bluuebunny/crossref_metadata_2025_split huggingface dataset:
GγAγ(β+) hereditary persistence of fetal hemoglobin: The Gγ – 158 C → T mutation in cis to the − 175 T → C mutation of the Aγ‐globin gene results in increased Gγ‐globin synthesis
Authors: Coleman M. B. , Adams J. G. , Steinberg M. H. , Plonczynski M. W. , Harrell A. H. , Castro O. , Winter W. P. 
Published: February 2007

AbstractHereditary persistence of fetal hemoglobin (HPFH) can be generally classified into deletional and nondeletional forms. The family described in the present study has characteristics of both types of HPFH. The proband is a healthy 30‐year‐old black woman. Analysis of her hemoglobin revealed 40.4% HbS, 40.9% HbF (Gγ/Aγ ratio 0.53), 16.8% HbA, and 1.9% HbA2. All of her hematologic indices were normal, and the distribution of HbF in her red cells was pancellular. Family studies demonstrated that the proband has one chromosome 11 bearing the βs‐globin gene and the other bearing a GγAγ(β+) HPFH determinant in cis to the βA‐globin gene. Gene mapping studies of the region between the Gγ‐ and β‐globin genes were normal. However, when the Aγ and Gγ promoters were amplified by polymerase chain reaction (PCR) and sequenced, the Aγ promoter was found to have the T→C mutation at −175, and the Gγ promoter region was found to have the C→T mutation at −158. The −158 C→T mutation has been associated with elevated Gγ levels and high HbF in hemolysis, although its role in causing these effects is unclear. The present study suggests that this mutation can also enhance Gγ‐globin expression in cis to the −175 T→C mutation in the absence of hemolysis. We suggest that the alteration of the Aγ gene octamer binding site by the −175 mutation, as well as the loss of a putative Gγ “silencer” caused by the −158 mutation may account for this phenotype. We propose calling these linked mutations the GγAγ(β+) HPFH. © 1993 Wiley‐Liss, Inc.
Improved or fatal acute disseminated intravascular coagulation in systemic lupus erythematosus
Authors: Shimamoto Yoshinori , Ohta Akihide , Sano Masayuki , Suga Kenji , Yamaguchi Masaya 
Published: February 2007

AbstractTo analyze the outcome of systemic lupus erythematosus (SLE) associated with acute disseminated intravascular coagulation (DIC) and also to clarify the clinical factor(s) contributing to the outcome, we retrospectively investigated 120 SLE patients treated between 1981 and 1991. Eight of these patients (6.7%) developed acute DIC; four recovered and the other four died within 2 weeks of onset. Infection preceded acute DIC in all these patients. Acute DIC associated with atypical pneumonia was always fatal, while the patients with pharyngitis or urinary tract infection survived when they were treated adequately. Comparison of the dead and surviving groups revealed that the activity of SLE before the onset of DIC, the severity of DIC, and the treatment given for DIC and the coexistent infection were not significantly related to a fatal outcome. However, severe infection such as atypical pneumonia in patients with secondary immunodeficiency was likely to be fatal irrespective of the presence of DIC. © 1993 Wiley‐Liss, Inc.
A mild type of Hb S‐β+‐thalassemia [‐92(C→T)] in a sicilian family
Authors: Divoky V. , Baysal E. , Schiliro G. , Dibenedetto S. P. , Huisman T. H. J. 
Published: February 2007

AbstractHematological data are presented for an adult Sicilian patient with a mild Hb S‐β+‐thalassemia caused by a C→T mutation at position −92 of the β promoter. This mutation was identified by sequencing of amplified DNA and was confirmed by dot‐blot analysis with specific probes. A comparison of levels of Hb S and Hb A in Hb S‐β‐thalassemia patients with different β‐thalassemia alleles showed great variations; the highest level of Hb A (45%) was recorded in the patient with Hb S‐β+‐thalassemia [‐92(C→T)] and the lowest (∼13%) in patients with Hb S‐β+‐thalassemia [IVS‐II‐745 (C→G)]. Clinical severity is directly related to the level of Hb A present. © 1993 Wiley‐Liss, Inc.
Long‐term high‐level expression of human β‐globin occurs following transplantation of transgenic marrow into irradiated mice
Authors: Himelstein Andrew , Ward Maureen , Podda Silvio , de la Flor Weiss Eduardo , Costantini Frank , Bank Arthur 
Published: February 2007

