuse reticuloendothelial system sequestration with a major component of platelets destroyed in the bone marrow. Splenectomy was performed in two patients. The pattern of 111In‐platelet sequestration was not predictive of response of glucocorticoid therapy or indicative of the necessity for splenectomy. Quantitative 111In‐labeled autologous platelet kinetic studies provide a new tool for the investigation of platelet disorders.
Measurement of human serum erythropoietin by erythroid colony‐forming technique using fetal mouse liver cells
Authors: Okamoto Takahiro , Kanamaru Akihisa , Hara Hiroshi , Nagai Kiyoyasu 
Published: February 2007

AbstractA quantitative bioassay for serum erythropoietin in anemic patients was established with erythroid colony‐forming technique using methyl cellulose. Fetal mouse liver contains many erythropoietin‐dependent erythroid colony‐forming cells (CFU‐E) with the concentration as well as sensitivity to erythropoietin being the highest at 13–14 days of gestation. Linearity in the erythropoietin response curve for the number of colonies from CFU‐E in fetal livers of 13–14 days gestation was obtained for erythropoietin concentrations in the culture medium of 3.9 to 125 mU/ml. When newborn liver cells were used instead, the linearity occurred at 31.2 to 250 mU/ml. Measurement of erythropoietin by the erythroid colony‐forming technique for sera from patients with various anemias was compared with that of the assay method using radioiron incorporation into heme in the suspension culture of fetal mouse liver cells. There was a good correlation between the values of both assay methods (r = 0.856, p < 0.001). Also, levels of erythropoietin measured by this assay method showed the expected inverse relationship to the hemoglobin concentration in the sera from the patients.
A case of chronic aneosinocytosis
Authors: Telerman Adam , Amson Robert B. , Delforge Alain , Collard Elizabeth , Stryckmans Pierre A. 
Published: February 2007

AbstractA 59‐year‐old woman, after complete recovery from an episode of drug‐induced agranulocytosis, was found to sustain a chronic absence of recognizable mature and immature eosinophils in blood and bone marrow during a follow‐up period of 8 years. Her bone marrow and peripheral blood cells cultured in vitro were able to produce normal numbers of eosinophil colonies. The present disorder was thus not caused by a lack of EO‐CFU‐C, nor by a defect in EO‐CSA, nor by an immunologically mediated mechanism acting on mature or immature eosinophils or on EO‐CSA producing cells. It is suggested that the marrow was not able to differentiate normally along the eosinophil pathway, possibly owing to a microenvironmental defect or, less likely, an intrinsic stem cell defect.
Phosphofructokinase (PFK) deficiency due to a catalytically inactive mutant M‐type subunit
Authors: Zanella A. , Mariani M. , Meola G. , Fagnani G. , Sirchia G. 
Published: February 2007

AbstractA case of M‐type PFK deficiency due to the synthesis of a structurally abnormal and catalytically inactive M‐subunit was reported. PFK activity was reduced (39% of normal) in red cells, normal in leukocytes and platelets, and absent in muscle. The red cell enzyme was not inhibited by antiserum to human muscle PFK and displayed normal biochemical properties (Km for ATP and fructose‐6‐phosphate, storage stability at + 4°C and −80°C, optimum pH, electrophoretic pattern and molecular weight).The complete lack of PFK activity in muscle was confirmed on both histological preparations and muscle extracts. Double immunodiffusion analysis using an antinormal M‐PFK serum revealed that the enzyme molecule was present and immunologically identical with normal, although it was catalytically inactive. The muscle abnormality was also confirmed by electromyography, ischemic exercise testing, histochemistry and electron microscopy. Moreover, PFK activity was investigated in myoblast cultures maintained up to 25 days, and it was found to be absent.
Phenotypic heterogeneity of human T‐cell malignancies: Demonstration by monoclonal antibodies and cytochemical markers
Authors: Knowles Daniel M. , Halper James P. , Machin Genevieve A. , Byeff Peter , Mertelsman Roland , Chess Leonard 
Published: February 2007

