bluuebunny/crossref_metadata_2025_split huggingface dataset:
In vitro endocytosis of benign and malignant human bone marrow plasma cells
Authors: Ohtake Ken‐Ichi , Yano Takahiro , Kameda Kazuo , Ogawa Tetsuhei 
Published: February 2007

AbstractWe report ultrastructural evidence of the phagocytic potential of plasma cells and myeloma cells. The incubation of plasma cells and myeloma cells in vitro with horseradish peroxidase (HRP) and cationized ferritin (CF) allows the tracing of fluid‐phase and receptor‐mediated pathways. Surface‐bound ligands (CF) and solutes (HRP) taken up in primary pinocytic vesicles are internalized to the endosomal compartment. After 1 hr of incubation, CF was found not only in plasma cells but also in myeloma cells. Reaction products of HRP were observed only in myeloma cells. In myeloma cells, however, HRP was located only in the lysosomal system, whereas CF was present within membrane cisternae as well as within lysosomes. These myeloma cells morphologically produced interleukin‐6 (IL‐6).
Hypertonic cryohemolysis: A diagnostic test for hereditary spherocytosis
Authors: Streichman S. , Gesheidt Y. , Tatarsky I. 
Published: February 2007

AbstractRed blood cells of subjects with hereditary spherocytosis are specifically susceptible to temperature changes while suspended in hypertonic solutions. Based on this property, a new diagnostic test for hereditary spherocytosis is presented. The suggested method is 100% sensitive in the diagnosis of all patients, including asymptomatic obligate carriers with hereditary spherocytosis, and is very specific in different control groups. Unlike other methods designed for the diagnosis of hereditary spherocytosis, this test does not depend on the cells' surface‐ area‐to‐volume ratio. Normal red blood cells that were induced to become spherocytes by different means (i.e., vinblastine, lysophosphatidyl choline, and heat) and showed increased osmotic fragility did not become susceptible to the hypertonic cryohemolysis conditions.
Transformation of bilineal hybrid acute leukemia to acute lymphoid leukemia: A case report with serial analyses of cytogenetics and gene rearrangement
Authors: Jinnai I. , Kusumoto S. , Shiomi Z. , Bessho M. , Saitoh M. , Hirashima K. , Minamihisamatsu M. , Kamiyama T. , Takase K. 
Published: February 2007

AbstractA 17‐year‐old male with bilineal hybrid acute leukemia is described. Two‐color flow cytometric analysis of blast surface phenotype revealed that there were two groups of blasts which showed either CD 10+ CD 19+ CD 13− CD 33− or CD 10− CD 19− CD 13+ CD 33+, but not both. He developed a complete remission by treatment with vincristine, prednisolone, adriamycin, and L‐asparaginase. After 8 months, however, leukemia relapsed and lymphoid blasts were dominant. Cytogenetic analysis at presentation showed 46, XY, t(3;9)(p21;p22), and at relapse it showed 46, XY, t(1;3;9)(1 pter→1 q32::3p25→3pter; 3qter→3p21::9p22→9pter;9qter→9p22::3p21→3p25::1q32→1qter), t(2;19)(p21;q13). Analysis of the heavy chain joining region at diagnosis showed three hybridizing bands, all rearranged, but at relapse only one rearranged band. Analysis of the constant region for the β T‐cell receptor gene (TCRβ) both at diagnosis and at relapse showed one rearranged and one germline band, suggesting that rearrangement of one allele of TCRβ of not only lymphoid but also myeloid blasts occurred. It is considered that the target cell of lymphoid leukemia cells and that of myeloid leukemia cells at diagnosis were the same, which differentiated to two lineages, and the clone which evolved from lymphoid lineage proliferated at relapse.
Chronic myelomonocytic leukemia: A test of a proposed staging system
Authors: Molica Stefano , Iannaccaro Piergiorgio , Alberti Antonio 
Published: February 2007

AbstractIn an attempt to characterize the prognostic significance of the main clinical and hematological features of chronic myelomonocytic leukemia (CMML), 30 such patients were allocated the “modified Bournemouth score”. The most significant separation of groups predictive of poorer survival was obtained by setting a cutoff at the median score value for the whole series (score,2). On this basis patients could be divided into two different groups whose survival probability as well as risk of acute transformation significantly differ each other (P < 0.05). In our opinion, this score could be of interest in treatment planning of CMML patients when the possibility of conservative or aggressive approach in envisaged.
Platelet derived growth factor messenger RNA is increased in bone marrow megakaryocytes in patients with myeloproliferative disorders
Authors: Katoh Osamu , Kimura Akiro , Kuramoto Atsushi , Itoh Takuji 
Published: February 2007

