bluuebunny/crossref_metadata_2025_split huggingface dataset:
Primary non‐Hodgkin lymphoma of the spleen
Authors: Brox Alan , Bishinsky Jonathan I. , Berry Gerald 
Published: February 2007

AbstractPrimary splenic lymphoma is a relatively infrequent cause of splenomegaly, its reported incidence being less than 1%. Nonetheless, various series have reported on its occurrence and its unfavorable prognosis, but these series have reported data on heterogenous populations, including many patients with nodal or hepatic disease.We report on a series of nine patients with primary splenic lymphoma. None of these patients had biopsy‐proven evidence of extrasplenic disease and were categorized as involving spleen only (stage 1) or spleen and splenic hilum (stage 2). In this series, classified by the recent N.C.I. working formulation, four patients had intermediate or high‐grade histology and five patients had low‐grade histology. There was no correlation between histological subtype and prognosis, nor could other factors be delineated to explain their favorable prognosis. The median survival in this group of patients was 7.48 years, and no evidence of relapse has been documented.The data suggest that primary splenic lymphoma, treated by splenectomy alone or combination therapy, may be potentially curative. Further studies should address the question of aggressive accurate staging in hopes of obtaining homogenous patient populations so that appropriate treatment in primary splenic lymphoma can be better defined.
Circulating nucleated red blood cells following splenectomy in a patient with congenital dyserythropoietic anemia
Authors: Adams Christopher D. , Kessler John F. 
Published: February 2007

AbstractA 24‐year‐old white female with a 10‐year history of juvenile rheumatoid arthritis and splenomegaly developed numerous circulating nucleated red blood cells (NRBC) following splenectomy for chronic abdominal pain. Subsequent evaluation revealed the presence of a congenital dyserythropoietic anemia (CDA) with atypical features. Circulating NRBCs have been reported following splenectomy in three other cases of CDA, each of which had atypical features and did not fit into the customary classification of types I–IV. Follow‐up of our patient at 4 years revealed no untoward consequences of persistent NRBCs in her circulation.
Thrombocytopenia associated with environmental exposure to polyurethane
Authors: Michelson Alan D. 
Published: February 2007

AbstractFew chemicals in the environment have been implicated as causes of isolated thrombocytopenia, and the evidence is usually less than convincing because the patients were not rechallenged with the chemical in vivo. In the present paper, a child is reported with the onset of thrombocytopenia in temporal association with environmental exposure to polyurethane. Five years after the initial thrombocytopenia had resolved, an inadvertent in vivo rechallenge with environmental polyurethane resulted in recurrence of the thrombocytopenia. This recurrence, together with the fact that only 1–4% of cases of idiopathic thrombocytopenic purpura in children recur, provided strong evidence for a causal role for the polyurethane exposure in this patient's thrombocytopenia. In summary, environmental exposure to polyurethane should be considered in the differential diagnosis of acquired thrombocytopenia in childhood.
Sickle cell disease and hemochromatosis
Authors: Conrad Marcel E. 
Published: February 2007

AbstractA 50 year‐old patient with sickle cell anemia was seen who had received only two units of blood during his lifetime. He had marked iron overloading, cirrhosis of the liver, arthralgia, and mild glucose intolerance. We believe the iron overloading was associated with hereditary hemochromatosis rather than sickle cell anemia because he had HLA‐A3 and B7 antigens, and hepatic iron deposits were primarily in parenchymal cells rather than Kupfer cells. The coexistence of either homozygous or heterozygous hemochromatosis should be suspected in sickle cell patients with organ damage from iron overloading.
Nutritional zinc increases platelet reactivity
Authors: Marx Gerard , Krugliak Judit , Shaklai Matityahu 
Published: February 2007

AbstractAfter ingestion of 220 mg zinc sulfate, platelet aggregation was evaluated at various time intervals (i.e., T = 0, 1, and 3 hr) and the autologous plasma analyzed by atomic absorption analysis. The zinc levels increased maximally some 0.4 ± 0.2 μg/ml within 3 hr after ingestion, which tor the entire blood pool corresponds to only 5% of the ingested zinc. Aggregation responses of platelet rich plasma (PRP), instigated with suboptimal levels of thrombin (<0.2 U/ml), ADP (<2 μM), epinephrine (<2 μM), collagen (<2 μg/ml), or PAF (<50 ng/ml), show significant improvement to at least one aggregant. Mean ± SEM values for Δ% aggregation increase are as follows: thrombin, 51 ± 10%; epinephrine, 21 ± 6%; ADP, 31 ± 6%; collagen 23 ± 6%; and platelet aggregating factor (PAF), 56 ± 6%. For controls, the platelets from one individual with Glanzmann thrombasthenia as well as four undosed volunteers exhibited no significant changes in platelet responsiveness. Increased platelet responsiveness to agonists after zinc sulfate ingestion was observed in PRP from blood collected in either citrate or heparin. We demonstrate that within a relatively short time period, single bolus of nutritional zinc intake can significantly increase platelet reactivity. These findings show that nutritional zinc availability is relevant to hemostasis and may pertain to the viability of platelet concentrates in blood banks.
A p‐nitrophenylphosphatase activity associated with the human erythrocyte membrane
Authors: Brissette Renee E. , Swislocki Norbert I. , Cunningham Earlene Brown 
Published: February 2007

