bluuebunny/crossref_metadata_2025_split huggingface dataset:
Detection of engraftment and chimerism after bone marrow transplantation by in situ hybridization using a Y‐chromosome specific probe
Authors: Agematsu Kazunaga , Kitahara Fuminori , Uehara Yoshio , Kawai Hiroshi , Miyagawa Yukiaki , Komiyama Atsushi , Nakahori Yutaka , Nakagome Yasuo , Akabane Taro 
Published: February 2007

AbstractAfter bone marrow transplantation (BMT), the recipient and donor cells must be distinguished from each other to document and characterize successful engraftment. In addition to dot blot and Southern blot analyses, we have performed in situ hybridization in two sex‐mismatched cases using a Y‐chormosome specific DNA probe (PHY10). In situ hybridization showed that >95% of the peripheral mononculear cells had clusters of grains indicative of male cell origin in a recipient girl (case 1), and no cells had clusters of grains in another recipient boy (case 2) at the time of engraftment and 3 months after BMT. In situ hybridization using the PHY10 probe appears to facilitate identification of individual cells of male and female origin, and it requires only 20 hr to obtain the results. The technique provides a powerful new method for the documentation of engraftment and the detection of mixed hematopoietic chimerism in peripheral blood and bone marrow cell compartments after BMT.
Treatment of patients with disseminated intravascular coagulation by protein C
Authors: Okajima Kenji , Imamura Haruo , Koga Shin , Inoue Masayasu , Takatsuki Kiyoshi , Aoki Nobuo 
Published: February 2007

AbstractThe therapeutic effect of highly purified protein C (PC) or activated protein C (APC) on three patients with disseminated intravascular coagulation (DIC) was tested. In two out of three cases, although therapeutic dose of heparin was not effective, PC or APC administration significantly improved the hypercoagulable state. In one case with acute leukemia who developed DIC with the severe gastrointestinal bleeding, APC corrected the hypercoagulable state without aggravating bleeding. These findings suggested that PC and/or APC might be effective in correcting hypercoagulable state without any adverse effect.
Treatment of pain in adults with sickle cell disease
Authors: Ballas Samir K. 
Published: February 2007

AbstractThe treatment of an adult patient with sickle cell disease whose clinical course is characterized by frequent painful crises creates a number of logistic problems in a tertiary care city hospital. Because such patients usually have no objective signs of painful crises, they are often considered to be malingerers and drug abusers. This paper reviews this controversial issue and presents one attempt at its resolution.
Phase II trial of α‐tocopherol (vitamin E) in the treatment of primary systemic amyloidosis
Authors: Gertz Morie A. , Kyle Robert A. 
Published: February 2007

AbstractPrimary systemic amyloidosis (AL) is a rare disorder characterized by deposition of a monoclonal immunoglobulin light chain or fragment thereof, resulting in dysfunction of the heart, kidney, liver, or nerves. Despite the use of melphalan, prednisone, colchicine, and dimethyl sulfoxide (DMSO), no improvement in median survival has been reported in prospective randomized studies.We undertook a study of α‐tocopherol acetate (vitamin E) in the treatment of 16 patients with AL because of its reported benefits in animal models of senile and secondary amyloidosis as well as reported benefit in the treatment of secondary amyloidosis in humans. None of the patients showed any objective regression of their disease. The median survival of the entire group was 19.4 months. This survival is not superior to that reported with other agents used in this disease. We conclude that α‐tocopherol is not a valuable agent in the treatment of AL.
“idiopathic thrombocytopenic purpura‐like syndrome” treated with interferon in a patient with lung cancer
Authors: Rapoport B. L. , Falkson G. , Alberts A. S. , Eloff P. 
Published: February 2007

AbstractA case of autoimmune thrombocytopenia associated with lung cancer is presented. The thrombocytopenia occurred 2 years before the diagnosis of lung cancer. Previous treatments include high doses of corticosteroids and splenectomy. The patient was managed with interferon, which resulted in an increase of the platelet count with control of the hemorrhagic manifestations with this syndrome.
Cobalamin as coenzyme: A twisting trail of research
Authors: Beck William S. 
Published: February 2007