AbstractWhen the human β‐globin gene is transferred into the bone marrow cells of live mice, its expression is very low. To investigate the reason for this, we transferred the bone marrow of transgenic mice containing and expressing the human β‐globin into irradiated recipients. We demonstrate that long‐term high level expression of the human β‐globin gene can be maintained in the marrow and blood of irradiated recipients following transplantation. Although expression decreased over time in most animals because of host marrow reconstitution, the ratio of human β‐globin transgene expression to endogenous mouse β‐globin gene expression in donor‐derived erythroid cells remained constant over time. We conclude that there is no inherent limitation to efficient expression of an exogenous human β‐globin gene in mouse bone marrow cells following marrow transplantation. © 1993 Wiley‐Liss, Inc.
Discordant LFA‐1/ICAM‐1 expression in a case of secondary plasma cell leukemia associated with subcutaneous plasmacytoma
Authors: Tsutani Hiroshi , Sugiyama Taeko , Shimizu Shiro , Iwasaki Hiromichi , Ueda Takanori , Ozaki Kenji , Konda Susumu , Nakamura Toru 
Published: February 2007

AbstractWe observed a unique case of multiple myeloma that was transformed into plasma cell leukemia presenting with subcutaneous and soft tissue infiltrates. Subcutaneous and minor pelvic soft tissue plasmacytomas and the leukemic transformation were diagnosed in a 72‐year‐old woman after she had completed 9 months of chemotherapy for IgG k multiple myeloma. Immunophenotypic study revealed that leukemic cells in her peripheral blood were positive for ICAM‐1 (CD54) but negative for LFA‐1 α (CD11a) and LFA‐1 β (CD18), whereas infiltrating leukemic cells in the subcutaneous plasmacytoma of the left thigh were positive for LFA‐1 α and LFA‐1 β but negative for ICAM‐1. In addition, intermingling capillary endothelial cells were positive for ICAM‐1. Extramedullary soft tissue plasmacytoma is uncommon in association with plasma cell tumors, and the exact mechanism of the development of plasmacytoma is not known. In the present case, however, the discordant expression of LFA‐1/ICAM‐1 adhesion molecules may have accounted for the distinct patterns of growth and the spread of the subcutaneous plasmacytoma through homing of the LFA‐1 α+, LFA‐1 β+ leukemic cells to ICAM‐1 endothelial cells. © 1993 Wiley‐Liss, Inc.
Spleen in sickle cell anemia: Comparative studies of Nigerian and U.S. patients
Authors: Adekile A. D. , McKie K. M. , Adeodu O. O. , Sulzer A. J. , Liu J.‐S. , McKie V. C. , Kutlar F. , Ramachandran M. , Kaine W. , Akenzua G. I. , Okolo A. A. , Asindi A. A. , Obinyan E. A. , Ogala W. N. , Ibrahim M. , Huisman T. H. J. 
Published: February 2007

AbstractAnecdotal reports have attributed persistent splenomegaly in African sickle cell anemia (SS) patients to the effects of malaria. However, no comparative studies of patients in malarial and nonmalarial regions have been conducted, and few studies of malaria antibody titers have been reported. In the present study, age‐ and sex‐matched Nigerian and U.S. steady‐state SS patients were compared. Splenomegaly was found in 22.3% of Nigerian patients (n = 310), while it was found only in 8% of U.S. patients (n = 100) from Georgia. There was significant linear correlation between spleen size and Hb levels and with serum immunoglobulins in the Nigerian group. However, serum complement levels (C3 and C4) were not affected by spleen size. In both groups, patients with splenomegaly had fewer circulating pitted red cells than their counterparts without splenomegaly. The mean ± SE of IgG‐specific malaria antibody titer among the Nigerian patients without palpable spleens was 9,386 ± 2,036; 9,334 ± 2,980 in those with spleens between 1 and 5 cm, 16,201 ± 4,502 in those with spleens between 6 and 10 cm, and 22,445 ± 8,456 in those with spleens above 10 cm. Coexistent α‐thalassemia did not influence the prevalence of splenomegaly among the Nigerian SS patients. This study provides additional evidence that malaria plays a significant role in the persistence of splenomegaly in African patients. © 1993 Wiley‐Liss, Inc.
Interleukin‐5 mRNA in three T‐cell lymphomas with eosinophilia
Authors: Samoszuk Michael , Ramzi Eiman , Cooper Dennis L. 
Published: February 2007

