bluuebunny/crossref_metadata_2025_split huggingface dataset:
Tartrate‐resistant acid phosphatase staining of monocytes in gaucher disease
Authors: Troy Kevin , Cuttner Janet , Reilly Margaret , Grabowski Gregory , Desnick Robert 
Published: February 2007

AbstractCytochemical studies were performed on peripheral blood from 30 patients with type 1 Gaucher disease. In 29 of the patients, peripheral blood monocytes stained positively for tartrate‐resistant acid phosphatase, whereas monocytes from 18 normal individuals and 14 patients with monocytosis did not. In the Gaucher patients, the percentage of monocyte positivity for tartrate‐resistant acid phosphatase ranged from 2 to 97. There was no correlation between the percent monocyte staining and the degree of disease severity, as measured by hepatosplenomegaly, pancytopenia, or extent of bone disease, for the group as a whole. In Gaucher patients who had not undergone splenectomy, however, there was a significant correlation between percent monocyte staining and the degree of hepatosplenomegaly, anemia, and thrombocytopenia. The presence of tartrate‐resistant acid phosphatase may be secondary to the lysosomal accumulation of glucosyl ceramide within these monocytes, although this remains to be confirmed. If so, these circulating cells may represent precursors of the Gaucher cells in tissues. Tartrate‐resistant acid phosphatase staining of peripheral blood monocytes may be useful as a diagnostic marker for Gaucher type 1 disease and for further studies on the pathogenesis of the disease.
Treatment of hemophilic arthritis with D‐penicillamine: A preliminary report
Authors: Corrigan James J. , Kolba Karen S. , Gall Eric P. , Trombley Joyce , Damiano Mary Lou , Meredith Keith , Jeter Monette 
Published: February 2007

AbstractCurrent medical management programs for established joint diseases in hemophiliacs are unsatisfactory and do not modify the eventual outcome. D‐penicillamine, a drug effective in the proliferative synovitis of rheumatoid arthritis, was evaluated in a rabbit model of hemarthroses‐induced arthritis and in four hemophiliacs with chronic synovitis. The animals had intra‐articular injections of citrate (left knees) and autologous citrated whole blood (right knees). Eight weeks later, the rabbits were divided into two groups: no treatment and D‐penicillamine (50 mg/kg/day, IM) until sacrificed at 6 months. The saline‐injected joints showed no inflammation and no iron deposition. The blood‐injected knees showed iron deposition in both groups, the D‐penicillamine animals had marked suppression of chronic inflammation. Of the four patients treated, three had clinical responses (reduction in synovial thickness, reduction in number of bleeds in the affected joint). One patient, who did not respond, developed mild‐moderate proteinuria. Those patients who responded received between 5.3 and 7.1 mg/kg/day of the drug. Mild abnormalities in platelet aggregation were seen in the responders. This preliminary study suggests that D‐penicillamine is beneficial in the chronic synovitis/arthritis induced by hemarthroses. Further trials are recommended.
Lupus anticoagulant: An analysis of the clinical and laboratory features of 219 cases
Authors: Gastineau Dennis A. , Kazmier Francis J. , Nichols William L. , Bowie E. J. Walter 
Published: February 2007

AbstractTo define clinical and laboratory characteristics of the lupus anticoagulant (LA), we reviewed our experience (219 subjects). Subjects were divided into group A, those with the LA and the diagnosis of lupus erythematosus, group B, those with the LA but nonlupus diagnoses, and group C, those with drug‐related lupus syndromes.The typical laboratory findings consisted of a prolonged and inhibited plasma clot time (an average of 1.9 times control time) which was proportionately more prolonged than the partial thromboplastin time or activated partial thromboplastin time (APTT) (average 1.3 times control). Ninety‐eight percent had a prolonged plasma clot time and 94% had a prolonged partial thromboplastin time. The prothrombin and thrombin times were prolonged in 33 and 25% of subjects, respectively. Washed platelets shortened the APTT in the 22 subjects so tested. Monoclonal protein peaks were seen in 7% of patients.Seventeen episodes of bleeding were observed, but in all but one instance there was another hemostatic defect present. In the 18 patients who underwent major operations, there were no hemorrhagic complications. Fifty‐eight episodes of thrombosis were observed with the same incidence in group A (25%) as in group B (26%).Bleeding is rare with the LA but thrombosis is common even without SLE and lupuslike syndromes. The plasma clot time in platelet‐rich plasma is more prolonged, and in our experience, is more sensitive in detecting the lupus anticoagulant than is the partial thromboplastin time.
A case of agnogenic myeloid metaplasia evolving into acute myelogenous leukemia associated with the development of trisomy 11 in bone marrow cells
Authors: Dang Chi V. , Stein Peter H. , Gress Daryl R. , Dallabetta Gina A. , Bender Walter L. 
Published: February 2007

