bluuebunny/crossref_metadata_2025_split huggingface dataset:
CD21 antigen in T‐lineage neoplastic lymphoid cells: Characteristic expression at thymic stage
Authors: Tatsumi Eiji , Yoneda Noriko , Kawano Seiji , Yamaguchi Nobuo 
Published: February 2007

AbstractThe expression of CD21 antigen, a receptor for the C3d fragment of complement and Epstein‐Barr vlrus (EBV), was Investigated in a total of 85 cases of neoplastic lymphoid cells Including 39 cases of T‐lineage acute lymphoblastic leukemia (ALL)/lymphoblastic lymphoma (LBL), although CD21 antigen is usually regarded as a pan‐B antigen. The CD21 antigen was expressed by one of the eight cases of neoplastic lymphoid cells expressing the CD7 antigen as a sole pan‐T antigen, by three of the 20 cases of pro‐ or early thymic stage (CD7+ CD5+ CD2‐, CD7+ CD5‐ CD2+, or CD7+ CD5+ CD2+), and ten of 11 cases of thymic stage (CD3r CD4+ CD8+), but not by one case of late thymic stage (CD3 ± CD4+ CD8‐) T‐ALULBL cells. The CD21 antigen was not expressed by any of the 11 cases of adult T‐cell leukemia (ATL) or two cases of chronic T‐lineage leukemia. At most 4% of the normal thymocytes obtained from seven infants or children expressed the CD21 antlgen. While only a very limited population of normal thymocytes expresses CD21 antigen, T‐ALL/LBL cells at the thymic stage characteristically express CD21 antigen in contrast to pro‐ or early thymic ALL/LBL or peripheral‐stage neoplastic T cells. The estimation of the expression of CD21 antigen is useful for delineating stages of differentiation in T‐ALL/LBL. Furthermore, these observation are notable, considering the possibiilty that the reported EBV‐carrying T‐cell lymphomas result from the penetration of EBV into EBV‐negative neoplastic T cells. © 1994 Wiley‐Liss, Inc.
Expression, of the thrombospondin receptor (CD36) on the cell surface in megakaryoblastic and promegakaryocytic leukemias: Increment of the receptor by megakaryocyte differentiation in vitro
Authors: Imamura Nobutaka , Ota Hiromi , Abe Kazuhiro , Kuramoto Atsushi 
Published: February 2007

AbstractIn this report we describe four cases of acute megakaryocytic leukemia demonstrated by the presence of megakaryocyteplatelet‐related cell‐surface antigens. These were detected utilizing flow cytometry and monoclonal antibodies in addition to both platelet peroxidase activity, which was shown by ultrastractural cytochemistry, and emergence of differentiation antigens, while culturing these leukemic cells. The blasts of one patient possessed both platelet Gplb and Gpllb/llla cell‐surface antigens detected by AN51(CD42b), J15, P2, and HPL2(CD41), respectively, whereas the remaining three patients almost completely lacked Gplb cell‐surface antigen. Hence the former were diagnosed as immature(pro)megakaryocytic leukemia and the latter as acute megakaryoblastic leukemia from the viewpoint of immunophenotypic analysis. While we cultured these leukemic cells in conditioned medlum prepared from phytohemagglutinin‐stimulated leukocytes and interleukin 3, expression of CD36(OKM5) antigen (thrombospondin receptor) increased gradually according to the differentiation and maturation of these cells. Finally, all leukemic cells differentiated to mature megakaryocytes. The function of CD36 on these cells remains to be elucidated. © 1994 Wiley‐Liss, Inc.
Reversible encephalopathy after cryopreserved peripheral blood stem cell infusion
Authors: Dhodapkar Madhav , Goldberg Stuart L. , Tefferi Ayalew , Gertz Morse A. 
Published: February 2007

AbstractWe report two patients who experienced severe reversible encephalopathy following infusion of peripheral blood stem cells cryopreserved in 10% dimethylsulfoxide (DMSO). In one patient, reduction of DMSO level with plasmapheresis resulted in marked improvement in encephalopathy. Infusion of large volumes of cryopreserved stem cells may result in a signiflcant toxlc reaction. Plasmapheresis may be a treatment option for patients with significant toxicity related to DMSO. © 1994 Wiley‐Liss, Inc.
Detection of PML‐retinoic acid receptor‐α fusion transcripts in acute promyelocytic leukemia with trisomy 8 but without t(15;17)
Authors: Ikeda Kazuma , Sasaki Kazunori , Tasaka Taizo , Nagai Masami , Kawanishi Koichi , Takahara Jiro , Irino Shozo 
Published: February 2007

