bluuebunny/crossref_metadata_2025_split huggingface dataset:
Microgranular promyelocytic leukemia: A multiparameter examination
Authors: Krause John R. , Stolc Viktor , Kaplan Sandra S. , Penchansky Lila 
Published: February 2007

AbstractSix cases of microgranular variant acute promyelocytic leukemia (M3v) were studied by use of a multiparameter approach including morphology, cytochemistry, flow cytochemistry, flow cytometry, cytogenetics, and gene rearrangement. Three of six cases demonstrated both myeloid and monocytoid associated surface markers by flow cytometry. One of six cases had strong alpha‐naphthyl‐butyrate esterase (α‐NBE) activity in addition to myeloperoxidase activity. There was no correlation between percentage of positive monocytoid surface markers and intensity of cytoplasmic α‐NBE activity. Four of six cases also had a T‐cell‐associated surface antigen. Further studies indicated that the T‐cell markers appeared to be on the promyelocytes and that the T‐B receptor gene was not rearranged. Similarly, cytogenetics studies indicated only one clonal abnormality t(15q+; 17q‐). Whether these cases represent true “lineage infldelity” remains to be answered. Future important studies are needed on normal hematopoietic progenitor cells at early stages of development and childhood to study lineage‐specific characteristics and to determine whether co‐expression normally exists during early development.
Modified bleeding time in the infant
Authors: Andrew Maureen , Castle Valerie , Mitchell Lesley , Paes Bosco 
Published: February 2007

AbstractThe present authors developed a modified template bleeding time for use in the newborn infant. The sensitivity of the bleeding time to the presence of antibiotics, indomethacin, generalized illness, and thrombocytopenia was tested in 242 infants. Both indomethacin and thrombocytopenia similarly prolonged the BT, and the latter could be corrected by raising the platelet count.
Detection of hypodiploidy using multi‐parameter flow cytometric analysis: A prognostic indicator in multiple myeloma
Authors: Morgan Robert J. , Gonchoroff Nick J. , Katzmann Jerry A. , Witzig Thomas E. , Kyle Robert A. , Greipp Philip R. 
Published: February 2007

AbstractWe analyzed 49 patients with multiple myeloma (MM) using propidium iodide (PI) staining and flow cytometry to assess DNA content of MM cells in bone marrow. In 12 patients the presence or absence of DNA content aneuploidy could not be determined by analysis of single‐parameter DNA histograms alone. Using forward light scatter measurement, resolution of ploidy was readily obtained in all but three cases. Assuming the DNA content of the low light scatter population to be diploid, we were able to classify 7 cases as hypodiploid and 39 as hyperdiploid or diploid. Hyperdiploid or diploid patients survived a median of 24 months and hypodiploid patients only 2.5 months. We conclude that multiparameter analysis of DNA content and light scatter provides easier analysis for the presence or absence of aneuploidy. Patients with DNA content hypodiploidy have a short survival.
Thrombocytosis as a presenting feature of acute lymphoblastic leukemia in childhood
Authors: Blatt Julie , Penchansky Lila , Horn Marianna 
Published: February 2007

AbstractTo determine the incidence of thrombocytosis at presentation in acute lymphoblastic leukemia (ALL), medical records of all children diagnosed at the Children's Hospital of Pittsburgh from 1980 to 1987 were reviewed. Out of 217 such patients, 7 (3.2%) had platelet counts greater than 400,000/mm3. All of the seven were boys compared with a male:female ration of 1.4:1 in the entire ALL population. Other than sex, no characteristics were clearly associated with thrombocytosis, including white blood cell count, hemoglobin, lymphoblast morphology, and immunologic or chromosomal markers. Apart from ALL, no inflammatory or infectious process which might have caused a thrombocytosis, was detected in any of these patients. The period of induction therapy was notable for the preservation of platelet counts greater than 20,000/mm3 in all patients. However, of the seven children with thrombocytosis, two had major induction complications: one, a cavernous sinus thrombosis; and the other, gastrointestinal bleeding with duodenal perforation.We conclude that thrombocytosis at diagnosis can be seen in children, particularly boys, with ALL. Based on small numbers, this group of patients may be at risk for major events during induction therapy. Large numbers, longer follow‐up, and platelet function studies on similar patients will be of interest.
Congenital erythroleukemia: A case report with morphological, immunophenotypic, and cytogenetic findings
Authors: Allan Randy R. , Wadsworth Louis D. , Kalousek Dagmar K. , Massing Bonnie G. 
Published: February 2007

