bluuebunny/crossref_metadata_2025_split huggingface dataset:
Severe fletcher factor (plasma prekallikrein) deficiency with partial deficiency of hageman factor (factor xii): Report of a case with observation on in vivo and in vitro leukocyte chemotaxis
Authors: Poon Man‐Chiu , Moore Michael R. , Castleberry Robert P. , Lurie Aubrey , Huang Shu Tsong , Lehmeyer Joyce 
Published: February 2007

AbstractA case of cross‐reacting material‐negative Fletcher trait with additional partial deficiency of Hageman factor (HF, Factor XII) is described. Although the patient presented with a recent history of frequent epistaxis, he had no other personal or family history of a tendency toward bleeding or infection. Similar to other cases of Fletcher trait, his plasma showed a markedly prolonged partial thromboplastin time which could be corrected by prolonged incubation with the surface‐activator kaolin. Surface‐induced fibrinolysis, amidolysis of α‐N‐benzoyl‐proline‐L‐phenylalanine‐L‐arginine‐p‐nitro‐ anilide, and cold‐promoted enhancement of factor VII activity, reactions requiring the presence in the plasma of Fletcher factor (pre‐kallikrein), in addition to Hageman factor and Fitzgerald factor (high‐molecular weight kininogen), were also defective. In vivo chemotaxis of polymorphonuclear leukocytes and monocytes (Rebuck's skin window technique) in response to skin abrasions was defective, but was normal when diphtheria‐tetanus toxoid was also applied. In vitro leukocyte chemotaxis (Boyden chamber technique) in response to normal or patient's own serum activated with zymosan was normal. Together with previous observations that kallikrein generated chemotactic activity, possibly via activation of C5, the present observations suggest that prekallikrein activation may be important for in vivo leukocyte chemotactic response to skin abrasion. The inheritance of Fletcher trait in this patient is unclear. Although the father was an apparent heterozygote, the mother was completely normal for Fletcher factor procoagulant activity and antigen. The mild Hageman factor deficiency in the patient did not contribute significantly to the plasma defects described and was likely inherited from the father who had a low HF procoagulant activity.
Diminished autologous mixed lymphocyte reaction in patients with hodgkin disease: Evidence for non‐t cell dysfunction
Authors: Zamkoff Kenneth W. , Dock Nancy L , Kurec Anthony S. , Davey Frederick R. 
Published: February 2007

AbstractIn the autologous mixed lymphocyte reaction (AMLR), T lymphocytes are stimulated to proliferate by autologous non‐T mononuclear cells. In five untreated patients with Hodgkin disease, the AMLR was diminished. In addition, in the same five patients, T cell response to PHA was inhibited by a cell in the non‐T cell fraction, the response of non‐T cells to PWM was diminished, and there was a diminished ability of the non‐T cell population to stimulate in allogeneic MLR. However, the response of T cells from patients with Hodgkin disease to allogeneic antigen was normal. The AMLR and allogeneic MLR were then studied in an additional five untreated patients before and after monocyte depletion of the stimulating non‐T mononuclear cell population. In this second group of Hodgkin disease patients, the AMLR was again diminished when T cells were incubated either with non‐T cells or non‐T cells depleted of monocytes. In the Hodgkin patients, monocyte depletion did not alter the T cell response in the AMLR. In the controls, monocyte depletion greatly diminished the proliferative response. The diminished AMLR in untreated Hodgkin disease patients may be the result of a failure of adequate monocyte stimulation of autologous T cells.
Detection of igg sensitization of red cells with 125I staphylococcal protein A
Authors: Yam Priscilla , Petz Lawrence D. , Spath Peter 
Published: February 2007

AbstractMost cases of immune hemolytic anemia are associated with a positive direct antiglobulin test. However, in some cases, the antiglobulin test is not sensitive enough to detect low levels of red‐cell bound antibodies. This report describes a method using radiolabelled purified staphylococcal protein A which is capable of detecting IgG sensitization of red cells beyond the threshold of serologic techniques. It is less cumbersome than previously described methods and does not require antibody purification procedures. Its effectiveness was demonstrated for the detection of red‐cell alloantibodies and in evaluation of patients with acquired hemolytic anemias associated with a negative direct antiglobulin test.
Hemoglobin E diseases: Hematological, analytical, and biosynthetic studies in homozygotes and double heterozygotes for α‐thalassemia
Authors: Wong S. C. , Ali M. A. M. 
Published: February 2007