AbstractCobalamin derivatives serve as coenzymes for the body's two cobalamin‐dependent enzymes—adenosylcobalamin‐dependent methylmalonyl CoA mutase, and methylcobalamin‐dependent methionine methyltransferase.This essay reviews, in brief form and in personal terms, the history, beginning in the mid‐1950s, of how these enzymes and coenzymes were discovered and what has been learned of their reaction mechanisms. It is clear that because of the fragility of the unique carbon‐cobalt bond in Cobalamin coenzymes, they serve primarily as free radical formers. This accounts for their efficiency in abstracting hydrogen from substrate molecules and for a subsequent chain of events that results in the isomerization of methylmalonyl CoA, the transfer of methyl groups, and (in certain bacteria) the reduction of ribonucleotides. Some thoughts are offered on the possible evolutionary significance of these facts.
Histidine‐rich glycoprotein as a negative acute phase reactant
Authors: Saigo Katsuyasu , Yoshida Akinori , Ryo Ryukichi , Yamaguchi Nobuo , Leung Lawrence L. K. 
Published: February 2007

AbstractSerum histidine‐rich glycoprotein was determined by the Laurell method in patients with acute inflammation, which negatively correlated with C‐reactive protein (P < 0.005). In sequential observations, this negative relationship was apparently confirmed. Thus histidine‐rich glycoprotein acts as a negative acute phase reactant.
Direct detection of β thalassemic mutations: Use of biotin‐labelled allele specific probes
Authors: Hendy J. G. , Cauchi M. N. 
Published: February 2007

AbstractMutations at positions βIVSS1–6, βIVS1–110, and β39 of the β globin gene are responsible for the three most common thalassemic genes in the Mediterranean population. The polymerase chain reaction (PCR) was employed to amplify a 536 base pair segment surrounding this region. Nonradioactive labelling of an oligonucleotide probe, specific for the βIVS1–110 mutation, was achieved by incorporation of biotin‐16‐dUTP into a standard 3′‐end labelling procedure. This probe was subsequently hybridized with the PCR amplification product and permitted detection of the mutant gene in a homozygous β thalassemic child by a simple colour detection method using a streptavidin‐alkaline phosphatase conjugate and NBT/BCIP (nitroblue tetrazolium/5‐bromo‐4‐chloro‐3‐indolyl phosphate) substrate. A known cloned mutant gene was similarly detected. Results could be obtained within 48 hr. These findings suggest that such an approach could provide a rapid and specific means for detection of β thalassemic mutations without the need for radioactive probes.
Structure of the granulocyte macrophage colony‐stimulating factor gene in patients with the myelodysplastic syndromes
Authors: Boultwood Jacqueline , Abrahamson Gail , Buckle Veronica J. , Rack Katrina , Oscier David G. , Wainscoat James S. 
Published: February 2007

AbstractDNA samples from 76 patients with the myelodysplastic syndromes, including 10 cases with a partial deletion of the long arm of chromosome 5 (5q‐), were examined for structural rearrangements of the granulocyte/macrophage colony‐stimulating factor (GM‐CSF) gene. No abnormalities were detected, indicating that structural aberrations of this gene are not a feature of the myelodysplastic syndromes.
Excess of red cell membrane proteins in hereditary high‐phosphatidylcholine hemolytic anemia
Authors: Lane Peter A. , Kuypers Frans A. , Clark Margaret R. , Andrews Deborah A. , Wagner Gail M. , Butikofer Peter , Shapiro Amy D. , Chiu Daniel T. Y. , Lubin Bertram H. , Mentzer William C. 
Published: February 2007

AbstractPrevious descriptions of hereditary high‐phosphatidylcholine hemolytic anemia (HPCHA) have highlighted the association of increased erythrocyte membrane phosphatidylcholine with abnormal membrane cation permeability. We studied the function and composition of erythrocyte membranes from three individuals with HPCHA to characterize further the membrane abnormalities in this disorder. Despite significant macrocytosis, HPCHA red cells were dehydrated and showed an increased surface area to volume ratio compared to normal red cells. The passive efflux of K+ from HPCHA erythrocytes was increased fourfold at 37°C. Total membrane phospholipid was increased 7–42%, largely due to excess phosphatidylcholine, which made up 35.8–37.2% of total phospholipid. Membrane cholesterol:phospholipid ratios were in the normal range. It appears that the excess phosphatidylcholine was not acquired during circulation, since plasma lipids were normal and all subpopulations of density‐separated HPCHA erythrocytes were similarly abnormal. The ratio of total protein to phospholipid in white ghosts was increased, indicating that membrane protein was increased to an even greater extent than membrane lipids. No abnormal membrane proteins were identified by Coomassie or periodic acid Schiff (PAS) staining. Quantitation of the major membrane proteins indicated that the total protein excess in HPCHA membranes was due to a proportional increase in all major proteins. We conclude that HPCHA erythrocytes have excess membrane proteins and hypothesize that the changes in lipid composition and cation permeability are secondary to underlying protein abnormalities, which remain to be defined.
Hb South milwaukee [β105 (G7) leu → Phe]: A newly‐identified hemoglobin variant with high oxygen affinity
Authors: Honig George R. , Divgi Ajit B. 
Published: February 2007