AbstractPlatelet derived growth factor (PDGF) has been suggested to play an important role in the pathogenesis of myelofibrosis, which often occurs in patients with myeloproliferative disorders (MPD). We examined the expression level of PDGF mRNA in bone marrow megakaryocytes from 13 MPD patients by in situ hybridization, using cDNA probes for both human PDGF A chain and B chain (c‐sis). The mRNA level for both chains in the patients was significantly higher than that in control patients, and was markedly higher for one patient with essential thrombocythemia and one with polycythemia vera. Transcripts for A chain and B chain were expressed with a positive correlation in the MPD patients. Using the marrow fibroblast proliferation assay, we found PDGF activity in purified megakaryocytes from one of the MPD patients with high mRNA level to be similar to that from one control patient. In addition, PDGF was previously shown to be decreased in circulating platelets from MPD patients. These results may suggest that, in some patients, PDGF is synthesized in megakaryocytes at a high rate, but some fraction is released into the bone marrow environment, if the level of PDGF mRNA is assumed to be linearly related to the protein synthesized. This might be one possible mechanism causing marrow fibrosis in MPD patients.
Selective Reduction of Serotonin Storage and ATP Release in Chronic Renal Failure Patient Platelets
Authors: Soslau Gerald , Parker Janet , Brodsky Isadore , Putatunda Bhaben , Schwartz Allan B. 
Published: February 2007

AbstractHemorrhagic complications, as monitored by skin bleeding times, occur in a significant number of chronic renal failure (CRF) patients. The etiology of hemostatic defects in these patients is complex and ill defined. Our studies demonstrate, for the first time, that activated platelets, derived from CRF patients, release significantly (P < 0.001) less ATP than controls while the percent of releasable serotonin (5HT), assumed to be co‐stored with ATP, is unaltered. Analysis of the CRF‐derived platelets reflects a selective acquired storage pool defect with significantly (P < 0.001) reduced 5HT levels while their dense granule contents of ATP and ADP are normal. The comparison of ATP release from platelets derived from CRF patients whose bleeding times were less than 9 min to those with bleeding times of 9 min or greater was significantly different (P < 0.02). This report demonstrates for the first time that there is a statistically significant correlation of ATP release and 5HT content to bleeding times (P < 0.001). The perturbation of platelet 5HT uptake, 5HT dense granule content, and ATP release appears to result from newly described altered plasma factors, detected by our in vitro mixing studies. It is proposed that the reduced level of releasable platelet 5HT and ATP contributes to bleeding disorders commonly encountered in CRF patients.
Diagnosis of pyruvate kinase deficiency in a transfusion‐dependent patient with severe hemolytic anemia
Authors: Rijksen Gert , Schipper‐Kester Gerda P. M. , Staal Gerard E. J. , Veerman Anjo J. P. 
Published: February 2007

AbstractIn a 2‐yr old girl a hemolytic anemia was present since birth requiring multiple blood transfusions. Pyruvate kinase deficiency was suspected on the basis of a marginal enzyme activity, but could not be established due to the presence of massive numbers of donor cells in her peripheral blood. However, by density fractionation we succeeded in the isolation of a small fraction of the patient's own cells, in which a severe pyruvate kinase deficiency could be detected. In contrast hexokinase and glucose‐6‐phosphate dehydrogenase activities were extremely high, which is indicative that a very immature cell population is present in this fraction.In immunofluorescence studies a clear crossreaction was apparent with anti M2‐type pyruvate kinase antibodies, whereas only a faint reaction with anti L‐type could be detected. Despite the presence of a slight amount of L‐type immunoreactive material, the residual activity in the patient's cell fraction could only be attributed to M2‐type pyruvate kinase as was shown by cellulose acetate electrophoresis.
New treatment strategies for multiple myeloma
Authors: Alexanian Raymond , Barlogie Bart 
Published: February 2007

AbstractDuring the past 5 years, several new treatments and strategies have been developed for patients with multiple myeloma. For patients with disease resistant to standard therapies, these include the VAD regimen, dexamethasone alone, high‐dose melphalan, and intensive chemoradiotherapy with bone marrow transplantation. Alpha interferon appears to have its greatest potential as part of early induction therapy or during remission maintenance. The role of hemopoietic growth factors or blood stem cells in support of high‐dose therapy and drugs that may overcome multiple drug resistance continues under study. A sequence of non‐cross‐resistant therapies early in the disease course seems worthy of investigation, especially in patients at high risk for early relapse.
FAB L3 Type of B‐cell acute lymphoblastic leukemia (B‐ALL) without chromosome abnormalities
Authors: Imamura Nobutaka , Mtasiwa Deodatus M. , Ota Hiromi , Inada Tominari , Kuramoto Atsushi 
Published: February 2007

