bluuebunny/crossref_metadata_2025_split huggingface dataset:
Vincristine therapy for thrombotic thrombocytopenic purpura
Authors: O'connor N. T. J. , Bruce‐Jones P. , Hill L. F. 
Published: February 2007

AbstractThrombotic thrombocytopenic purpura (TTP) is a rare disorder characterized by micro‐ angiopathic haemolytic anemia, consumption thrombocytopenia, renal impairment, neu‐ rological dysfunction, and fever, but all these features are not present in every patient. Although the prognosis of TTP has improved since it was demonstrated that large volumes of fresh frozen plasma (FFP) lead to a remission in most patients, the disease remains fatal in many instances. Therefore, other therapies are often used in TTP, but there is skepticism about their value, since such treatments are often used in combination, making evaluation difficult. This report describes three patients with TTP and a consistent therapeutic response occurring 5 days after vincristine in each of four instances.
Epidermal growth factor binding sites on human erythrocytes in donors with different ABO blood groups
Authors: Engelmann Bernd , Schumacher Udo , Haen Ekkehard 
Published: February 2007

AbstractAnalysis of epidermal growth factor (125J‐EGF) binding to human red cells revealed the presence of two classes of binding sites with apparent equilibrium dissociation constants (app.Kd) in the 10−10−10−9 M and in the 10−8 M range, respectively. The number of binding sites/cell ranged between 600 and 2,400 for the high‐affinity binding site and between 7,200 and 23,000 for the low‐affinity site. No differences were seen in the apparent Kd values for both types of binding sites between red cells obtained from donors with different ABO‐blood groups. An increase in the number of high affinity EGF binding sites was observed in donors with blood group A,‐erythrocytes as compared to red cells taken from donors with blood groups o and B.
Effects of interleukin‐5 on acute myeloid leukemias
Authors: Baumann Michael A. , Paul Cassandra C. , Grace Michael J. 
Published: February 2007

AbstractBecause of the known activity of interleukin‐5 (IL‐5) as a growth factor for human eosinophils, we performed studies to ascertain whether M4Eo acute leukemia cells might be capable of responding to IL‐5. Surprisingly, short term incubation of freshly isolated M4Eo blasts with rhIL‐5 induced differentiation of the cells to macrophages. To determine whether other variants of acute myelogenous leukemia (AML) might also be capable of responding to IL‐5, we studied cells from four additional unselected cases and found that one responded to IL‐5 by vigorous proliferation. Using biotinylated rhIL‐5, second labelling with streptavidin‐fluorescein isothiocyanate (FITC) and flow cytometric analysis, specific binding of IL‐5 to both responsive leukemias was demonstrated, suggesting the presence of specific IL‐5 receptors. Additional study will be needed to assess the frequency of IL‐5 responsiveness in AML and to determine the relationship of these phenomena to the physiologic role of IL‐5 in the regulation of hematopoiesis.
Marrow transplantation for paroxysmal nocturnal hemoglobinuria
Authors: Kawahara K. , Witherspoon R. P. , Storb R. 
Published: February 2007

AbstractBetween 1971 and 1990, nine patients ranging in age from 14‐38 years received marrow transplants for paroxysmal nocturnal hemoglobinuria (PNH). Six were transplanted for aplastic complications of PNH. Four of these were from HLA‐identical siblings, and the patients were conditioned with cyclophosphamide. One graft was from a syngeneic twin without conditioning, and one from a two HLA‐antigen nonidentical father after conditioning with cyclophosphamide and total body irradiation. Three of the four recipients of allogeneic marrow developed acute and two chronic graft‐versus‐host disease (GVHD). Five of six transplanted for severe aplastic anemia are long‐term survivors with follow‐up ranging from more than 6.2 to more than 19.1 years. The HLA nonidentical transplant recipient experienced graft rejection and died of a pulmonary hemorrhage. Three patients were transplanted for nonaplastic complications of PNH consisting of life threatening recurrent thromboses or refractory hemolysis. Two of these patients received marrow grafts from HLA‐identical siblings after conditioning with busulfan and cyclophosphamide. They are surviving with normal hemograms >2.2 and >2.5 years and had mild chronic GVHD which resolved, although one has biochemical evidence of PNH in 15% of the red cells. One received a syngeneic marrow graft without conditioning but reverted to PNH. He is alive >8.6 years after transplantation. Marrow transplantation for aplastic complications of PNH is successful, well tolerated, and compatible with long‐term survival when an HLA‐identical sibling or a syngeneic donor is available. For patients without aplasia, one must weigh the complications of transplantation with the life threatening nature of thrombotic episodes and hemolysis. © 1992 Wiley‐Liss, Inc.
A new gene deletion involving the α2‐, α1 ‐, and 81‐globin genes in a black family with Hb H disease
Authors: Fei Y. J. , Liu J. C. , Walker E. L. D. , Huisman T. H. J. 
Published: February 2007