AbstractFifteen individuals among four generations of a family of English ancestry demonstrated elevated hemoglobin levels accompanied by leftward‐shifted whole blood oxygen equilibrium curves. Five of the affected family members have required phlebotomies for relief of symptoms attributable to erythrocytosis. An abnormal hemoglobin or globin chain could not be isolated, but 43% of the β chains of the affected individuals contained a Leu → Phe substitution at position 105 (G7). Oxygen equilibrium curves demonstrated a normal Bohr effect but decreased cooperativity.
Acetylcholinesterase in the human erythron. III. Regulation of differentiation
Authors: Barr Ronald D. , Koekebakker Marijke 
Published: February 2007

AbstractAcetylcholinesterase (AChE) is present in both primitive and mature erythroid cells, but a role for the enzyme in human hematopoiesis has not been defined. This prospect represented the primary objective of the following study. In clonal culture of normal human bone marrow cells, a “wave” of AChE activity was demonstrated, rising from undetectable levels to a peak (of 1.48 femto‐moles per min per cell) at 10 days in the course of progressive erythroid clonogenesis. At concentrations of enzyme inhibitor that clearly reduced AChE activity in a dose‐dependent fashion, there was no overall effect on erythropoiesis in vitro, but the clones were generally smaller and significantly more often multi‐focal than in control cultures. Furthermore, in the presence of AChE inhibitors, a concentration‐dependent increase in the myeloid‐erythroid ratios of the culture harvests was observed. Likewise, a clear reduction in hemoglobination was revealed, in cells of 10 day cultures, from a mean hemoglobin concentration of 35.0 pg per cell in controls to 20.1 pg per cell in the presence of the maximal concentration of the inhibitor (10−6M eserine). These data point to a role for AChE in the regulation of differentiation in the human erythron.
Micromethod for demonstrating increased platelet surface immunoglobulin G: Findings in acute, chronic, and human immunodeficiency virus‐1‐related immunologic thrombocytopenias
Authors: Sthoeger Dahlia , Nardi Michael , Travis Susan , Karpatkin Margaret , Karpatkin Simon 
Published: February 2007

AbstractA method has been developed for the demonstration of increased platelet surface IgG that uses 1 ml of blood regardless of the platelet count. Platelets are gel filtered to remove plasma and contaminating lymphocytes. They are then reacted with fluorescein‐conjugated antihuman IgG and analysed by flow cytometry. Percent positive staining cells vary from 10% to 80% of total cells examined. A platelet antibody index is derived from the product of percent positive staining cells X mean fluorescence intensity of positive staining cells. All patients studied with chronic idiopathic thrombocytopenia purpura (IIP) or human immunodeficiency virus‐1 (HIV‐1)‐related thrombocytopenia had increased platelet surface IgG. Twelve acute children and 11 chronic children had indices averaging 3.5‐and 8.9‐fold greater than 12 normal children, respectively. Five of 12 children with acute ITP had normal platelet IgG. There was no linear correlation between the platelet antibody index and platelet count. Platelets of patients with acute, chronic, or HIV‐1‐related ITP displayed autofluorescence. In chronic ITP, the percentage of platelets displaying auto‐fluorescence had a significant negative correlation with the platelet count. This technique will be a valuable diagnostic tool in the pediatric population.
Malignant mastocytosis with circulating mast cells
Authors: Torrey Elizabeth , Simpson Karen , Wilbur Stephen , Munoz Phillip , Skikne Barry 
Published: February 2007