AbstractThe objective of this controlled pilot study was to determine if mRNA coding for interleukin‐5 (IL‐5), a cytokine that promotes eosinophil differentiation, growth, and migration, could be detected in three T‐cell lymphomas that were infiltrated extensively by eosinophils. To detect mRNA coding for IL‐5, we performed an RNA polymerase chain reaction on mRNA extracted from three T‐cell lymphomas with eosinophilia and from 29 positive and negative validation controls. Using this procedure, we detected a 293‐base pair, IL‐5‐specific amplification product in the three cases of T‐cell lymphoma with eosinophilia and in 11 of 12 positive validation controls, including 10 cases of Hodgkin's disease with eosinophilia. IL‐5 mRNA was not detectable in the 17 negative validation controls. This preliminary study suggests that IL‐5 mRNA is detectable by polymerase chain reaction in three cases of T‐cell lymphoma with eosinophilia. © 1993 Wiley‐Liss, Inc.
Pilot study of ascorbic acid for the treatment of refractory immune thrombocytopenic purpura
Authors: Jubelirer Steven J. 
Published: February 2007

AbstractThe treatment of corticosteroid‐ and/or splenectomy‐refractory Immune thrombocytopenic purpura (ITP) includes vinca alkaloids, immunosuppressives, Danazol, intravenous gammaglobulin, and α‐interferon. However, these treatments have often been associated with toxic side effects. Brox et al. (Br J Haematol 70:341–344, 1988) reported the efficacy of ascorbic acid in the treatment of ITP; the platelet count normalized in seven of 11 patients studied, and tolerance was excellent. However, other investigators have reported less impressive results. These conflicting reports prompted a pilot study of ascorbic acid in 12 patients with refractory ITP. Patients were given 2 g every morning for at least 10 weeks. All have received glucocorticoids, three had undergone splenectomy, and six received other treatments. The maximal increase in platelet count above baseline (i.e., the prestudy platelet count) achieved was <20,000 in 11 patients and 25,000 in one patient. Therapy was well tolerated, with only two patients complaining of dyspepsia. The results of this study suggest that ascorbic acid is not very effective in patients with refractory ITP.
Hematologic remission and cytogenetic improvement after treatment of stable‐phase chronic myelogenous leukemia with continuous infusion of low‐dose cytarabine
Authors: Robertson Michael J. , Tantravahi Ramana , Griffin James D. , Canellos George P. , Cannistra Stephen A. 
Published: February 2007

