bluuebunny/crossref_metadata_2025_split huggingface dataset:
Chronic granulocytic leukemia: Correlation of elastic transformation type with karyotypic evolution
Authors: O'Malley F. M. , Garson O. Margaret 
Published: February 2007

AbstractOver a 3‐year period, 26 patients with Philadelphia chromosome‐positive chronic granulocytic leukemia were studied cytogenetically in both the chronic and blastic transformation phases of the disease; a further three patients were studied only after blastic transformation. Sixteen were considered to have adequate evidence of the type of transformation and form the basis of this report, where chromosome changes have been correlated with the morphological type of blastic transformation.Seven patients developed a myeloblastic transformation, seven a lymphoblastic transformation, and two an erythroblastic transformation. All patients in the myeloid group acquired one or more of the nonrandom changes associated with CGL blastic transformation, viz. +8,i(17q), + 19,+22q‐. Patients in the lymphoblastic group acquired structural abnormalities, apparently random in nature and usually in a small percentage of cells. The two patients with erythroblastic transformation developed markedly hyperdiploid cells (> 50 chromosomes) with both numerical and structural abnormalities. Patients in the lymphoblastic group appeared to have a slightly better prognosis than the myeloid group, whilst the patients with erythroblastic transformation had a very poor prognosis.
Hypertonic cryohemolysis of pathologic red blood cells
Authors: Streichman S. , Kahana E. , Tatarsky I. 
Published: February 2007

AbstractHuman erythrocytes suspended in hypertonic solutions undergo hemolysis when the temperature of the suspension is changed from 37°C toward 0–4°C. It has been suggested that the hypertonic environment causes some proteins of the skeletal network to be changed in such a way that their normal adaptation to temperature changes is prevented, thus resulting in cryohemolysis. In the present study, we compared the cryohemolysis of some pathologic red blood cells in hypertonic sucrose and NaCl to normal cells. Erythrocytes of hereditary spherocytosis (HS) were found to be significantly more fragile than all others in hypertonic sucrose, while they behaved normally in hypertonic NaCl. In contrast, erythrocytes of thalassemic patients showed decreased susceptibility to cryohemolysis, both in hypertonic sucrose and in NaCl. Autoimmune hemolytic anemia samples behaved like normal samples, both in NaCl and in sucrose. The erythrocytes of congenital dyserythropoietic anemia‐type II patients showed two types of cryohemolysis; one pattern was similar to that of HS, and the other one presented normal levels in sucrose and reduced levels in NaCl. The different patterns of cryohemolysis described for the pathologic cells are thought to reflect different lesions in the membranes of the erythrocytes of the various hemolytic disorders. It is hoped that studying the cryohemolysis of abnormal red cells may contribute some illumination as to molecular interactions in intact cells in health and in disease.
Marrow mass and distribution in murine skeletons cleaned by beetles as compared to cut up carcasses and a further simplification of the latter technique
Authors: Boggs Dane R. , Patrene Kenneth D. 
Published: February 2007

AbstractDistribution of 59Fe into various bone groups of the complete murine skeleton was studied using two methods of dividing up the bones: 1) our previously reported technique of simply cutting up a skinned, eviscerated carcass and 2) separating bones from skeletons cleaned of overlying tissue by beetles, Dermestes species. The total percentage of injected 59Fe recovered in the sum of all skeletal parts, the percentage of total skeletal 59Fe found in each bone group, and the overall accuracy of determining these values were quite similar for the two techniques. The only statistically significant difference shown was a modest decrease in the percentage of total skeletal iron found in ribs plus sternum plus cervical and thoracic spine in beetle‐cleaned as compared to cut up groups and we would not consider this to be of biological significance. Cutting up carcasses is the simpler of the two techniques but there are circumstances in which beetle digestion would be advantageous.In addition, we collected data on the reproducibility and precision of determining the percentage of 59Fe injected which is found in a „pulled off”︁ foreleg plus scapula and of the distribution of 59Fe within three cut up pieces from the leg and within the scapula. These data can be used as a measure of overall changes in marrow mass and/or distribution, or at least they can be used as a screening procedure to detect such. This simple procedure adds potentially useful values for fully interpreting hematopoietic changes in the mouse.
Erythrocyte incorporation of ingested stable isotope of iron (58Fe)
Authors: Janghorbani Morteza , Ting Bill T. G. , Fomon Samuel J. 
Published: February 2007