AbstractWe report a lethal case of congenital erythroleukemia presenting on the first day of life with peripheral blast cells and a leukemic infiltrate in the placenta. Although initial bone marrow examination did not fulfill the French‐American‐British (FAB) cooperative group criteria for acute myelogenous leukemia (AML), including M6, a malignant clone was confirmed by cytogenetic analysis: 49,XX,+8,+19,+21. Evolution to erythroleukemia (M6) occurred over a two‐month period. The diagnosis of erythroleukemia was supported by immunophenotyping employing an antibody to glycophorin A. The clinical course was complicated by liver failure of unknown etiology. Comparison to previously reported cases of early childhood erythroleukemia is made.
Artefactual low lymphocyte activity caused by platelet contamination in the mononuclear cell preparations
Authors: Rueda F. , Marti F. , Pinol G. , Remacha A. 
Published: February 2007

AbstractPlatelet (PLT) contamination in preparations of mononuclear cells (MNC) produces a significant decrease (P<0.02) in lymphoproliferative activity and interleukin 2 (IL2) production. This effect was observed in MNC preparations from either healthy blood donors or polyglobulic patients. In a dose‐response curve carried out with increasing PLT number per MNC, progressive inhibition was found, reaching to 62.6% and 49.5%, respectively, to phytohemagglutinin and IL2 lymphoproliferative response and 87.8% in IL2 production, at 80 PLTs/MNC.
Prolymphocytic transformation of chronic lymphocytic leukemia: A case report of lengthy survival after intensive chemotherapy
Authors: Roberts John D. , Tindle Barbara H. , Macpherson Bruce R. 
Published: February 2007

AbstractA case of prolymphocytic transformation of chronic lymphocytic leukemia (CLL) is presented in which complete peripheral morphometric remission and lengthy survival were observed after intensive chemotherapy. The case is discussed within the context of the reported therapeutic experience.
Spontaneous regression in non‐hodgkin's lymphoma: Clinical and pathogenetic considerations
Authors: Drobyski William R. , Qazi Raman 
Published: February 2007

AbstractThe spontaneous regression (SR) of tumor has been noted in a variety of neoplastic conditions. In non‐Hodgkin's lymphoma, this phenomenon has been reported in indolent histologic subtypes, with a frequency of 10–20% in selected series. Invstigators evaluating new therapies for lymphomas with a favorable histology need to be cognizant of SR's impact. Mechanisms which have been proposed to explain SR have included the role of contemporaneous bacterial or viral infection, as well as an augmented host immune response which is able to mediate tumor regression via humoral and cellular effector mechanisms. The ability to recapture immunoregulatory control is aptly illustrated by lymphomas developing after organ transplantation where reduction of immunosuppression has, on occasion, resulted in tumor regression. The importance of immune regulation of B‐cell lymphoma is also suggested by the tumor's responses to immunotherapy and interferons in vivo and by the biologic and pathologic characteristic of indolent lymphomas being analogous, in may respects, to benign neoplasms. Indolent lymphomas which differ from aggressive lymphomas in their clinical and biological behavior may be more responsive to these host immunoregulatory influences. Review of clinical experience as well as proposed mechanisms of spontaneous regression in non‐Hodgkin's lymphoma will be explored in this report.
Role for platelet von willebrand factor in supporting platelet‐vessel wall interactions in von willebrand disease
Authors: Castillo R. , Escolar G. , Monteagudo J. , Ordinas A. , Garrido M. , Moia M. , Federici A. B. , Mannucci P. M. 
Published: February 2007

AbstractTwelve infusions of plasma concentrates of von Willebrand factor (vWF) were given to four patients with severe (type III) von Willebrand disease (vWD). Their prolonged bleeding times were either completely or partially corrected after five infusions and had not changed after the remaining seven. In contrast, the low platelet coverage of the subendothelial surface of rabbit aorta perfused with normal washed platelets and red cells resuspended in preinfusion patient plasma was completely or partially corrected in ten instances by replacing preinfusion plasma with postinfusion plasma and remained unchanged in two. Postinfusion improvement in surface coverage was greater than that in bleeding time, suggesting that vWF from normal platelets is needed to support optimal platelet‐vessel wall interactions in vWD. This possibility was further explored through other perfusion experiments. The subendothelial surface covered by platelets from an untreated patient with type III vWD (containing no measurable vWF) or from a type IIA vWD patient (containing dysfunctional vWF) resuspended in normal plasma was much smaller than that covered by normal platelets resuspended in normal plasma. These results establish that platelet vWF is important in supporting platelet‐vessel wall interactions in vWD and also provide experimental support in favour of the therapeutic transfusion of normal platelets in addition to vWF concentrates to correct the bleeding time in vWD patients.
Immunodiagnosis of acute leukemia displaying ectopic antigens: Proposal for a classification of promiscuous phenotypes
Authors: Vecchio Luigi Del , Schiavone Ettore Mariano , Ferrara Felicetto , Pace Emilio , Pardo Catia Lo , Pacetti Maurizio , Russo Maria , Cirillo Dora , Vacca Clemente 
Published: February 2007