AbstractAn 83‐year‐old woman who had a 5‐year history of agnogenic myeloid metaplasia and a previous normal marrow cell karyotype presented with acute myelogenous leukemia (AML) associated with the development of trisomy 11 in 100% of cultured marrow cells. Isolated trisomy 11 in marrow cells has been reported previously in five cases of AML, suggesting that trisomy 11 is a nonrandom chromosomal aberration associated with AML.
Transformation of myeloma into Ph1‐negative CML with plasma cell antigens
Authors: Paletta Elisabeth , Goldstein Mervyn , Papenhausen Peter , Ciobanu Niculae , Wiernik Peter H. 
Published: February 2007

AbstractWe report a patient in whom Ph1‐negative chronic myelogenous leukemia (CML) developed 15 years after the diagnosis of myeloma. Combined staining of morphologically myeloid elements in the peripheral blood for myeloid and plasmacytoid antigens revealed double‐marker expression, suggesting that the two neoplasms arose from a common originator cell.
Molecular aspects of erythroenzymopathies associated with hereditary hemolytic anemia
Authors: Miwa Shiro , Fujii Hisaichi 
Published: February 2007

AbstractSince the discovery of glucose 6‐phosphate dehydrogenase (G6PD) and of pyruvate kinase deficiences, erythroenzymopathies associated with hereditary hemolytic anemia have been extensively investigated. Kinetic and electrophoretic studies have shown that most, if not all, erythroenzymopathies are caused by the production of a mutant enzyme. Except for a few enzymes that are abundant in blood and tissues, it is difficult to obtain enough sample to study the functional and structural abnormalities of mutant enzymes associated with genetic disorders in man. The primary structures of only two normal red cell enzymes which can cause hereditary hemolytic anemia, phosphoglycerate kinase (PGK) and adenylate kinase, have been determined. Single amino acid substitutions of PGK variants have been found, and the identification of the exact molecular abnormalities of such variants has helped us to understand the accompanying functional abnormality. Gene cloning makes possible the identification of the DNA sequence that codes for enzyme proteins. Recently, human complementary DNA (cDNA) for aldolase, PGK, G6PD, and adenosine deaminase (ADA) have been isolated, and the nucleotide sequences for PGK and ADA determined. In the near future, human cDNA sequencing should permit identification of the gene alteration that gives rise to the mutant enzymes.
Hematopoiesis and aging III: Anemia and a blunted erythropoietic response to hemorrhage in aged mice
Authors: Boggs D. R. , Patrene K. D. 
Published: February 2007

AbstractWhether the hematocrit normally declines in the aged or whether such a decline represents inapparent disease in addition to aging is a matter of dispute. Female B6D2f1 mice were studied at ages 3, 13, or 27‐28 months, and there was no difference in hematocrit between the younger groups. The hematocrit of 45 aged mice was slightly lower than that of 66 younger mice; mean 43 % vs 49% (p < 0.001). However, rather unexpectedly, the total red cell mass was not decreased in the aged; rather, the plasma volume was expanded. Survival of mature red blood cells did not differ significantly between young and aged mice. Mice were bled 0.4 ml from the orbital sinus for 4 days, reducing the hematocrit of all groups to a nadir of 20‐25%. Recovery of hematocrit began more slowly in aged than in young mice. That this reflected a difference in erythropoiesis rather than a difference in plasma volume equilibration was suggested by studies with 59Fe. 59Fe was given following the second bleed, and 1 day later RBC 59Fe was more than twice as high in young mice than in groups of aged mice. Aged mice that did not appear healthy had been excluded. Aged mice were divided into a group with significant amounts of gray hair and/or patches of hair loss and two groups with normal‐appearing hair; the latter was subdivided into those weighing less (25‐26 g) or more (30‐34 g) than most aged mice. Neither hair condition nor weight influenced hematocrit or response to bleeding. These results suggest, but do not prove, that a mild “dilutional” anemia and a blunted erythropoietic response to hemorrhage may be an expected part of the murine aging process.
Role of arachidonic acid metabolism in human platelet activation and irreversible aggregation
Authors: Rao Gundu H. R. , White James G. 
Published: February 2007

