bluuebunny/crossref_metadata_2025_split huggingface dataset:
Investigation of plasma von Willebrand factor and circulating platelet aggregating activity in mitomycin C‐related hemolytic‐uremic syndrome
Authors: Monteagudo Juan , Pereira Arturo , Roig Susana , Reverter Juan C. , Ordinas Antonio , Castillo Ricardo 
Published: February 2007

AbstractWe have studied the ability of the plasma to induce aggregation of both homologous and heterologous platelets in four patients with hemolytic‐uremic syndrome (HUS) associated with chemotherapy with mitomycin C (MMC). Neither platelet aggregation was elicited by patients' plasmas nor the in vitro addition of purified von Willebrand factor (vWF) had any effect on the aggregation pattern. In addition, ristocetin‐induced binding of patients' vWF to formaldehyde‐fixed platelets was normal, and multimeric vWF analysis revealed a normal structure of patients' plasmatic vWF whatever the clinical stage in which it was studied. These findings suggest that, in spite of the existence of common clinical and biological features in the various forms of HUS, the pathogenesis of MMC‐related HUS may be, at least in part, different from that of the other forms of HUS in which both platelet‐aggregating activity and alterations in the vWF are found.
Treatment of severe β‐thalassemia (patients) with myleran
Authors: De‐pei Liu , Chih‐chuan Liang , Zao‐hui Ao , Pei‐chen Jia , Song‐sen Chen , Rong‐xin Wang , Li‐juan Liu , He‐qian Jin , Dan‐yu Zha , You‐wen Huang 
Published: February 2007

AbstractWe previously reported that myleran, a cell cycle nonspecific drug, can stimulate γ‐globin gene expression in anemic adult rhesus monkeys. This finding prompted us to treat two patients with severe β‐thalassemia with myleran. Both patients received an initial course of therapy, constantly of myleran at a dosage of 0.2 mg/kg/d for 9 days followed by 0.15 mg/kg/d for the next 11 days. One patient received an additional 20‐day course of myleran at a dosage of 0.2 mg/kg/d beginning 44 days after completion of the first course. No severe ill effects related to the drug were observed during or after drug administration. After 20 days of myleran treatment, levels of HbF and reticulocytes increased in both patients and level of F cells increased in patient 1. In patient 1, Hb concentration rose from 42g/L (9 days after transfusion) before treatment to a maximum of 65g/L afterward; in patient 2, it rose from 74g/L to a maximum of 106g/L. A value of 15g/L above baseline lasted for about 5 months in both patients. Hypomethylation of bone marrow DNA near the γ‐globin gene was demonstrated in patient 1.
Transient engraftment of syngeneic bone marrow after conditioning with high‐dose cyclophosphamide and thoracoabdominal irradiation in a patient with aplastic anemia
Authors: Matsue Kosei , Niki Takeo , Shiobara Shintaro , Ueda Mikio , Ohtake Shigeki , Mori Takao , Matsuda Tamotsu , Harada Mine 
Published: February 2007

AbstractWe describe the clinical course of a 16 year old girl with aplastic anemia who was treated by syngeneic bone marrow transplantation. Engraftment was not obtained by simple infusion of bone marrow without immunosuppression. The patient received a high‐dose cyclophosphamide and thoracoabdominal irradiation, followed by second marrow transplantation from the same donor. Incomplete but significant hematologic recovery was observed; however, marrow failure recurred 5 months after transplantation. Since donor and recipient pairs were genotypically identical, graft failure could not be attributed to immunological reactivity of recipient cells to donor non‐HLA antigens. This case report implies that graft failure in some cases of aplastic anemia might be mediated by inhibitory cells resistant to cyclophosphamide and irradiation.
Protein C deficiency and anticardiolipin antibodies in a family with premature stroke
Authors: Haire William D. , Newland James R. 
Published: February 2007

AbstractAnticardiolipin antibodies and, rarely, protein C deficiency have been described in patients with stroke. The familial coexistence of these two prothrombotic defects has not been previously reported. We describe a family with debilitating strokes occurring at an early age in whom both anticardiolipin antibodies and protein C deficiency were found. The propositus and her deceased mother both suffered a stroke at age 50 years. The propositus had both anticardiolipin antibodies and protein C deficiency. After a detailed study of the remainder of the family, the tendency to form anticardiolipin antibodies appeared to be inherited independently of the protein C deficiency. The simultaneous occurrence of these abnormalities in one family suggests that it may be useful to test for both anticardiolipin antibodies and inherited anticoagulant protein deficiencies in patients with unusual or unexplained stroke. The differential expression of both protein C deficiency and anticardiolipin antibody production in various members of the described family may allow insight into the relative contributions of these two prothrombotic abnormalities for the development of thrombotic complications, including stroke.
Postoperative thrombocytopenia in type IIB von Willebrand disease
Authors: Hultin Mae B. , Sussman Ira I. 
Published: February 2007