AbstractThe present study sought to delineate the phenotypic heterogeneity of the human T‐cell malignancies. Twenty T‐cell neoplasms were investigated for reactivity with the OKT hybridoma monoclonal antibodies and expression of acid α‐naphthyl acetate esterase (ANAE), β‐glucuronidase (BG), and acid phosphatase (AP) activity. Twelve cases (Mycosis fungoides, Sezary syndrome, cutaneous T‐cell lymphoma, chronic lymphocytic leukemia) were OKT3+T4+, ie, expressed the phenotype commonly associated with mature T‐helper cells. These cases were further divisible into ANAE+BG+ (6 cases), ANAE‐BG+ (5 cases), and ANAE‐BG‐ (1 case) phenotypes. In contrast to the 12 OKT3+T4+ cases, the remaining 8 cases showed considerable inter‐ and intratumor heterogeneity with respect to reactivity with the OKT antibodies. Six of these cases (acute lymphoblastic leukemia, lymphoblastic lymphoma) expressed phenotypes consistent with various intrathymic stages of T‐cell differentiation. Five of the latter 6 cases were AP+BG+ANAE‐, analogous to the majority of normal cortical thymocytes; an OKT3+T4‐T8+T10+ neoplasm was ANAE+, analogous to normal medullary thymocytes. Two cases expressed the previously undescribed OKT3+T4‐T8‐T10+ phenotype. These studies demonstrate that the T‐cell malignancies are divisible into phenotypes which correspond to normal maturational stages of T‐cell differentiation and functionally distinct T‐cell subsets. Phenotypic analysis of the human T‐cell malignancies may provide a basis for understanding their biological heterogeneity and may aid in the identification of transitional stages of T‐cell differentiation and minor T‐cell subsets.
Familial T‐cell lymphoblastic lymphoma: Association with von recklinghausen neurofibromatosis and gardner syndrome
Authors: Kaplan Joseph , Cushing Barbara , Chang Chung‐Ho , Poland Ronald , Roscamp Jacqueline , Perrin Eugene , Bhaya Nirmala 
Published: February 2007

AbstractA family is described in which three of seven siblings developed a T‐cell lymphoblastic lymphoma. Tumor cells formed rosettes with sheep erythrocytes, lacked surface Ig, and expressed human T‐ but not B‐lymphocyte antigens. They lacked the enzyme terminal transferase suggesting a lymph node rather than thymic T‐cell origin. Autopsy findings supported this conclusion. All three sibs, like their father, had numerous cafe'‐au'lait spots indicative of Von Recklinghausen neurofibromatosis. One child had a subcutaneous fibroma, and another had multiple colonic polyps and exostoses characteristic of Gardner syndrome. Both are autosomal dominant conditions known to predispose to malignancies. The genetic factors responsible for these conditions may also predispose to the development of “postthymic” T‐cell lymphoblastic lymphoma.
Acute myeloid leukemia as the first hematologic manifestation of fanconi anemia
Authors: Auerbach Arleen D. , Weiner Michael A. , Warburton Dorothy , Yeboa Kwame , Lu Li , Broxmeyer Hal E. 
Published: February 2007

AbstractA six‐year‐old girl with Fanconi anemia (FA) developed acute myeloid leukemia (AML) as the first hematologic manifestation of the syndrome. She remains in remission 18 mo after diagnosis although her management is complicated by unusual sensitivity to chemotherapeutic agents. Marrow cells studied prior to initiation of leukemia therapy showed increased chromosome breakage and an abnormal clone in which a number 7 and a number 8 chromosome were replaced by two marker chromosomes. During the present remission her cultured lymphocytes, bone marrow cells, and fibroblasts showed increased “spontaneous” chromosome breakage as well as enhanced sensitivity to the clastogenic effect of the difunctional alkylating agent diepoxybutane (DEB), features characteristic of FA. Eight months into remission 50% of her marrow cells comprised an abnormal clone, which was monosomic for the number 7 chromosome but had both copies of number 8; in addition a variable number of unique marker chromosomes were present in clonal as well as nonclonal cells. This same marrow sample, upon culture, showed an abnormal growth pattern of CFU‐GM, absence of detectable CFU‐GEMM and BFUe, non‐responsiveness of CFU‐GM to inhibition by acidic isoferritins, increased bone marrow acidic isoferritin inhibitory activity, and absence of detectable bone marrow cell‐derived GM‐CSF. The implications of these findings to leukemogenesis in FA are discussed.
Rosette formation between chronic T‐lymphocytic leukemia cells and macrophages: A facsimile of normal T‐lymphocyte—macrophage cooperation
Authors: Jennette J. Charles , Benson John D. , Wilkman Alice S. 
Published: February 2007