AbstractThe nature of the blast cells in 163 cases of acute leukemia was investigated by immunophenotyping, with particular emphasis on the expression of “ectopic” surface membrane structures. Although no antigen included in our panel except CD3 revealed absolute lineage restriction, immunological typing allowed a definite characterization of blast cells in more than 90% of cases. Four groups of patients were identified (A, B, C, D) with different degrees of antigen ectopic expression. We classified as group A leukemias (74%) those expressing conventional antigenic patterns, in absence of cross‐lineage markers. Samples classified as group B (18%) showed a single ectopic membrane specificity, apparently discordant with the overall composite phenotype; such a “low‐grade deviation” did not prevent a definite immunodiagnosis. Pattern C specimens (5%) revealed a promiscuous coexpression of markers related to different lineages (biphenotypic leukemias), whereas group D included unclassifiable phenotypes, characterized by no antigen or DR‐only expression. Our findings suggest the possibility of interpreting complex phenotypic constellations of membrane markers in a consistent and logical manner.
T‐cell non‐hodgkin lymphoma in human immunodeficiency virus‐1‐infected individuals
Authors: Lust John A. , Banks Peter M. , Hooper W. Craig , Paya Carlos V. , Kueck Brian D. , Hanson Gerald A. , Ritch Paul S. , Woloschak Gayle E. 
Published: February 2007

AbstractWe present two patients with human immunodeficiency virus‐1 (HIV‐1) infection in whom T‐cell non‐Hodgkin lymphoma developed, based on pathologic diagnosis, immunophenotyping, and T‐cell receptor gene rearrangement. Both cases were positive for human immunodeficiency virus‐1 by enzyme‐linked immunosorbent assay and immunoblot methods. Histologic sections from each patient showed a high‐grade pleomorphic T‐cell non‐Hodgkin lymphoma, and immunophenotyping demonstrated a prevalence of reactivity for CD4 (helper) over CD8 (suppressor) antigens. T‐cell receptor β‐chain gene rearrangement studies revealed a rearranged pattern with either the Hindlll or BamHI enzymes, whereas immunoglobulin heavy chain genes retained a germ‐line configuration. Viral sequences specific for human T‐cell leukemia virus‐1, human T‐cell leukemia virus‐II, or HIV‐1 were not detected. Thus, although rare, T‐cell non‐Hodgkin lymphoma may be observed in HIV‐1‐infected individuals.
Hyponatremia and seizures in young children given ddavp
Authors: Smith Thomas J. , Gill Joan C. , Ambruso Daniel R. , Hathaway William E. 
Published: February 2007

AbstractDesmopressin (DDAVP), a synthetic vasopressin, temporarily corrects bleeding abnormalities associated with mild hemophilia A, von Willebrand disease, and disorders of platelet function. The side effects of DDAVP are considered benign although most of its use has been in adults and older children. We report four children under the age of 2 years who became hyponatremic after intravenous DDAVP administration (0.3 μg/kg). Three of them developed grand mal seizures. A review of the literature and these cases indicate that associated risk factors for hyponatremia after DDAVP administration include stress, surgery, anesthesia and narcotics (endogenous release of antidiuretic hormone), vomiting (loss of Na+), liver disease (hindered metabolism of DDAVP), renal tubular acidosis (chronically low serum Na+), multiple doses of DDAVP, and overhydration with hyponatremic fluids. DDAVP is not a benign drug in this age group and shows a serious potential for hyponatremia and seizures. Fluid restriction, avoidance of hyponatremic solutions, and close monitoring of serum electrolytes and urine output for at least 15–20 hr after the administration of DDAVP, when used in children under the age of 2 years, is warranted.
Hemolysates reduce iron released from transferrin
Authors: Zhan Hui , Pollack Simeon , Weaver Janet 
Published: February 2007