AbstractA case of malignant mastocytosis with peripheral blood involvement is presented. The course of the patient's illness was complicated by recurrent hypotensive episodes, presumed to have been caused by mast cell degranulation. Treatment with hydroxyurea was associated with persistent hypotension which resulted in death. It has been proposed that the diagnosis of mast cell leukemia be given to patients presenting with greater than 10% atypical mast cells in the blood. However, review of 16 published cases of malignant mastocytosis with circulating mast cells reveals that the clinical manifestations, complications, and survival do not vary significantly with the percentage of peripheral blood mast cells. Patients with malignant mastocytosis with significant involvement by atypical mast cells in the bone marrow and peripheral blood should be considered as having an aggressive disease, regardless of the precentage of circulating mast cells.
Richter syndrome with two B cell clones possessing different surface immunoglobulins and immunoglobulin gene rearrangements
Authors: Tohda Shuji , Suzuki Toshiya , Nagata Kaoru , Kamiyama Takashi , Imai Yasufumi , Nara Nobuo , Aoki Nobuo , Morio Tomohiro 
Published: February 2007

AbstractA patient with two populations of B cell malignancy in the bone marrow is reported. One population consisted of mature small lymphocytes, expressing surface IgM + D, λ and proliferating very slowly. The other population consisted of abnormal large lymphoid cells, expressing surface IgM, K and proliferating actively. We considered the former as chronic lymphocytic leukemia cells and the latter as malignant lymphoma cells. Therefore, this case was considered as Richter syndrome. The analysis of immunoglobulin heavy chain gene rearrangement showed the different patterns between the two populations. It suggested that the two populations arose from the different origins. We discussed the genetic relationship between the two clones of Richter syndrome referring to other reported cases.
Heparin‐like anticoagulant associated with systemic candidiasis
Authors: Horne McDonald K. , Chao Elizabeth S. , Wilson Olga J. 
Published: February 2007

AbstractA 15 year old girl with aplastic anemia developed a heparin‐like anticoagulant during the course of systemic candidiasis. This was initially detected in the laboratory by an elevation of the thrombin clotting time which corrected with toluidine blue but not by mixing with normal plasma. In vivo and in vitro the anticoagulant was remarkably resistant to neutralization by protamine sulfate. Nevertheless, its heparin‐like nature was confirmed by its sensitivity to heparinase and its dependence on antithrombin III.
Hemostasis in malignancy
Authors: Nand Sucha , Messmore Harry 
Published: February 2007

AbstractHemostatic abnormalities are present in a majority of patients with metastatic cancer. These abnormalities can be categorized as 1) increased platelet aggregation and activation, 2) abnormal activation of coagulation cascade, 3) release of plasminogen activator, and 4) decreased hepatic synthesis of anticoagulant proteins like Protein C and antithrombin III. The abnormal activation of coagulation cascade is mediated through release of Tissue Factor, Factor X activators, and other miscellaneous procoagulants from the plasma membrane vesicles of tumor cells. Macrophages of a tumor‐bearing host also produce increased amounts of Tissue Factor. Production of Factor X activators and macrophage Tissue Factor is decreased by warfarin. The ability of the tumor cells to produce platelet‐aggregating activity and plasminogen activator parallels their metastatic potential in animal and experimental systems. These studies also show that antiplatelet agents and antibodies against plasminogen activator can suppress the metastatic process. One or more laboratory abnormalities of hemostasis can be shown in up to 95% of patients with metastatic cancer. These abnormalities, however, are unable to predict subsequent development of thromboembolic or hemorrhagic complications.Clinical complications occur in 9–15% of the patients in the form of thrombotic or hemorrhagic disorders. The therapy of tumor‐related coagulopathy should be guided by its clinical expression. Subclinical DIC should not be treated. Coumadin is generally ineffective for therapy of thrombosis in cancer patients. There is no consensus regarding the use of heparin in acute promyelocytic leukemia (APL). The defibrination in APL may be from disseminated intravascular coagulation as well as systemic fibrinolysis, as shown by decreased alpha 2 antiplasmin levels. In such cases, epsilon aminocaproic acid plus heparin therapy may be of benefit.
Distribution of cell surface glycoprotein CD9 (P24) antigen on megakaryocyte lineage leukemias and cell lines
Authors: Imamura Nobutaka , Mtasiwa Deodatus M. , Ota Hiromi , Inada Tominari , Kuramoto Atsushi 
Published: February 2007

