bluuebunny/crossref_metadata_2025_split huggingface dataset:
Edta‐dependent pseudothrombocytopenia: A clinical study of 18 patients and a review of the literature
Authors: Berkman Neville , Michaeli Yossef , Or Reuven , Eldor Amiram 
Published: February 2007

AbstractEDTA‐dependent pseudothrombocytopenia (EDTA‐PTCP) is the phenomenon of a spurious low platelet count due to the appearance of antibodies that cause platelet agglutination in blood anticoagulated with EDTA. We review here the clinical features of 18 patients with EDTA‐PTCP treated in our hospital from 1984 to 1987 as well as those of 34 patients reported in the literature. This phenomenon appears more frequently in severely ill patients, in association with autoimmune, neoplastic, atherosclerosis‐related, and liver diseases. In the majority of our patients, EDTA‐PTCP appeared during hospitalization, indicating that the antibody is an acquired one. Neither splenomegaly nor the presence of autoimmune markers were features of this entity. Unlike true thrombocytopenias, EDTA‐PTCP is associated with a normal mean platelet volume. Awareness of this entity is essential since EDTA‐PTCP is frequently misdiagnosed and therefore incorrectly treated.
CD71 phenotype and the value of gallium imaging in lymphomas
Authors: Feremans Walter , Bujan Willem , Neve Pierre , Delville Jean‐Pierre , Schandene Liliane 
Published: February 2007

AbstractTumor cells of 14 cases of non‐Hodgkin lymphomas and 2 cases of Hodgkin disease were tested for the presence of the transferrin receptor (CD71) by flow cytofluorimetry before 67gallium imaging. It appeared that expression of CD71 phenotype was closely related to the positivity of gallium scan before therapy. We feel that this test is able to predict the avidity for 67gallium and the clinical implications are discussed.
Arsenic intoxication presenting as a myelodysplastic syndrome: A case report
Authors: Rezuke William N. , Anderson Crawford , Pastuszak William T. , Conway Stephen R. , Firshein Stephen I. 
Published: February 2007

AbstractA case of arsenic intoxication presenting as a myelodysplastic syndrome is reported. A 41‐year‐old woman with a 6‐month history of gastrointestinal and neurological symptoms was noted to be pancytopenic at presentation. A bone marrow aspirate revealed dysmyelopoietic changes involving all three marrow cell lines. Subsequent analysis of urine for heavy metals demonstrated very high levels of arsenic. Treatment with British anti‐Lewisite (BAL) resulted in the resolution of gastrointestinal symptoms and hematologica abnormalities although the neurological complications progressed. This case emphasizes that heavy metal intoxication should be considered in the differential diagnosis of any individual presenting with the hematological features of myelodysplasia especially when accompanied by clinical features considered atypical for primary or secondary myelodysplasia.
Analysis of tumor‐specific immunoglobulin gene rearrangement in peripheral blood B‐cells of multiple myeloma patients
Authors: Baldini L. , Cro L. , Delia D. , Chiorboli O. , Neri A. , Maiolo A. T. 
Published: February 2007

AbstractThe search for bone marrow clone neoplastic precursors in the peripheral blood of multiple myeloma (MM) patients has been the subject of a number of studies which, using different methodological approaches, have led to conflicting results. With the aim of contributing toward resolving this controversy, immunoglobulin (lg) gene rearrangement in the bone marrow mononuclear cells (BMMC) and B‐lymphocyte enriched peripheral cells (BePBC) of 11 appropriately selected advanced MM patients was studied by means of Southern blotting.Peripheral mononuclear cells (PBMC) and BePBC were studied immunophenotypically by evaluating the presence of cytoplasmic Ig positive cells (Cylg+) and CD19/PCA‐1 reactivity. A pattern of Ig gene rearrangement identical to that observed in the respective BMMC was found in only two patients who, moreover, had a significant number of Cylg+ cells. Ig gene rearrangement was not found in any of the remaining patients who showed no evidence of the presence of Cylg+ cells, although there were CD19/PCA‐1 positive cells. Consequently, the significance of the presence of malignant pre‐plasma cell peripheral B‐lymphocytes needs to be reconsidered and, in any case, these cells should be looked for in cell populations greatly enriched in B‐lymphocytes and, above all, completely lacking in plasma cells or Cylg+ cells.
Spontaneous endocytosis in human neonatal and adult red blood cells: Comparison to drug‐induced endocytosis and to receptor‐mediated endocytosis
Authors: Colin Francine C. , Schrier Stanley L. 
Published: February 2007