AbstractTransferrin donates iron to reticulocytes as follows: it binds to a receptor on the reticulocyte surface; the complex is endocytosed; both irons are released and the transferrin is recycled to the cell exterior. It has been proposed that the trigger for iron release after transferrin endocytosis is acidification of the endocytic vesicle. But this could account for removal of only one of transferrin's two irons, since only one of the irons is labile at acid pH. Moreover, iron continues to be removed from transferrin when acidification of the vesicle is blocked by a chloride‐transport inhibitor. Thus a detailed explanation of iron removal from transferrin remains elusive. In earlier work we showed that iron can be removed from transferrin by whole hemolysates and also by the combined action of hemoglobin and ATP at pH 7. We now show that the iron released from transferrin by hemolysates, and by hemoglobin and ATP, is in the Fe(II) oxidation state. We also show that ADP and DPG can substitute for ATP and that NADH and NADPH can substitute for the hemoglobin, although with these substitutions Fe(II) is generated less efficiently. The reductive release of iron from transferrin is rapid enough to account for all the iron processed by a young reticulocyte. We speculate that transferrin iron may be reduced to Fe(II) before reaching the mitochondria.
Serum and red cell folate and serum vitamin B12 levels in hyperthyroidism
Authors: Ford H. C. , Carter J. M. , Rendle M. A. 
Published: February 2007

AbstractSerum and red blood cell folate levels and serum B12 concentration were determined by radioassay in 20 hyperthyroid patients and compared with values obtained when the same patients had been euthyroid for at least 4 months. In hyperthyroidism, the levels of serum and red blood cell folate were significantly (P < .01) higher than when euthyroidism was achieved. There was no significant change in serum B12 concentration. Declines in serum and red blood cell folate levels between hyperthyroidism and euthyroidism occurred in 15 and 16 of the 20 patients, respectively. Although the explanation for the relative elevations of serum and red blood cell folate levels in hyperthyroid patients is unclear at present, our findings do not support the view that hyperthyroidism in man is associated with depletion of folate stores or subclinical deficiency of the vitamin.
Complete remission in acute promyelocytic leukemia despite the persistence of the 15;17 translocation
Authors: Wallace Paul J. 
Published: February 2007

AbstractAcute promyelocytic leukemia (APL) is a morphologically distinct subtype of acute non‐lymphocytic leukemia (ANLL) characterized cytogenetically by the presence of a trans‐location between chromosomes 15 and 17 (t(15;17)). In contrast to other subtypes of ANLL, morphologic examination of the bone marrow in the early postinduction period fails to identify patients adequately who have a high likelihood of entering remission. Remission has frequently been achieved in APL despite the failure to attain marrow aplasia or eliminate dysplastic progranulocytes after initial induction chemotherapy. In a patient with APL who failed to achieve cytoreduction after a single course of induction chemotherapy, serial cytogenetic studies demonstrated persistence of metaphases with the t(15;17) up to 23 days after induction. However, complete morphologic and cytogenetic remission was subsequently attained by day 29 without further therapy. In APL remission may occur even when induction therapy fails to achieve marrow aplasia or to eradicate replicative cells with abnormal karyotype promptly. In order to avoid excessive exposure to toxic therapy, new strategies may be necessary to identify, early in treatment, patients with APL who will require only one course of induction chemotherapy to enter remission successfully.
Adult T‐cell leukemia initially manifesting as facial diplegia
Authors: Sawada H. , Matsui M. , Udaka F. , Nishimura M. , Fujita M. , Kameyama K. 
Published: February 2007

AbstractWe report an unusual case of adult T‐cell leukemia (ATL) initially manifesting as facial palsy due to ATL meningitis, which was caused by lymphoid cells in the cerebrospinal fluid (CSF) with a different phenotype from those in the blood. A DNA southern blot analysis of the blood cells confirmed the diagnosis of ATL. The aberrant cells in the CSF contained nuclei with simple indentations and responded well to the initial chemotherapy, while leukemic cells in the blood showed lobulated or convoluted nuclei and were resistant to treatment. Flow cytometry performed before treatment demonstrated that the majority of the cells in the CSF were CD3 + CD4 + CD8‐CD25 +, consistent with ATL, whereas they expressed the CD45R antigen, which the blood ATL cells did not. These differences in the subphenotype, cell morphology, and responsiveness to treatment among the ATL cells in blood and CSF suggest that a single clone or subclone with heterogeneous nature was not generated, before the final development of ATL.
Ferrokinetic study of splenic erythropoiesis: Relationships among clinical diagnosis, myelofibrosis, splenomegaly, and extramedullary erythropoiesis
Authors: Beguin Yves , Fillet Georges , Bury Jean , Fairon Yvette 
Published: February 2007

