bluuebunny/crossref_metadata_2025_split huggingface dataset:
Platelet function in acute respiratory failure
Authors: Carvalho Angelina C. , Quinn Deborah A. , Demarinis Sandra M. , Beitz Julie G. , Zapol Warren M. 
Published: February 2007

AbstractTo assess the role of platelets in thrombohemorrhagic complications of acute respiratory failure (ARF), we studied platelet function in 13 ARF patients admitted for intensive care, in six acutely ill intensive care patients without evidence of acute lung injury (non‐ARF), and in 10 normal subjects. Platelet counts in ARF and non‐ARF patients were similar to the normal range. The bleeding time of the ARF patients (8.5 ± 0.9 min) was significantly longer (p < 0.01) than the normal (4.8 ± 0.2 min) but similar to non‐ARF patients (5.4 ± 0.8 min). The bleeding time prolongations in ARF patients were unrelated to platelet concentration. Platelet aggregation induced by ADP and thrombin was normal in both ARF and non‐ARF patient groups. The epinephrine response was impaired in one non‐ARF patient and in three ARF patients; collagen‐induced aggregation was absent in two ARF patients, with a prolonged bleeding time. Levels of VIII:C and vWF in both groups of patients were similar to the normal level, but VIIIR:Ag levels in ARF patients (407 ± 45% of normal) were higher (p < 0.01) than in both non‐ARF patients (210% ± 10%) and normal subjects (106% ± 4). The electrophoretic mobility of VIIIR:Ag was abnormal in ARF patients. The prolonged bleeding time in ARF patients appears to result from the qualitative and quantitative VIIIR:Ag defect. β‐Thromboglobulin levels were greater (p < 0.01) in ARF patients (87.6 ± 6.9 ng/ml; p < 0.001) than in non‐ARF patients (46.2 ± 3.1 ng/ml) or in normal subjects (25.3 ± 2.5 ng/ml p < 0.0001). However, platelet factor 4 plasma levels in ARF patients (18 ± 1.6 ng/ml) did not differ from those in non‐ARF patients (15.0 ± 3.0 ng/ml), but both were significantly different from normal (6.1 ± 0.8 ng/ml). Plasma thromboxane B2 (T × B2) levels were not different from normal values in either ARF or non‐ARF patients, but 6‐keto‐PFG1α levels were significantly reduced (p < 0.01) in ARF patients (215 ± 43 pg/ml) compared to normal values (381 ± 34 pg/ml). Non‐ARF patients had 6‐keto‐PGF1α levels (285 ± 111 pg/ml) midway between the normal values and those of ARF patients. Our results suggest that in vivo platelet activation occurs in ARF. ARF patients have quantitative and qualitative platelet defects that may contribute to thrombotic and hemorrhagic complications.
In vitro induction of myeloid surface markers in a rare case of acute leukemia
Authors: Drexler Hans G. , Coustan‐Smith Elaine , Gignac Suzanne M. , Jani Harsha , Victor Hoffbrand A. 
Published: February 2007

AbstractThe phenotypic marker profile of a case of acute leukemia is described; its immunophenotype is unique in that the blast cells were initially negative for a wide panel of monoclonal antibodies (McAbs) to surface and intracytoplasmic antigens and for the nuclear enzyme terminal deoxynucleotidyl transferase. Morphological and cytochemical examination suggested an acute myeloid leukemia (AML), but the cells were unreactive with anti‐myeloid McAbs. Treatment with the phorbol ester 12‐0‐tetradecanoylphorbol 13‐acetate (TPA) induced the cells to differentiate morphologically to macrophage‐like cells and led to the expression of surface antigens that could be detected by antimyeloid McAbs. It is not clear whether these cells represent a rare subclass of leukemic cells that are void of any characteristic surface markers but have the potential to differentiate along the myeloid axis, or whether the antigens were masked by an unknown process.
Fibrinogen giessen I: A congenital homozygously expressed dysfibrinogenemia with Aα 16 Arg← his substitution
Authors: Alving Barbara M. , Henschen Agnes H. 
Published: February 2007