AbstractNeonatal RBC contain many more spontaneous endocytic vacuoles than do adult RBC. It is not known if this difference is a result of an increase in production of vacuoles in the neonatal RBC (as is the case in drug‐induced endocytosis), or is the result of a less effective neonatal macrophagic “pitting” process.Using an in vitro model of spontaneous endocytosis, we compared the rate and quantity of vacuoles and the shape of cord and adult RBC containing pits, visible by interference contrast microscopy (Nomarski method). The mechanism of the spontaneous endocytosis was explored using different inhibitors: sodium vanadate an inhibitor of ATPases, sodium fluoride which inhibits the generation of ATP and sodium cyanide a potent inhibitor of oxidative phosphorylation. We then compared spontaneous endocytosis with two other forms of RBC endocytosis: drug‐induced endocytosis and receptor‐mediated endocytosis.Spontaneous endocytosis is in fact increased in neonatal RBC initially but the increase in number of RBC containing pits after 144 hr of incubation is almost the same in adult RBC and neonatal RBC. Comparing spontaneous endocytosis with drug‐induced endocytosis, it appears that their mechanisms are different in that spontaneous endocytosis is not preceded by stomatocytic shape change and is not inhibited by sodium vanadate or sodium fluoride as is the case for drug‐induced endocytosis. Spontaneous endocytosis is different than transferrin receptor‐mediated endocytosis because it occurs in many RBC, not only in the motile R1 reticulocytes and is not inhibited by sodium cyanide as is receptor‐mediated endocytosis.Thus spontaneous endocytosis appears to be different than drug‐induced endocytosis and transferrin receptor‐mediated endocytosis. The increase in spontaneous endocytosis in cord RBC seen in vivo is probably a consequence of an immaturity of the neonatal macrophage pitting process.
Erythrocyte webb‐type glycophorin C variant lacks N‐glycosylation due to an asparagine to serine substitution
Authors: Telen Marilyn J. , Le Van Kim Caroline , Guizzo Mary Lou , Cartron Jean‐Pierre , Colin Yves 
Published: February 2007

AbstractWe have analyzed part of the sequence of the human glycophorin C (GPC) gene carried by a Webb blood‐group positive donor. Our results indicate that the lack of N‐glycosylation of the variant GPC associated with the Webb phenotype is due to a point mutation resulting in an asparagine to serine substitution at amino acid position 8.
Erythrocyte glycophorin B deficiency may occur by two distinct gene alterations
Authors: Rahuel Cecile , London Jacqueline , Vignal Alain , Ballas Samir K. , Cartron Jean‐Pierre 
Published: February 2007

AbstractThe genomic DNA from rare persons whose erythrocytes are deficient in glycophorin B (GPB) (S‐s‐U‐ phenotype), was examined by Southern hybridizations using glycophorin B probes and was subdivided into two main categories. In the type I variant (Fav., M.H., S.K.), we found that the S‐s‐U‐ condition is generated by a large gene deletion extending from exons B2 to B4 of glycophorin B gene. Conversely, in the type II variant (Del.), the entire gene is present, and its promoter is almost similar to common Glycophorin A(GPA) and GPB as well as to type I promoters, except for four‐point mutations, which do not occur in potential cis‐acting elements. We concluded that the same phenotypic glycophorin B deficiency may occur by different gene alterations, including either a gene deletion or a mutation that might alter transcription or translation of the gene.
Increased production of tumor necrosis factor‐α by peripheral blood mononuclear cells in the patients with aplastic anemia
Authors: Shinohara K. , Ayame H. , Tanaka M. , Matsuda M. , Ando S. , Tajiri M. 
Published: February 2007

AbstractThe activity of tumor necrosis factor‐α: (TNF‐α) in the supernatant of cultured peripheral blood mononuclear cells (PBMC) was measured in patients with aplastic anemia. It was significantly higher in patients with aplastic anemia than in normal controls, both when PBMC were unstimulated or when they were stimulated with PHA. Results from aplastic anemia patients were also significantly higher than patients who had received allogeneic bone marrow transplants. In aplastic anemia patients, the TNF‐α: value produced by PBMC upon stimulation and the platelet count were inversely correlated, as well those patients who had high TNF‐α values tended to have lower hemoglobin and leukocyte values although this was not significant statistically. These results suggest that the increased production of TNF‐α by PBMC plays a role in the severe suppression of hematopoiesis in aplastic anemia.
Granulocyte proteases do not process endothelial cell‐derived unusually large von willebrand factor multimers to plasma vWF in vivo
Authors: Phillips Martin D. , Vu Chau , Nolasco Leticia , Moake Joel L. 
Published: February 2007

