bluuebunny/crossref_metadata_2025_split huggingface dataset:
Erythropoietin affects the maturation pattern of fetal G‐CSF‐responsive progenitors
Authors: Christensen Robert D. , Rothstein Gerald 
Published: February 2007

AbstractA transient hyporegenerative neutropenia has been reported in neonates, but not in older children or adults, undergoing treatment with recombinant erythropoietin (epo). Monocytopenia has not been reported. We postulated that epo might selectively reduce the responsiveness of neonatal progenitors to Granulocyte Colony‐Stimulating Factor (G‐CSF), while not similarly affecting their responsiveness to Macrophage Colony‐Stimulating Factor (M‐CSF). To test this hypothesis two types of experiments were performed. First, progenitors of adult or fetal origin were pre‐incubated with epo (or control), then washed, and their responsiveness to G‐CSF and M‐CSF evaluated in clonogenic culture assays. Second, clonogenic maturation was initiated using either G‐CSF or M‐CSF, after which the effect of a late addition of epo to the developing clones was evaluated. Indeed, pre‐incubation with epo resulted in production of fewer neutrophils from fetal progenitors grown in G‐CSF (P<0.001), but it did not reduce the number of macrophages generated from progenitors grown in M‐CSF. Adding epo to the already‐developing G‐CSF‐responsive and M‐CSF‐responsive adult and fetal clones did not alter colony development. Thus, epo appears to have an action on G‐CSF‐responsive, but not‐M‐CSF‐responsive fetal progenitors, resulting in reduced production of neutrophils. This effect is no longer apparent, however, when progenitors have matured to the 8‐cell clone stage.
Erythromelalgia in thrombocythemia of various myeloproliferative disorders
Authors: Michiels Jan J. , ten Kate Fiebo J. W. 
Published: February 2007

AbstractErythromelalgia is caused by platelet‐mediated acral inflammation and arteriolar thrombosis in thrombocythemia in its primary form or associated with polycythemia vera. The prompt and lasting relief of burning pain by low‐dose aspirin is a prerequisite for the diagnosis of thrombocythemic erythromelalgia.Here we extend observations on the occurrence of erythtromelalgia in thrombocythemia associated with primary myelofibrosis, Philadelphia‐chromosome positive micromegakaryocytic myelofibrosis, and myelodysplastic syndrome type II. It is concluded that erythromelalgia may occur in thrombocythemia of all variants of chronic myeloproliferative disease as well as myelodysplastic syndrome if platelet counts are sufficiently high.
Immunophenotype and DNA content of myeloma cells in primary plasma cell leukemia
Authors: Shimazaki Chihiro , Gotoh Hideo , Ashihara Eishi , Oku Naritoshi , Inaba Tohru , Murakami Satoshi , Itoh Kunihiko , Ura Yasuaki , Nakagawa Masao , Fujita Naohisa 
Published: February 2007

AbstractTo clarify the biological characteristics of the myeloma cells in primary plasma cell leukemia (PCL), we studied the immunological phenotype and DNA content of cells from five patients with primary PCL as compared to those from the patients with typical multiple myeloma (MM). In two of five patients with PCL myeloma cells had B‐cell‐associated antigens (B1, J5) and surface immunoglobulin in addition to plasma cell associated antigens, suggesting that these cells are immunologically immature as compared to mature plasma cells. Concerning the DNA content of myeloma cells, two of four patients had hypodiploid while two had diploid myeloma cells. In contrast, 24 of the 37 (65%) patients with typical MM had hyperdiploid and 1 had hypodiploid myeloma cells. These observations suggest that the myeloma cells in PCL are immunologically heterogenous. The increased incidence of hypodiploidy in PCL may explain its relatively poor prognosis as previously shown in those with typical MM and hypodiploid DNA content.
Polysaccharide encapsulated bacterial infection in sickle cell anemia: A thirty year epidemiologic experience
Authors: Wong Wing Yen , Powars Darleen R. , Chan Linda , Hiti Alan , Johnson Cage , Overturf Gary 
Published: February 2007