AbstractA new deletion of ∽ 8.5 kb that includes the α2‐, α1‐, and θ1‐globin genes was detected in a father and newborn son of a black family from Georgia. In the newborn baby the chromosome with the deletion occurred together with a rightward (‐α3.7) deletion chromosome. The resulting Hb H disease was detected at birth by the high level of Hb Bart's; a moderate anemia with severe microcytosis and hypochromia was present at the age of 5 months. A review of cord blood testing results for a period of over 20 years, involving at least 200,000 black newborns, indicated that this baby was the first with a level of Hb Bart's in excess of 10‐15%; this underscores the rarity of α‐thalassemia‐1 in this population. © 1992 Wiley‐Liss, Inc.
Delivery of infants with Glanzmann thrombasthenia and subsequent blood transfusion requirements: A follow‐up of 39 patients
Authors: Awidi Abdalla S. 
Published: February 2007

AbstractThe delivery and transfusion requirements of 39 patients with Glanzmann thrombasthenia are described. Of these, type I thrombasthenia was found in 21 females and 12 males, and type II thrombasthenia was found in four females and two males. Eighteen of these patients were born in the hospital and 21 at home. All were delivered vaginally. Absence of excessive hemorrhagic symptoms was noted in neonates affected with thrombasthenia. The patients were followed for a total of 220 patient years and received a total of 276 units of blood during this period. In females, the dominant reason for transfusion was menorrhagia followed by gum bleeding and epistaxis, while in males epistaxis, gum bleeding, and circumcision accounted for most of the transfusions. © 1992 Wiley‐Liss, Inc.
Total sialic acid in human and canine platelets does not change with the platelet age
Authors: Mezzano Diego , Aranda Eduardo , García María Eugenia , Pereira Jaime , Quiroga Teresa , Pérez Marcos 
Published: February 2007

AbstractTotal platelet sialic acid (SA) was measured in three experimental conditions: (1) human and canine platelet density subpoputations obtained by centrifugation in arabinogalactan gradients, (2) circulating canine platelets during recovery from experimental immune and mechanical thrombocytopenias, and (3) platelets obtained from a patient with chronic immune thrombocytopenic purpura before and after splenectomy. The density of human and canine platelets is, in part, determined by their age. We found no significant differences in total SA between high‐density (HD) and low‐density (LD) platelets (9.32 ± 2.0 vs. 9.55 ± 1.3 μg/mg of platelet protein in dogs and 9.02 ± 2.3 vs. 9.10 ± 2.9 ng/mg in humans). In the human and canine thrombocytopenic models, the entrance of new platelets from the bone marrow is followed by their aging in the circulation. In these models, no significant changes in total SA content were detected in sequential measurements during the recovery of the thrombocytopenia. Accordingly, we conclude that total SA in human and canine platelets is unrelated to their age in circulation. These results do not support the notion that the loss of SA from membrane glycoproteins determines the recognition and removal of platelets from the circulation. © 1992 Wiley‐Liss, Inc.
Pulmonary leukostasis without hyperleukocytosis: A clinicopathologic study of 16 cases
Authors: Soares Fernando Augusto , Landell Glaucia A. Magnani , Cardoso Maria Camila de Miranda 
Published: February 2007

AbstractPulmonary leukostasis is a serious, almost always fatal, complication usually reported to occur in patients with acute granulocy tic leukemias when the peripheral white blood cells exceed 50,000/mm3. We report a Clinicopathologic study of 16 leukemic patients with pulmonary leukostasis and with < 50,000/mm3 circulating leukocytes. The results demonstrated that hyperleukocytosis per se cannot be the cause of pulmonary leukostasis, but that other factors, such as the presence of circulating blasts and the affinity of neoplastic cells for the pulmonary endothelium, may be related to the development of acute respiratory distress in patients with leukemia. © 1992 Wiley‐Liss, Inc.
Bone marrow necrosis in lymphoma studied by magnetic resonance imaging
Authors: Weissman David E. , Negendank William G. , Al‐Katib Ayad M. , Smith Mitchell R. 
Published: February 2007