AbstractThe unusually large von Willebrand factor (ULvWF) multimers present within endothelial cells and platelets are larger than the vWF multimers normally found in adult human plasma. Furthermore, ULvWF multimers are cleared rapidly from the circulation if they are released by intense endothelial cell stimulation. The mechanisms by which the ULvWF multimers are processed to large plasma vWF multimers are not known. It has been demonstrated that granulocyte proteases are capable of decreasing vWF multimer size in vitro, and that some patients with myeloproliferative syndromes have a relative absence of large plasma vWF multimers in sodium citrate‐anticoagulated plasma samples. In order to assess the influence of granulocyte proteases on vWF multimer size, we evaluated the vWF multimeric patterns in 94 plasma samples from 60 patients with neutrophil counts that were either considerably elevated or extremely reduced. In 83 of 94 plasma samples, the vWF multimeric patterns were normal. No patients with very low neutrophil counts had ULvWF multimers present. These observations suggest that granulocyte proteases are not likely to be involved in vivo in the processing of ULvWF multimers from endothelial cells to the smaller vWF forms in circulation.
CD3+, CD4−, CD8− large granular T‐cell lymphoproliferative disorder
Authors: Sun Tsieh , Cohen Neil S. , Marino John , Koduru Prasad , Cuomo Joanne , Henshall Joan 
Published: February 2007

AbstractLarge granular T‐cell lymphoproliferative disorder (LGTLD) is a heterogeneous disorder covering a broad spectrum of diseases and requiring further subdivision. Most reported cases emphasized its suppressor phenotype (Tγ cell or CD8+), but we encountered two cases of CD3+, CD4−, CD8− LGTLD. Both cases had a benign clinical course and required no chemotherapy despite persistent lymphocytosis. This unique phenotype has been reported in a few cases of acute lymphoblastic leukemia expressing the T‐cell receptor (TcR) gamma chain gene and is considered the counterpart of thymocytes at the intermediate stage between early precursors and mature thymocytes. Our case 1 provides further evidence that the CD3+, CD4−, CD8− phenotype, indeed, expresses the TcR gamma chain gene. However, the negative reaction to terminal deoxynucleotidyl transferase in our case 1 indicates that this phenotype represents proliferation of peripheral T‐cells, in which about 2% bear the CD3+, CD4−, CD8− phenotype in the normal population. The selective use of CD3, CD4, CDS, HNK‐1 monoclonal antibodies and of cytochemical stains (acid phosphatase and alpha‐naphthyl butyrate esterase) for characterization of this disorder is discussed.
Platelet aggregation in Hyperapobetalipoproteinemia
Authors: Miller M. , Bell W. , Plano S. , Connors J. , Kennedy S. , Kwiterovich P. 
Published: February 2007

AbstractHyperapobetalipoproteinemia (HyperapoB) is a lipid disorder characterized by premature coronary disease, although the mechanisms have not been elucidated. Because abnormalities in platelet function may represent an enhanced susceptibility to coronary thrombosis, the aggregability of platelets was examined in hyperapoB subjects. Compared to controls, there were no significant differences in either platelet lipid composition or in aggregation to epinephrine, ADP, or collagen. In contrast to other dyslipidemias, platelet function does not appear to be abnormal in this well defined lipid disorder.
Isolated relapse of acute myelogenous leukemia presenting as a gastric ulcer
Authors: Roy Jean , Vercellotti Gregory , Fenderson Mary , Mulvahill Amy , Snover Dale , Weisdorf Daniel 
Published: February 2007