AbstractWe herein describe CD9 (p24) antigen as existing on the cell surface of megakaryocyte lineage leukemias as well as megakaryocytic leukemia cell lines, MEG‐01 and HEL, by means of fluorescence‐activated cell sorter (FACS IV) with a panel of monoclonal antibodies (Mabs).We found CD9 antigen expression on the cell surface of megakaryoblastic leukemias as well as MEG‐01 and HEL cells. Furthermore, CD9 antigen expression increased while culturing these cells with phorbol esters, and was also found in the cytoplasm by means of indirect immunofluorescence test.These findings clearly showed that CD9 antigen exists on the cell surface of megakaryocytic cells which are capable of synthesizing the CD9.
The persistence and heterogeneity of factor VIII:C inhibitors as demonstrated by preparative isofocusing
Authors: Coots Macie C. , Glueck Helen I. 
Published: February 2007

AbstractThe purpose of this study was to determine if factor VIII:C inhibitors completely disappear during remission and if they recur on exacerbation, are they similar to or different from the original inhibitors? To answer these questions, isofocusing, which separates proteins on the basis of their p/s, was utilized to recover inhibitors from the plasmas of six patients, five nonhemophiliacs with acquired VIII:C inhibitors and the sixth with classic hemophilia. Initially, all plasmas were studied during the presenting hemorrhagic episodes and, subsequently, following the disappearance or recurrence of the inhibitors. Following isofocusing, each fraction was tested for inhibitory activity. The method enabled us to determine that inhibitors to factor VIII:C persisted even when they could not be demonstrated by conventional methods. The inhibitory activity resulted from a composite of inhibitors, each of the group identified by its own p/. In many patients, some peaks persisted throughout the entire interval of study, whereas others disappeared and new ones appeared, suggesting that various groups of cells were capable of producing the inhibitors.
Treatment of relapsed and refractory acute myeloid leukemia with diaziquone and mitoxantrone: A CALGB phase I study
Authors: Amrein Philip C. , Davis Roger B. , Mayer Robert J. , Schiffer Charles A. 
Published: February 2007

AbstractTwenty‐one patients with relapsed or refractory acute myeloid leukemia (AML) were treated with mitoxantrone (12 mg/m2/day, days 1–3) and diaziquone (continuous infusion days 1–5). The dosage of diaziquone was increased for sequential cohorts of seven patients from 20 mg/m2/day to 24 mg/m2/day, and finally to 28 mg/m2/day to determine the maximum tolerated dose for this chemotherapy combination. Myelosuppression was the dose‐limiting toxicity. The median time to recovery of blood counts was greater at the highest dose of diaziquone (48 days) than at the lower two doses (31 and 28 days). Other toxic effects were minimal. Overall, 9/21 (43%, 95% confidence interval, 0.22 to 0.66) patients achieved complete remission. We conclude that this combination of drugs shows sufficient antileukemic activity with acceptable toxicity to warrant further trials.
Detection of interleukin‐6 (IL‐6) in human bone marrow myeloma cells by light and electron microscopy
Authors: Ohtake Ken‐Ichi , Yano Takahiro , Kameda Kazuo , Ogawa Tetsuhei 
Published: February 2007

AbstractWe used an Avidin‐Biotin peroxidase complex (ABC) immunoperoxidase technique to evaluate the localization of IL‐6 of human bone marrow cells in multiple myeloma (MM). The cellular distribution of IL‐6 was determined at light and electron microscopic levels. The author's study indicated that cytoplasmic IL‐6 was detected only in the myeloma cells of bone marrow cells. Immunoelectron microscopic (immuno‐EM) study showed positive reactivity mainly in the perinuclear space (PNS), well‐developed rough endoplasmic reticulum (RER), and Golgi area.
Cytogenetic findings in 21 cases of peripheral T‐Cell lymphoma
Authors: Inwards David J. , Habermann Thomas M. , Colgan Joseph P. , Banks Peter M. , Dewald Gordon W. 
Published: February 2007

AbstractAlthough numerous publications have described the chromosome abnormalities in B‐cell non‐Hodgkin lymphoma and their significance, sparse literature exists pertaining to the chromosome abnormalities in T‐cell lymphoma. We did cytogenetic analyses in 21 cases of peripheral T‐cell lymphoma (PTCL). Chromosomally abnormal clones were identified in 15 (71%) of the cases, including 7 of the 10 cases in which the histologic distinction between a malignant and benign process was difficult. Abnormalities of chromosome 1 were observed in 10 cases; a breakpoint at 1p36 was demonstrated in 5 of these cases. Chromosome abnormalities previously attributed to B‐cell malignancies were infrequent. These results suggest an association between 1p36 breakpoints and PTCL and emphasize the utility of cytogenetic analysis for documenting clonality among the histologically diverse groupings of PTCL.