AbstractTwo families with Hb E diseases are described. In the Laotian family S, three homozygous Hb E were found. In the Vietnamese family H, double heterozygous Hb E‐α‐thalassemia‐2 and Hb E‐Hb H diseases were found. Anemia or hemolysis was absent in Hb E carriers, unless complicated by iron deficiency, the presence of severe α‐thalassemia gene (Hb H disease), or oxidative drug (paraaminosalicylic acid). Moreover, iron deficiency or concurrent α‐thalassemia genes resulted in a decreased amount of Hb E in its heterozygous carriers. Mild microcytosis and hypochromia were observed in Hb E heterozygotes, whereas the microcytosis and hypochromia were more pronounced in Hb E homozygotes. Globin chain synthesis studies yielded unbalanced α/non‐α ratios in both heterozygotes and homozygotes (average ratios were 1.13 and 1.56, respectively). The unbalanced biosynthetic ratios with microcytosis and hypochromia in Hb E carriers represented a β‐thalassemia phenotype, which could be a result of reduced synthesis of βE‐globin mRNA, as suggested by recent hybridization studies.
Clinical, hematological, and biochemical features of Hb SC disease
Authors: Ballas Samir K. , Lewis Cynthia N. , Noone Ann Marie , Krasnow Steven H. , Kamarulzaman Embi , Burka Edward R. 
Published: February 2007

AbstractTwenty‐seven patients were seen and followed at our Sickle Cell Center over a period of seven years. Their clinical, hematological, and biochemical features were determined and compared to those of patients with sickle cell anemia who were concurrently investigated. The data indicate that the mild anemia of hemoglobin (Hb) SC disease is slightly microcytic and hyperchromatic. Parameters of hemolysis and the complications of chronic hemolytic anemia (cholelithiasis, leg ulcers, hepatomegaly, and cardiomegaly) are milder in Hb SC disease than in sickle cell anemia. Asplenia and its sequelae (increased platelet count and reduced serum IgM levels) are less frequent in Hb SC disease. Cerebrovascular accidents and the decreased leukocyte alkaline phosphatase scores are similar in both diseases. Thromboembolic complications, retinopathy, and renal papillary necrosis are more frequent in Hb SC disease.
Results of induction therapy with vincristine and prednisone alone in adult acute lymphoblastic leukemia: Report of 43 patients and review of the literature
Authors: Hess Charles E. , Zirkle John W. 
Published: February 2007

AbstractThe complete remission (CR) rate in children with acute lymphoblastic leukemia (ALL) who received vincristine (VCR) plus prednisone (PRED) alone for induction is > 90%, and has not improved with the use of more intensive regimens. In adults with ALL, using the same regimen, a CR rate of only 50% is frequently quoted, and is apparently based mainly on unpublished data on only 13 patients from an early Acute Leukemia Group B protocol. We reviewed the case records of 43 adults with previously untreated ALL seen between July 1, 1966 and December 31, 1978 who received VCR + PRED alone for induction. A CR rate of 66% was achieved and compares to an overall figure of 61% from 162 patients collected from the literature. The CR rate was age dependent with 21/29 (76%) < 50 years of age achieving a CR compared to 4/19 (29%) who were < 50 years of age (P < 0.02). The median duration of CR was 8.3 months. For those who achieved a CR, the median survival was 19 months compared to five months for those who either failed to achieve a CR or who achieved only a partial remission (P = 0.004). All patients who achieved a CR received maintenance therapy, and approximately one‐half also received CNS prophylaxis with intrathecal methotrexate. The incidence of isolated CNS relapse was 11% in those who received CNS prophylaxis compared to 20% in those who did not.From these data and a review of the literature, we conclude that approximately two‐thirds of adults with ALL will achieve a CR with the use of VCR + PRED alone. The median duration of CR, however, is < one year and the median survival is approximately two years. Whether the use of more intensive induction, consolidation, and maintenance regimens will improve these results remains to be established.
Inhibitory effect of short‐chain aliphatic acids on deoxythymidine incorporation into DMA in mitogen‐stimulated and leukemic lymphocytes
Authors: Stolc Viktor 
Published: February 2007

