bluuebunny/crossref_metadata_2025_split huggingface dataset:
Treatment of myelodysplastic syndrome with 1.25‐dihydroxy‐vitamin D3
Authors: Richard C. , Mazo E. , Cuadrado M. A. , Iriondo A. , Bello C. , Gandarillas M. A. , Zubizarreta A. 
Published: February 2007

Abstract1.25‐dihydroxy‐vitamin D3 (1.25 (OH)2D3) was tested in seven patients with myelodysplastic syndrome. The study was undertaken because 1.25 (OH)2D3 promotes differentiating myeloid cells in vitro and because of a prior report of potential benefit in a clinical study. The drug was given orally at a dose of 2.5 μg/day for a minimum of 8 weeks (range 8–28). After therapy, there were no significant changes in any of the parameters observed in peripheral blood or bone marrow. We did not observe any feature of granulocytic‐monocytic differentiation. Treatment was well tolerated. One patient died because of bone marrow failure. Survivors have persisting myelodysplastic syndrome and continue to be transfusion dependent. 1.25 (OH)2D3 has no beneficial effect in patients with myelodysplastic syndrome with this dose regimen.
Monoclonal antibodies to CALLA do not alter polymorphonuclear functions
Authors: Kaplan S. S. , Pesando J. M. , Basford R. E. , Zdziarski U. E. 
Published: February 2007

AbstractThe common acute lymphoblastic leukemia antigen (CALLA) is present on the malignant cells of most patients with acute lymphoblastic leukemia (ALL). Monoclonal antibodies (MoAbs) to CALLA have been useful for differentiating lymphoblastic from nonlymphoblastic leukemias as well as for serotherapy in ALL. Since this MoAb also reacts with polymorphonuclear (PMN) leukocytes, it was of interest to determine whether or not MoAbs to CALLA affected PMN function. We therefore evaluated four MoAbs to CALLA for their effect on PMN function. Two of the MoAbs were IgG and two were IgM. Chemotaxis was studied using Micro‐Boyden chambers. Intraleukocyte bacterial killing was studied using Staph: PMN of 2: 1 and 10: 1, and metabolic activation was evaluated by hexose monophosphate shunt activity. The results showed that these MoAbs to CALLA did not impair the parameters of PMN function studied. These findings have relevance to the usefulness of MoAbs to CALLA for serotherapy and also as a probe for understanding the surface molecule properties that have a bearing on PMN host defense functions.
Organ distribution and fate of human platelets: Studies of asplenic and splenomegalic patients
Authors: Hill‐Zobel Robin L. , McCandless Brian , Kang S. Andrew , Chikkappa Gundabhaktha , Tsan Min‐Fu 
Published: February 2007

AbstractLittle is known about the organ distribution and fate of human platelets. We investigated the kinetics, organ distribution, and fate of autologous 111 In‐oxine‐labeled platelets in 12 normal volunteers, four asplenic subjects, and four patients with splenomegaly.The initial recovery of infused 111 In‐platelets from the circulation was 97.8 ± 9.8% (means ± SD) for asplenic subjects and 26.3 ± 5.9% for splenomegalic patients as compared to 59.2 ± 9.3% for normal controls. The mean platelet survival times as derived from the multiple‐hit model were 9.2 ± 1.0 days for asplenics and 6.2 ± 0.6 days for splenomegalic subjects (8.4 ± 0.8 days for normals).At 30 min postinfusion, 79.4 ± 19.2% of the infused 111 In‐platelets pooled in the spleen of splenomegalic subjects and 42.7 ± 12.2% in normal controls. There was 7.1 ± 2.0, 12.6 ± 3.7, and 29.3 ± 8.4% pooling in the liver of splenomegalic, normal, and asplenic subjects, respectively. At 10 days postinfusion, 37 and 24% of the 111 In‐platelets were sequestered in the spleen and liver of normal control subjects, respectively. Similar figures for splenomegalic subjects were 71 and 14%, respectively. In asplenic subjects, 89% was sequestered in the liver.We conclude that spleen and liver are the primary sites of platelet destruction, accounting for 61% of infused 111In‐platelets in normal volunteers and 85% in splenomegalies, while the liver is the primary site of platelet destruction, accounting for 89% in asplenic subjects.
Spectrum of HTLV‐III infection in a hemophilic cohort treated with blood products from a single manufacturer
Authors: McGrath Katherine M. , Spelman Denis , Barnett Mark , Kellner Sybil 
Published: February 2007