AbstractIsolated gastrointestinal (GI) infiltrate is unusual at presentation or relapse of acute myelogenous leukemia (AML). We report a case of acute myelogenous leukemia (FAB‐M4) whose isolated relapse presented as a bleeding gastric ulcer. The patient was a 30‐year‐old male who had been diagnosed to have AML in June 1988. While in third complete remission, he underwent a sibling allogeneic HLA‐matched bone marrow transplant. Five months after transplantation, he was readmitted for pneumonia. While in the hospital, he had an episode of upper GI bleeding. The endoscopy revealed a leukemic gastric ulcer, with morphology and immunophenotyping identical to his initial AML. There was no evidence of leukemia in the blood or bone marrow. Although different types of leukemic infiltrates have been recognized at post‐mortem examination, our case is unique because AML presenting as an isolated malignant ulcer has not been described previously. We conclude that relapsing AML may present as an isolated gastric ulcer and suggest that any suspicious lesion on upper GI endoscopy should be biopsied after aggressive platelet support.
Stability of peripheral hematological parameters after chronic acetylcholinesterase inhibition in man
Authors: Moriearty Pamela L. , Womack Cindy L. , Dick Barrett W. , Colliver Jerry A. , Robbs Randall S. , Becker Robert E. W. 
Published: February 2007

AbstractAcetylcholinesterase (AChE; EC 3.1.1.7) is present in both primitive and mature erythroid cells, and a role has been suggested for the enzyme in regulation of differentiation in the human erythron. AChE is also a major enzyme in the central nervous system; alteration of its activity has been proposed as a therapeutic strategy in Alzheimer disease. We recently treated 18 Alzheimer disease patients with metrifonate, a long‐acting AChE inhibitor, over periods up to 7 months, with resulting erythrocyte AChE inhibition as high as 82 per cent of baseline values. Despite chronic reduction of enzyme activity, no significant alterations were noted in erythrocyte, leukocyte or platelet characteristics or numbers that would suggest a deleterious effect of AChE inhibition on normal differentiation. Thus, any modification of developmental pathways appears to be compensated by other regulatory mechanisms in the intact organism.
Detection of rearrangement of immunoglobulin heavy chain and T‐cell receptor beta chain in leukemic cells by restricted polymerase chain reaction
Authors: Stole V. , Uhrmacher J. , Krause J. R. 
Published: February 2007

AbstractRearrangement of the immunoglobulin heavy chain and of the T‐cell receptor beta subunit was analyzed by using restricted polymerase chain reaction (PCR). To differentiate between the germline configuration and the rearranged genome in a DNA sample extracted from lymphocytes, we compared the ratio of the amplified products. The intensity of amplification of the intron region (JHF) upstream of the first joining region was compared to the intensity of joining region 6 of the immunoglobulin heavy chain. The number of the amplification cycles in the PCR was designated in such a way that the ratio of JHF/JH6 was less than one in the rearranged configuration. As the concentration of clonal B‐lymphocytes with the rearranged genome in the sample increased the amplification of the JHF intron proportionally decreased. We used the same approach for the two constant regions of the T‐cell receptor beta chain. As one of the intron regions of the constant sequence became depleted by rearrangement so the amplification of the particular region decreased. Therefore, the absence or decreased concentration of a particular product of amplification indicated deletion and thus rearrangement of the genome in the leukemic B‐ or T‐lymphocytes. The threshold of detection of cells with the rearranged genome on a photograph of agarose gel loaded with the particular amplified regions and staining with the ethidium bromide is less than 10% by densitometric tracing and 25–50% by visual evaluation.This novel approach allows the detection of the rearranged DNA sequences in a 2 day span. Hence, it can serve as a diagnostic tool for the identification of clonal expansion of lymphocytes in acute leukemias and lymphomas in particular and for the detection of deleted genomic regions in general.
Molecular characterization of Hb S(C) β‐thalassemia in American blacks
Authors: Gonzalez‐Redondo J. M. , Kutlar A. , Kutlar F. , McKie V. C. , McKie K. M. , Baysal E. , Huisman T. H. J. 
Published: February 2007