AbstractClinical reports are published for only two patients with homozygously expressed congenital dysfibrinogenemia. The patients, both of whom have a bleeding diathesis, have amino acid substitutions in the fibrinogen molecule at Aα 16 Arg←Cys and Aα 19 Arg←Ser, respectively. We report that a third patient with dysfibrinogenemia (fibrinogen Giessen I) is homozygous for Aα 16 Arg← His. Although this patient has had excessive postpartum bleeding, she has had normal hemostasis throughout several minor surgical procedures and hysterectomy. Elucidation of the amino acid alterations in patients with dysfibrinogenernia may expand our understanding of structural determinants of fibrinogen that are critical to its function in vivo.
Establishment of a common acute lymphoblastic leukemia cell line (LC4‐1) and effects of phorbol myristate acetate (PMA) on the surface antigen expression of the cell line
Authors: Yoshimura Tomohide , Mayumi Mitsufumi , Yorifuji Tohru , Kim Kwang‐Myong , Heike Toshio , Miyanomae Takeshi , Shinomiya Keisuke , Mikawa Haruki 
Published: February 2007

AbstractA common acute lymphoblastic leukemia (ALL) cell line, designated LC4‐1, was established from peripheral blood mononuclear cells of a patient with acute non‐T‐cell ALL. LC4‐1 cells were characteristically positive for Ia, B4, and common ALL antigens (CALLA), but negative for B2, Tac, T3, T4, T8, T11, and M1 antigens and E‐rosette formation. Approximately 30% of LC4‐1 cells expressed detectable amounts of B1 antigens. LC4‐1 cells expressed neither Epstein‐Barr‐virus–associated nuclear antigen (EBNA), cytoplasmic immunoglobulins (cIg) nor surface immunoglobulins (sIg). Gene rearrangements had already occurred in LC4‐1 in the D‐J region of immunoglobulin heavy chain genes, but not in T‐cell receptor (β‐chain) genes, suggesting that LC4‐1 is a progenitor cell line of B‐cell lineage earlier than pre‐B‐cells.The expression of cell surface antigens of LC4‐1 was changed by treatment with 4‐phorbol 12‐myristate 13‐acetate (PMA) (0.1 ng/ml) for 2 days. Before treatment with PMA, about 98% of LC4‐1 cells were positive for B4, CALLA, and Ia. However, following treatment they lost CALLA expression without any change in expression of Ia and B4. There was no change in B1‐positive population. The change in surface antigens on LC4‐1 cells seems to be due to differentiation induced in the cells by PMA. These results support the hypothesis that CALLA is a differentiation antigen and suggest one possible differentiation pathway for pre‐B‐cells.
Modified end‐point glycerol hemolysis assay as a screening test for hereditary spherocytosis that requires no venipuncture
Authors: Judkiewicz Luba , Szczepanek Andrzej , Bugała Irena , Bartosz Grzegorz 
Published: February 2007

AbstractThe high specifity of the “pink test” for the detection of hereditary spherocytosis has been confirmed. A modification of the test is proposed that requires only 10 μl of blood taken without anticoagulant (a “direct pink test”), thus eliminating the necessity of venipuncture, especially cumbersome in newborns and infants.
High‐dose chemoradiotherapy with syngeneic bone marrow transplantation for multiple myeloma: A case report and literature review
Authors: Wolff Steven N. , McCurley Thomas L. , Giannone Leonard 
Published: February 2007

AbstractWe had the opportunity to treat a patient with progressive heavily pretreated multiple myeloma with high‐dose chemoradiotherapy with hematopoietic rescue by syngeneic bone marrow transplantation. The patient was a 53‐year‐old male who had previously received melphalan, prednisone, 1,3‐bis (2‐chloroethyl)‐l‐nitrosourea (BCNU), vincristine, and standard radiation therapy. At the time of bone marrow transplantation, he had increasing bone pain, increasing M‐protein (IgG kappa), and a bone marrow diagnostic of myeloma. The transplant regimen consisted of cyclophosphamide, 60 mg/kg intravenously for 2 days, and total body irradiation—1,200 rads given as 200‐rad fractions, twice daily for three days. The transplant course was complicated by confusion, herpes simplex mucositis, fever, and two episodes of idiopathic diffuse interstitial pneumonia. Over the next 2 years the patient did well and was in immunologic and bone marrow complete remission. Unfortunately, 3 years after treatment, the myeloma relapsed with detectable M‐protein. Three and one‐half years after transplant, clinical relapse occurred with bone pain and lytic lesions necessitating additional radiation and chemotherapy. Salvage therapy has produced clinical improvement and the patient is alive almost 4 years from transplant and almost 7 years from diagnosis. Although intense chemoradiotherapy did not cure this patient, substantial control of a refractory tumor was observed. This case, together with other cases of intense therapy for myeloma which are reviewed in this paper, support the concept of high‐dose therapy and should foster further investigation of high‐dose therapy.
High‐dose intravenous gammaglobulin (IVG) in neonatal immune thrombocytopenia
Authors: Suarez Carlos R. , Anderson Craig 
Published: February 2007