AbstractBecause of a possible hazard from the use of radioisotopes to determine iron absorption by infants, the use of stable isotopes for this purpose has much appeal. We have applied the method of inductively coupled plasma mass spectrometry (ICP/MS) to determine the mass ratio, 58Fe/57Fe, in blood before and after oral administration of 58Fe. From the increase in erythrocyte enrichment with 58Fe, we have calculated percentage absorption of iron. We have shown that the coefficient of variation of measured mass isotope ratio is 0.1‐1.0%, depending on the conditions of the measurement.The method has been applied to a feasibility study involving four infants. Each infant was given 58Fe either as a single dose or as one dose on each of two consecutive days. Each dose provided 1.945 mg iron and 1.440 mg 58Fe. Samples of blood were obtained before isotope administration and at 14, 42, and 60 days thereafter. Isotopic analysis of the samples demonstrates that this approach results in a sufficiently large isotope enrichment to permit satisfactory measurement of iron availability.It is concluded that this new method is highly promising for studies of iron availability in infants and children.
Determination of ectoenzyme activities in leukemic cells and in established hematopoietic cell lines
Authors: Morell Andreas , Losa Gabriele , Carrel Stephan , Heumann Didier , Von Fliedner Vladimir E. 
Published: February 2007

AbstractEctoenzyme activities were determined in peripheral blood cells from patients with acute leukemias, from normal controls, and in cells of hematopoietic cell lines. In common acute lymphoblastic leukemia, cell membrane‐associated 5′‐nucleotidase (5′‐N) activity was significantly higher than in acute T and unclassified lymphoblastic leukemias. In acute myeloblastic and myelomonocytic leukemias, cells contained significantly higher γ‐glutamyl transpeptidase (γ‐GT) activity than in lymphoblastic leukemias. Normal B lymphocytes differed from T cells and monocytes mainly in their 5′N activity, whereas in monocytes, γ‐GT activity was more pronounced than in other normal blood cells. Hematopoietic cell lines showed some distinct patterns of ectoenzyme activity. Most B cell lines had high 5′N and (Na‐K‐Mg) adenosine triphosphatase activities. In lines of myeloid origin, elevated γ‐GT values were found. In lymphoid stem cells and in T lymphoblast lines, most ectoenzyme activities were lower than in the other cell lines. In some cell lines, characteristic high‐activity marker enzymes were detected.
Acquired von willebrand syndrome due to an inhibitor specific for von willebrand factor antigens
Authors: Lazarchick John , Pappas Alexandros A. , Kizer Joette , Hall Sam A. 
Published: February 2007

AbstractA patient with acquired von Willebrand syndrome associated with polycythemia rubra vera is described. Her plasma factor VIII procoagulant activity (67 U/dl) and factor VIII‐related antigen (117 U/dl) were normal but no von Willebrand factor activity could be detected. Factor VIII crossed immunoelectrophoresis revealed decreased levels of less anodic polymeric forms of factor VIII. Mixture of her plasma or immunoglobulin G (IgG) fraction with normal plasma resulted in complete recovery of factor VIII activity and related antigen but no measurable von Willebrand factor activity, confirming the presence of an unique inhibitor. The limited specificity of this inhibitor to antigenic sites solely on the von Willebrand portion of the factor VIII bimolecular complex is distinct from all previous reports of this syndrome. This unique inhibitor offers a molecular probe to examine the von Willebrand factor: platelet interaction.
Hemopoietic marrow function in chronic neutropenia of blacks: Cure of aplastic anemia by allogeneic marrow transplantation from a neutropenic sibling donor
Authors: Ash Robert C. , Mendelsohn Larry A. , Marshall M. Ernest 
Published: February 2007