AbstractNeoplastic T lymphocytes from a patient with chronic T‐lymphocytic leukemia formed rosettes with macrophages. It is proposed that this phenomenon represents retention by these abnormal cells of the normal ability of T lymphocytes to associate physically with macrophages. Recognition of rosettes between benign macrophages and leukemia or lymphoma‐derived lymphocytes may indicate T‐cell lineage of the neoplastic lymphoproliferation.
Aryl sulfatase a in acute nonlymphocytic leukemia
Authors: Pillai M. V. , James G. W. , King M. E. , Fox F. W. , Choi S. C. 
Published: February 2007

AbstractTo ascertain whether estimation of urinary aryl sulfatase A (ASA) is a reliable noninvasive technique for monitoring disease activity in adult acute nonlymphocytic leukemia, we studied the excretion pattern of this enzyme during active disease, partial remission, and complete remission in ten patients and compared the results with those of ten healthy volunteers. There was no significant difference in enzyme excretion between complete remission cases and the control group. Patients with partial remission and active disease had significantly different urinary enzyme levels from each other and from the control and complete remission groups.Estimation of urinary ASA is a useful noninvasive method of monitoring disease activity in adult acute nonlymphocytic leukemia.
Neutrophil marrow cellularity in neutropenia
Authors: Dancey John T. , Brubaker Leonard H. 
Published: February 2007

AbstractIn order to identify individuals in whom marrow abnormalities might be contributing to or responsible for neutropenia, we quantitatively examined the number and distribution of cells comprising neutrophil marrow in patients with blood neutrophils less than 2,000/μl. Neutrophil marrow cellularity was determined from ferrokinetic estimation of normoblast numbers and neutrophil‐normoblast ratios obtained from marrow biopsy sections. Only two of 30 patients exhibited the change in cellularity expected of a normal marrow responding to removal of circulating neutrophils: reduced numbers of segmented cells, an expanded mitotic pool, and a normal ratio of metamyelocytes and band forms to promyelocytes and myelocytes. Twenty‐three patients had basal mitotic pool size or increased numbers of segmented marrow cells despite neutropenia, a hypoplastic mitotic pool, or a reduction in the number of metas and bands relative to promyelocytes and myelocytes. The results in individual patients were consistent with hypoplasia, subnormal proliferative or release responses, loss of cells during ontogeny, or combinations thereof. In five cases the results could not be so classified. Clinical observations seldom predicted marrow cellularity. Diverse disorders of marrow function appear to be common among neutropenic patients. Neutropenia constitutes a rich field for study of neutrophil marrow physiology.
The role of autologous helper and suppressor T cells in the regulation of human granulopoiesis
Authors: Barr Ronald D. , Stevens Catherine A. 
Published: February 2007

AbstractHelper and suppressor T cells play a physiological role in immunoregulation and possibly in other biological systems. Previous studies have suggested that unfractionated T cells influence human blood cell formation in vitro. Subpopulations of such cells (TM and TG cells) were prepared by immune ox erythrocyte resetting techniques. Co‐cultures were established in semi‐solid agar with autologous null lymphocytes as a source of granulocyte—macrophage progenitor cells (CFU‐GM). TM (helper) cells produced an increase and TG (suppressor) cells a decrease in the expression of CFU‐GM as reflected in the number of GM colonies generated by comparison with cultures of null lymphocytes alone. These data point to a contribution from T lymphocyte subpopulations to the physiological regulation of human granulopoiesis.
Long survival in sickle cell anemia
Authors: Shurafa Muhammad S. , Prasad Ananda S. , Rucknagel Donald L. , Kan Yuet Wai 
Published: February 2007