AbstractPrevious studies from our laboratory have demonstrated that the aggregation response of platelets inhibited by agents blocking cyclooxygenase activity could be restored to a normal state of sensitivity by prior stimulation of α‐adrenergic receptors. Since cyclooxygenase activity and thromboxane synthesis are not absolutely required for irreversible platelet aggregation, it is important to define precisely what role this pathway serves in platelet physiology. The present study has evaluated the influence of agents that selectively block arachidonic acid conversion at different steps of synthesis. Inhibition of peroxidase, cyclooxygenase, lipoxygenase, and thromboxane synthetase blocked the second wave response of platelets to several agonists, but did not cause dissociation of aggregates preformed by prior exposure to arachidonate (AA) or adenosine diphosphate. Phospholipase (A2/C) inhibitors, similar to prostaglandin inhibitors, blocked the second wave response of platelets to the action of agonists and, in addition, caused dissociation of aggregates induced by aggregating agents. Results of our study demonstrate that when single agonists are tested at threshold concentrations, products of arachidonate metabolism may play a role in the activation process. However, continued generation of these metabolites does not appear to be essential for the maintenance of irreversible aggregation. When a combination of agents or high concentration of physiological agonists are used, both activation and irreversible aggregation can be secured independent of prostaglandin synthesis or the release reaction.
Two arrangements of the human fetal globin genes may be responsible for high Gγ values in chinese newborns: ‐Gγ‐Gγ‐δ‐β and ‐Gγ‐AγGγ‐Aγ‐δ‐β
Authors: Harano T. , Hulsman T. H. J. , Zeng Y. T. , Huang S. Z. 
Published: February 2007

AbstractHigh Gγ values (78% and higher) have been observed in about 3% of Chinese newborns from the Shanghai area. We describe here two arrangements different from the normal ‐Gγ‐Aγ‐δ‐β arrangement which have been characterized in the DNA from three of these babies. One baby was heterozygous for a chromosome with two linked Gγ globin genes (‐Gγ‐Gγ‐δ‐β), which has also been observed in Black newborns [Powers et al, Nucleic Acids Res 12: 7023, 1984], while the two other babies were heterozygous for a chromosome with triplicated γ globin genes, presumably of the ‐Gγ‐AγGγ‐Aγ‐δ‐β arrangement. A similar triplication has been observed in natives of the New Hebrides [Trent et al, Nucleic Acids Res 9: 6723, 1981].
Neonatal and maternal fibrinolysis: Activation at time of birth
Authors: Suarez Carlos R. , Walenga Jeanine , Mangogna Lynn C. , Fareed Jawed 
Published: February 2007

AbstractUtilizing chromogenic synthetic substrate‐based methods, determination of the plasma concentration of plasminogen, α‐antiplasmin, α‐macroglobulin, and Bβ15‐42‐related peptides were made at the time of birth in both newborns and mothers. Plasminogen levels were increased in the maternal group (150 ± 26%), and markedly decreased in the newborn group (67 ± 14%). The major inhibitors of fibrinolysis, α2‐antiplasmin and α2‐macroglobulin, were within normal range in both groups. Determination of Bβ15‐42‐related peptide showed markedly increased levels in the maternal group above control values (115 ± 102, normal 29 ± 12); the newborn group showed values only mildly elevated above control values (39 ± 21). The results demonstrate increased fibrinolytic activity in both groups, though the degree of activation is significantly higher in the maternal group, as reflected by the higher levels of Bβ15‐42‐related peptides. Fibrinopeptide A levels confirm an activation of coagulation in both maternal and newborn groups (22 ± 4 ng/ml and 138 ± 22 ng/ml, respectively) with a significantly increased level in newborns (2‐5 ng/ml normal).
Multiple cerebral thrombosis in fletcher factor (prekallikrein) deficiency: A case report
Authors: Harris Margot G. , Exner Thomas , Rickard Kevin A. , Kronenberg Harry 
Published: February 2007