AbstractProlonged exposure to low concentrations of cytarabine preferentially inhibits in vitro growth of neoplastic myeloid progenitors from patients with chronic myelogenous leukemia (CML) compared to that of normal myeloid progenitors. Continuous infusions of cytarabine in doses of 15–30 mg/m2/day were therefore administered for extended periods to patients with CML in stable phase to determine if this treatment could achieve selective cytoreduction of Philadelphia chromosome (Ph)‐positive cells. Five patients demonstrating >90% Ph‐positive metaphases before treatment received a total of 43 cycles of cytarabine infusional therapy. Cytarabine was administered on an outpatient basis using a portable, battery‐operated syringe pump until the total leukocyte count reached 2500/μl or the platelet count reached 75,000/μl. A new cycle was begun when the total leukocyte count exceeded 4,000/μl and the platelet count exceeded 100,000/μl. The median duration of cytarabine administration per cycle was 29 days (range 15–72 days). Leukocytosis was readily controlled by low‐dose cytarabine therapy in all patients. All five patients experienced complete hematologic responses during cytarabine therapy. The fraction of Phpositive metaphases in the marrow of the five patients was reduced to 0, 10%, 43%, 72%, and 84%, respectively, during therapy. The median time to achieve optimal cytogenetic response was 4.8 months (range 2.8–8.6 months). One patient demonstrated a complete cytogenetic response after three cycles of cytarabine. Another patient demonstrated persistent cytogenetic improvement during 20 cycles of cytarabine, with a median 38% Ph‐positive marrow metaphases (range 10–53%) over 32 months. Cytarabine therapy was generally well‐tolerated, but was discontinued in one patient because of persistent asymptomatic elevations in hepatic enzymes, which resolved within 2 months after discontinuing therapy. There were no episodes of fever during neutropenia, and platelet transfusions were not required. However, symptomatic anemia requiring transfusion of red cells occurred during most cycles of treatment. In summary, treatment of CML with low‐dose cytarabine can induce prolonged cytogenetic improvement in some patients with acceptable toxicity. Further evaluation is needed to ascertain the effects of this treatment on duration of stable phase and overall survival.
Measurement of β2‐glycoprotein i (apolipoprotein h)‐independent anticardiolipin antibody in human immunodeficiency virus‐1‐positive and‐negative hemophiliacs
Authors: Matsuda Juzo , Saitoh Noriko , Tsukamoto Miyo , Gohchi Kengo , Asami Katsuhisa , Hashimoto Masakatsu 
Published: February 2007

AbstractThe authors studied whether or not anticardiolipin antibody (aCL), which is frequently detected in hemophiliacs with human immunodeficiency virus‐1 (HIV‐1) infection, is dependent on β2‐glycoprotein I (GPI), a cofactor of cardiolipin (CL). GPI‐independent aCL was positive in 27 of 36 hemophiliacs with HIV‐1 antibody (75%) and in 23 of 29 patients without HIV‐1 antibody (79%). However, only six HIV‐1‐positive and four HIV‐1‐negative patients were positive for GPI‐dependent aCL. Thus the aCL in these hemophiliacs was GPI independent and therefore different from the aCL found in autoimmune diseases, such as systemic lupus erythematosus.
Comparison of the behavior of normal factor IX and the factor IX BM variant hilo in the prothrombin time test using tissue factors from bovine, human, and rabbit sources
Authors: Lefkowitz Jerry B. , Monroe Dougald M. , Kasper Carol K. , Roberts Harold R. 
Published: February 2007

AbstractA subset of hemophilia B patients have a prolonged bovine‐brain prothrombin time. These CRM+ patients are classified as having hemophilia Bm. The prolongation of the prothrombin time has been reported only with bovine brain (referred to as ox brain in some literature) as the source of thromboplastin; prothrombin times determined with thromboplastin from rabbit brain or human brain are not reported to be prolonged. Factor IX from a hemophilia Bm patient (factor IX Hilo) was isolated. The activity of factor IX Hilo was compared to that of normal factor IX in prothrombin time assays when the thromboplastin source was of bovine, rabbit, or human origin. Factor IX, either normal or Hilo, prolonged a prothrombin time regardless of the tissue factor source. However, unless thromboplastin was from a bovine source, this prolongation required high concentrations of factor IX. Further, factor IX normal was as effective as factor IX Hilo in prolonging the prothrombin time when rabbit or human thromboplastin was used. With bovine thromboplastin, factor IX Hilo was significantly better than factor IX normal at prolonging the prothrombin time. The amount of prolongation was dependent on the amount of factor IX Hilo added. In addition, the prolongation was dependent on the concentration of factor × present in the sample. The prothrombin time changed as much as 20 seconds when the factor × concentration was varied from 50% to 150% to normal (fixed concentration of factor IX Hilo). These results demonstrate the difficulty of classifying the severity of a hemophilia Bm patient based on the bovine brain prothrombin time unless both the factor IX and factor × concentrations are known.
Four years' experience with the treatment of all‐trans retinoic acid in acute promyelocytic leukemia
Authors: Wu Xingzhong , Wang Xuewen , Qien Xiaoping , Liu Hainin , Ying Jianshang , Yang Zhicheng , Yao Haiqien 
Published: February 2007