AbstractChromosome translocation t(15;17), the breakpoints of which are in the PML gene on chromosome 15 and retinoic acid receptor‐α (RARα) gene on chromosome 17, is specifically found in acute promyelocytlc leukemia (APL). Clinically typical APL without t(15;17) and with the PML‐RARα fusion transcripts or rearrangements in PML and/or RARα gene has been reported, suggesting submicroscopic changes at the molecular level without apparent t(15;17) or observation of normal metaphases. Trisomy 8 is common in APL as a secondary chromosomal abnormality in addition to t(15;17), as well as in acute myelogenous leukemia in general, but it is rare as a sole chromosomal anomaly in APL. PML‐RARα fusion transcript was detected in an APL case with trisomy 8 but without t(15;17), indicating that the leukemic cells lacked t(15;17) and still expressed the PML‐RARα fusion transcripts. This indicates that the same submicroscopic molecular changes as in APL with t(15;17) do occur in APL without t(15;17) and supports the use of molecular analysis for PML‐RARα fusion in APL. © 1994 Wiley‐Liss, Inc.
Malignant histiocytosis associated with autoimmune thrombocytopenia
Authors: Ohno Tatsuharu , Sugiyama Tateo , Furukawa Hiroo , Ishibashi Takafumi , Okuma Minoru 
Published: February 2007

AbstractWe describe a patient with malignant histlocytosis whose serum contained an autoantlbody against platelet protein. The patient was admitted because of nasal bleeding and high‐grade fever. The clinical course was fulminantly progressive and terminated with cerebral hemorrhage. Bone marrow aspirate showed the proliferation of large atypical cells, some of which exhibited phagocytic activity. At postmortem examination, there was diffuse infiltration of these atypical cells In the liver, spleen, kidney, and bone marrow. Morphological, cytochemlcal, immunohistochemlcal, and genotypic characteristics suggested that the proliferating cells were derived from the monocyte‐rnacrophage system. Western blot analysis revealed the presence of autoantibody against an ∼88 kd molecule of platelet proteins. Although the relationship of cause and effect remains to be clarified, this autoantibody appeared to have stimulated thrombophagocytosls of the neoplastic cells. © 1994 Wiley‐Liss, Inc.
Mild β+(−87)‐thalassemia CACCC box mutation is associated with elevated fetal hemoglobin expression in cis
Authors: Gilman John G. , Manca Laura , Frogheri Laura , Pistidda Paola , Guiso Luciana , Longinotti Maurizio , Masala Bruno 
Published: February 2007

AbstractThe β°‐thalassemia codon 39 nonsense mutation predominant in Sardinia is severe, and homozygotes are transfusion dependent. Two‐thirds of β° 39 alleles are linked to AγT (haplotype II). Onefourth are linked to AγI (haplotypes I and IX), as is the mild β+‐thalassemia −87 C→G mutation (haplotype VIII). β+/β°‐Thalassemla VIII/II compound heterozygotes have Significantly higher AγI:AγT (23:7) than β°‐thalassemia I/II (24:20) or IX/II (16:17) cases. This suggests that the β+ −87 mutation is associated with elevated γ expression in cis, which may contribute to the lack of transfusion‐dependence in β+/β° Cases. © 1994 Wiley‐Liss, Inc.
Alpha thalassemia and stroke risk in sickle cell anemia
Authors: Adams Robert J. , Kutlar Abdullah , McKie Virgil , Carl Elizabeth , Nichols Fenwick T. , Liu Jin Cal , McKie Kathleen , Clary Anthony 
Published: February 2007

AbstractIn an effort to identify possible risk factors for stroke in Sickle Cell Anemia (Hb SS), we analyzed the distribution of alpha‐globin gene deletions in a group of Hb SS patients with and without stroke. The group with stroke consisted of 44 patients, (27 male, 17 female) with a mean of 7.5 years at time of stroke. The control group (non‐stroke) had 256 Hb SS patients (126 male, 130 female) with a mean age of 7.7 years. There were 9 patients with heterozygous α‐thalassemia in the stroke group (20.5%). In the control group, there were 93 patients with heterozygous α‐thalassemia and 5 with homozygous α‐thalassemia. The incidence of α‐thalassemia in Hb SS patients without stroke (38%) was comparable to that reported for the African‐American population in general. The incidence in the stroke population (20.5%) was significantly lower (P = 0.02) These results indicate that α‐thalassemia is associated with a lower risk of stroke in Hb SS. This observation should be confirmed in studies involving larger numbers of patients. Possible protective effects of α‐thaiassemia are unknown but may be related to decreased hemolysis and more favorable rheologlc properties of red blood cells. © 1994 Wiley‐Liss, Inc.
Serum transferrin receptor in hereditary hemochromatosis and African siderosis
Authors: Baynes Roy D. , Cook James D. , Bothwell Thomas H. , Friedman Brian M. , Meyer Theo E. 
Published: February 2007