AbstractAnnual age‐specific incidence rates of Streptococcus pneumoniae or Haemophilus influenzae bacterial septicemia in sickle cell anemia (SS) were determined for the years of 1957 through 1989. Forty‐nine patients had 64 episodes of septicemia among a population of 786 SS patients observed for 8,138 person‐years. Peak frequency of infection occurred between 1968‐1971 and 1975‐1981 with a conspicuous absence of episodes in 1972, 1973, 1982‐1984, and 1986‐1987, thus demonstrating cycles of high and low attack rates. The annual age‐specific incidence rate of septicemia varied from 64.5 (1965) to 421.1 (1980) per 1,000 person‐years for those under 2 years of age and never exceeded 10.2 per 1,000 in those over 4 years of age. Following the introduction of pneumococcal polyvalent vaccine in 1978, incidence of infection decreased in SS children greater than 2 years of age. No modification of the risk of infection was observed in immunized children less than 2 years of age.During these three decades, there has been a ten‐fold increase in the number of SS adults over 20 years of age. The relative risk of chronic sickle complications comparing the survivors of septicemia to the non‐infected patients was: subsequent death 1.76, retinopathy 4.06, avascular necrosis 1.95, symptomatic cholelithiasis 1.33, stroke 1.30, and priapsim 1.26. These data suggest that prognosis for lifetime severe SS is initially manifested as an increased risk of septicemia during childhood.
Increased levels of plasma thrombomodulin in chronic myelogenous leukemia
Authors: Morishita Eriko , Saito Masanori , Asakura Hidesaku , Jokaji Hiroshi , Uotani Chika , Kumabashiri Ichiro , Yamazaki Masahide , Matsuda Tamotsu 
Published: February 2007

AbstractCirculating blood plasma contains proteinase‐degraded forms of thrombomodulin that are soluble. We quantitatively assayed the plasma levels of thrombomodulin in 15 patients with chronic myelogenous leukemia (CML) in chronic phase by method of an enzymelinked immunosorbent assay using a monoclonal antibody to protease‐degraded products of thrombomodulin. Plasma levels of thrombomodulin in patients with CML at diagnosis were significantly increased (19.5 ± 6.2 ng/ml: means ± SD) compared with the levels in normal controls (8.0 ± 1.9 ng/ml, n = 20) (P < 0.001). Fibrin degradation products (D‐dimer), thrombin‐antithrombin III complex, and plasmin α2‐antiplasmin complex were almost normal, suggesting that intravascular coagulation or plasmin‐mediated fibrinolysis little occurred in these patients. On the other hand, the plasma levels of elastase‐α1‐proteinase inhibitor (E‐α1PI) complex, which was the indicator of released leukocyte elastase, were significantly increased in CML (P < 0.0001). The plasma levels of thrombomodulin and E‐α1 PI complex were decreased in parallel with decline of leukocyte counts in 10 patients with CML following anti‐leukemic therapy. Furthermore, a statistically significant correlation was observed between the plasma levels of thrombomodulin and E‐α1PI complex obtained at 39 time points in 15 patients with CML (r = 0.81, P < 0.001).These results suggest that the increased plasma levels of thrombomodulin in CML may be partly caused by leukocyte elastase, which may split the surface thrombomodulin and release protease‐degraded fragments of it into the circulation.
Hemolytic anemia in protoporphyria: Possible precipitating role of liver failure and photic stress
Authors: Key Nigel S. , Rank Jeffrey M. , Freese Deborah , Bloomer Joseph R. , Hammerschmidt Dale E. 
Published: February 2007