AbstractOne hundred fifty‐eight hemophilia A, B, and von Willebrand disease (VWD) patients treated with clotting factor concentrates from a single manufacturer were tested for antibody to the human T‐lymphotropic virus type III (HTLV‐III). Antibody was detected in 63% and 40% of those with severe hemophilia A and B, respectively 12% and 0% of those with mild hemophilia A and B, and two patients with recessive VWD. Forty‐two antibody‐positive and 20 antibody‐negative patients were studied for clinical and laboratory features of infection. Eleven seropositive patients had clinical signs of infection including Pneumocystis carinii pneumonia, lymphadenopathy, splenomegaly or diarrhea; however, only one patient had developed acquired immune deficiency syndrome (AIDS), and only two had significant impairment of their performance status. Thirty‐one patients remained totally asymptomatic. Eight patients had a history suggestive of acute HTLV‐III infection. Thrombocytopenia was observed in 18% of seropositive patients, lymphopenia in 60%, depressed T‐helper cells in 43%, reduced T‐helper: T‐suppressor ratios (TH: TS) in 33%, and elevated platelet‐bound immunoglobulin in 53%. The antibody‐negative group had normal T‐helper cell levels (except one patient) and TH: TS ratios, and normal platelet immunoglobulin levels. Both groups demonstrated a significant elevation of immunoglobulin levels and a high prevalence of antinuclear factor and antismooth muscle antibodies. The mean level of IgG was significantly higher in the antibody‐positive group. This study confirms the correlation between HTLV‐III infection and reduced T‐helper cells in hemophiliacs but demonstrates a low incidence of clinical symptomatology. There was evidence of polyclonal B‐cell hyperactivity in the antibody‐negative group as well as the seropositive group.
Characterization of purine nucleoside phosphorylase in leukemia
Authors: Morisaki Takayuki , Horiuchi Naomi , Fujii Hisaichi , Miwa Shiro 
Published: February 2007

AbstractPurine nucleoside phosphorylase (PNP) activity was determined in mononuclear cells from 49 patients with various types of leukemia. A low level of PNP activity was found in mononuclear cells from patients with acute myeloid and lymphoblastic leukemia and with chronic lymphocytic leukemia. Enzymatic and immunological studies on PNP from leukemic cells of these patients revealed no differences in Michaelis constant for inosine, thermostability, electrophoretic mobility, immunological reactivity, or specific activity between the PNP of leukemic cells and that of normal mononuclear cells. These results suggest that the decrease in PNP activity of leukemic cells is due to a decreased rate of enzyme synthesis. Thus, the abnormality of PNP activity might be due to an alteration in the regulatory mechanism of enzyme synthesis in the purine metabolism in the leukemic clone.
Human parvovirus and aplastic crisis in chronic hemolytic anemias: A study of 24 observations
Authors: Lefrère Jean‐Jacques , Couroucé Anne‐Marie , Bertrand Yves , Girot Robert , Soulier Jean‐Pierre 
Published: February 2007

AbstractFrom March 1984 through November 1985, 24 children and adults with aplastic crises were admitted in several Parisian hospitals. Twelve patients had known hemolytic anemia. Aplastic crisis revealed hemolytic anemia in the remaining patients. The detection of human parvovirus antigen was performed by counter‐immunoelectrophoresis, and specific IgM antibodies were detected by IgM‐antibody‐capture‐radioimmunoassy, in order to establish the incidence of human parvovirus infection in the genesis of the aplastic crisis. Twenty‐one patients had acute infection with human parvovirus. In the three remaining patients, no marker of human parvovirus infection was found. The features of the human parvovirus linked aplastic crisis are described. We consider that human parvovirus infection, and unknown hemolytic anemia, must be systematically researched in any case of unexplained acute aplastic anemia.
Is sickle cell crisis a thrombotic event?
Authors: Green David , Scott John Paul 
Published: February 2007