AbstractWe report studies of a large kindred with type IIb von Willebrand disease and manifestations of thrombocytopenia. While only one member of the family was thrombocytopenic routinely, three members of the family who underwent various surgical procedures demonstrated thrombocytopenia and platelet clumping postoperatively. Platelet clumps were found on peripheral blood smear only in the immediate postoperative specimens and did not appear to be a technical artifact. In the one patient who received no preoperative prophylactic therapy, postoperative plasma specimens showed the transient appearance of high molecular weight von Willebrand factor multimers. These results support the hypothesis that surgery, or some related aspect such as stress, led to the release of high molecular weight multimers, resulting in platelet clumping and removal from the circulation, and subsequent thrombocytopenia. Thrombocytopenia under conditions of stress may be a more common manifestation of type IIb vWd than is currently appreciated.
Transformation of refractory anemia with excess of blasts into acute myelogenous leukemia with Ph‐negative chronic myelogenous leukemia‐like characteristics
Authors: Yamauchi Kunihiko , Arimori Shigeru , Nagao Tadami 
Published: February 2007

AbstractWe report a 56 year old patient with acute myelogenous leukemia (FAB classification: M2), in whom the number of mature myeloid cells similar to those seen in Ph‐negative chronic myelogenous leukemia increased markedly 2 months after the diagnosis of refractory anemia with excess of blasts (RAEB). This is a rare case of leukemic evolution as a terminal event of RAEB.
B‐cell surface phenotypes of proliferating myeloma cells: Target antigens for immunotherapy
Authors: Chan Clara S. P. , Wormsley Susan B. , Pierce Lawrence E. , Peter James B. , Schechter Geraldine P. 
Published: February 2007

AbstractDual‐parameter flow cytometric analysis of B‐cell antigens and DNA content was used to determine the phenotypes of proliferating tumor cells (S‐phase cells) from 30 patients with multiple myeloma. B4 (CD19), J5 (CALLA, CD10), B1 (CD20), and monotypic surface immunoglobulin (Sig) were expressed heterogeneously in 24 patients. J5 and monotypic Slg were found most frequently but were always expressed on a significantly lower percentage of cells than the antigens typically associated with plasma cells, cytoplasmic immunoglobulin (Clg) and T10 (CD38). S‐phase cells were found in each antigen(+) subset. B antigen(+) cycling cells were demonstrated in 16 patients whose marrow or blood cells expressed B antigens exclusively in the hyperdiploid fraction and therefore were certainly part of the myeloma clone. Similar to the low level of proliferative activity of the T10(+), Clg(+), and PCA1(+) subsets, the percentages of cycling cells of the preplasma cell B‐antigen‐bearing myeloma subsets ranged from < 1% to 12%. The tumor cells of four patients were also studied with dual‐color surface antigen analysis and demonstrated independent expression of B antigens, with only rare coexpression of T10 and monotypic Slg, J5, or B4. These findings are consistent with the presence of distinct myeloma subsets bearing differing B phenotypes in the same tumor and provide evidence that the proliferation in myeloma is occurring at various developmental stages in the malignant B lineage. These antigens may be important targets for immunologic therapy aimed at eliminating the entire proliferating compartment of this B‐cell tumor.
Prognostic factors at relapse for adults with acute myeloid leukemia
Authors: Uhlman Dorothy L. , Bloomfield Clara D. , Hurd David D. , Peterson Bruce A. 
Published: February 2007