AbstractHemolytic anemia is not a common clinical feature in protoporphyria. In this report, we describe two patients in whom we have encountered severe hemolytic anemia. Both individuals had advanced hepatic disease as a complication of their porphyria and were undergoing orthoptic liver transplantation. The onset of hemolysis appeared to be related to the development of liver disease, and in both cases, the operative procedure acutely exacerbated the red cell destructive process. We suggest that liver involvement in protoporphyria may unmask hemolytic anemia, and that red cells in these individuals are hypersensitive to photooxidative stress, such as might occur during a prolonged operative procedure.
Application of immunocryoultramicrotomy to free cells: Ultrastructural localization of myeloperoxidase in neutrophils of human peripheral blood
Authors: Saito Nagahito , Takemori Nobuo , Hirai Katsuyuki , Kubota Noriko , Onodera Ryuichi , Namiki Masayoshi 
Published: February 2007

AbstractImmunocryoultramicrotomy is generally considered the most suitable method for observing the immunostainings by monoclonal antibodies to intracellular antigens by electron microscopy. However, this procedure with the free cells such as peripheral blood cells or cultured cells is not widely used because of the difficulty in preparing ultrathin frozen sections. We report here a new technique for making ultrathin frozen sections of peripheral blood cells by positioning the cell in a semigelatinized medium for ultrasectioning. Monoclonal antibody against myeloperoxidase (MPO) was reacted with the ultrathin sections made with this new technique to observe the ultrastructural localization of intracellular MPO. MPO was observed in the primary granules of the neutrophils.
Human parvovirus B19 infection in hemophiliacs first infused with two high‐purity, virally attenuated factor Vlll concentrates
Authors: Azzi A. , Ciappi S. , Zakvrzewska K. , Morfini M. , Mariani G. , Mannucci P. M. 
Published: February 2007

AbstractHuman parvovirus B19 can be transmitted by coagulation factor concentrates and is highly resistant to virucidal methods. To evaluate whether the additional removal of virus by chromatographic methods during the manufacture of high‐purity concentrates re‐duces the risk of B19 transmission, we have prospectively evaluated the rate of anti‐B19 seroconversion in two groups of susceptible (anti‐B19 negative) hemophiliacs infused with high‐purity, heated (pasteurized) or solvent‐detergent‐treated factor Vlll concen‐ trates. Both products infected a relatively high proportion of patients (nine of 20).
Granulocyte fcγ receptor recognition of cell bound and aggregated IgG: Effect of γ‐interferon
Authors: Ruiz Pedro , Gomez Francisco , Lopez Rafaela , Chien Paul , Rossman Milton D. , Schreiber Alan D. 
Published: February 2007

AbstractGranulocyte Fcγ receptors are important components in the recognition of IgG‐coated cells and immune complexes. Two proteins have been identified on resting human granulocytes which function as Fcγ receptors, Fcγ RII (CD32) and Fcγ RIII (CD16). A third protein, Fcγ RI (CD64), is not constitutively expressed on resting granulocytes, but can be induced by activation with γ‐interferon. We examined the role of these three Fcγ receptors on human granulocytes in the binding of both IgG‐sensitized erythrocytes and soluble oligomeric IgG. In these studies we employed anti‐Fcγ receptor antibodies which complete for the Fcγ RII and Fcγ RIII ligand binding sites. Preincubation of granulocytes with saturating concentrations of high‐affinity anti‐Fcγ RII monoclonal antibody did not alter the recognition of IgG sensitized human cells by granulocytes. Furthermore, ligand binding studies demonstrated that anti‐Fcγ RII antibody altered neither the number nor the affinity of granulocyte binding sites for human trimeric IgG. In contrast, Fab anti‐Fcγ RIII inhibited the binding of both IgG (anti‐D) sensitized human RBCs and IgG sensitized sheep RBCs. Similarly, a reduction in the expression of Fcγ RIII by treatment with phosphatidyl‐inositol specific phospholipase C reduced PMN recognition of IgG‐sensitized cells. Also, anti‐Fcγ RIII decreased the number of granulocyte binding sites for human IgG trimer without a change in receptor affinity. Fcγ RI, which was induced by γ‐IFN, increased granulocyte recognition of both IgG sensitized RBCs and IgG trimer. These data suggest that Fcγ RIII is the primary Fcγ receptor on granulocytes which recognizes IgG sensitized RBCs and low molecular weight complexes of IgG. With γ‐interferon activated granulocytes, Fcγ RI appears to enhance this recognition process.
Familial association of autoimmune thrombocytopenia and hyperthyroidism
Authors: Bizzaro Nicola 
Published: February 2007