AbstractA p‐nitrophenylphosphatase activity has been identified as a component of the human erythrocyte membrane. This activity is distinct from that associated with the cell's Na+ + K ‐dependent ATPase, Ca2+ ‐dependent ATPase, or spectrin phosphatase. The activity described here is stimulated by Mn2+ but not by Ca2+ with or without calmodulin. A potential erythrocyte membrane substrate for this activity is a 95 kDa phosphoprotein that can be shown to undergo Mn2+ ‐stimulated but not Mg2+ ‐stimulated dephosphorylation.
Origin of hematopoietic progenitor cells after bone marrow transplantation: Analysis by means of a Y‐chromosome specific DNA probe
Authors: Nakatsumi Tomoko , Nakao Shinji , Ohtaguro Mamiko , Chujo Tatsuya , Tsuchiya Haruo , Niki Takeo , Mori Takao , Matsuda Tamotsu , Shiobara Shintaro , Nagai Kozo 
Published: February 2007

AbstractTo characterize hematopoietic cells in mixed hematopoietic chimeras after allogeneic bone marrow transplantation (BMT), the authors examined the origin of progenies derived from hematopoietic progenitor cells of male recipients who received a marrow graft from female donors, by use of a Y‐chromosome specific DNA (YDNA) probe in combination with an in vitro colony assay. Host‐type hematopoietic cells were detected in cultured bone marrow mononuclear cells (BMMC) from 4 out of 6 patients studied, who were all in complete remission. In 2 patients of the mixed chimeras, the relative amount of host‐derived YDNA from BMMC increased after methyl cellulose cultures for 14 days. Analysis of individual colonies derived from granulocyte‐macrophage colony forming units (CFU‐GM) from these mixed chimeras, including 2 patients with chronic myelogenous leukemia (CML), revealed approximately 30% of total colonies were host‐type, although no evidence for the existence of residual Ph1 positive cells was obtained by using polymerase chain reaction for detecting bcr‐abl chimeric messenger RNA in the 2 CML patients. These findings provide direct evidence that considerable numbers of host‐derived normal hematopoietic progenitors survive and persist for a long term in a certain population of marrow recipients, after BMT following supralethal radiochemotherapy.
Continuous, low‐dose therapy with interferon‐α for human immunodeficiency virus (HIV)‐related immune thrombocytopenic purpura
Authors: Northfelt Donald W. , Kaplan Lawrence D. , Abrams Donald I. 
Published: February 2007

AbstractA patient with HIV‐related immune thrombocytopenic purpura (HIV‐ITP) had a rapid rise in platelet count when treated with interferon‐α2b, 3 million units three times weekly. There were no significant toxicities with therapy. His platelet count fell to pretreatment levels when therapy was discontinued, then increased again when therapy was reinstituted at 1.5 million units three times weekly. Interferon‐α2b, administered continuously at low doses, is well tolerated, effective, and possibly less immunosuppressive than other treatments for HIV‐ITP.
Epirubicin and cytosine arabinoside for the induction therapy of childhood acute nonlymphocytic leukemia
Authors: Liang Der‐Cherng , Jiang Chang‐Jen 
Published: February 2007

AbstractEpirubicin, a new anthracycline, was used in combination with cytosine arabinoside for the induction therapy of de novo acute nonlymphocytic leukemia in childhood. The treatment consisted of epirubicin 20 mg/m2/day for 3 days and cytosine arabinoside 100 mg/m2/day for 7 days. The treatment could be repeated every 3 weeks. Remission induction rate was 80% (20/25). Moreover, in 13 patients, the remissions were obtained after a single course. In general, the side effects of epirubicin and cytosine arabinoside were tolerable. However, the main causes of all the three deaths were infections. Our study suggests that epirubicin is acceptable and effective for the induction therapy for de novo acute nonlymphocytic leukemia in childhood.
Characterization of novel platelet and endothelial cell target antigens in a family with genetic susceptibility to autoimmunity
Authors: Hashemi Sofia , Trudel Elise , Ganz Peter R. , Drouin Jeanne , Couture Roger , Page Denis , Aye M. T. 
Published: February 2007