AbstractRecent reports suggest that intravenous gammaglobulin (IVG) may be an effective treatment modality in patients with immune thrombocytopenia (ITP). Two newborns with isoimmune thrombocytopenia secondary to HLA‐A2 and PLA1 platelet antigen incompatibilities with their respective mothers and two newborns with thrombocytopenia secondary to maternal ITP were treated with IVG 400 mg/kg/day × 5 days. One patient was exposed to steroids in utero; only one mother was thrombocytopenic at the time of delivery.All patients were severely thrombocytopenic on day 1 of treatment with mean platelet count of 5.7 × 109/L. All had petechiae and positive quaiac stools, and patients with isoimmune thrombocytopenia had CT scan evidence of intracranial bleeds. The mean platelet count after 24 hr was 26.7 × 109/L and the average platelet increase was 21 × 109/L and 33 × 109/L at 24 and 48 hr, respectively. The two cases with isoimmune thrombocytopenia had sustained platelet increases; the two cases secondary to maternal ITP had transient platelet elevations. IVG can rapidly elevate the platelet count in these patients, especially those with severe bleeding manifestations.
Partial deficiency of protein 4.1 in hereditary elliptocytosis
Authors: Lambert S. , Zail S. 
Published: February 2007

AbstractProtein 4.1, an important component of the red cell membrane skeleton, was quantitated relative to protein 3 after sodium dodecyl sulphate polyacrylamide gel electrophoresis (SDS‐PAGE) of membranes isolated from red cells of members of 14 kindreds with hereditary elliptocytosis (HE) who reside in South Africa. A partial deficiency of protein 4.1 (mean 30% reduction) was inherited in autosomal dominant fashion in five white kindreds giving a frequency of 0.36 of HE families studied. Immunoblots of membrane proteins separated by SDS‐PAGE and probed with a monoclonal antibody to protein 4.1 did not reveal any proteolytic fragments in the 4.1‐deficient subjects that could account for the reduction of this protein. These studies draw attention to the relatively high frequency of this condition as a cause of HE in white subjects in this country.
Reliability of absent platelet dense bodies as a diagnostic criterion for Hermansky‐Pudlak syndrome
Authors: Witkop Carl J. , Krumwiede Marcy , Sedano Heddie , White James G. 
Published: February 2007

AbstractThe clinical, pigmentary, and ceroid storage manifestations of the Hermansky‐Pudlak syndrome (HPS) triad of albinism, hemorrhagic diathesis, and ceroid storage disease are variable. Therefore, a rapid and accurate method of diagnosing HPS is needed. Platelets of 66 albinos were examined by electron microscopy for the presence or absence of dense bodies. Results show that patients reexamined over a period of 1 year had consistent findings. Those lacking dense bodies (15) when first examined also lacked dense bodies when reexamined a year later, and they had evidence of ceroid storage. Those with dense bodies when first examined (8) also had dense bodies when reexamined, did not have evidence of storage disease, and had types of albinism other than HPS. Of 20 propositi lacking dense bodies, all 32 albino relatives also lacked dense bodies, while 6 albino relatives of 6 propositi with dense bodies also had dense bodies in their platelets. The evidence supports the concept that HPS is a distinct genetic and biochemical disease in which the components of the triad are the result of a single genetic defect, either a point mutation or a small deletion. Comparison of whole mount preparations with thin section preparations of 13 albinos shows that whole mount preparations are an accurate and rapid method for diagnosing HPS. The most consistent diagnostic feature of HPS is lack of platelet dense bodies.
Dysfunctional α‐globin genes in hemoglobin H disease in blacks: Variation in restriction fragment size permits the detection of the ‐α/‐αT genotype
Authors: Safaya S. , Mullaney P. , Rieder R. F. 
Published: February 2007

AbstractHemoglobin H (HbH) disease is most often due to deletion of three of the four α‐globin genes (genotype ‐ ‐ ‐ ‐/‐α). In black subjects although the ‐α/chromosome is common, the ‐ ‐/haplotype is very rare and few examples of HbH disease have been detected. We have studied three black siblings with HbH by restriction endonuclease mapping of the α‐like gene complex (5′‐ζ‐Ψζ‐Ψα2‐Ψα1‐α2‐α1‐3′) using ζ‐ and α‐ specific probes. The presence of size differences in the previously described hypervariable region between the ζ and ζΨ genes results in a restriction fragment length polymorphism which permitted the detection of single α genes on both number 16 chromosomes in these subjects. Quantitative DNA hybridization by a slot‐blot technique confirmed that their genomes contained two α‐globin genes. The results establish that in these black subjects HbH disease is associated with dysfunctional α‐globin genes (genotype: ‐α/‐αT).
Cause of death in hemophilia a patients in the United States from 1968 to 1979
Authors: Aronson David L. 
Published: February 2007