AbstractAn extension of previous reports describing the molecular defects and hematological abnormalities in black patients with Hb S(C) β‐thalassemia living in the Southeastern United States is presented. As many as 58 patients with Hb S‐β+‐thalassemia, 16 with Hb C‐β+‐thalassemia and 12 with Hb S‐β°‐thalassemia have been studied. Patients with Hb S(C) β+‐thalassemia type 2 (high Hb A values) were most common; the thalassemia was due to mutations in the promoter of the β‐globin gene [−88 (C → T) and −29 (A → G)] or at the polyadenylation signal (T → C). Two patients with lower Hb A values (type 1) carried a mutation in the first intron of the β‐globin gene (IVS‐1‐5: G → T). The simultaneous presence of an α‐thalassemia −2( −αl) resulted in some modifications of the hematological parameters, but had a minimal effect on the clinical condition. Patients with Hb S‐β°‐thalassemia had lower hemoglobin values, lower number of red blood cells, and lower MCHC values and suffered more frequently from complications than the patients with Hb S‐β+ ‐thalassemia. A total of 17 different β‐thalassemia mutations were observed in 128 chromosomes; two mild β+ ‐thalassemia mutations [−88(C → T) and −29(A → G)] account for more than 80% of the thalassemic chromosomes.
A glycosaminoglycan inhibitor of thrombin: A new mechanism for abnormal hemostatic assays in cancer
Authors: Liebman Howard A. , Comenzo Raymond , Allen Suzanne T. , Dilorio Jere M. 
Published: February 2007

AbstractThe isolation and partial characterization of a novel anticoagulant from the plasma of a patient with metastatic prostate cancer is described. The patient had a prolonged activated partial thromboplastic time, prothrombin time and thrombin time which did not correct by mixing with normal plasma. The reptilase time was normal and the prolonged thrombin time was corrected with protamine sulfate suggesting a heparin‐like anticoagulant. A glycosaminoglycan anticoagulant (GAC) was isolated from the patient's plasma. The inhibitory activity of the GAC was destroyed by treatment with chondroitinase ABC. The GAC migrated on agarose gel electrophoresis between keratin sulfate and heparan sulfate. Purified GAC possessed only 2% (W/W) of the antithrombin III cofactor activity of porcine heparin. In assays using purified fibrinogen, the GAC was shown to directly inhibit fibrinogen proteolysis by thrombin. It is concluded that this glycosaminoglycan anticoagulant directly inhibits thrombin clotting of fibrinogen and is a new mechanism for abnormal hemostatic assays in cancer.
Transient reversal of bone marrow aplasia associated with lymphocyte depleted hodgkin's disease after combination chemotherapy
Authors: Johnston P. G. , Sullivan F. J. , Ruscetti F. W. , Longo D. L. , Connaghan D. G. 
Published: February 2007

AbstractThis report describes a patient with lymphocyte depleted Hodgkin's disease who presented with bone marrow aplasia. The aplastic marrow reverted to normal after initiation of MOPP chemotherapy; however, 4 months after completion of therapy, bone marrow aplasia recurred in the absence of recurrent Hodgkin's disease. The patient remains free of Hodgkin's disease 34 months after completion of chemotherapy. Bone marrow abnormalities in Hodgkin's disease are reviewed and the current understanding of the pathological mechanisms leading to aplastic anemia is discussed.
Ultrastructural evidence for the origin of mast cells in normal human bone marrow
Authors: Saito Nagahito , Takemori Nobuo , Tachibana Noriko , Hayashishita Naoyuki 
Published: February 2007

AbstractWhether the origin of the progenitor of mast cells occurs in the basophil/mast cell system or the myeloid cell system of neutrophils and monocytes/macrophages remains controversial.Lactoferrin or lysozyme is known to be present in the myeloid cell system. By electron microscopy, antibodies against lactoferrin and lysozyme were observed to stain the granules of normal bone marrow mast cells. This supports the proposal that the precursor cell of bone marrow mast cells is nearer to the myeloid cell system than the basophil/mast cell system.
Effect of estrogens and progesterone on human peripheral erythroid burst‐forming unit (BFU‐E) growth
Authors: De Feo T. M. , Cappellini M. D. , Fiorelli G. 
Published: February 2007

AbstractVariations in the number of peripheral burst‐forming unit—erythroid (BFU‐E) of healthy women were observed during a prolonged period of observation. These differences were related to different phases of the menstrual cycle. A peak in the number of BFU‐E occurred on day 14 of the cycle corresponding to the serum 17‐β‐estradiol peak. The effect of estrogens and progesterone on the in vitro growth of peripheral BFU‐E of healthy women was assayed. Estrogens demonstrated a stimulatory and progesterone an inhibitory effect in total lymphomonocyte cultures, whereas neither hormone had an effect in monocyte‐depleted cultures. Prostaglandin E1 (PGE1) which is known to be secreted by monocytes, stimulated the in vitro growth of peripheral BFU‐E. These data suggest that estrogens and progesterone could have a role in the in vitro growth of peripheral BFU‐E, probably mediated by monocytes.