AbstractThe effect of short‐chain aliphatic acids on [3H]deoxythymidine incorporation into DNA was studied in human mitogen‐stimulated lymphocytes. Butyric acid at 1‐‐2 mM level was strongly inhibitory; however, its hydroxy or amino derivatives were ineffective. Valeric and propionic acids were less inhibitory. Formic, acetic, and hexanoic acids did not have any inhibitory effect. The effect of butyrate persisted in washed lymphocytes for 24‐‐48 hr depending on the concentration used and the time of its addition into the incubation medium. The inhibitory effects of butyrate, valerate, and propionate on deoxythymidine and amino acid incorporation into DNA and protein were also found in human leukocytes in myelocytic and lymphocytic leukemias.
Exposure to phorbol diester (TPA) in vitro as an aid in the classification of blasts in human myelogenous and lymphoid leukemias: In vitro differentiation, growth patterns, and ultrastructural observations
Authors: Polliack Aaron , Leizerowitz Rachael , Korkesh Aviva , Gurfel Dorit , Gamliel Haim , Galili Uri 
Published: February 2007

AbstractLeukemic cells from the peripheral blood of 52 patients with acute and chronic leukemias were incubated with 12‐0‐tetradecanoyl phorbol ester (TPA). Thirty‐one cases of lymphocytic leukemia (18 cases of acute lymphoblastic and 13 cases of chronic lymphocytic leukemia), 13 cases of acute nonlymphoblastic (myelo or myelomonoblastic) leukemia, and eight cases of blastic crisis of CGL (seven cases of predominantly myeloblastic crisis, and one case of lymphoblastic crisis) were studied. In all cases of lymphoid leukemia, cells formed clumps or aggregates after exposure to TPA, while in all cases of myeloid leukemia cells became adherent to the substrate. Seven of the eight cases of blastic crisis of CGL were predominantly myeloid in type and cells adhered to the substrate, while in a single case of lymphoid crisis in CGL cells formed clumps after TPA exposure. Functional, cytochemical, and ultrastructural studies showed altered cell differentiation and continuing in vitro maturation of leukemic cells after exposure to TPA. In the light of the above results, it is concluded that this simple test employing TPA exposure in vitro serves as a reliable means of distinguishing blasts from different origins in human leukemias.
Effects of divalent cations on the conversion of fibrinogen to fibrin and fibrin polymerization
Authors: Kanaide Hideo , Uranishi Takako , Nakamura Motoomi 
Published: February 2007

AbstractEffects of divalent cations on fibrinogen and its reaction with thrombin were re‐examined and correlated to improve definition of mechanisms underlying the acceleration of clot formation by the ions. The rate of release of fibrinopeptides from fibrinogen was not affected by any of the specific ions studied, but increased rates of fibrin monomer polymerization were obtained with all but magnesium ions. Maximal acceleration of monomer polymerization was observed with the divalent ions at concentration of 2.5–5 mM, and no added specific ion effects were observed with much higher levels. The acceleratory ions were found to have a corresponding effect on the solubility of fibrinogen, as judged from acceleration of its precipitation at low temperature. Although magnesium ions had no effect on fibrin monomer polymerization, they did have a low ranking effect on cryoprecipitation. These data confirm that the principal effect of divalent cations in the fibrinogen‐fibrin transformation is to accelerate fibrin monomer polymerization, and suggest that the acceleration is associated with effects on the solubility of fibrinogen.
Studies of a patient with recurring thrombotic thrombocytopenic purpura
Authors: Kitchens Craig S. 
Published: February 2007

AbstractA young woman with recurring bouts of thrombotic thrombocytopenic purpura presented an opportunity to study a single patient serially and in depth. This patient underwent 12 remissions in response to fresh frozen plasma, and as little as 250 ml of plasma or plasma from which cryoprecipitate had been extracted was efficacious. Neither the Lian factor nor alterations in either cold‐insoluble globulin or protein C could be demonstrated. Evidence in support of the concept of “exhausted” platelets was generated, in that immediately before a thrombocytopenic relapse, platelet function was altered as manifested by bruising, prolongation of the bleeding time, and decreased platelet aggregation. The in vitro addition of normal plasma to such platelets did not improve aggregation. The antiplatelet agent sulfinpyrazone did not seem to add efficacy. Serial observations support the hypothesis that the progression of pathophysiologic events in this patient is: exhausted platelets, thrombocytopenia, hemolysis, renal perturbations, and central nervous system dysfunction.
Nonsecretory multiple myeloma
Authors: Dreicer Robert , Alexanian Raymond 
Published: February 2007