AbstractAn association between thrombocytopenia and thyrotoxicosis in a single individual is well documented, and the theories for this event include a common immunologic cause or a thyrotoxic‐induced decrease in platelet survival.We report the first description of the coexistence of autoimmune thrombocytopenic purpura (AITP) and Graves' disease in several members of the same family, in which four females were thrombocytopenic and two of these were also hyperthyroid. All four patients had high titers of antiplatelet antibodies, and the two hyperthyroid cases were positive for thyroid‐stimulating immunoglobulins (TSI).The familial occurrence of two autoimmune disorders is very uncommon, and suggests a genetic etiology. The HLA phenotype was determined and the antigens B8 and DR3, which are reported with high frequency in both diseases, were present in three patients. Although the etiologic cause is still unknown, our findings further support the theory that a genetic predisposition underlies autoimmune disease.
Further evidence for recessive inheritance of von willebrand disease with abnormal binding of von willebrand factor to factor VIII
Authors: Lopez‐Fernandez M. F. , Blanco‐Lopez M. J. , Castiñeira M. P. , Batlle J. 
Published: February 2007

AbstractA new family with a bleeding diathesis and FVIII deficiency secondary to abnormal binding of von Willebrand factor (vWF) to factor VIII (FVIII) is described. Two propositi of this family, an 18‐year‐old male and a 33‐year‐old female, both with a history of epistaxis, bruising, bleeding from the gums, epistaxis, hemarthrosis, and hematoma, were analyzed. Also additional members of the same family with no bleeding history were also studied. The propositi showed normal vWF activities, low FVIII activity; one of them had been diagnosed as having hemophilia A and the other was a hemophilia A carrier. Both showed a very poor response to treatment with FVIII concentrates and desmopressin (DDAVP) but a good clinical response to cryoprecipitate. APTT was prolonged and no inhibitory activity was noticeable in their plasmas. Thirty‐five units per kilogram body weight of Hemofil M was infused to both propositi and FVIII reached basal level within 60 minutes of the infusion. No FVIII response at all was observed in the female after intravenous DDAVP administration. However, the male who received the infusion of 35 U/kg body weight of Humate‐P achieved a normal FVIII level that was maintained for 12 hours. Multimeric analysis of vWF was normal in all the members studied. Von Willebrand factor domain for FVIII binding was assayed in the two propositi and in six other members of the same family by using a non‐isotopic and sensitive method, a modification of the one previously described, using the Hemofil M concentrate as exogenous FVIII. The data obtained showed that both propositi had similar binding to that observed by using plasma of a patient with severe von Willebrand disease. Furthermore, five siblings had a decreased binding of vWF to FVIII, when compared with plasma from normal individuals or patients with hemophilia A. We also observed that, for screening purpose, the ratio of bound FVIII/immobilized vWF (at saturation of the anti‐vWF and offering of 1 U/ml of exogenous FVIII) distinguished two levels of abnormality (normal range 0.70–1.15, propositi 0,004–0.007, and remaining members affected 0.25–0.42). The most probable explanation is that the propositi are homozygous or double heterozygous, the other five siblings affected being heterozygous for a recessive vWF defect. This more accessible assay presented here may be of help in routine analysis for diagnosing this type of von Willebrand disease, which has important implications for therapy and genetic counseling. © 1992 Wiley‐Liss, Inc.
μ‐heavy chain disease: Presentation as a benign monoclonal gammopathy
Authors: Wahner‐Roedler Dietlind L. , Kyle Robert A. 
Published: February 2007