AbstractA retrospective analysis was done on 43 patients with acute promyelocytic leukemia (APL) at our hospital from June 1987 to August 1992. All‐trans retinoic acid was used to induce these patients to differentiation. In the early period of induction there were risks of severe hemorrhage, which was the main cause of early death. Treatments combined with platelets and heparin or aminomethylbenzoic (PAMBA) were given to patients with abnormal coagulation. As a result only 4 out of 43 patients died of intracranial bleeding at 4–12 days when their white blood cell (WBC) counts peaked. The combination of retinoic acid (RA) and HA chemotherapy could reduce hyperleukocytosis during the RA induction course. None of 7 patients died at early stage with this treatment combination. Our studies showed that it could predict the onset of remission at early stage through observations on the successive changes of karyotypes and morphology of the bone marrow and peripheral blood cells. Our studies also showed that the growth of CFU‐F could be inhibited by RA. We think that it may play a role in the RA‐induced differentiation. In 4 years of follow‐up the overall leukemia‐free survival (LFS) was 80% with a relapse rate of 45%. Thirty‐five patients out of 43 cases were still alive in remission, and one was alive in relapse. All 11 out of 43 patients relapsed within 3 years, but the relapses occurred later, after 3 years duration of remission (P < .01). Retinoic acid failed to induce 5 patients who relapsed with the continuation treatment of RA and chemotherapy alternatively. In order to overcome the resistance to RA, the continuation treatment of simple chemotherapy had been administered following CR. Two cases achieved remission in this way. The difference of resistant events to RA reached significance between these 2 groups of different continuation treatment.
In vivo antimalarial action of a lipophilic iron (III) chelator: Suppression of plasmodium vinckei infection by reversed siderophore
Authors: Lytton Simon D. , Loyevsky Mark , Mester Brenda , Libman Jacqueline , Landau Irene , Shanzer Abraham , Cabantchik Z. Ioav 
Published: February 2007

AbstractWe assessed in vivo antimalarial action of a lipophilic iron (III) chelator belonging to a new synthetic family of biomimetic siderophores previously termed reversed siderophores (RSFs). The family member, RSF ileum2, was chosen for its high membrane permeability and fast irreversible inhibition of human malaria parasite growth in vitro. [Shanzer A, et al., Proc Natl Acad Sci USA 88:6585, 1991 and Lytton SD, et al., Blood 81:214, 1993]. The lipophilic drug was administered to Swiss mice by subcutaneous route in fractionated coconut oil at a dosage of 0.37 g/kg every 8 hr with no adverse reactions observed. After 3–4 injections demonstrable suppression of Plasmodium vinckei petteri infection was observed and an additional 3–4 injections resulted in 2–3‐fold lower parasitemia with prolonged survival time over sham‐injected control mice.
Dipyridamole enhancement of drug sensitivity in acute lymphoblastic leukemia cells
Authors: Sotomatsu Manabu , Yugami Shin‐Ichirou , Shitara Toshiji , Kuroume Takayoshi 
Published: February 2007