AbstractA black patient with severe aplastic anemia is described who underwent successful bone marrow transplantation from a sibling with chronic neutropenia. During an evaluation to identify a suitable donor, it was found that the majority of famiily members tested had neutropenia, with no familial history of significant infections or related hospitalizations. In vitro hemopoietic culture studies of marrow from the patient's HLA‐MLC‐matched siblings showed normal numbers of pluripotential and committed hemopoietic progenitors; in vitro hemopoietic colony formation from the patient was markedly subnormal, consistent with the clinical picture of severe aplastic anemia. Following appropriate conditioning therapy, marrow transplanted from one of these neutropenic sibs produced full hematopoietic reconstitution. Posttransplant marrow culture studies of the patient showed restoration of a normal pattern of in vitro hemopoiesis. The in vitro culture studies and clinical experience in this patient support the concept that chronic neutropenia of blacks is not primarily a marrow progenitor cell disorder but, more likely, a manifestation of a genetically determined alteration in granulocyte kinetics.
DDAVP in acquired von Willebrand syndrome associated with multiple myeloma
Authors: Takahashi Hoyu , Nagayama Reizo , Tanabe Yasuhiko , Satoh Ken , Hanano Masaharu , Mito Masao , Shibata Akira 
Published: February 2007

AbstractThe response to a single intravenous infusion of 1‐deamino‐8‐D‐arginine vasopressin (DDAVP, desmopressin) was studied in two patients with acquired von Willebrand syndrome associated with IgG‐k myeloma. Following infusion of DDAVP (0.3–0.4 μg/kg), prolonged bleeding time was normalized; plasma ristocetin cofactor activity, von Willebrand factor antigen, and factor VIII activity were remarkably increased; and high‐molecular‐weight forms of von Willebrand factor were demonstrated by crossed immunoelectrophoresis in both patients. Excellent hemostasis was achieved following adminstration of DDAVP in one patient when it was used for the treatment of gum bleeding and for the prophylaxis of bleeding during and after dental extractions. These observations suggest that DDAVP is an effective alternative to blood products for at least some patients with acquired von Willebrand syndrome in addition to patients with inherited von Willebrand disease, hemophilia A, and uremia.
Acute nonlymphocytic leukemia (M7 subtype) with erythrocytic internalization
Authors: Woessner S. , Lafuente R. , Giralt M. , Molina J. , Florensa L. 
Published: February 2007

AbstractA case of acute nonlymphocytic leukemia of megakaryocytic lineage or M7 with a conspicuous phenomenon of erythrocytic internalization present in nearly 3% of the leukemic megakaryocytic precursors (LMP) is reported. The phagocytosis was detected at light microscopy and ultrastructural levels. The erythrocyte might have reached the interior of the LMP through cytoplasmic vacuoles that are connected with the extracellular space and that can be considered rudimentary equivalents of the demarcation membrane system (DMS). Erythrocytic internalization is not a specific feature of the mononuclear phagocytic system and can be misleading in the classification of undifferentiated blast cells.
Heterogeneity in type IIB von Willebrand disease: Two unrelated cases with no family history and mild abnormalities of ristocetin‐induced interaction between von willebrand factor and platelets
Authors: Federici A. B. , Mannucci P. M. , Bader R. , Lombardi R. , Lattuada A. 
Published: February 2007

AbstractTwo patients from two separate families were diagnosed as having type IIB von Willebrand disease, because they had lifelong bleeding tendencies, prolonged bleeding times, no large von Willebrand factor multimers, and low levels of ristocetin cofactor in plasma with heightened ristocetin‐induced platelet aggregation. There was no history of bleeding, and no laboratory abnormalities were found in the parents and sibship of either propositi, in contrast with the autosomal dominant pattern of inheritance usually observed in type IIB von Willebrand disease. Abnormalities of ristocetin‐induced von Willebrand factor‐platelet interactions were less severe than in a patient from a previously reported family with type IIB von Willebrand disease studied in parallel. The peculiar features of these cases provide additional evidence of the existence of heterogeneity within this variant.
Hemostatic defects in response to aspirin challenge in hypothyroidism
Authors: Zeigler Zella R. , Hasiba Ute , Lewis Jessica H. , Vagnucci Anthony H. , West Valerie A. , Bezek Elizabeth A. 
Published: February 2007