AbstractThe present investigation evaluated the serum transferrin receptor concentration in subjects with nontransfusional iron overload who were identified in two separate studies on the basis of a serum ferritin level above 400 μg/L. Subjects with preciinical hereditary hemochromatosis were evaluated in the first study and those with the African form of iron overload in the second. in the first study, hereditary hemochromatosis was identified in 14 white men on the basis of a persistent elevation in transferrin saturation above 55%. The serum receptor concentration was elevated above the upper cut‐off of 8.5 mg/L in two of the subjects, but the mean receptor of 6.1 ± 1.4 mg/L (mean ± 2 SE) did not differ significantly from the normal mean for this assay of 5.6 ± 0.3 mg/L. In the same study, 60 control subjects with secondary iron overload were identified on the basis of a serum ferritin persistently above 400 μg/L, with a normal serum C‐reactive protein concentration but with a transferrin saturation <55%. Three of these subjects had an elevated serum receptor concentration but the mean value of 5.5 ± 0.4 mg/L did not differ from normals nor from subjects with hemochromatosis. In the second study, 49 black Africans with iron overload were divided into those with or without an elevated transferrin saturation. The mean serum receptor concentration of 5.0 ± 0.8 mg/L and 4.5 ± 0.4 mg/L, respectively, did not differ statistically. It was concluded that there is no evidence of generalized dysreguiation of the transferrin receptor in hemochromatosis or African siderosis. © 1994 Wiley‐Liss, Inc.
Thrombotic microangiopathy and retroviral infections: A 13‐year experience
Authors: Ucar Antonio , Fernandez Hugo F. , Byrnes John J. , Lian Eric C‐Y , Harrington William J. 
Published: February 2007

AbstractEleven of fifty serum samples collected from patients with a diagnosis of thrombotic microangiopathy (TMA), from 1979 to 1991, tested positive for antlretroviral antibodies. Seven had human immunodeficiency virus (HIV) infection, and four had human iymphotrophic virus, type I (HTLV‐I) infection. All patients were treated with plasma exchange and for infusion, but only two of the HIV‐infected patlents obtained a complete response (CR) and one of them died after a few months. Combined results from the ilterature indicate that most patients with HIV infection survive less than one year from the initial diagnosis of TMA. In the setting of HIV infection, TMA Is a treatable condition, but survivai for most patients is less than 12 months. Three of the four HTLV‐I infected patients with TMA had a CR. These observations strongly suggest that both HIV and HTLV‐I infections are associated with TMA, but rigorous epidemiologic studies will be needed to determine the relative risk for each. Retrovirai Infections should be considered In patients with TMA, especially If the patient has associated risk factors and demographic characteristics. © 1994 Wiley‐Liss, Inc.
Studies on the platelet surface‐associated von Willebrand factor
Authors: Martin S. Eric , Johnson Margaret M. , Abel Robert F. 
Published: February 2007

AbstractThe contribution of platelets to the prevention and control of bleeding depends not only on the plasma and vessel wall yon Willebrand (vW) factor, but also on the vW factor originating from platelets. Although platelet vW factor is mainly stored in the α‐granules, small amounts of vW factor have been detected on the platelet surface under conditions without direct stimulation. This communication focuses on the small amount of vW factor associated with the surface of platelets.EDTA‐washed platelets in a calcium‐free medium containing prostaglandin E1 (PGE1) were exposed to anti‐vW factor F(ab')2 fragments. The platelet‐associated anti‐vW factor antibodies were separated from the free fragments by gel chromatography. Normal platelets were compared to platelets from patients with Bernard Soulier syndrome, Glanzmann's thrombasthenia, and Hermansky‐Pudlak syndrome. These experiments showed that, in the absence of extracellular calcium, vW factor can be detected on the surface of washed platelets independent of glycoproteins lb and llb‐llla, and despite markedly reduced intracellular calcium content. This particular platelet surface expression of vW factor is probably the result of disturbing the platelet during the in vitro manipulation.von Willebrand factor is an extremely important element in the multiple molecular interactions required to stop and anchor the platelet in areas of high flow rates. Mechanical perturbation of platelets leading to surface expression of the vW factor helps increase its availability for such critical interactions. © 1994 Wiley‐Liss, Inc.
Perioperative administration of a single dose of conjugated oestrogen to uraemic patients is ineffective in improving haemostasis
Authors: Jacobs Peter , Jacobson Jack , Kahn Delawir 
Published: February 2007