AbstractDespite markedly prolonged activated partial thromboplastin times (APTT) patients with Fletcher factor (prekallikrein) deficiency do not clinically bleed. However, there is one reported case of myocardial infarction associated with prekallikrein deficiency. These clinical observations suggest that the contact mechanism has a minor role in normal in vivo hemostatic function. We report a further two cases of prekallikrein deficiency in Caucasian siblings. The propositus presented with multipie cerebral thrombosis. Cautious anticoagulation resulted in massive cerebral hemorrhage and death of this patient. The sibling was investigated in an attempt to establish a possible defective fibrinolytic pathway in vivo. New sensitive tests for fibrinolysis were used. These included radioimmunoassay of fibrin degradation product “D,” Bβ 15‐42, and in vitro 125I‐labelled fibrin lysis assays. The plasma half‐life of prekallikrein in this patient was calculated to be 58 hr. Family studies of heterozygotes were also performed. Prekallikrein deficiency is found not to be important in normal in vivo fibrinolysis, which possibly operates via tissue or vessel wall fibrinolytic activator release.
Impaired immune function in hemophilia patients treated exclusively with cryoprecipitate: Relation to duration of treatment
Authors: Pollack Shimon , Atias Dina , Yoffe Galina , Katz Rina , Shechter Yael , Tatarsky Ilana 
Published: February 2007

AbstractRecently, abnormalities of cell‐mediated immunity were found in hemophiliac patients receiving factor VIII concentrate therapy. Contradictory results were reported concerning cellular immune functions in hemophiliacs treated only with cryoprecipitate or fresh frozen plasma. Therefore, we evaluated the immunological status of 15 Israeli patients with severe classic hemophilia‐A who were treated only with cryoprecipitate and never exposed to factor VIII concentrate whether of commercial source or blood bank prepared. As a group, only mildly depressed cellular immune functions and slight reduction in the helper to suppressor cell ratio were found. However, when patients treated more than 15 years were analyzed separately, a significant reduction in proportion of T cells, T‐helper cells, helper to suppressor ratio, and proliferative response to phytohemaglutinin and pokeweed mitogen were observed compared to patients treated for less than 15 years and normal controls. Proportion of T‐suppressor cells, Con A‐activated suppressor activity, and IgG and IgA levels were significantly elevated in patients treated for more than 15 years. These results may support the view that derangement of immune function in hemophiliacs results from infusion of foreign proteins or an ubiquitous virus rather than contracting AIDS infectious agent.
Acute nonlymphocytic leukemia in a glue sniffer
Authors: Caligiuri Michael A. , Early Amy P. , Marinello M. J. , Preisler Harvey D. 
Published: February 2007

AbstractA 17‐year‐old while male with a past history of chronic inhalational abuse of plastic glue was referred to our institution for sore throat, cervical adenopathy, and an abnormal peripheral blood smear. A diagnosis of acute myelomonocytic leukemia was made and abnormalities in cytogenetic studies were demonstrated. Specific inquiry regarding this form of drug exposure should be pursued when searching for possible etiologies of malignant disease.
Human polymorphonuclear leukocytes of the bone marrow, circulation, and marginated pool: Function and granule protein content
Authors: Hetherington Seth V. , Quie Paul G. 
Published: February 2007

AbstractPolymorphonuclear leukocytes (PMN) demonstrate altered function during acute infections and after administration of corticosteroids. We questioned whether or not such changes are due to population shifts from functionally different compartments of the granulocyte pool. Volunteers were given epinephrine to induce demargination or hydrocortisone (HC) to promote egress of PMN from the bone marrow. PMN obtained before and after drug administration were compared for adherence, chemotaxis, luminol‐enhanced chemiluminescence, and total content and release of lactoferrin (LF), myeloperoxidase (MPO), and β‐glucuronidase (β‐glu). Epinephrine induced a significant neutrophilia of mature PMN (segmented neutrophils), but there were no changes in function or granule protein content. HC induced a significant neutrophilia with segmented neutrophils and immature PMN (bands). Circulating PMN obtained 4 hr after HC administration demonstrated less adherence, increased chemiluminescence, increased MPO release, and decreased MPO content. Band neutrophils, however, were more adherent than segmented PMN and showed a similar decrease in adherence following HC in vivo. Thus alteration of PMN adherence following intravenous corticosteroids is not due to an influx of immature neutrophils. On the other hand, it is possible that MPO content and release and capacity for oxidative metabolism change as PMN mature.
B‐prolymphocytic leukemia cells that form rosettes with sheep red blood cells through monoclonal surface immunoglobulin
Authors: Maruta Atsuo , Motomura Shigeki , Ogawa Koji , Matsuzaki Michio , Asada Hiroko , Takahashi Kenichi , Fukumura Motonori , Sakurai Masako , Ita Akira , Ookubo Takao , Mouri Hiroshi , Watanabe Shinichiro 
Published: February 2007