AbstractAlthough large vessel thrombi are occasionally reported in patients with homozygous sickle cell disease, the role of intravascular coagulation in typical pain crises is controversial. Therefore, we studied 24 sickle cell patients during and between episodes of pain crisis, using several sensitive tests of hemostasis. Fibrinogen was measured by a clotting assay, β3‐thromboglobulin (β‐TG) and fibrinopeptide A (FPA) were quantitated by radioimmunoassay, and protein C was determined by absorbing the zymogen from test plasma, activating it with thrombin‐thrombomodulin complex, and measuring activity with a selective synthetic substrate. Fibrinogen was elevated in asymptomatic patients (355 ± 145 mg/dl) but was no different from the value in these same patients during crisis (333 ± 180 mg/dl, p > 0.1). Similarly, β‐TG 136 ± 52 ng/ml vs 118 ± 56; FPA 3.7 ± 4.8 ng/ml vs 5.2 ± 4.5, and protein C 71 ± 20% vs 66 ± 19 showed no important changes during crisis. However, all these values were significantly different from those in age‐ and sex‐matched healthy controls. (β‐TG, fibrinogen, and FPA were elevated (p < 0.001, 0.005, and 0.05, respectively), and protein C was decreased (p < 0.003). We conclude that while chronic intravascular coagulation is common in patients with sickle cell disease, there is no evidence that the pain crisis per se is a thrombotic event.
Intravenous gammaglobulin (Gamimune) for treatment of chronic idiopathic thrombocytopenic purpura (ITP): A two‐year follow‐up
Authors: Warrier Indira A. , Lusher Jeanne M. 
Published: February 2007

AbstractThirteen subjects 5‐20 years of age with the chronic, autoimmune form of idiopathic thrombocytopenic purpura (ITP) were given intravenous gammaglobulin (Gamimune; Cutter Biological, Berkeley, CA) in a dose of 400 mg/kg per day for 5 consecutive days. Two of the 13 children had undergone splenectomy; the other 11 had not. Eight of these 13 children had also received corticosteroid therapy with no sustained increase in platelet of counts. Six of 13 children had a good or excellent response to the first 5 day course of gammaglobulin therapy, and one had a fair response. The peak platelet count occured within 7 days of the start of therapy except in one patient, whose platelet count peaked on day 12. Six of seven patients who initially responded to Gamimune required booster doses to maintain platelet counts at a safe level. All children had marked increases in serum IgG following Gamimune except one (who had undergone splenectomy for chronic ITP), who had high baseline levels of immunoglobulin G (IgG). No untoward reactions necessitating cessation of therapy were encountered during this study. The most common side effect observed was headache. During the first year of follow‐up after Gamimune, three of seven initial responders became refractory to Gamimune therapy. Two of these three refractory subjects later underwent splenectomy with excellent response. The third refractory patient who was splenectomized prior to gammaglobulin therapy had spontaneous remission of his ITP 5 months after the last dose of Gamimune. Three of the four other initial responders have continued to do well and have maintained platelet counts above 40,000/mm3 (one without booster). The fourth subject dropped out of the study. Thus our observations indicate that Gamimune is an effective form of treatment for some children with chronic ITP, and can be considered as an alternative to splenectomy or as a potential therapeutic modality in those who have failed to respond to splenectomy.
Efficacy of danazol in pure red cell aplasia
Authors: Lippman Scott M. , Durie Brian G. M. , Garewal Harinder S. , Giordano Gerald , Greenberg Bernard R. 
Published: February 2007

AbstractTwenty‐one unselected patients with refractory chronic anemias of various etiologies were treated with danazol, a synthetic attenuated androgen. All had previously failed treatment with hematinics, androgens, corticosteroids, high‐dose intravenous immunoglobulin, antithymocyte globulin, cytotoxic immunosuppressive agents, and/or plasmapheresis. Three patients with pure red cell aplasia and one with aplastic anemia responded. No responses were observed in 11 patients with myelodysplastic syndromes, two patients with myelofibrosis, and two with paroxysmal nocturnal hemoglobinuria. Remission in pure red cell aplasia was maintained with danazol alone in one patient and required combined low‐dose prednisone in two. Objective responses occurred in 2 to 3 weeks and therapy generally was well tolerated. To date, one patient with pure red cell aplasia remains in complete remission at 9 months on lowdose danazol alone. We conclude that danazol alone or combined with prednisone may induce and maintain remission in severe refractory pure red cell aplasia and possibly other chronic cytopenias characterized by immunologic marrow suppression. Further trials of danazol in treating these disorders are indicated.
Acute leukemia with t(4;11) following treatment for breast cancer
Authors: Archimbaud Eric , Charrin Christiane , Sebban Catherine , Fiére Denis 
Published: February 2007