AbstractAcute lymphocytic leukemias (ALLs) are morphologically classified into L1, L2, and L3. The former two types are phenotypically constituted of quite heterogeneous ALLs.In the present study, phenotypes of cells from five L3 type ALL were analysed in FACS‐IV using a panel of monoclonal antibodies (MAbs). The leukemic cells of all these patients coexpressed la (HLA‐DR), CD19, CD20, CD21, CD24, CD38, and surface immunoglobulins, whereas a negative reaction with MAbs CD1, CD2, CD3, CD4, CD7, CD8, and Ti (WT31), TiγA, δTCS 1, and anti TCR‐γ/δ was observed. Neither myeloid‐monocyte‐erythroid nor megakaryocyte related cell surface antigens were detected in these cases with L3 type ALL.Chromosomal analysis of the ALL cells from two cases revealed a normoploid karyotype with specific translocation t(8;14)(q24;q32), whereas it was normal (46XY or 46XX) for the remaining three cases. Expression of myc oncogene was high in the former group, but low in the later one.Basing from our findings, we conclude that L3 type ALL is heterogeneous with respect to immunophenotypes, cytogenetics and oncogene analysis.
Necrobiotic xanthogranuloma with IgA multiple myeloma: A case report and literature review
Authors: Valentine Elizabeth A. , Zamkoff Kenneth W. , Friedman Henry D. , Streeten Barbara W. 
Published: February 2007

AbstractThe association between necrobiotic xanthogranuloma of the dermis and paraproteinemia and/or B‐cell malignancy is best described in the ophthalmologic literature. We report a case which occurred in the eyelid and orbit of a 64 year old man that led to the diagnosis of an IgA multiple myeloma. To our knowledge, this is the first report of an IgA type paraproteinemia and IgA type multiple myeloma associated with necrobiotic xanthogranuloma.
Increased protein binding to a −530 mutation of the human β‐globin gene associated with decreased β‐globin synthesis
Authors: Berg Patricia E. , Mittelman Moshe , Elion Jacques , Labie Dominique , Schechter Alan N. 
Published: February 2007

AbstractAlthough some cases of the syndrome of hereditary persistence of fetal hemoglobin (HPFH) have been correlated with mutations causing a change in the binding of trans‐acting factors to DNA sequences flanking the γ‐globin gene, this mechanism has not been described in β‐thalassemias upstream of the canonical promoter of the β‐globin gene. In this report we describe such a change in binding of a protein that may explain a silent carrier phenotype of β‐thalassemia. We have previously demonstrated the binding of a protein (BP1) derived from a nuclear extract of human K562 cells to DNA 5′ to the human β‐globin gene in a region having a negative regulatory function. The binding of BP1 in this region can be detected by DNAse I footprinting and by gel mobility shift analysis. We have now compared binding of BP1 to the normal sequence and a mutated sequence (+ATA/‐T at −530 bp from the cap site) from the silent carrier of β‐thalassemia. Using mobility shift assays we show that BP1 binds about nine times more strongly to the mutated sequence than the normal sequence. These results suggest the possibility that the decreased expression of the β‐globin gene exhibited by the carrier may be due, at least in part, to tighter binding of a protein which functions as a negative control element or represser.
Maternal and fetal iron measurements in a hemochromatotic pregnancy
Authors: Baynes Roy D. , Meyer Theo E. , Bothwell Thomas H. , Lamparelli Rosario D. 
Published: February 2007

AbstractThe findings in the cord blood sample of an infant from a treated hemochromatotic mother of a raised transferrin saturation (88%) and a raised ferritin concentration (250.2 μg/L) together with elevated maternal values (66% and 91.6 μg/L, respectively) yet a normal total placental iron content (26.9 mg) suggested that in common with gastrointestinal mucosal cells and reticuloendothelial cells in hemochromatosis, the placental cell may exhibit an abnormality of iron storage and transport.
Radiotherapy of pseudotumors of bone in hemophiliacs with circulating inhibitors to factor VIII
Authors: Castaneda Victoria L. , Parmley Richard T. , Bozzini Miguel , Feldmeier John J. 
Published: February 2007