Abstractμ‐Heavy chain disease (HCD) is a rare monoclonal lymphoid disorder characterized by the failure to assemble a complete IgM immunoglobulin. The μ‐heavy chains analyzed to date revealed absence of the variable region and a shortened constant domain. We report the first case of μ‐HCD presenting as a benign monoclonal gammopathy. The literature on the 27 reported μ‐HCD cases is reviewed, and important clinical and laboratory findings are discussed. The ages of the patients ranged from 15 to 80 years (median, 57.5 years). Twenty‐two of 27 patients had an associated lymphoplasma cell proliferative disorder. A monoclonal spike on routine serum protein electrophoresis was found in only 8 of 19 patients. Fourteen of 22 patients had Bence Jones proteinuria, but μ‐HCD protein was reported in the urine of only two patients. The survival ranged from <1 month to 11 years (median, 24 months). © 1992 Wiley‐Liss, Inc.
Treatment of relapsed or refractory Hodgkin disease and non‐Hodgkin lymphoma with high‐dose chemoradiotherapy followed by unstimulated autologous peripheral stem cell rescue
Authors: Rapoport Aaron P. , Rowe Jacob M. , Heal Joanna M. , Tanner Martin A. , Dipersio John F. 
Published: February 2007

AbstractNine patients with relapsed or refractory Hodgkin disease or non‐Hodgkin lymphoma underwent peripheral stem cell autografting because of a history of marrow involvement with visible lymphoma. Peripheral stem cells were collected during a process of unstimulated leukapheresis. Recovery to a neutrophil count of 0.1 × 109/L was seen at a median of 14 days compared with a median of 18 days for a concurrent series of marrow recipients (P = .12). Recoveries to a neutrophil count of 0.5 × 109/L and a platelet count of 50 × 109/L occurred at medians of 25 days and 28 days, respectively. These figures are not significantly different from those obtained in the marrow recipients. Of the original nine patients, six are surviving free of disease progression or relapse at a median of 240 days post‐transplant. Two patients died of transplant‐related complications and one relapsed 250 days post‐transplant. All surviving patients remain independent of transfusions and six have attained almost complete hematological reconstitution.Although administration of cytotoxic therapy and granulocyte—macrophage colony‐stimulating factor (GM‐CSF) may enhance the yield of early progenitor cells during leukapheresis, unstimulated leukapheresis results in a stem cell product capable of rapidly restoring and sustaining marrow function even after multiple courses of intensive “salvage” therapy. Additional follow‐up will be needed to determine whether preliminary survival figures continue to compare favorably with those of patients with similar extramedullary disease states undergoing marrow transplants. © 1992 Wiley‐Liss, Inc.
Coincident Kaposi sarcoma and T‐cell lymphoma in a patient with the Wiskott‐Aldrich syndrome
Authors: Meropol Neal J. , Hicks David , Brooks John J. , Siminovitch Katherine A. , Fishman Neil O. , Kant Jeffrey A. , Bennett Joel S. 
Published: February 2007