AbstractThe excessive bleeding that occurs in uraemic patients undergoing surgery is reported to be reversed by at least one schedule of prior oestrogen administration. To assess this possibility a single intravenous injection of 25 mg of the conjugated hormone was given to ten uraemic patients undergoing regular haemodialysis and randomly compared to eight matched patients who received only the diluent. Two hours later there was no reduction in bleeding time, improvement in platelet aggregation, nor an increase in plasma levels of factor VIII (VIII:C), von Willebrand factor (VWF:Ag), or the factor VIII‐related ristocetin cofactor state. Furthermore, there was no reduction in intraoperative blood loss, being a median of 558 ± 154 (range 225‐1, 260) mL in the test group and 412 ± 55 (range 270‐555) mL in the control and additional median postoperative portovac drainage was 123 ± 27 (range 0‐170) mL and 153 ± 41 (range 45‐240) mL, respectively. These findings differ from the shortening in bleeding time achieved by others with repeated administration of the same agent, and emphasize the importance of dose and duration of oestrogen therapy when used for this indication. © 1994 Wiley‐Liss, Inc.
Elevated soluble CD4 levels in the cerebrospinal fluid in patients with adult T‐cell leukemia
Authors: Uozumi Kimiharu , Hanada Shuichi , Ishitsuka Kenji , Ohno Nobuhito , Otsuka Maki , Shimotakahara Shigemi , Nakahara Katsushi , Takeshita Taketsugu , Chyuman Yoshiko , Kuwazuru Yasuo , Makino Torahiko , Saito Takeshi , Ishibashi Kazuaki , Iwahashi Masahito , Utsunomiya Atae , Arima Terukatsu 
Published: February 2007

AbstractLevels of the soluble form of the leukocyte surface antigen CD4 (sCD4) were measured by enzyme linked immunosorbent assay (ELISA) in the cerebrospinal fluid (CSF) of patients with adult T‐cell leukemia (ATL) and other malignant and non‐malignant diseases. All patients with ATL and meningeal infiltration had markedly elevated levels of sCD4 in the CSF (53.7 ± 34.9 U/ml). ATL patients without CSF pleocytosis often had elevated levels of sCD4 (15.1 ± 9.2 U/ml). Non‐ATL patients with CSF pleocytosis had elevated levels of sCD4 (23.3 ± 12.2 U/ml) and those without CSF pleocytosis also showed elevation of sCD4 levels (16.8 ± 9.3 U/ml). However, the mean levels of sCD4 in CSF from these patients were significantly lower than ATL patients with meningeal infiltration. Soluble CD4 in the CSF from healthy volunteers were below the detectable limit. We conclude that meningeal infiltration of CD4(+) ATL cells is strongly associated with elevated sCD4 levels in CSF, and some part of sCD4 in CSF may be originated from the native cells in the CNS as a response of inflammatory stimulations. Therefore, measurement of sCD4 may be useful in the diagnosis of meningeal infiltration and/or meningeal irritation in patients with ATL. © 1994 Wiley‐Liss, Inc.
Fetal hemoglobin synthesis in sickle cell anemia: Some molecular considerations
Authors: Bhaumik Kabita 
Published: February 2007

AbstractAn ability to maintain high levels of fetal hemoglobin (Hb F) has been associated with the amelioration of the clinical severity of the sickle cell disease (SS). Clinical efforts to increase the Hb F level of the patients have, however, yielded variable therapeutic response. In an attempt to further elucidate the underlying molecular basis, in vitro Hb F synthesis was studied in erythroid progenitor (BFU‐E) cells obtained from SS patients and their heterozygous (AS) relatives with varying genetic backgrounds. This allows us to study the Hb F biosynthesis pattern uncomplicated by the influence of the preferential survival of “F cells” In vivo. The Hb F levels and the relative concentrations of its constituent gamma globin chains, Gγ and Aγ, were assayed using the reversed phase HPLC method. A percentage increase in the fetal hemoglobin content was observed in the lysates of the erythroid progenitor cells relative to the circulating peripheral blood erythrocyte values in SS patients and their AS relatives with different βs haplotypes reflecting the intrinsic capacity of fetal hemoglobin synthesis in these subjects. No such increase was observed in the patient with the Mor haplotype. Furthermore, the Hb F synthesized in the BFU‐E colonies was more of the adult type, as evidenced by the decrease in the percent Gγlevel relative to the corresponding peripheral blood values of the subjects in all the haplotype groups studied. The Mor haplotype was again an exception, synthesizing fetal hemoglobin more of the fetal type. © 1994 Wiley‐Liss, Inc.
Genetic linkage studies in antithrombin‐deficient kindreds using a highly polymorphic trinucleotide short tandem repeat (str) within the human antithrombin gene
Authors: Ni H. , Waye J. S. , Sheffield W. P. , Eng B. , Blajchman M. A. 
Published: February 2007