AbstractTo identify prognostic factors for patients with acute myeloid leukemia in first relapse we have analyzed the courses of 57 patients undergoing intensive reinduction therapy. The median duration of first remission was 9 months. A second complete remission was achieved by 31 patients (54%). The median duration of second remission was 5 months (range 1‐142 + months). Two patients are still disease‐free with 42 months and 142 months follow‐up. Clinical, laboratory, and treatment variables at the time of original diagnosis and at relapse were examined for possible associations with the outcome of reinduction chemotherapy. Only duration of first remission consistently correlated with ability to achieve a second remission, second remission duration, and overall survival from reinduction. The second complete remission rate was 69% for those with a first remission longer than 9 months but only 39% for those with a first remission less than 9 months (P < 0.05). The patients with longer first remissions also had longer second remissions (8 months median vs. 4 months, P = 0.02) and a longer median survival following reinduction chemotherapy (8 months vs. 5 months, P = 0.02). We conclude that the duration of a patient's first remission should be considered a useful prognostic factor both in treatment planning and in the interpretation of clinical trials of patients in relapse.
Detection of monosomy 7 in interphase cells of patients with myeloid disorders
Authors: Kolluri Rukmini V. , Manuelidis Laura , Sait Sheila , Cremer Thomas , Gezer Sefer , Raza Azra 
Published: February 2007

AbstractSix patients, five with acute myeloid leukemia (AML) and one with a myelodysplastic syndrome (MDS), were found to have monosomy 7 by conventional cytogenetics at diagnosis. Repetitive DNA sequences from the heterochromatic region of human chromosomes 1 and 7 were used as probes for in situ hybridization experiments on interphase cells of these patients. A double hybridization protocol was used to reveal the particular chromosomes as distinct spots or clusters of signals within interphase nuclei. The chromosome 1 sequence served as an internal control. Simultaneous detection of the sequences showed the presence of two normal number 1 chromosomes and a missing 7 chromosome from individual cells. While cytogenetic preparations showed only −7 metaphases in 3 AML and 1 MDS patients, in situ hybridization of interphase cells showed many normal cells as well as the presence of ‐7 in fully mature granulocytes. One AML patient studied in remission showed only normal metaphases yet had 9% interphase cells with a missing 7 and relapsed within 3 months. We conclude that examination of interphase cells by in situ hybridization provides clinically useful data since every cell including mature granulocytes can be examined, the lineage of a cell can be determined, and efficacy of differentiation therapy can be evaluated.
Spectrin modifications in a heterozygous case of both hereditary elliptocytosis and β‐thalassemia
Authors: Streichman Sara , Herz Ethy , Tatarsky Ilana 
Published: February 2007

AbstractThe clinical and hematological parameters of a patient described here, who inherited the genes of both hereditary elliptocytosis (HE) and β‐thalassemia, seem to reflect a mutual enhancement of the two diseases. The coexistence of the two pathologies is probably also responsible for the observed changes in spectrin: the appearance of an extra spectrin band between tetramers and dimers on denaturing gel electrophoresis and the metabolic‐dependent reduction in spectrin amount. It is assumed that the instability of the skeletal network that results from the HE pathology caused increased exposure of the spectrin molecule to oxidative damage that usually occurs in thalassernic red cells. The products of such oxidation may have led to abnormal spectrin associations which finally resulted in the above changes.
Immunogenetic analysis of bone marrow aspirates in patients with non‐hodgkin lymphomas
Authors: Ellison Douglas J. , Hu Eddie , Zovich Dan , Pinter‐Brown Lauren , Pattengale Paul K. 
Published: February 2007

AbstractThe immunogenetic analysis (IGA) on the staging bone marrow aspirates in 15 patients with non‐Hodgkin lymphoma (NHL) is reported. We found the sensitivity of IGA and morphologic examination in detecting bone marrow involvement by malignant lymphoma to be 91% and 82%, respectively. In 11 cases there was agreement between the morphologic findings and IGA. In 8 of these 11 cases, IGA confirmed the morphologic involvement of the bone marrow by demonstrating clonal rearrangement of either the immunoglobulin heavy‐ and/or light‐chain or the T‐cell receptor beta chain (TCR) genes. In 3 of these 11 cases, morphology showed no involvement and IGA showed germline configurations for both the immunoglobulin heavy‐ and light‐chain or the TCR genes. In 2 additional cases the techniques proved to be complementary, as involvement was detected by only 1 of the 2 procedures. In 1 of these 2 cases, IGA showed gene rearrangement while morphologic examination was negative for involvement by NHL, by NHL while in the other case, morphologic examination showed involvement by NHL, but IGA did not show gene rearrangement. IGA was also useful in determining the clonality of solitary lymphoid nodules in the 2 remaining cases when morphologic interpretation was equivocal. In the 12 cases with bone marrow involvement, the immunophenotype and immunogenotype agreed in 11 cases. In the one case in which there was a discordance between the immunophenotype and immunogenotype, the immunophenotype was incorrectly interpreted as B‐cell lineage, while the immunogenotype demonstrated a T‐cell lineage. IGA also demonstrated a clonal population in 1 case of T‐chronic lymphocytic leukemia where other techniques could not demonstrate the clonality of the pathologic process. IGA analysis may detect bone marrow involvement in NHL which may not be detected by morphologic examination because of patchy distribution.
Synergy of inhibition of DNA synthesis in human bone marrow by azidothymidine plus deficiency of folate and/or vitamin B12?
Authors: Herzlich Barry C. , Ranginwala Mohammad , Nawabi Ismat , Herbert Victor 
Published: February 2007