AbstractPseudotumors of bone in two hemophiliacs with severe factor VIII deficiency and a high level of circulating inhibitors are reported. Both had favorable response to radiation therapy after unsuccessful treatment with factor concentrates and remain free of recurrence at 18‐62 months. A review of the literature of cases in which radiotherapy has been used is presented. Radiotherapy in the treatment of pseudotumors of bone in hemophiliacs should be strongly considered, particularly if coagulation factor inhibitors are present.
Antiphospholipid antibodies and adrenal hemorrhage
Authors: McCroskey Robert D. , Phillips Anthony , Mott Frank , Williams Eliot C. 
Published: February 2007

AbstractWe recently encountered two cases of massive bilateral adrenal hemorrhage in patients with antiphospholipid antibodies. A search of the records of this institution covering the past 15 years revealed two additional patients with massive adrenal hemorrhage associated with evidence of antiphospholipid antibodies. Three of these four patients were receiving anticoagulant drug therapy. The presence of the antiphospholipid antibodies may increase the risk of massive adrenal hemorrhage, particularly in patients who receive anticoagulant drugs.
Occurrence of high‐grade T‐Cell lymphoma in a patient with philadelphia chromosome‐negative chronic myelogenous leukemia with breakpoint cluster region rearrangement: Case report and review of the literature
Authors: Djulbegovic Benjamin , Hadley Terence , Yen Frank 
Published: February 2007

AbstractIn this report, we describe a patient with Philadelphia chromosome‐negative chronic myelogenous leukemia (CML) with breakpoint cluster region gene rearrangement who developed T‐cell lymphoblastic lymphoma. The occurrence of T‐cell lymphoblastic lymphoma coincided with the appearance in the bone marrow of the cytogenetic abnormality, trisomy 22q11.2 22qter. This is the first report of high‐grade T‐cell lymphoma in a patient with documented CML.
H‐ras‐1 polymorphism in leukemia
Authors: Wadleigh Robert G. , Chan Clara S. , Cohen Martin H. , Krasnow Steven H. , Anderson Anita , Detera‐Wadleigh Sevilla D. 
Published: February 2007

AbstractWe have examined leukocyte DNAs obtained from 21 patients with various types of leukemia for the Sacl polymorphism of H‐ras‐1. The patients included ten with B‐lineage leukemia, two with T‐cell leukemia, and nine with acute myelogenous leukemia. Using a genomic probe for H‐ras‐1 in Southern hybridizations, we found that 78% of the DNA from leukemias of myeloid origin were heterozygous. In contrast, only three of ten B‐cell‐derived leukemias showed heterozygosity. This difference in the number of heterozygotes vs. homozygotes in the two patient samples studied is statistically significant (P = 0.04 by Fisher's exact test). These results suggest the possibility that the H‐ras‐1 allelic pattern that is associated with acute myelogenous leukemia is distinct from that found in B‐cell‐derived malignancies.
Erythrocyte folate levels: A clinical study
Authors: Jaffe Jeffrry P. , Schilling Robert F. 
Published: February 2007

AbstractPURPOSE: 1) To determine if there are significant differences in laboratory parameters and patient characteristics between patients with low erythrocyte (RBC) folate levels and those with normal RBC folate levels; 2) to determine the correlation between serum and RBC folate levels; 3) to determine if the RBC folate level changed the diagnosis, treatment, and outcome of these patients if the serum folate level was available.PATIENTS AND METHODS: The study is a retrospective review of all inpatients and outpatients at a public teaching hospital who had an RBC folate level >175 ng/ml (N = 57) over the study period (69 months) and a blindly selected group of patients with RBC folate levels >175 ng/ml (N = 53) during the same period.RESULTS: Patients' with low RBC folate levels had higher mean corpuscular volume (MCV) and red cell distribution width (RDW) values and lower serum folate and B12 values than patients with normal RBC folate levels, but there was no difference in degree of anemia, presence of oval macrocytes and/or hypersegmented neutrophils on the peripheral blood smear, LDH, alcohol use, diet, or any other measured clinical parameter. Serum and RBC folate levels were highly correlated and a low RBC folate affected the clinical outcome of three patients (5% of the low RBC folate group).CONCLUSIONS: Based upon these retrospective data and a review of the literature, we cannot define significant differences between patients with low RBC folate and randomly selected patients with normal RBC folate that could not have been equally well defined using serum folate values. The sensitivity and specificity of a low RBC folate level in the diagnosis of ill or healthy individuals are undefined, and until prospective studies utilizing some of the newer, more specific biochemical indicators of tissue folate are completed, the interpretation of low RBC folate levels will remain problematic.
Hemoglobin aggregation and pseudosickling in vitro of hemoglobin setif‐containing erythrocytes
Authors: Noguchi C. T. , Mohandas N. , Blanchette‐Mackie J. , Mackie S. , Raik E. , Charache S. 
Published: February 2007