AbstractWe used magnetic resonance imaging (MRI) to examine the extent of necrosis in the marrow space in three patients with iymphoma who had necrosis in bone marrow biopsies taken from the posterior iliac crest. Two of the patients had multiple focal lesions observed in the spine, pelvis, and femurs. These lesions had MRI characteristics of fluid‐filled necrosis within marrow or Iymphoma. The third patient exhibited a unique pattern of anatomically extensive marginated lesions otherwise typical of avascular necrosis of bone. These cases provide evidence that MRI can document the extent of necrosis within the marrow space and can differentiate two distinct patterns of necrosis. © 1992 Wiley‐Liss, Inc.
Relapsing polychondritis: A paraneoplastic syndrome associated with myelodysplastic syndromes
Authors: Van Besien Koen , Tricot Guido , Hoffman Ronald 
Published: February 2007

AbstractWe report an unusual case of a patient with a myelodysplastic syndrome associated with life threatening relapsing polychondritis. The improvement in symptoms attributable to the relapsing polychondritis during treatment of the hematologic disorder suggests that relapsing polychondritis is a paraneoptastic syndrome associated with myelodysplastic syndromes. © 1992 Wiley‐Liss, Inc.
Successful treatment of T‐γ lymphoproliferative disease with human‐recombinant granulocyte colony stimulating factor
Authors: Lang David F. , Rosenfeld C. S. , Diamond H. S. , Shadduck R. K. , Zeigler Z. R. 
Published: February 2007

AbstractA trial of recombinant human granulocyte colony‐stimulating factor (rhG‐CSF) was attempted in a male with agranulocytosis, infection, and T‐gamma lymphoproliferative disease (T‐γ‐LPD). During five days of rhG‐CSF (960 μg/day), the absolute neutrophil count (ANC) increased from 0.0 to 4.5 K/μI. There were no changes in eosinophil or lymphocyte counts. In addition, there was no toxicity. Bone marrow cytotoxic/suppressor cells (CD57+/CD8+) were elevated (21.9%) before and decreased to 10.6% (normal < 12%) following rhG‐CSF. By contrast, there was no change in activated T cells (CD3+DR+) or T cell gene rearrangements. These findings suggest rhG‐CSF can improve granulopoiesis in T‐γ‐LPD, possibly by altering T‐cell mediated marrow suppression. © 1992 Wiley‐Liss, Inc.
Clinical relevance of immunological dissection in T‐ALL: A report on 20 cases with stem cell (CD7+, CD4‐, CD8‐, CD1‐) phenotype
Authors: Ferrara Felicetto , Cimino Renato , Antinolfi Iolanda , de Rosa Carlo , Mele Giuseppina , Mettivier Vincenzo , Fusco Caterina , Vecchio Luigi Del 
Published: February 2007

AbstractIn a prospective study on 44 cases of T‐cell origin acute lymphoblastic leukemia, 20 patients were found to display an immature immunophenotype (CD7+, CD4‐, CD8‐, CD1‐) and were classified as T‐stem cell leukemia (T‐SCL). Twenty‐four patients expressed CD4 and/or CD8 antigens on their blast cells, designated T acute lymphoblastic leukemia (T‐ALL). The T‐SCL subset showed a significantly higher median age, a more frequent incidence of extramedullary leukemia, a morphology L1 in most cases, and a poor response to treatment in terms of either complete remission rate or median survival duration. In addition, significant differences between the two groups were found in evaluating the number of days of blast disappearance from peripheral blood, of CR achievement, and of neutrophils and platelets recovery. We conclude that T‐SCL represents a distinct clinical entity, characterized by a poor response to ALL conventional chemotherapy. Alternative therapeutic approaches should be developed for patients suffering from this form of leukemia, to modify its severe prognosis. © 1992 Wiley‐Liss, Inc.
Prevention of carryover contamination in the detection of βS and βC genes by polymerase chain reaction
Authors: Wang Xiuhua , Chen Timothy , Kim David , Piomelli Sergio 
Published: February 2007