AbstractSplenic erythropoiesis was demonstrated by surface counting of 59Fe in 129 of 1,350 ferrokinetic studies performed over a 15 year period. These 129 studies were carried out in 108 patients, including 40 with chronic myelogenous leukemia (CML), 24 with agno‐genic myeloid metaplasia (AMM), 18 with polycythemia vera (PV), six with a myelodys‐plastic syndrome, five with acute leukemia, three with prostate or breast carcinoma, two each with aplastic anemia or Hodgkin's disease, and one each with idiopathic thrombo‐cythemia, multiple myeloma, chronic renal failure, or treated hypopituitarism. Splenomegaly was present in 83% of the studies and hepatomegaly in 72%. Grade II‐III myelofi‐brosis was demonstrated in 62% of the cases. Hepatic erythropoiesis was present in 77% of the studies (only 38% in PV), and marrow erythropoiesis was undetectable in 33%. Total erythropoiesis was about twice normal (range 0.2 to 8 times normal) but was ineffective to varying degrees in 86% of the studies. Relationships between organomegaly, myelofibrosis, and extramedullary erythropoiesis, as well as differences among clinical disorders, are discussed. Differences observed between CML in chronic or blastic phase suggested that the erythroid cell line was involved in the proliferative process. It is concluded that splenic erythropoiesis 1) is encountered in a variety of clinical conditions; 2) is not necessarily associated with splenomegaly or myelofibrosis, even in the myelo‐proliferative disorders; 3) is part of a predominantly extramedullary (in the liver as well as in the spleen), expanded, and largely inefficient total erythropoiesis; and 4) can be evaluated in a semiquantitative manner by surface counting.
Whole blood aggregation in von willebrand disease
Authors: Sweeney Joseph D. , Hoernig Lynne A. , Fitzpatrick John E. 
Published: February 2007

AbstractPlatelet function testing in von Willebrand disease is generally performed in platelet rich plasma using an optical system. The characteristic abnormality is an abnormal response to the agglutinating agent, ristocetin. Platelet aggregation can be also studied in whole blood using either an impedance aggregometer or a particle counter. Fifteen patients with von Willebrand disease and fifteen normal subjects were studied using both whole blood methods. In the impedance system, normal subjects responded to ristocetin (1 mg/ml) with short lag phases (<70 sec) and normal maximum aggregation (>5 Ω). Only three patients with von Willebrand disease responded with normal maximal aggregation but each of these had a prolonged lag phase, and this may be a useful diagnostic parameter. In the particle counter, discrimination between normals and some von Willebrand disease patients was possible but an overlap between normals and von Willebrand patients is evident. It is concluded that the platelets in patients with von Willebrand disease exhibit the same abnormalities in whole blood as in platelet rich plasma and that the combination of impedance aggregometry and a factor VIII procoagulant assay is a time‐efficient and sensitive method to screen for von Willebrand disease.
Distribution of breakpoint within the breakpoint cluster region (bcr) in chronic myelogenous leukemia with a complex philadelphia chromosome translocation
Authors: Nishigaki Hikari , Inazawa Johji , Misawa Shinichi , Nishida Kazuhiro , Okuda Tsukasa , Horiike Shigeo , Tsuda Shoichiro , Taniwaki Masafumi , Abe Tatsuo 
Published: February 2007

AbstractWe analyzed, by Southern blot hybridization, the site of breakpoint within the breakpoint cluster region (bcr) in six patients with a complex Philadelphia chromosome (Ph) translocation and in 23 unselected patients with a standard Ph. The breakpoint was found within the 5.8 kb bcr in all 29 patients. When the bcr was subdivided into four parts, fragments I–IV, based on the restriction enzyme sites, among the six patients with a complex Ph, two had a breakpoint at fragment I, three at fragment II, and one at fragment III. This distribution of breakpoints in patients with a complex Ph did not differ significantly from that in patients with a standard Ph. A deletion of an allele within the bcr was found in three patients (50%) with a complex Ph and in three (13%) with a standard Ph. The internal bcr deletion may be more common in patients with a complex Ph.