AbstractThe effect of dipyridamole (DPM) on cell sensitivity to anticancer drugs was examined in acute lymphoblastic leukemia (ALL) cell lines. We established two ALL cell lines (KMO‐90 and KMO‐R) from bone marrow samples of a 12‐year‐old girl with ALL. The drug concentrations needed to reduce optical density to 50% of that of control cells (IC50) showed that KMO‐R was about twofold more resistant to doxorubicin (DOX), mitxantrone (MIT), vincristine (VCR), and etoposide (VP‐16) than was KMO‐90. Considering that both KMO‐90 and KMO‐R were established from a patient with ALL at the time of presentation and relapse, respectively, these two cell lines might be novel and useful models for research into the acquisition of drug resistance in ALL cells. Although cytotoxicity of DPM in KMO‐90 was about 6% at 1 μg/ml, DPM enhanced cell sensitivity to DOX, MIT, VCR, and VP‐16 at this concentration. Cytotoxicity of DPM in KMO‐R was less than 5% at 1,5, and 10 μgg/ml. In KMO‐R, DPM enhanced cell sensitivity to these four drugs in a dose‐dependent manner. The plasma concentrations achieved by oral administration of DPM is about 1 μg/ml. At clinically achievable concentrations, DPM enhanced cell sensitivity to DOX, MIT, VCR, and VP‐16 in both KMO‐90 and KMO‐R, thus showing DPM to be a useful agent for potentiating anticancer chemotherapy of hematopoietic malignancy.
Diagnostic value of clonality of surface immunoglobulin light and heavy chains in malignant lymphoproliferative disorders
Authors: Batata Ai , Shen Bo 
Published: February 2007

AbstractCell suspensions from the peripheral blood of B‐chronic lymphoid leukemias (B‐CLL, n = 274) and reactive lymphocytosis (RLC, n = 132) and from solid tissue samples of B‐non‐Hodgkin's lymphoma (B‐NHL, n = 466) and reactive lymphadenopathy (RLA, n = 324) were analyzed to evaluate the diagnostic value of clonality of L‐ and H‐ chains in B‐CLL and B‐NHL. Cutoff levels for monoclonal L‐chain (mono‐L) and monoclonal H‐chain (mono‐H) were defined. In B‐CLL, the association patterns of L‐ and H‐ chains were as follows: mono‐L/mono‐H, 245 cases (89.42%); mono‐L/polyclonal H chain (poly‐H), 4 (1.46%); polyclonal L chain (poly‐L)/mono‐H, 2 (0.73%); poly‐L/poly‐H, 2 (0.73%); undetected (und) ‐L/mono‐H, 6 (2.19%); and und‐L/und‐H, 15 (5.47%). In B‐NHL, the association patterns were mono‐L/mono‐H, 433 cases (92.92%); mono‐L/poly‐H, 4 (0.86%); poly‐L/ mono‐H, 8 (1.72%); poly‐L/poly‐H, 2 (0.43%); und‐L/mono‐H, 4 (0.86%); and und‐Lund‐H, 15 (3.22%). Monoclonality of H chains are complementary to L‐chain restriction, especially in the cases with poly‐L or und‐L, and should be considered as a positive criterion in determining surface immunoglobulin (Slg) clonality. Monoclonality of Slg assessed by both L and H chains is both sensitive and specific for the diagnosis of B‐CLL and B‐NHL, and their differentiation from RLC and RLA, since none of the cases of RLC and RLA showed monoclonal Slg.
Prevalence of pyruvate kinase deficiency among the chinese: Determination by the quantitative assay
Authors: Feng Chi‐Shun , Tsang Sophia S. , Mak Ying‐Tat 
Published: February 2007

AbstractThe prevalence of pyruvate kinase (PK) deficiency among the Chinese population has not been established. Fung et al. (Arch Dis Child 44:373–376, 1969) and Wu et al. (Am J Hematol 20:139–144, 1985) indicate 3.4% and 2.1% PK deficiency prevalence rates, respectively, the higher figure based on Beutler's screening test [3] without confirmatory testing. Neither figure is consistent with the occurrence of hemolytic anemia from this cause in the experiences of hematologists in Hong Kong. Using the standard quantitative assay, we measured PK activity in blood samples from 1,100 local Chinese people. The assay was automated on a centrifugal analyzer, and the results were expressed in IU per gram of hemoglobin (IU/g Hb). Blood samples from 497 healthy male adults were measured, and PK activity was found to have a range of 15.2 ± 5.2 (mean ± 2 SD). A total of 100 cord blood samples were also measured, and the reference interval for this subgroup was 17.7 ± 4.8. Additionally, samples from 503 anaemic patients were measured, and all were found to have values above the lower limit of the reference interval. The prevalence of PK deficiency among the Hong Kong Chinese population determined by this study was < 0.1%.