AbstractThe effect of azidothymidine (Zidovudine, AZT) on pyrimidine (thymidine, deoxyuridine, and thymidine triphosphate) incorporation into DNA in folate‐ and/or vitamin B12‐deficient and normal human bone marrow cells was studied to Investigate whether such vitamin deficiency affects susceptibility to AZT‐induced hematologic toxicity. Bone marrow cells from 12 patients were studied: 5 had folate and/or vitamin B12 deficiency; 7 controls included 5 with anemia related to chronic disease and 2 with iron deficiency.At 0.2 μM AZT (3 hr, 37°C), the approximate pharmacologic serum trough level, pyrimidine incorporation into DNA was suppressed by 12 to 19% in folate‐ and/or vitamin B12‐deficient cells and by 16 to 23% in normal cells. At 2.0 μM AZT (3 hr, 37°C), the approximate pharmacologic serum peak level, this was suppressed by 15 to 40% in folate‐ and/or vitamin B12‐deficient cells and by 32 to 47% in controls. Deoxyuridine incorporation into DNA was inhibited signlficantly greater than thymidine at 2.0 μM AZT (3 hr, 37°C) in both groups. Inhibition of deoxyuridine incorporation was not reversed with methyltetrahydrofolate or vitamin B12. There tended to be less striking suppression by AZT of deoxyuridine incorporation into DNA in bone marrow cells from vitamin B12‐deficient patients, which was made more striking by adding vitamin B12. This suggests that some of what passes for “AZT damage” to bone marrow cells may in fact be coincident deficiency of vitamin B12.AZT Inhibition of DNA synthesis in 3 hr bone marrow cultures is relatively consistent in a varlety of hematologic disorders. As approximately two‐thirds of AIDS patients appear to be in negative balance with respect to folate and/or vitamin B12, the fact that AZT‐induced inhibition of pyrimidine incorporation into DNA is occurring in cells which may be megaloblastic, i. e., In a state of Impaired DNA synthesis, suggests that these cells may be more susceptible to AZT toxicity. The data also support the notion that AZT inhibition results predominantly from termination of DNA chain elongation.Whether folate or vitamin B12 supplementation may partially overcome apparent “AZT inhibition” of DNA synthesis (hematologic toxicity) and whether the benefit of such therapy exceeds the risk will require further study.
Effect of adherent cells on the regulation of BFU‐E in patients with myeloproliferative disease
Authors: Shabbad Evelyne , Cassel Aliza , Froom Paul , Aghai Esther 
Published: February 2007

AbstractThe interaction between adherent cells and red cell progenitors from peripheral blood of patients with polycythemia vera (PV), essential thrombocytosis (ET), and healthy controls was studied. Various combinations of adherent and nonadherent cells were co‐cultured in a semisolid system. Adherent cells from controls, when added at low concentrations, stimulated BFU‐E proliferation, whereas high concentrations (40% of total cells in the culture) caused a significant decrease in the number of BFU‐E colonies in 6/8 PV patients, 4/4 ET patients, and 8/12 controls. On the other hand, low and high concentrations of adherent cells from both patients with PV and ET caused a significant increase in BFU‐E from either patients or controls. Moreover, adherent cells from these patients induced endogenous BFU‐E proliferation (independent of erythropoietin) in nonadherent cells of 12/12 normal controls.The results show that BFU‐E from patients with PV and ET are sensitive to suppression by normal adherent cells. On the other hand, adherent cells from these patients possess stimulatory activity on BFU‐E from peripheral blood at all concentrations and are devoid of the inhibitory activity. This suggests a possible defect in the functioning of adherent cells in PV and ET patients which may contribute to the abnormal regulation of hematopoiesis in these disorders.
Hematological abnormalities in hemophilic patients with human immunodeficiency virus infection
Authors: Bello Jose L. , Burgaleta Carmen , Magallon Manuel , Herruzo Rafael , Villar Jose M. 
Published: February 2007