AbstractDeath data on 949 hemophiliacs for the years 1968–1979 have been analyzed. The median age at death has increased from 33 to 55 years. There was no evidence of new diseases caused by the more intensive therapy during this time interval. The leading infectious disease was hepatitis, accounting for eight deaths. Only one acute hepatitis death was listed after 1974, when sensitive tests for hepatitis B antigen screening of plasma were implemented. Cirrhosis was a primary or associated cause of death in 76 cases (8%) and pneumonia was a primary or associated cause of death in 62 deaths (6.5%). The types of malignancies in hemophiliacs were similar to those in the male US population with no evidence of excessive retrovirus malignancies prior to infection with HIV‐1.
Prolonged activated partial thromboplastin time of unknown etiology: A prospective study of 100 consecutive cases referred for consultation
Authors: Kitchens Craig S. 
Published: February 2007

AbstractThe activated partial thromboplastin time (aPTT) is frequently used to assess overall competency of the intrinsic pathway of coagulation. An abnormal value may be caused by any of several abnormalities along this pathway or by many other variables including the presence of inhibitors, poor collection of the sample, or variables in the laboratory. When the cause for the prolongation is unknown to the requesting physician, the hematologist may be consulted. In this prospective study, the cause and perceived hemostatic risk to 100 consecutive patients referred to us for consultation regarding a prolonged aPTT of previous unknown cause were evaluated. We found that these abnormal aPTTs may be either indicative of a hemostatic defect, in 50% of the cases, or of no particular risk, in 36% of the cases. In 14%, the aPTT was artifactually prolonged. Most (81%) patients with a prolonged aPTT due to a hemostatic defect had an abnormal hemostatic history but some (19%) did not. Even among true abnormal tests, the degree of abnormality indicated little or nothing about hemostatic competency. We conclude that the cause of an abnormal aPTT is more important than the result itself. These data may be of use to those who consult on such matters.
Sickle cell trait in a white Jewish family presenting as splenic infarction at high altitude
Authors: Shalev Oded , Boylen Alice L. , Levene Cyril , Oppenheim Ariella , Rachmilewitz Eliezer A. 
Published: February 2007

AbstractWe report the presence of sickle cell trait in several members of a white Jewish family. The trait was discovered when the propositus developed massive splenic infarction at high altitude. No erythrocyte markers characteristic of African ancestry were detected in any of the family members. This is the first bona fide documentation of sickle trait among white Jews.
Human T‐lymphotropic virus type I‐associated benign transient immature T‐cell lymphocytosis
Authors: Ehrlich Garth D. , Han Tin , Bettigole Richard , Merl Stuart A. , Lehr Bruce , Tomar Russell H. , Poiesz Bernard J. 
Published: February 2007

AbstractWe describe a case of human T‐lymphotropic virus type I (HTLV‐I)‐associated transient benign immature T‐cell lymphocytosis in a black female patient, which over the course of several months underwent spontaneous complete remission. The patient presented with a white blood cell count of 20,000/μl and a T4/T8 ratio of 1.7:1. The majority of cells appeared to be lymphoid in origin, and cell marker analyses established that the circulating lymphocytes were predominantly immature T‐cells. HTLV‐I was detected at this time by a p19 indirect immunofluorescent slide assay. Over a 1‐month period of time the patient's clinical status evolved into a mature T‐lymphocytosis with a T4/T8 ratio of 4.5:1. HTLV‐I was detected by anti‐p19 immunofluorescence by cell sorter analyses and by dot‐bloc nucleic acid hybridization. Serological testing demonstrated that the patient had anti‐HTLV‐I antibodies and antimembrane antibodies specific for an HTLV‐I producing cell line. In a competitive HTLV‐I ELISA assay only HTLV‐I proteins could effectively compete out the seroreactivity. The patient also had a high serum level of soluble interleukin‐2 (IL‐2) receptors, which is associated with HTLV‐I infection. This is the first reported case of immature T‐lymphocytosis in a patient infected with HTLV‐I. The patient's HTLV‐I markers disappeared with time, and her lymphocytosis subsequently spontaneously resolved. She remains disease free and virus negative after 2 years of follow‐up study.
Rapid response to 2′‐deoxycoformycin in advanced hairy cell leukemia after failure of interferons alpha and gamma
Authors: Lembersky Barry C. , Ratain Mark J. , Westbrook Carol , Golomb Harvey M. 
Published: February 2007