AbstractTen patients with sickle cell anemia surviving beyond the age of 40 were compared to 11 subjects with sickle cell anemia who died before that age. Hematologic and biochemical data as well as clinical and physical parameters of the two groups were compared. There was no statistically significant difference between the two groups with respect to the degree of anemia, severity of hemolysis, or hemoglobin A2 levels. A statistically significant difference was found between the two groups with respect to fetal hemoglobin, red cell zinc, and secondary sexual characteristics. Although the long survivors had fewer crises per year than the short survivors, the long‐term complications such as leg ulcer, congestive heart failure, and aseptic necrosis of the hip were more common in the older patients. Cerebrovascular accidents were the cause of death in 9/11 short survivors and are absent in the long survivors. The α/β chain synthesis ratio was normal in the long‐survivor group and α gene mapping in five subjects in that group revealed the genotype αα/αα in four and — α/αα in one. The older patients as a group had higher red cell zinc values. The secondary sexual characteristics were also better developed in the older subjects. The overall significance of zinc status and of a higher HbF on longevity of sickle cell anemia patients remains unknown.
Gamma‐chain heterogeneity of fetal hemoglobin in nonblack β‐ and δβ‐thalassemia and hpfh heterozygotes and homozygotes
Authors: Efremov G. D. , Ibarra B. , Gurgey A. , Sukumaran P. K. , Altay C. , Huisman T. H. J. 
Published: February 2007

AbstractThe fetal hemoblobin (Hb F) of a few hundred nonblack patients with a heterozygosity or homozygosity for β‐thalassemia (β‐thal), δβ‐thalassemia (δβ‐thal), and some forms of the hereditary persistence of Hb F (HPFH) was isolated by DEAE‐cellulose chromatography and further characterized by high‐pressure liquid chromatography. Quantitative data for the three types of γ chain (AγT, AγI, and G‐γ) were compared with those obtained for the Hb F from black patients with similar conditions.The Gγ chain levels in nonblack β‐thal heterozygotes varied greatly and did not fall into two distinct groups with high or low levels, as has been observed in blacks. The level of the AγT chain in AγT heterozygotes did not differ significantly when this anomaly was in cis or in trans to the β‐thal determinant. Beta‐thalassemia homozygotes from Turkey and Yugoslavia, had Gγ values varying between 40% and 80%. Only 13 of 34 patients carried the AγT gene. Nine were AγT heterozygotes with an AγT/total Aγ level averaging 39% and four were AγT homozygotes.The Gγ chain levels in nonblack δβ‐thal heterozygotes varied between 28% and 46%. An additional AγT chain heterozygosity in cis to the δβ‐thal determinant demonstrated that over 90% of the γ chains is produced by genes in cis to this anomaly. Analyses of members of two relatively large families with β‐thal, δβ‐thal, and the AγT chain heterozygosities and homozygosities occurring in different combinations allowed a more or less quantitative evaluation of the production of γ‐chain genes in cis or in trans to either of the two types of thalassemia determinants. Such calculations were possible both in simple heterozygotes and in persons with the β‐δβ‐thalassemia condition.
Activated partial thromboplastin time after heparin removal (aPTT/HR) in a new scheme of anticoagulant monitoring
Authors: Branson Herman E. , Gupta Naveen , Slater Lewis M. , Fagin Awilda , Armentrout Steven A. 
Published: February 2007

AbstractActivated partial thromboplastin time after heparin removal (aPTT/HR), a test employing anion exchange chromatography, was devised as an alternative to the prothrombin time after heparin removal (PT/HR) to monitor simultaneous anticoagulation with heparin and coumarins. The potential utility of the aPTT/HR was assessed by performing parallel PTs and aPTTs on 62 consecutive plasmas from coumarin‐treated outpatients. All samples had 0.2 units/ml of heparin added and then removed to see if the maneuver influenced therapeutic group assignment. In no instance did reassignment occur. A conditional Irwin‐Fisher test (P = 0.000604) and a special multinomial trial analysis (P = 0.002) indicated that the aPTT would be at least comparable to the PT for following coumarin antithrombotic prophylaxis. Since the heparin removal procedure had no influence on therapeutic categorization, the same statistical proof could be applied to the relationship between aPTT/HR and PT/HR. This study indicates that the aPTT can be used to monitor all stages of heparin and/or coumarin anticoagulation.
Red cell enzyme activity during blood storage and reactivation of phosphofructokinase
Authors: Noble Nancy A. , Tanaka Kouichi R. , Myhre Byron A. , Johnson Delores E. 
Published: February 2007