AbstractAs the polymerase chain reaction (PCR) process becomes a common tool in genetic diagnostic laboratories, prevention of carryover contamination from previous PCR amplifications has become an urgent topic. A PCR carryover prevention technique, utilizing deoxyuridine triphosphate (dUTP) and uracil DNA glycosylase (UDG), has been described recently. We report on its adaptation to a diagnostic system for detecting hemoglobin SS and SC diseases. Excellent amplification was achieved by increasing the dUTP and MgCl2 concentrations. dU‐containing DNA could be analyzed by restriction endonucleases to distinguish the βS from βA gene using Ddel, but two other restriction enzymes, Bsu36l (replacement for Mstll by the manufacturer) and Cvnl, can no longer be used. The dU‐containing PCR products retained their hybridization specificity with allele‐specific oligonucleotide (ASO) probes for the βA, βS, and βC genes. The PCR carryover prevention technique is easy to use and takes only 20 additional minutes. It should be extremely useful to genetic diagnostic laboratories where PCR is repeated daily and carryover contamination may thus lead to misdiagnoses. © 1992 Wiley‐Liss, Inc.
Clonality of cold agglutinins in patients with hemolytic anemia: An analysis by high‐resolution two‐dimensional gel electrophoresis
Authors: Tissot Jean‐Daniel , Clément François , Schifferli Jüirg A. , Frei Philippe C. , Hochstrasser Denis F. , Schneider Philippe 
Published: February 2007

AbstractHigh‐resolution two‐dimensional gel electrophoresis (2‐DGE) was used to analyse plasma samples and partially purified cold agglutinins (CA) obtained from two selected patients. Both presented an acute hemolytic anemia with CA of high thermal amplitude, normal immunoglobulin levels, no detectable paraproteinemia, and no clinical evidence of a malignant B‐cell disorder. The electrophoretograms of their plasma showed evident alterations of the “normal” protein profile, which were directly related to hemolysis (absence of the spots of haptoglobin and in one case of those of hemopexin), but no monoclonal gammopathy. The electrophoretograms of their purified CA revealed two clearly different spot patterns respectively corresponding to a monoclonal IgM and to polyclonal IgM. These results show that the clonality of CA associated with hemolytic anemia can be easily determined by 2‐DGE. This technique may be very useful to discriminate chronic cold agglutinin disease in the early phase from “parainfectious” CA. © 1992 Wiley‐Liss, Inc.
Coincidental finding of May‐Hegglin anomaly in a patient with end‐stage renal failure
Authors: Nel N. , van Rensburg B. W. J , Plessis Lizette Du , Potgieter C. D. , Stevens K. 
Published: February 2007

AbstractWe present two cases of May‐Hegglin anomaly incidentally discovered in a patient and his brother during investigation of the patient for end‐stage renal failure and workup for renal transplantation. Routine laboratory tests were performed and included a basically normal clotting profile. Ultrastructural studies of the May‐Hegglin inclusions proved diagnostic, findings were compared with those of two similar granulocyte inclusion bodies, and nomenclature discrepancies that still exist in most references are again emphasized. The finding of the May‐Hegglin anomaly in our patient appears to be incidental to the underlying renal disease. A successful renal transplant has been carried out in this patient.We now report on a patient and his brother in which the MHA was discovered during workup of the patient for end‐stage renal failure and renal transplantation. No association between the underlying renal disease and the MHA could be demonstrated. © 1992 Wiley‐Liss, Inc.
Ifosfamide in plasma cell leukemia: A report of two cases and review of the literature
Authors: Malhotra Hemant , Dhabhar Boman N. , Saikia Tapan K. , Gopal Ramkrishnan , Nadkarni Kanchan S. , Nair Chandrika N. , Advani Suresh H. 
Published: February 2007

AbstractWe report two patients with primary plasma cell leukemia (PCL) treated with a single agent, ifosfamide. One patient had a total disappearance of plasma cells (PC) from the peripheral blood and the bone marrow and disappearance of the myeloma protein, is disease free 8 months after completion of treatment, and alive 14 months after diagnosis. The second patient had a partial response with persistence of plasma cells in the bone marrow lasting 7 months, after which she had a frank relapse of the disease. We suggest that ifosfamide may be an active agent in plasma cell malignancies and needs further evaluation in multiple myeloma. © 1992 Wiley‐Liss, Inc.
Secondary T‐cell lymphoma in the setting of nodular lymphocyte predominance Hodgkin's disease
Authors: Tefferi A. , Wiltsie J. C. , Kurtin P. J. 
Published: February 2007