AbstractThe clinical features and disease course of 29 consecutive multiple myeloma patients without monoclonal globulins are described. This variant accounted for 4.7% of all patients with this disease referred over a 17 year time span. Despite bone marrow plasmacytosis and multiple bone lesions in all, hemoglobin levels were less frequently depressed and immunoglobulin values more frequently preserved in comparison with typical myeloma patients. A low tumor mass was defined in 69%, in comparison with 24% of those with evidence of myeloma protein production. Such earlier disease contributed to the long median survival of 39 months in these patients, a duration about 1 year longer than that of typical patients.
Enzyme alterations in leukemic cells
Authors: Dunn Nancy L. , Maurer Harold M. 
Published: February 2007

AbstractRecently, much attention has been focused on various enzyme alterations found in leukemic cells. Most of the data generated thus far has involved the study of terminal deoxynucleotidyl transferase, the purine pathway enzymes, and hexosaminidase and other lysosomal enzymes. Differences in both total enzyme activities and isoenzyme patterns have been found to occur among the various leukemia types and subtypes. These changes may prove to be useful aids in diagnosing, classifying, detecting subclinical recurrent or residual disease, and as therapeutic determinants in hematopoietic neoplasia, especially in the lymphoid malignancies.
Familial and constitutional bleeding disorder due to platelet cyclo‐oxygenase deficiency
Authors: Horellou M. H. , Lecompte T. , Lecrubier C. , Fouque F. , Chignard M. , Conard J. , Vargaftig B. B. , Dray F. , Samama M. 
Published: February 2007

AbstractThree family members from two successive generations had a bleeding tendency. Their template bleeding time was prolonged and platelet aggregation induced by ADP and adrenaline showed no second wave; collagen at low to moderate concentrations failed to aggregate and release ATP, whereas higher amounts aggregated and released. Aggregation and release due to thrombin, ristocetin, and synthetic epoxy derivatives (U 44069 and U 46619) were normal. Arachidonate (AA) was inactive, and was not converted either in thromboxane (TX) A2activity evaluated on the rabbit aorta strip, nor in TXB2evaluated by radioimmunoassay and by radiochromatography. The parallel impairment of TXB2and PGE2formation by the patient's platelets are compatible with a platelet cyclo‐oxygenase deficiency. This study suggests that transmission is autosomal dominant. and confirms that cyclo‐oxygenase is not needed for aggregation and ATP release by high amounts of collagen.
Beta‐thromboglobulin and platelet‐factor 4 in patients with cancer: Correlation with the stage of disease and the effect of chemotherapy
Authors: Al‐Mondhiry Hamid 
Published: February 2007

AbstractPlasma level of beta‐thromboglobulin (BTG) and platelet factor 4 (PF4), two platelet‐specific proteins, were measured in 20 patients with cancer. None of the patients had evidence of thromboembolism or impaired renal function, and none was taking drugs known to interfere with platelet function. In ten patients who were in complete remission or whose disease was thought to be inactive, the levels of both proteins were essentially within normal limits. The other ten patients had active or progressive symptomatic disease. BTG level was elevated in all but one patient, and PF4 was raised in two patients. These results suggest that in patients with clinically active cancer, a state of ongoing in vivo platelet activation may be present, a phenomenon which may contribute to the known increased occurrence of thromboembolism in patients with malignancy. The intravenous administration of various cytotoxic drugs used in cancer chemotherapy produced no immediate measurable changes in BTG and PF4 level.
A family of hereditary stomatocytosis associated with normal level of Na+‐K+‐ATPase activity of red blood cells
Authors: Mutoh Shigeaki , Sasaki Ryuhei , Takaku Fumimaro , Aoyama Masako , Moriyama Shinichi , Yoshimoto Masahiro , Yawata Yoshihito 
Published: February 2007

AbstractA rare familial case of hereditary stomatocytosis with hemolytic anemia, increased auto‐hemolysis, increased osmotic fragility, and shortened erythrocyte survival is described. The erythrocytes were abnormally permeable to sodium and potassium. In addition, “Na‐K pump” rate of the red blood cells was increased, while Na+‐K−‐ATPase, Mg2+‐ATPase and Mg2+‐Ca2+‐ATPase activities were within normal limits. Splenectomy induced marked improvement of anemia.
The occurrence of different levels of Gγ chain and of the AγT variant of fetal hemoglobin in newborn babies from several countries
Authors: Huisman T. H. J. , Reese A. L. , Gardiner M. B. , Wilson J. B. , Lam H. , Reynolds A. , Nagle S. , Trowell P. , Yi‐tao Zeng , Shu‐zheng Huang , Sukumaran P. K. , Miwa S. , Efremov G. D. , Petkov G. , Sciarratta G. V. , Sansone G. 
Published: February 2007