AbstractActivities of 21 red cell enzymes were measured in 15 units of CPD‐A1 anticoagulated blood before and after 35 days of storage. Paired t‐tests revealed that five of the enzyme activities decreased significantly after 35 days of storage (P < 0.05) while four increased significantly after storage (P < 0.05). The most striking change was in phosphofructokinase (PFK), which decreased an average of 33% (P < 0.005). Mean lactate dehydrogenase activity increased to 128% of its zero‐day activity (P < 0.005). Percent red cell survival, determined by reinfusion of a 51Cr‐tagged aliquot of stored blood into the autologous donor, was highly correlated with 35‐day ATP levels (r = 0.97; P < 0.001). No significant relationships between two measures of red cell survival and any of the nine significantly altered enzymes were found.Studies of reactivation of PFK in hemolysates using 4 mM concentrations of numerous compounds indicate that the compounds or combinations of compounds used which reactivate PFK significantly all contain at least one high‐energy phosphate group. These data suggest that loss of PFK activity during storage may be due to loss of organic phosphates. Whether the changes described have an important role in blood transfusion remains to be determined.
Acquired cyclic neutropenia: Successful treatment with prednisone
Authors: Rodgers George M. , Shuman Marc A. 
Published: February 2007

AbstractA previously healthy woman developed severe, periodic neutropenia after ingestion of phenylbutazone. Oscillations in the monocyte count and hemoglobin concentrations also occurred. The neutropenic episodes were associated with severe bacterial infections requiring hospitalization. Lithium induced a transient interruption in the neutropenia, but continued use was ineffective. Prednisone in a dosage of 100 mg daily successfully interrupted the neutrophil cycling and prevented infection. The patient has remained in remission on 10 mg of prednisone on alternate days.
Iron overload in hereditary spherocytosis: Association with hla‐linked hemochromatosis
Authors: Edwards Corwin Q. , Skolnick Mark H. , Dadone Mary M. , Kushner James P. 
Published: February 2007

AbstractHeavy iron loading is a rare clinical finding in patients with hereditary spherocytosis. A pedigree was studied in which the proband, a 38‐year‐old man, had both hereditary spherocytosis and overt hemochromatosis. He had never received blood transfusions. The 8‐year‐old son of the proband also had hereditary spherocytosis and mildly increased iron stores. The 39‐year‐old brother of the proband did not have spherocytosis but did have overt hemochromatosis. Since hemochromatosis is transmitted as an HLA‐linked autosomal recessive disorder, HLA haplotypes serve as markers of hemochromatosis alleles. In this pedigree only individuals with two hemochromatosis alleles (homozygosity) had heavy iron loads, whether hereditary spherocytosis was present or not. The presence of hereditary spherocytosis may have contributed to the magnitude of the iron loading but was not a major factor. Our findings suggest that nontransfusional hemochromatosis found in association with hereditary spherocytosis is due primarily to homozygosity for hemochromatosis.
Chronic lymphocytic leukemia: A monoclonal disease
Authors: Solanki Dilip L. , McCurdy Paul R. , Macdermott Richard P. 
Published: February 2007

AbstractA black woman with chronic lymphocytic leukemia (CLL) was found to have monoclonal B lymphocytes with one type of surface immunoglobulin and one variant of G6PD (glucose‐6‐phosphate dehydrogenase) (G6PD A). Erythrocytes and T cells contained both G6PD A and G6PD B and hence were of polyclonal origin. The CLL cells in this patient likely arose from a developmental stage later than the step of differentiation into T and B lymphocytes. Furthermore, her erythrocytes did not arise from a stem cell affected by the CLL process.
Transfer of pelger‐huet anomaly by bone marrow transplantation
Authors: Barton Ann M. , Macleod Beth C. , Thomas E. D. 
Published: February 2007

AbstractA marrow transplant was performed on a patient with refractory acute myelomonocytic leukemia using marrow from a sibling donor with congenital Pelger‐Huet anomaly (P‐H). The patient, free of P‐H prior to transplant, subsequently acquired the anomaly, thus providing a useful means of demonstrating engraftment of donor cells.