AbstractThis report describes a French Canadian family whose members exhibit a high incidence of allo‐ and autoantibodies to antigens present on both platelets and endothelial cells. This is correlated with various HLA specificities known to be associated with autoimmunity, such as A1, B8, DR3, and, in some cases, with clinical disorders, including nephritis, hypertension, and thrombocytopenia. Immunoblot analysis using platelet and endothelial cell lysates showed serum antibodies to a 75 kDa endothelial cell surface polypeptide and to polypeptides with apparent mass of 115 kDa and 26 kDa found on both platelets and endothelial cells. This 115 kDa internal platelet protein was also found in a variety of other cell types, such as mononuclear cells, and increased following cell activation. Monoclonal antibody immunobilization assays were used to characterize the 26 kDa polypeptide; in three of the four patients tested, an antibody to leukocyte differentiation antigen CD9 was identified. The asymptomatic child of the propositus also exhibited an autoantibody against an 80 kDa platelet protein which was sensitive to thrombin digestion, suggesting that this polypeptide may be platelet glycoprotein V. In addition, PIA1 alloantibody was identified in one sister who had given birth to a severely thrombocytopenic boy and who herself had a severe vascular rejection to a cadaver kidney 2 years prior to this study. The propositus also developed hypertensive renal disease following a pregnancy and became dialysis dependent. Thus, members of this family have developed a variety of antibodies, particularly to platelet and endothelial cell antigens. Some subjects have remained asymptomatic in spite of having autoantibodies. However, others have been seriously ill, and their immune response to these antigens is believed to have played a role in the pathogenesis of their neonatal alloimmune thrombocytopenic purpura, hypertensive renal disease, renal graft rejection, and thrombocytopenia.
The single ζ globin gene rearrangement: Hematologic phenotype and its frequent occurrence in polynesian niueans
Authors: Trent R. J. , Yakas J. , Rutherford J. , Mickleson K. N. P. 
Published: February 2007

AbstractThe single ζ globin gene rearrangement (−ζ/) is an unusual mutation associated with the α globin gene cluster on chromosome 16p13.3. It has been detected regularly among West Africans (gene frequency ˜0.05) and sporadically among other populations. DMA samples from 186 Polynesian Niueans living in Auckland, New Zealand, were studied. Eighteen (gene frequency 0.05) were shown to have the −ζ/ gene rearrangement, which results from loss of the Ψζ1 gene. Hematological phenotypes in six cord blood samples and 12 affected adults were normal. The high frequency for −ζ/ in Niueans may represent another example of genetic drift and a founder effect in Polynesians or may reflect a different founding population.
Disseminated toxoplasmosis in a severely immunodeficient patient: Demonstration of cysts in bone marrow smears
Authors: Soulier‐Lauper Mirella , Zulian Gilbert , Pizzolato Gianpaolo , Cox Jeremy , Helg Claudine , Beris Photis 
Published: February 2007

AbstractA 30‐year‐old woman was treated with unrelated HLA‐compatible bone marrow transplantation for acute myeloid leukemia. Examination of her bone marrow smears because of fever and pancytopenia revealed the presence of Toxoplasma cysts. Although Toxoplasma cysts are rarely found in the bone marrow, bone marrow examination in the immunocompromised patient offers rapid diagnosis of systemic toxoplasmosis.
Molecular characterization of hemoglobin C in sicily
Authors: Travi M. , Cremonesi L. , Primignani P. , di Benedetto S. , Testa R. , Schilirò G. , Ferrari M. 
Published: February 2007