AbstractThe neoplastic cells from a patient with B‐cell prolymphocytic leukemia exhibited sheep red blood cell (SRBC) rosette formation. Immunochemistries revealed no reactivity with T‐cell monoclonal antibodies OKT 3, 4, 8, and 11. However, the neoplastic cells expressed characteristics of B‐cells including surface IgM and IgD associated with lambda light chains, Ia‐like antigenicity, and reactivity to monoclonal antibodies against B‐cell antigens B1, B2, and B4. Inhibition procedures revealed that SRBC rosette formation was the result of binding activity of surface immunoglobulin to SRBC. Pretreatment of the leukemic cells with antihuman IgM or lambda antisera, or pronase or trypsin blocked rosette formation with SRBC. Circulating antibody to SRBC was also suspected by the fact that preincubation of SRBC with heat‐inactivated patient's serum resulted in agglutination of the SRBC.
Transient Passovoy defect during a febrile illness
Authors: Nand Sucha , Jordan James V. , Merchut Michael P. , O'Keefe J. Paul 
Published: February 2007

AbstractThe Passovoy defect is a recently characterized hemorrhagic diathesis. We describe a patient with a febrile illness, possibly from Epstein‐Barr (EB) virus, who acquired this defect transiently. Prothrombin time; assays for factors VIII, IX, XI, XII; and Fletcher (prekallikrein) and Fitzgerald (high molecular weight kininogen) factors were normal. No definite circulating inhibitor could be demonstrated. The transient Passovoy defect could possibly be ascribed to the infectious process or sulfisoxazole, which the patient had received.
Five adults with mild sickle cell anemia share a βs chromosome with the same haplotype
Authors: Bakioglu I. , Hattori Y. , Kutlar A. , Mathew C. , Huisman T. H. J. 
Published: February 2007

AbstractFive adult SS patients from Qatar, Turkey, and South Africa with mild disease, had greatly elevated Hb F and specific patterns of polymorphic sites on their (βs chromosomes. One subject had an α‐thalassemia (–α/–α). The haplotypes were the common type # 19, associated with severe disease, and type # 31, not seen thus far in an SS patient (numbering system of Antonarakis et al [2]). The data suggest that modifications in the DNA of the βs # 31 chromosome promotes the synthesis of γ chains.
Determination of glucocorticoid receptors in leukemic myeloblasts by isoelectric focusing in polyacrylamide gel
Authors: Ogier Claudio , Humla Solveig , Nordenskjöld Bo , Skoog Lambert 
Published: February 2007

AbstractWe investigated the optimal conditions for measuring glucocorticoid receptor in blast cells from patients with acute nonlymphocytic leukemia.Cytosol receptor measured with isoelectric focusing was saturated after 60 min of incubation at 0°C with 100 nM of either dexamethasone or triamcinolone. Saturation was achieved when cytosol from at least 7 × 106 cells was used for incubation. Trypsin treatment of the cytosol resulted in a sharpened peak of receptor focusing at pH 5.6 with no loss of receptor‐bound radioactivity.The two physical forms of glucocorticoid receptor were isolated with DEAE cellulose chromatography. They were both found to focus at pH 5.6 during isoelectrc focusing.
Hypocellular bone marrow with increased blasts
Authors: Gladson Candece L. , Naeim Faramarz 
Published: February 2007

AbstractTwenty patients with hypocellular bone marrow and increased blasts (HBMIB) were reviewed. The median age was 60 years with a male:female ratio of 17: 3. History of alcohol abuse was noted in 30%, potential exposure to toxic chemicals in 20%, second malignancies in 20%, and aplastic anemia in 25%. Pancytopenia with marrow hypocellularity and increased marrow blast cells were characteristic hematopathologic features. Marrow hypocellularity was moderate to severe (>25%) in over half of the cases and mild to moderate (>25, >35%) in the remainder. Blast cells were the predominant cellular elements in the marrow displaying scanty to moderate amounts of cytoplasm, round to oval nuclei, and one or more nucleoli. Special stains were performed in 19 cases. Blast cells morphologically displayed myeloid features, but Sudan black B and/or peroxidase positivity was noted in only ten patients. The overall mortality was high, especially in patients undergoing chemotherapy. At 1 year follow‐up, 11 patients had received chemotherapy and eight of these eleven were dead compared to three of nine patients dead in those not receiving chemotherapy. Only two patients developed „overt”︁ leukemia evidenced by hypercellular marrow and over 30% blast cells in the peripheral blood. HBMIB is a distinct clinicopathologic entity characterized by severe marrow failure and a low response rate to chemotherapy.