AbstractErythrocytes from individuals heterozygous for hemoglobin Setif (α94 Asp→Tyr) sickle in vitro without deoxygenation when incubated in chloride buffer due to hemoglobin aggregation. We now report quantitative studies of hemoglobin polymerization and deformability in these cells. Hemoglobin polymer gradually increased in intact cells during a 24 h incubation period at 24°C. After 24 hr, about 80% of the cells in 290 mosm sodium chloride buffer contained polymer which appeared as short rods compared to >99% containing polymer at 450 mOsm. Similar proportions of cells were morphologically sickled. Deformability of erythrocytes with 40% hemoglobin Setif incubated in 290 mOsm buffer at 37°C decreased to 80% of normal by 210 min but in 450 mOsm decreased to 50% after only 30 min as measured by the ektacytometer. However, at 4°C deformability remained normal even in 450 mOsm buffer. The solubility of gelled hemolysate containing 40% hemoglobin Setif was 24 g/dl and 21 g/dl at 290 and 459 mOsm buffer respectively. The gel persisted at 4°C with a solubility of 25 g/dl, but melted when dialyzed into sodium phosphate or potassium phosphate buffer. These data suggest that hemoglobin polymerization, reduced deformability, and sickling of hemoglobin Setif‐containing erythrocytes are related to reduced hemoglobin solubility. The rate and extent of intracellular polymerization in vitro are considerably reduced (as in the case of sickle trait) compared with erythrocytes from individuals with sickle cell anemia. Hence, the slower kinetics of hemoglobin aggregation in hemoglobin Setif‐containing cells provide an alternate system for studying hemoglobin aggregation in hemoglobin Setif‐containing cells provide an alternate system for studying hemoglobin polymerization and abnormal rhelogy.
Therapy of aplastic anemia with sequential antithymocyte globulin and cyclosporin
Authors: Doolittle Gary C. , Bodensteiner David C. , Skikne Barry S. , Amare Mammo 
Published: February 2007

AbstractFive consecutive patients with severe aplastic anemia were treated with antithymocyte globulin followed by cyclosporin A. All received antithymocyte globulin initially, and because of lack of response within a 4 week period, cyclosporin was administered subsequently. Hematologic improvement occurred within four months of initiation of cyclosporin. Four patients no longer require blood product support, while one remains transfusion‐dependent. In two patients, thrombocytopenia developed when the cyclosporin was tapered but re‐institution of the drug resulted in a prompt improvement of counts. These observations indicate that the sequential use of antithymocyte globulin and cyclosporin may be an effective therapeutic approach in the treatment of severe aplastic anemia.
Hemophagocytosis by small cell lung carcinoma
Authors: Molad Yair , Stark Pinhas , Prokocimer Miron , Joshua Henry , Pinkhas Jack , Sidi Yechezkel 
Published: February 2007

AbstractA 60‐year‐old woman with disseminated small cell carcinoma of the lung and hemophagocytosis by the metastatic cells in the bone marrow is presented, it is the first clinicopathologic report on phagocytosis of erythrocytes by lung tumor cells in concordance with a recently described evidence of a macrophage origin of small cell carcinoma of the lung.
Changes in plasma levels of tissue‐plasminogen activator/inhibitor complex and active plasminogen activator inhibitor in patients with disseminated intravascular coagulation
Authors: Asakura Hidesaku , Jokaji Hiroshi , Saito Masanori , Uotani Chika , Kumabashiri Ichiro , Morishita Eriko , Yamazaki Masahide , Matsuda Tamotsu 
Published: February 2007

AbstractPlasma levels of tissue‐plasminogen activator · plasminogen activator inhibitor (t‐PA · PAI) complex and active PAI were assayed in 58 cases of disseminated intravas‐cular coagulation (DIC). A significant elevation of both parameters was observed in most cases of DIC, especially in patients with non‐Hodgkin lymphoma, sepsis, or some patients with acute leukemia, but no such elevation was observed in patients with acute promyelocytic leukemia (APL). The levels of both parameters were higher in cases of DIC with multiple organ failure (MOF) than in those without MOF. Since no elevation of t‐PA · PAI complex was observed in most cases of APL, t‐PA did not seem to play an important role in the activation of fibrinolytic system in APL. Active PAI, which reflects the inhibitory regulation in fibrinolytic system, was considered to play a role in the progression of MOF. Plasma levels of active PAI were low in the cases of APL, which had no complication of MOF.