AbstractAnalysis of polymorphisms of the β‐globin gene cluster was performed on 12 families and on one unrelated individual of Sicilian origin who carried hemoglobin C (Hb C). Two different haplotypes were found in association with βc Sicilian alleles, corresponding to haplotypes I and II previously described in American blacks. In our population, the more frequent one (haplotype I) was linked to the lack of a polymorphic Hpal site 3′ to the β gene (13.0‐kb fragment), similarly to haplotype I in blacks, while the less frequent one was linked to a 7.0‐kb Hpal fragment attributable to a site that had never been previously described in linkage with βc alleles. In Italy, these two haplotypes have been found in rare cases in association with βA alleles. These findings provide new insights into the origin of Hb C present in Sicily, suggesting that (1) the βc mutation detected in Sicily derived from African black chromosomes and does not represent a new mutation; and (2) Hb C may have originated either by multiple mutational events on separate chromosomes or by mutation in the Hpal site 3′ to the β gene in a pre‐existing βc choromosome.
Deficiency of P62, a putative collagen receptor, in platelets from a patient with defective collagen‐induced platelet aggregation
Authors: Ryo Ryukichi , Yoshida Akinori , Sugano Wataru , Yasunaga Mutsumi , Nakayama Katsuji , Saigo Katsuyasu , Adachi Masashi , Yamaguchi Nobuo , Okuma Minoru 
Published: February 2007

AbstractRecently, we described a platelet antibody against a putative collagen receptor (P62), which was found in a patient with idiopathic thrombocytopenic purpura (ITP) (Blood 69:1712). We now report a deficiency of the P62 receptor in a young man whose platelets showed defective collagen‐induced platelet aggregation. He had a mild bleeding tendency and slight thrombocytopenia. The results of coagulation and fibrinolysis studies were normal. The patient's platelets were partially unresponsive to collagen, although aggregation in response to ADP, thrombin, ristocetin, and calcium ionophore (A23187) was almost normal. Adhesion of his platelets to bovine collagen was markedly reduced. Addition of collagen caused no synthesis of thromboxane (TX)B2, in platelet rich plasma (PRP) from this patient. Furthermore, collagen produced no rise of cytosolic free calcium ([Ca2+]i) in fura2‐loaded platelets. In contrast, thrombin caused TXB2, formation and an increase of [Ca2+]i in his platelets. These results suggest defective interaction between the platelets and collagen. The lgG from the ITP‐patient induced irreversible aggregation in normal PRP, but caused no aggregation of the young man's platelets. Immunoblot studies showed that normal platelets had antigens with a molecular weight of 62 KDa under reducing conditions and of 57 KDa under nonreducing conditions. In contrast, the young man's platelets had no P62 band, although GPla/lla and thrombospondin were normally present. These results indicate that impaired collagen‐induced aggregation in the patient's platelets was due to a deficiency of P62 and confirm that P62 may play a crucial role as a collagen receptor in platelet activation.
Low‐dose cytosine arabinoside for treating hypocellular acute leukemia in the elderly
Authors: Kanamori Heiwa , Maruta Atsuo , Miyashita Hiroko , Harano Hiroshi , Fukawa Hitoshi , Matsuzaki Michio , Motomura Shigeki , Mohri Hiroshi , Kodama Fumio , Okubo Takao 
Published: February 2007

AbstractTen previously untreated elderly patients with hypocellular acute leukemia received a low dose of cytosine arabinoside (Ara‐C), 10 mg/m2 injected subcutaneously every 12 hours for 14‐28 days. Six patients achieved a complete remission (CR) for periods ranging for 6‐23 months (median 8.5 months) and the others had a partial remission (PR). Relapse has occurred in three patients to date. The median survival ranged from 10‐24 months (median 14.5 months). Only two of the six patients, in whom bone marrow biopsy was performed at CR, had a normal cellularity. This change, however, did not appear to be a significant prognostic factor in those patients. In seven patients who achieved a CR or PR low‐dose Ara‐C was administrated as maintenance therapy at the same low dose for 10 days each month. Treatment was well tolerated in all patients despite for myelosuppression. There were no drug‐related deaths. These observations suggest that low‐dose Ara‐C is effective in treating elderly patients with hypocellular acute leukemia.
Low‐dose α‐interferon treatment of chronic myeloid leukemia
Authors: Sanchez Angel , Robinson William A. , Cohn Allen , Gonzalez Rene , Adlakha Arun 
Published: February 2007

AbstractWe treated 29 patients with chronic‐phase and accelerated chronic myeloid leukemia (CML) with α‐interferon in a dose of 2 mU/m2 given subcutaneously daily for 30 days and then three times per week. Most had received prior treatment. Three patients have had sustained hematologic and karyotypic remissions after a median of 22 months of treatment. Three further patients have had complete hematologic and partial karyotypic remissions. At a median follow‐up of 48 months, the median survival has not been reached. This dose of α‐interferon may induce sustained remissions even in pretreated patients and may prolong survival in CML.
Differential induction responses of δ‐aminolevulinate synthase mRNAs during erythroid differentiation: Use of nonradioactive in situ hybridization
Authors: Mitani Kinuko , Fujita Hiroyoshi , Hayashi Norio , Yamamoto Masayuki , Sassa Shigeru 
Published: February 2007