AbstractA patient with advanced hairy cell leukemia initially had a short‐lived minor response to interferon alpha therapy and failed to respond to interferon gamma. Subsequent treatment with 2′‐deoxycoformycin (dCF) administered biweekly for 12 wk resulted in a complete hematological remission which has continued for 16 months without additional therapy.
Clinical presentation and natural history of patients with essential thrombocythemia and the Philadelphia chromosome
Authors: Stoll D. B. , Peterson P. , Exten R. , Laszlo J. , Pisciotta A. V. , Ellis J. T. , White P. , Vaidya K. , Bozdech M. , Murphy S. 
Published: February 2007

AbstractSix women presented with the clinical picture of essential thrombocythemia (ET) without the anemia, marked splenomegaly, and extreme leukocytosis characteristic of chronic myelogenous leukemia (CML). All had the Philadelphia chromosome on karyotype analysis of the bone marrow. Peripheral basophilia was present in four cases, providing a clinical clue that the Philadelphia chromosome might be present. Marrow biopsy showed granulocytic hyperplasia and either small megakaryocytes or sheets of megakaryocytes with marked atypia, findings that are more typical of CML than ET. The clinical importance of finding the Philadelphia chromosome in patients who seem to have ET is in assessing prognosis. ET generally follows a chronic, indolent course. However, five of these six patients who had the Philadelphia chromosome underwent clinical transition to the accelerated phase of CML or blastic leukemia in 4–7 years.
Effect of methotrexate on the deoxyuridine suppression test: Results with different cell preparations
Authors: Kanazawa Shoichiro , Iwasa Saori , Iseki Toru , Okuda Keiko , Kondo Haruki , Okuda Kunio 
Published: February 2007

AbstractRecently, the deoxyuridine suppression test was used to detect folate deficiency. Several cell preparations were used in this test, and the sensitivity of the test seems to vary with them. We used whole blood (micro‐dU test) and bone marrow cells for the deoxyuridine suppression test, and the effects of methotrexate were compared. [3H]Thymidine incorporation into DNA was highest when the concentration of methotrexate was 1 μM in the whole blood culture and bone marrow culture. Methotrexate concentrations from 100 nM to 1 mM did not affect the results of the micro‐dU test. However, the bone marrow deoxyuridine test became abnormal with methotrexate, and the abnormality in test values was corrected by the addition of N5‐formyltetrahydrofolate. Differences in the results between the micro‐dU test and the bone marrow deoxyuridine suppression test seem to represent specificity of each dU test.
Combined assay of adenosine deaminase, purine nucleoside phosphorylase, and lactate dehydrogenase in the early clinical evaluation of B‐chronic lymphocytic leukemia
Authors: Corrons Joan Lluis Vives , Rozman Ciril , Pujades Maria Assumpció , Colomer Dolors , Vila Encarna Perez , Anegon Ignacio , Gallart Teresa , Puiggrós Jordi Vives , Viñolas Nuria , Montserrat Emili 
Published: February 2007

AbstractThe levels of adenosine deaminase (ADA), purine nucleoside phosphorylase (PNP), lactic dehydrogenase (LDH), and LDH isoenzyme patterns (LD1 to LD5) have been measured in lymphocyte extract from 28 patients with B‐chronic lymphocytic leukemia (B‐CLL). The activities of ADA, PNP, and LDH have been correlated with two morphological groups of B‐CLL classified according to the percentage of large, nongranular, atypical lymphocytes (AL) in peripheral blood: “typical” B‐CLL (less than 10% of AL, 21 cases) and “atypical” B‐CLL (10–25% of AL, seven cases). Patients with atypical B‐CLL had significantly (P < 0.001) higher activities of ADA (0.46 ± 0.17 U/109 cells), PNP (1.74 ± 1.0 U/109 cells), and LDH (48.3 ± 9.7 U/109 cells) than patients with typical B‐CLL (ADA, 0.29 ± 0.1 U/109 cells; PNP, 0.58 ± 0.23 U/109 cells; and LDH, 29 ± 10 U/109 cells). In addition, the “treatment‐free period” was also signficantly (P < 0.025) shorter in the group of atypical B‐CLL compared with the typical B‐CLL group. No clear‐cut statistical differences in lymphocyte surface markers or in several other prognostic factors between the two subgroups of B‐CLL were found. The present study supports the idea that in B‐CLL the simultaneous determination of ADA, PNP, and LDH might be helpful in better understanding the pathophysiology, prognosis, and natural history of the disease.