AbstractWe report the case of a 51‐year‐old woman presenting t(4;11) acute undifferentiated leukemia 19 months after initiation of adjuvant radio‐chemotherapy for a breast cancer. Morphology of the blasts was FAB L2 and cytochemical and immunological studies were in favor of an undifferentiated leukemia. A possible relationship between t(4;11) leukemia and exposure to carcinogens is discussed.
Evaluation of the nature of mildly prolonged prothrombin times
Authors: Mazzucconi M. G. , Mariani G. , Chistolini A. , Lasagni R. Pasquali , Motta M. , Ghirardini A. , Altieri D. , Mannucci P. M. , Mandelli F. 
Published: February 2007

AbstractTwenty‐one asymptomatic individuals with a mildly prolonged prothrombin time ( > 2 SD from the prothrombin time of the reference plasma) were found to have a mild isolated factor VII (F VII) defect (x 38.8 U/dl; SD 13.2). Factor VII antigen levels were also found to be reduced (x 45.5 U/dl; SD 7.8) in 13 of them. These figures were compared with those of 50 normals and 28 obligatory heterozygotes for F VII deficiency. The phenotypical behaviors in the propositi were found to be equal to those of the F VII congenital deficiency heterozygotes: the discrepant one (VII+) and the nondiscrepant one (VII−/R).Fifteen families of the propositi could also be studied, totalling 55 additional individuals; in 25 of them (ten pedigrees) a mild F VII deficiency was found showing the same phenotypical features of the corresponding propositi.We therefore believe that these individuals with mild F VII deficiency can be considered as heterozygotes for the defect, since (1) the other vitamin K‐dependent clotting factors were normal; (2) the defect is transmitted throughout the kindred with the same mode of inheritance as F VII congenital deficiency; and (3) F VII: C and F VII:Ag levels are highly comparable with those of known obligatory heterozygotes for F VII deficiency.On the grounds of a careful statistical analysis we propose a formula which allows a discrimination between the two phenotypes of the heterozygotes for F VII congenital deficiency.In addition it is suggested that sensitive tissue thromboplastins should be used to pick up these mild defects.
Functional analysis of the marginating pool of human polymorphonuclear leukocytes
Authors: Berkow Roger L. , Dodson Robert W. 
Published: February 2007

AbstractThe intravascular pool of human polymorphonuclear leukocytes (PMN) is composed of one compartment which is circulating and another that is marginated to the vascular endothelium. Administration of B‐adrenergic agonists leads to a rapid demargination with an increase in the circulating PMN pool. The marginating PMN has previously been stated to represent an older PMN based on a higher cytochemical alkaline phosphatase activity. With the understanding that circulating PMN are heterogeneous with respect to function and size we undertook the present study to evaluate the contribution of the marginating PMN to functional and volume‐dependent heterogeneity. We found that PMN isolated 7 min after epinephrine administration, presumably enriched by marginating PMN, were not different in volume, biochemically measured alkaline phosphatase activity, stimulated superoxide anion release, degranulation, or phagocytosis. These data suggest that the circulating and marginating pools of PMN are interchangeable and that the marginating PMN are not enriched by a particular subpopulation of PMN.
Search for eosinopenia in hospitalized patients with normal blood leukocyte concentration
Authors: Krause John R. , Boggs Dane R. 
Published: February 2007

AbstractHospitalized patients were studied prospectively in an attempt to determine whether idiopathic eosinopenia in the absence of other major changes in blood cells is rare or is more frequent than is commonly recognized. Strict criteria for eosinopenia were used; patients with more than 10.0 × 109/liter total leukocyte count, or less than 4.0, as well as those with any form of hematologic cancer, or those receiving any form of cancer chemotherapy, were excluded from the study. With those criteria and exclusions, only 24 patients with eosinopenia were found among 24,300. Twenty were receiving some form of adrenal glucocorticosteriod (steroid) and of the other four, three had serious organic diseases for which they were receiving various drugs. The remaining patient, whose primary problem was depression, could have had druginduced eosinopenia. Thus, unexpected eosinopenia appears to be a very rare event or syndrome.A normal range for eosinophils was defined from studies of 740 medical students, which was 0.015 to 0.65 × 109/liter and was quite similar to previously reported values. The effect of acute or chronic steroid administration on eosinophils in normal human subjects was studied. Confirming studies reported for man and many other mammals, eosinopenia developed promptly, but disappeared within hours unless repeated doses were given. Literature on various types of eosinopenia was reviewed.
Role of immune stimulation in the etiology of multiple myeloma: A case control study
Authors: Cohen Harvey Jay , Bernstein Rosyln Julie , Grufferman Seymour 
Published: February 2007