AbstractExpression of mRNAs encoding the erythroid‐specific δ‐aminolevulinate synthase (ALAS‐E) and the nonspecific δ‐aminolevulinate synthase (ALAS‐N) were examined in murine Friend virus‐transformed erythroleukemia (MEL) cells using nonradioactive in situ hybridization. Following dimethyl sulfoxide (DMSO) treatment, ALAS‐E mRNA increased markedly, while ALAS‐N mRNA did not increase in wild‐type MEL cells. In contrast, in a DMSO‐resistant clone of MEL cells, ALAS‐E was not detectable before and after DMSO treatment. These findings suggest that ALAS‐E and ALAS‐N mRNAs are under separate controls and that the expression of ALAS‐E mRNA is a critical event in erythroid differentiation.
Cyclosporin A treatment for diamond‐blackfan anemia
Authors: Splain James , Berman Brian W. 
Published: February 2007

AbstractDiamond‐Blackfan anemia (DBA) is characterized by a variable response to corticosteroid therapy. Patients poorly responsive to acceptable doses of steroid treatment require long‐term transfusion therapy. We have treated three patients with DBA with the immunosuppressive agent cyclosporin A with limited success. Patients 1 (DS) and 2 (LS), halfsiblings, were 13 and 9 years old, respectively, and remained transfusion independent for many years on steroid therapy. Both patients manifest steroid‐associated growth failure and osteopenia, with resultant orthopedic complications. Oral cyclosporin therapy sufficient to achieve trough serum levels of 100‐200 ng/ml was associated with a brisk 50‐100% increase in hematocrit within 1 month of initiation of treatment and allowed for a gradual tapering of prednisone dose to approximately 20% of prior established maintenance dose. After 7‐8 months, both patients developed progressive decline in hematocrit level requiring increased prednisone dose and, ultimately, transfusion support. Patient 3 (RD), a 5‐year‐old child with steroid refractory, transfusion‐dependent DBA, was entirely unresponsive to cyclosporin therapy. No cyclosporin‐associated toxicity occurred. Our observations indicate that cyclosporin A can transiently ameliorate the hematologic course of some patients with DBA. Further studies are in order to determine its mechanism of action and potential clinical utility in patients unresponsive to acceptable doses of steroid.
Nuclear localization and characterization of alkaline phosphatase in neutrophils from normal controls and pregnant women
Authors: Vergnes Henri A. , Brisson‐Lougarre Andrée , Grozdea Jean G. , Blum Claude J. , Kihn Y. , Sevely J. 
Published: February 2007

AbstractThere were controversial data concerning localization of alkaline phosphatase (AP) in neutrophil nuclei under physiological conditions. In this context, the AP pattern has been determined on nuclei preparations from normal human neutrophils. Blood cells were isolated from 10 healthy adults and from 3 women in the third trimester of an uncomplicated pregnancy. Purity of nuclear suspension was checked by electron microscopy and assay of organelle marker enzymes. Electron microscope cytochemistry and immunocytochemistry studies were carried out on WBC. Enzyme characterization was performed by the usual biochemical procedures. AP was found in nuclear preparations from four of ten normal controls. When present, AP was detected in approximately two‐thirds of the nuclei examined, representing an average of 20% of the total cell activity. Conversely, a large amount of nucleus‐bound enzyme (55% of total AP activity) was recognized in all pregnant women samples. Biochemical and immunological characteristics clearly differentiate AP forms in the two groups of subjects. Normal controls have an heterogeneous enzyme pattern. AP positive preparations contain a mixture of isoenzymes: a prominent heat labile form and a relatively heat stable minor component. The heat stable fraction displays some properties similar to those previously described in leukocyte AP. Pregnant women express a unique very heat labile isoenzyme identical in its main characteristics to the early placental type. © 1992 Wiley‐Liss, Inc.
Change of thyroid function in 50 patients with aplastic anemia
Authors: Cao Qingxue , Liu Jianbin 
Published: February 2007

AbstractThe serum thyroid hormones were measured in 50 patients with aplastic anemia (AA). Serum T3 and T4 levels were significantly lower, especially in the elderly and in those in chronic AA, while they were normal in acute AA. There was no obvious difference with thyroid‐stimulating hormone (TSH). It was suggested that the result may be caused by euthyroid sick syndrome, a protective adaptation that helps in classifying and evaluating the prognosis of the disease. © 1992 Wiley‐Liss, Inc.