AbstractThe γ chain compositions of the fetal hemoglobins of 2453 newborn babies from East Asian countries (1350 babies), from Italy, Yugoslavia, Bulgaria, and Georgia (417 Caucasian babies), and 686 black babies from Georgia were determined by high pressure liquid chromatography. Unusual results for a limited number of babies were confirmed by chemical analyses, and were evaluated further by family studies.Statistical analyses indicated high gene frequencies for the AγT chain in Italian (f = 0.237), Yugoslavian and Bulgarian (f = 0.238), and white Georgia babies (f = 0.224), a lower frequency in Japan (f = 0.178), and India (f = 0.173), and particularly in mainland China (f = 0.079). The AγT gene frequency in normal (AA) Black babies was 0.102. When a βS or βC mutation was also present this frequency was greatly decreased, particularly in babies with the AC condition (f = 0.036). These results suggest the near absence of the AγT mutation on the chromosome also carrying the βC determinant.Most babies had the expected Gγ values which vary between 60 and 80%, but several (mainly black) babies had higher values (between 80 and 90%), while one normal black baby had a Gγ value of (nearly) 100%. This condition may be a form of Aγ+1‐thalassemia and has been discussed in detail elsewhere (Blood 58:491‐500, 1981). Thirty‐five clinically normal (mainly Chinese, Indian, and Japanese) babies had Gγ values of about 40%. Twenty‐six babies had Aγ1 values of about 60%, while the remaining nine babies had AγT and AγI chains in a ratio of either 1 to 2 or 1 to 1. Two additional newborns did not produce any Gγ chains, but had only AγI chains or AγI chains. Family studies failed to indicate a specific hematological abnormality. These unusual ratios between the Gγ and (either AγI or AγT) chains have led to speculations regarding possible genetic abnormalities present in these infants.
Altered Immunoregulation in Waldenström's macroglobulinemia. I. The absence of con A‐induced and spontaneous T suppressor cell activity in a patient and his kindred
Authors: Keller Robert H. , Feldman Eric , Libnoch Joseph A. 
Published: February 2007

AbstractIn vitro lymphocyte functions in a patient with Waldenström's macroglobulinemia and his kindred were examined. The percentage and number of total peripheral blood T cells and the absolute number of FCγ bearing T cells were decreased in the patient and family members compared to controls but the B cells and monocytes were normal. In addition, PHA and PWM stimulated proliferation were decreased. Decreased responsiveness to PWM was abrogated by preculturing PBMC in media alone before PWM stimulation. Decreased responsiveness to PHA was not reversed by a similar period of preculture.To further define the above abnormalities in immunoregulation we examined mononuclear subpopulations. In controls, adding purified T cells or Con A activated lymphocytes to autologous cultures which had been stimulated with PHA and PWM significantly decreased proliferative activity. No such suppressive activity of T cells or Con A activated cells from the patient or family members was noted.These data demonstrate an absent or functionally inactive spontaneous and Con A activated T suppressor cells in the family of a patient with Waldenström's macroglobulinemia.
Simultaneous occurrence of mycosis fungoides and hodgkin disease: Clinical and histologic correlations in three cases with ultrastructural studies in two
Authors: Hawkins Katherine A. , Schinella Roger , Schwartz Melvin , Ramsey David , Weintraub Arthur H. , Silber Robert , Amorosi Edward L. 
Published: February 2007

AbstractWe present three patients who manifested both Hodgkin disease and mycosis fungoides. Ages ranged from 39 to 66 and two were male. Skin lesions were present from 3 to 40 years before the diagnosis of Hodgkin disease. In all cases, mycosis fungoides was confirmed histologically by skin biopsy; the clinical course of the mycosis fungoides was indolent in all cases. Hodgkin disease was confirmed histologically in three, and confirmed by electron microscopy in two. All three patients responded to appropriate treatment for Hodgkin disease and are alive and well at the present time.