AbstractAs hospital‐based case‐control study of 153 multiple myeloma (MM) cases and 459 controls was conducted to evaluate the hypothesis that chronic or frequent infections or allergic and autoimmune diseases might be of higher prevalence in individuals who develop MM. Information was obtained by direct interviews of subjects. Controls were matched to cases on age, sex, race, and hospital. “Immune‐stimulating conditions” included chronic infections such as pyelonephritis, urinary tract infections (UTIs), prostatitis, rheumatoid arthritis and other collagen vascular diseases, allergies, bronchitis, tuberculosis, cholecystitis, diverticulitis, and osteomyelitis. The overall odds ratio (OR) (odds of history of immune‐stimulating conditions in cases versus controls) was 0.4 (95% confidence interval = 0.3 − 0.7) which suggested that cases had significantly less immune‐stimulating conditions than did controls. The exposure rate for these conditions was high for cases (0.7) as well as for all control groups (0.8). These findings suggest that immune‐stimulating conditions alone are not the causative factor in the etiology of MM, though they may play a role in the predisposed individual.
Effects of zinc supplementation on linear growth in beta‐thalassemia (A new approach)
Authors: Arcasoy A. , Çavdar A. , Cin Ş. , Erten J. , Babacan E. , Gözdasoglu S. , Akar N. 
Published: February 2007

AbstractLinear growth was evaluated in 32 patients with beta‐thalassemia major. At the beginning of the study of 40.6% of the patients were below the 10th percentile with biochemical evidence of zinc deficiency. Effects of zinc supplementation on growth velocity (height) were assessed in a controlled manner. Twenty‐one children received oral zinc sulphate for a period of 1 to 7 years (15 early‐ and 6 late‐supplemented cases), while the remaining 11 thalassemics were maintained only on conventional transfusion therapy. The mean height velocity of early‐zinc supplemented children was significantly greater than that of normal children (P < 0.01). An increase in height was also observed in the patients who received delayed zinc retardation. The present study demonstrated that zinc deficiency is one of the factors responsible for retarded linear growth in beta‐thalassemia major. Only the patients who received zinc supplementation showed an acceleration of growth in height. Administration of zinc could, therefore, be considered as an effective adjuvant therapy in homozygous betathalassemia.
Effect of alpha interferon on the altered t‐b‐cell immunoregulation in patients with thalassemia major
Authors: Nualart Patricia , Estevez Maria E. , Ballart Isidro J. , De Miani Susana A. , Peñalver Jorge , Sen Luisa 
Published: February 2007

AbstractWe studied the ability of B‐lymphocytes to differentiate to immunoglobulin‐producing cells in 14 patients with thalassemia major, 11 parents of the patients, and 86 normal subjects. In comparison with the parents of the patients and with the normal individuals, the patients were found to have 1) an increased number of cells spontaneously secreting immunoglobulin; 2) a decreased number of plaque‐forming cells (PFC) when induced with pokeweed mitogen (PWM) and a deficient helper T‐cell function for the induction of B‐cell differentiation; 3) an increase in the number of PFC after the addition of alpha interferon (100 IU/ml) to the cell cultures with PWM. Previous incubation of non‐T‐cells with alpha interferon for 1 hr and subsequent coculture with T‐cells from the same patients or from normal subjects in the presence of PWM increased the number of PFC. On the contrary, previous incubation of T‐cells with alpha interferon followed by coculture with normal non‐T‐cells or cells from the same patient did not modify the number of PFC.These results suggest that patients with thalassemia major possess hyperreactive spontaneous B‐cell responses and that these B‐cells are unable to differentiate to immunoglobulin‐secreting cells in the presence of PWM. This would be due to a T‐helper‐cell deficiency with an inability to produce lymphokines (interferon being one of them), while the B‐cell function seems to remain intrinsically intact.