AbstractA patient with hypothyroidism had ingested aspirin and bled profusely following dental extraction. The only abnormalities noted on coagulation survey were a prolonged aspirin bleeding time and reduced retention of platelets on glass beads. Based on this index case, an additional 12 hypothyroid patients, on no medications, were evaluated for a mild bleeding disorder, including studies with aspirin challenge. The results indicate that thrombin‐induced platelet serotonin release, following aspirin ingestion, was subnormal in most hypothyroid patients. However, only hypothyroid individuals with TSH levels of > 60 μ U/ml have an increased hemostatic sensitivity to aspirin, as measured by the bleeding time test. These findings did not appear to result from decreased synthesis of the factor VIII‐von Willebrand factor complex. As this drug is commonly ingested, the effects of aspirin on bleeding may explain the easy bruising and menorrhagia that occur in hypothyroid individuals.
Effect of serum from patients with idiopathic thrombocytopenic purpura on cultured endothelial cell growth
Authors: Nakajima Tokuo , Kakishita Eizo , Nagai Kiyoyasu 
Published: February 2007

AbstractThis study examined the effects of platelet‐derived serum factors from patients with idiopathic thrombocytopenic purpura (ITP) on human umbilical cord vascular endothelial cell growth and DNA synthesis. Vascular endothelial cell growth factors were identified in the platelet extracts, and platelet‐derived vascular endothelial cell growth factors with molecular weights of less than 10,000 were also identified in the sera. These growth factors were stable even after 30 minutes of heat treatment at 56°C and acid treatment.When ITP patient serum was added to vascular endothelial cells, their growth capacity and DNA synthesis capacity were lower than when serum from normal subjects or patients with ITP in remission was added. This indicated that in ITP, impairment of vascular integrity and abnormal repair of the vascular walls that accompany thrombocytopenia may be causes of the predisposition to hemorrhage. The decrease in ITP of platelet‐derived vascular endothelial cell growth factors with molecular weights of less than 10,000 was hypothesized to be a contributory factor.
DNA damage induced by chloramphenicol and its nitroso derivative: Damage in intact cells
Authors: Yunis A. A. , Arimura G. K. , Isildar M. 
Published: February 2007

AbstractWe have postulated that the p‐NO2 group of chloramphenicol (CAP) is the structural feature underlying aplastic anemia from this drug. In a series of studies to examine this hypothesis we have demonstrated the toxic nature of the CAP‐reduction intermediate nitroso CAP (NO‐CAP) and its damaging effect on isolated DNA in vitro. The present study was designed to examine the comparative effects of CAP, NO‐CAP, and thiamphenicol (TAP) on the integrity of DNA in intact cells. By using the alkaline elution technique of Kohn, DNA damage in the form of single strand breaks could be readily demonstrated in cultured Raji cells and in PHA‐stimulated normal human lymphocytes by small concentrations of NO‐CAP (0.05‐0.1 mM). A small but reproducible effect was observed from large concentrations of CAP (2 mM). In contrast, TAP, lacking the p‐NO2 group, was without effect.
Bone marrow transplantation in a patient with myelodysplasia associated with diffuse eosinophilic fasciitis
Authors: Tallman Martin S. , McGuffin Robert W. , Higano Celestia S. , Starkebaum Gordon , Collins Steven J. , Johnston Harold , Singer Jack W. , Perry David J. , Kunath Arthur 
Published: February 2007

AbstractA 34‐year‐old man with diffuse eosinophilic fasciitis and a hypocellular myelodysplastic syndrome underwent marrow transplantation from an HLA‐identical brother. Prompt hematopoietic reconstitution was observed, strongly suggesting that the marrow hypocellularity was caused by neither a serum inhibitory factor nor a microenvironmental disorder. The patient died of disseminated cytomegalovirus infection too early to evaluate the impact of hematopoietic reconstitution on the eosinophilic fasciitis. Nevertheless, marrow transplantation may offer a therapeutic option for those patients with this disorder who develop severe hematopoietic dysfunction and who have a suitable marrow donor.
In vitro correlates of low dose Ara‐C efficacy: Clinical, cytogenetic, and bone marrow culture analysis
Authors: Weisdorf Daniel J. , Perri Robert T. , Oken Martin M. , Miller Wesley J. , Arthur Diane C. , Machnicki Joy L. 
Published: February 2007