AbstractHematological abnormalities are common in patients with AIDS or AIDS‐related complex. We studied cytological characteristics in peripheral blood and bone marrow samples of 33 hemophilic patients with HIV Infection and in six HIV negatives. The HIV‐positive patients presented leukopenia (60.6%), thrombocytopenia (69.9%), and anemia (57.5%). Bone marrow showed abnormalities of maturation in one or more cell lines similar to those described in other HIV‐infected groups of patients. These findings were more prominent in megacaryocytes and granulocytic series. Lymphocytosis, plasmocytosis, and increased hemophagocytosis were also common. These alterations do not appear in HIV‐negative patients and seem related to a direct effect of HIV on bone marrow cells or to alterations in T‐cell regulatory functions.
Platelet peroxidase‐like activity detected in mature erythroblasts from a patient with pernicious anemia
Authors: Kawaguchi Masato , Nakano Masaru , Enomoto Yasuhiro , Watanabe Younosuke , Toyama Keisuke 
Published: February 2007

AbstractWe report a case of pernicious anemia in which the possession of platelet peroxidase (PPO)‐like activity was proved in its erythroblasts. PPO‐positive cells were found in a distinct minor population of hemoglobinized (as a mean of differentiated) erythroid cells in the bone marrow before treatment, but these cells disappeared following therapy with vitamin B12. The present report is considered to be the first case to identify PPO‐like activity in mature erythroblasts from the nonmalignant clone. The existence of PPO in erythroid lineage in cases with pernicious anemia is discussed.
Familial macrothrombocytopenia associated with decreased glycosylation of platelet membrane glycoprotein IV
Authors: Yufu Yuji , Ideguchi Hiroshi , Narishige Takahiro , Suematsu Eiichi , Toyoda Kazunori , Nishimura Junji , Nawata Hajime , Oda Susumu 
Published: February 2007

AbstractA case of hereditary thrombocytopenia with large platelets (familial macrothrombocytopenia, FM) is reported. Studies on the platelets from the propositus showed decreased glycosylation of platelet membrane glycoprotein IV, which would distinguish the case from other FM previously described.
Effects of 2′‐deoxycoformycin on hl‐60 cell differentiation, adenosine deaminase, and oligoadenylate synthetase
Authors: Triozzi Pierre L. , Vicstein Frederick 
Published: February 2007

AbstractThere is evidence that 2′‐deoxycoformycin (DCF) functions as a differentiating agent. We examined the effects of DCF on the differentiation of HL‐60 cells, a standard model of leukemic cell differentiation. DCF did not induce morphologic or histochemical evidence of differentiation, nor did it interact with retinoic acid, 1,25‐dihydroxyvitamin D3, or interferon‐gamma in inducing differentiation. Furthermore, DCF and agents that differentiated HL‐60 cells had opposing effects on adenosine deaminase and 2′, 5′‐oligoadenylate synthetase activity.
Pulmonary embolism and splenic infarction in a patient with sickle cell trait
Authors: Sugarman Jeremy , Samuelson Wayne M. , Wilkinson Robert H. , Rosse Wendell F. 
Published: February 2007

AbstractA 43 year‐old black man with sickle cell trait documented by hemoglobin electrophoresis presented with severe pleuritic chest pain and hypoxemia three weeks after discharge following abdominal surgery. A pulmonary embolus was diagnosed by angiography and he was treated with heparin; the minimum arterial pO2 was 55 torr while O2 was being administered at a rate of 3 L/min. During this therapy, he developed abdominal pain. Computerized tomography suggested splenic infarction, which was documented by radionuclide liver‐spleen scan and magnetic resonance imaging (MRI); the patient's spleen had been normal at exploratory laparotomy three weeks previously. No source for emboli was identified in the deep venous system by MRI.Although splenic infarction has been reported in patients with sickle cell trait at high altitude, this is the first reported case of splenic infarction secondary to the hypoxemia of pulmonary embolism in a patient with sickle cell trait. The spleen is subject to infarction in sickle cell trait because blood flow is slow through a hypoxemic and acidemic environment. The additional hypoxemia due to pulmonary embolism is presumed, in our patient, to have created a local splenic environment which permitted infarction to occur.