AbstractPCR amplification and analysis of short tandem repeats (STR) have provided a useful tool for genetic linkage studies and for the diagnosis of genetic disorders. We have recently identified a novel trinucleotide STR, (ATT) (TAA), in the fifth intron of the human antithrombin gene (AT3) located on chromosome 1q23. PCR amplification, cloning, and sequence analysis revealed this AT3‐STR to be highly polymorphic with repeat units ranging in size from (ATT)5 to (ATT)18 Ten distinct alleles were found in 81 unrelated Caucasian individuals (162 alleles) with an observed heterozygosity of 81%. Genetic linkage studies using the AT3‐STR in two previously described antithrombin (AT)‐deficient kindreds, AT‐Hamilton (Ala 382 Thr) and AT‐Amiens (Arg 47 Cys), demonstrate, in a given kindred, that a specific AT3‐STR polymorphism is strongly associated with a particular AT mutation. Thus, this highly polymorphic AT3‐STR should be very useful in performing linkage studies in AT‐deficient kindreds as well as in investigating other chromosome 1‐related genetic disorders. © 1994 Wiley‐Liss, Inc.
Increased binding of SP1 to the γ‐globin gene promoter upon site‐specific cytosine methylation
Authors: Sengupta Pritam K. , Lavelle Donald , Desimone Joseph 
Published: February 2007

AbstractWe have studied the effect of site‐specific cytosine methylation on the binding of nuclear proteins to the γ‐globin promoter region from −71 to −34. This sequence was recently shown to contain a stage selector element responsible for increased transcription from the γ compared to the β promoter when both are linked to Locus Control Region enhancer sequences. We observed that Sp1 binding to this sequence is increased upon site‐specific cytosine methylation, such that only methylation of the cytosine in the −50 CpG dinucleotide effects Sp1 binding. Methylation of the distal C of the Hpall site has no effect. The possible functional role of Sp1 as a repressor of γ‐globin synthesis is discussed. © 1994 Wiley‐Liss, Inc.
Reversal of nephrotic syndrome due to reactive amyloidosis (AA‐type) after excision of localized Castleman's disease
Authors: Perfetti Vittorio , Bellotti Vittorio , Maggi Anna , Arbustini Eloisa , De Benedetti Fabrizio , Paulli Marco , Marinone Maria Gabriella , Merlini Giampaolo 
Published: February 2007

AbstractThe patient (TAL), a chronic asymptomatic HBV carrier with HBsAg‐anti‐HBsAg circulating immune complexes, was admitted to our hospital because of a nephrotic syndrome due to renal amyloidosis. There was no family history of hereditary amyloidosis. Recurrent arthralgias, asthenia, and weight loss were the prominent clinical features. Laboratory test results showing that severe chronic inflammatory activity had been present for 6 years. Interleukin‐6 (IL‐6) serum concentration was 10 times normal and C‐reactive protein was 1.9 mg/ml. A complex immunological picture was also present (immune complex formation, exuberant B‐cell reactivity, and decrease in the number of CD4 T cells). A localized form of Castleman's disease (CD) (plasma‐cell type) was diagnosed by surgical excision of a giant axillary lymph node. AA amyloid was present in the blood vessels. Within 60 days after excision of the mass, the systemic symptoms subsided, laboratory signs of inflammatory activity disappeared and IL‐6 serum concentration returned to normal, thus establishing a causal relationship between the localized Castleman's disease, elevated IL‐6 concentration and the chronic inflammation responsible for AA amyloidosis. At 10 months of follow‐up, the nephrotic syndrome has reversed, kidney function has slowly ameliorated, and the patient has gained 12 kg. Abdominal fat aspirates drawn to search for amyloid, positive before surgery, were subsequently negative. The latter finding, and the remission of the nephrotic syndrome, provided strong evidence for regression of the amyloid deposits. However, the HBsAg‐anti‐HBsAg immune complexes and depression of T‐helper cell activity persist. This immunological derangement is therefore not a consequence of CD. Chronic stimulation of the immune system due to the patient's inability to eliminate HBV, in the contest of perturbed immunity, may have favored the genesis of the lymphadenopathy. © 1994 Wiley‐Liss, Inc.