AbstractA 24 year old male with a history of eczema, recurrent mild infections, and thrombocytopenia consistent with the Wiskott‐Aldrich syndrome (WAS) presented with a mediastinal mass, generalized lymphadenopathy, splenomegaly, and severe thrombocytopenia. Studies of immune function including immunoglobulin levels and T‐cell subsets were normal. Furthermore, his T lymphocytes proliferated normally in response to phytohemagglutinin, concanavalin A, and the combination of neuraminidase/galactose oxidase. However, their proliferative responses to anti‐CD43 antibody and periodate were diminished, consistent with the clinical diagnosis of WAS. An initial inguinal lymph node biopsy surprisingly revealed Kaposi sarcoma. However, following splenctomy to increase the platelet count, biopsy of the mediastinal mass revealed T‐cell large cell lymphoma. Studies of biopsied tissue for the presence of Epstein‐Barr virus and cytomegalovirus were negative, as were studies of blood, including the polymerase chain reaction, for the presence of the human immunodeficiency virus (HIV). This is the first report of Kaposi sarcoma arising in a patient with a congenital immunodeficiency syndrome. Although Kaposi sarcoma can arise in the face of the severe immunosuppression that follows allograft transplantation and in patients infected with HIV, we postulate that longevity in the face of mild immunosuppression was the major factor in the development of Kaposi sarcoma in this patient. © 1992 Wiley‐Liss, Inc.
Plasma cytokine levels in thrombotic thrombocytopenic purpura
Authors: Wada Hideo , Kaneko Toshihiro , Ohiwa Michiaki , Tanigawa Motoaki , Tamaki Shigehisa , Minami Nobuyuki , Takahashi Hoyu , Deguchi Katsumi , Nakano Takeshi , Shirakawa Shigeru 
Published: February 2007

AbstractPlasma cytokine levels were examined in 13 patients with thrombotic thrombocytopenic purpura (TTP). Auto‐antibodies, platelet‐associated immunoglobulin G, and platelet aggregating factor were detected in many of these patients and high‐molecular‐weight bands of von Willebrand factor multimers were reduced in 9 of 10 patients examined. Complete remission (CR) was attained in 7 of the 13 patients, but 6 died. Tumor necrosis factor (TNF), Interleukin (IL)‐1β, IL‐6, and soluble IL‐2 receptor showed marked increases at onset and decreased at CR. The prognosis tended to be poor in patients with increased IL‐6 and soluble IL‐2 receptor levels. These findings suggest that immunological mechanisms, such as the activation of macrophage, are involved in the pathogenesis of TTP and are reflected in the plasma cytokine levels. © 1992 Wiley‐Liss, Inc.
Bleeding tendency, platelet function, and pharmacokinetics of ibuprofen and zidovudine in HIV(+) hemophilic men
Authors: Ragni Margaret V. , Miller Barbara J. , Whalen Robert , Ptachcinski Richard 
Published: February 2007

AbstractThe use of ibuprofen (IBP) in hemophilic men for chronic hemophilic arthropathy is associated with transient coagulation abnormalities, but usually does not cause bleeding symptoms. However, when hemophilic men are treated with ibuprofen while also receiving zidovudine (ZDV), excess bleeding has occurred in some. In order to evaluate platelet function and pharmacokinetics of combination IBP and ZDV, we measured platelet aggregation, platelet adhesive index, bleeding time, and IBP and ZDV drug levels by high performance liquid chromatography on five patients receiving chronic oral IBP, 400 mg every 6 hr, and on ten patients receiving both IBP and ZDV, 100 or 200 mg every 4 hr five times daily. Samples were obtained at baseline (ZDV alone), acutely (IBP + ZDV acutely), and chronically (2 weeks on IBP and ZDV). Abnormal platelet aggregation with arachidonic acid occurred in four of five (80%) of those receiving IBP alone and in 7 of 10 (70%) at baseline, 9 of 10 (90%) at acute, and 8 of 10 (80%) at chronic IBP and ZDV treatment, most commonly at 2 hr following dosing, when peak IBP levels occurred, and persisting 4 hr in those on chronic dosing. Half or more of those on combination IBP + ZDV showed a lowered platelet adhesive index and/or prolonged bleeding time. Excess bleeding symptoms occurred in three on chronic combination IBP + ZDV, two with increased frequency of spontaneous hemorrhages, and one with prolonged traumatic bleeding. Bleeding tendency was unrelated to the degree of platelet function abnormality, to peak drug levels of IBP or ZDV, or degree of liver function abnormality (SGPT). The clearance of IBP alone and ZDV alone were not different from the clearance of combination IBP and ZDV. Caution is urged regarding potential enhanced bleeding tendency in hemophiliacs receiving both IBP and ZDV in combination. © 1992 Wiley‐Liss, Inc.