AbstractLow‐dose Ara‐C (10 mg/m2 subcutaneously bid) has been used as an alternative therapy for acute nonlymphocytic leukemia (ANLL) and myelodysplastic syndromes. We sought to define its therapeutic mechanism by assessing clinical and cytogenetic responses to treatment in conjunction with careful in vitro study of both morphologic and functional characteristics of bone marrow cells cultured with Ara‐C. Sixteen patients (12 ANLL, four myelodysplastic syndrome) were treated. All developed pancytopenia and 11 of 12 had bone marrow hypoplasia during treatment. Four had a meaningful clinical response while five more showed in vivo leukemic cell sensitivity to low‐dose Ara‐C. Seven showed no reponse. Cells with cytogenetic abnormalities were either decreased in number or eradicated during clinical improvement. Liquid culture of marrow mononuclear cells with Ara‐C (.033‐.333 μg/ml × 7 days) produced little evidence of morphologic or functional differentiation (ten of 11 studied). No functional maturation was observed in cells from clinically responding patients. We conclude that low‐dose Ara‐C is modestly effective for some patients with ANLL or myelodysplasia. However, no evidence for in vivo leukemic differentiation is suggested by either in vitro culture studies or cytogenetic correlates of clinical response. In vitro marrow culture studies failed to predict clinical response to Ara‐C.
Antithymocyte globulin therapy for pure white cell aplasia
Authors: Firkin F. C. , Prewett E. J. , Nicholls K. , Moran J. 
Published: February 2007

AbstractSevere neutropenia due to selective loss from the bone marrow of cells of the entire neutrophil maturation sequence developed in a patient with Goodpasture's Syndrome and was associated with serious infections complicating continuous ambulatory peritoneal dialysis. Involvement of T‐lymphocytes in the process affecting the neutrophil series was implicated by the relation between recovery from neutropenia and treatment with antithymocyte globulin (ATG). Azathioprine and corticosteroid administration failed to sustain recovery from neutropenia induced by ATG. It is concluded that ATG can provide a nonmyelotoxic form of therapy for pure white cell aplasia whose effectiveness is independent of responsiveness to other immunosuppressive agents.
Porphyria cutanea tarda associated with the acquired immune deficiency syndrome
Authors: Wissel Paul S. , Sordillo Peter , Anderson Karl E. , Kappas Attallah , Sassa Shigeru , Savillo Robert L. 
Published: February 2007

AbstractThree male subjects with cutaneous symptoms and biochemical signs typical of porphyria cutanea tarda (PCT) developed acquired immune deficiency syndrome (AIDS). All three were in a classic high risk group for the latter disease and developed a typical progressive illness. Two patients succumbed to opportunistic infections; the third is alive but critically ill. The symptomatic prodrome of AIDS developed concurrently with or followed the onset of symptoms of PCT in all three individuals. PCT and AIDS are both uncommon disorders; their association in three patients is thus of inherent clinical interest. If this association is not coincidental, it raises the possibility that the occurrence of photosensitivity, skin lesions, and evidence of biochemical changes characteristic of PCT may, in certain patients at risk for AIDS, presage the subsequent full clinical expression of the latter disease.
Platelet kinetics in human immunodeficiency virus induced thrombocytopenia
Authors: Bel‐Ali Zoher , Dufour Valérie , Najean Yves 
Published: February 2007

AbstractA platelet kinetic study was performed in 34 patients (33 homosexuals and/or drug addicts) with (in most of them) severe and isolated thrombocytopenia and human immunodeficiency virus positive serological tests. The reported data indicate that the thrombocytopenia is due to an extracorpuscular hyperdestruction. The sequestration of the labeled platelets is exclusively splenic in most of the cases. Splenectomy was successful in the ten cases in which it was done; however, corticoids showed no or low effect, and Ig‐G infusion had only a temporary efficacy.
Pure red cell aplasia as possible early manifestation of chronic myeloid leukemia
Authors: Haas Oskar A. , Hinterberger Wolfgang , Mörz Reinhard 
Published: February 2007

AbstractCytogenetic studies as well as erythroid and myeloid progenitor cell assays were performed in a 29‐yr‐old epileptic man with pure red cell aplasia (PRCA) who had been treated with primidone for several years. Despite clinical evidence of preleukemia, our studies indicated an underlying atypical Philadelphia chromosome‐positive myeloproliferative disorder. These laboratory findings were confirmed by the subsequent development of chronic myeloid leukemia (CML) which terminated in a CALLA‐positive lymphoblastic crisis 32 months later.The rare concurrent occurrence of PRCA and CML and the possible inducing role of the preceding antiepileptic treatment are discussed.