AbstractLymphocyte predominance Hodgkin's disease (LPHD) can be histogically subdivided into a nodular and diffuse variety. The two subtypes differ in immunophenotypic characteristics but have a similar long‐term clinical outcome. Nodular LPHD has immunophenotypic and histological characteristics suggestive of a B‐cell derived neoplastic process. Nodular LPHD is associated with an increased risk of secondary large cell lymphoma (LCL). Gene rearrangement studies in some of these cases have revealed a B‐cell clonal process, further supporting the association between nodular LPHD and the B‐cell system. In addition, it has been suggested that the apparent secondary LCL, at least in some cases, may represent a histologic progression of nodular LPHD. We report a unique case of T‐cell lymphoma, confirmed by T‐cell receptor gene rearrangement studies, which developed in the setting of nodular LPHD. Our observation demonstrates that the association of nodular LPHD and LCL is complex and that LCL developing in the context of nodular LPHD may be an independent secondary process sometimes involving T‐cell lymphomas. © 1992 Wiley‐Liss, Inc.
Cyclosporine and prednisone therapy for pure red cell aplasia in patients with chronic lymphocytic leukemia
Authors: Chikkappa G. , Pasquale D. , Zarrabi M. H. , Weiler R. J. , Divakara M. , Tsan M. F. 
Published: February 2007

AbstractWe describe the characteristics of response to treatment with cyclosporine (CYA) plus prednisone in seven episodes of pure red cell aplasia (PRCA) in four patients with B cell chronic lymphocytic leukemia (CLL). Fourteen episodes of PRCA occurred in four patients with CLL. Eleven episodes were treated with conventional therapies which included an alkylating agent and prednisone. Four episodes that failed to respond to conventional therapies and an additional three episodes were treated with CYA and prednisone. Six of the seven episodes, including three of four which had failed conventional therapies, responded to CYA plus prednisone compared with six of eleven episodes treated with conventional therapies. Response to CYA and prednisone occurred without a reduction in leukemic mass. In contrast, PRCA remission did not occur until after leukemic mass reduction in three of four patients treated successfully with conventional therapies. Time to response was shorter (14 ± 3 days) with CYA plus prednisone than with conventional therapies (154 ± 97 days) in three of four patients. These results indicate that CYA plus prednisone is an effective therapy for the induction of remission from PRCA in patients with CLL. © 1992 Wiley‐Liss, Inc.
Epidemiology of AIDS in females with hemophilia and other chronic bleeding disorders in the united states: Comparisons with males with chronic bleeding disorders and AIDS and with nonhemophilic female blood‐transfusion recipients with AIDS
Authors: Holman Robert C. , Clarke Matthew J. , Evatt Bruce L. , Chorba Terence L. 
Published: February 2007

AbstractFrom January 1, 1981 through June 30, 1990, 32 females with chronic bleeding disorders were diagnosed with acquired immunodeficiency syndrome (AIDS) in the United States. Most (81.3%) were white and ≧30 years of age, with a median age of 37.5 years. Eighteen (56.3%) had von Willebrand's disease. Pneumocystis carinii pneumonia was reported for 16 (50%). None had Kaposi sarcoma. The median survival time was 10.8 months, with a cumulative probability of survival at 1 year of 47.3% and at 2 years of 27.6%. We compared the demographic data and survival times of these females with those of males with a chronic bleeding disorder and AIDS, and with those of nonhemophilic females with AIDS whose exposure to the human immunodeficiency virus (HIV) was through receipt of blood transfusions, blood components, or tissue. The principal demographic difference was age distribution. The females with chronic bleeding disorders tended to be younger than the transfused, nonhemophilic females, but older than the males. The survival time from AIDS diagnosis to death for the females with chronic bleeding disorders did not differ statistically from that of the other two groups, although older nonhemophilic females whose exposure was transfusion may progress more rapidly to AIDS. © 1992 Wiley‐Liss, Inc.
Recombinant interferon‐α2A as maintenance treatment for patients with advanced stage chronic lymphocytic leukemia responding to chemotherapy
Authors: Ferrara Felicetto , Rametta Vincenzo , Mele Giuseppina , Antinolfi Iolanda , Mettivier Vincenzo , Cimino Renato 
Published: February 2007

AbstractForty‐five patients suffering from advanced B‐CLL were randomized to receive interferon‐α (IFNα) or no treatment after achieving complete remission or partial response, following a chemotherapy protocol called MINa. The two groups were fully comparable in terms of clinical characteristics and level of response obtained by chemotherapy. IFNα was given at a dose of 3 megaunits three times a week intramuscularly for 1 year. The IFN‐treated patient group showed a significantly longer duration of response and a less frequent incidence of infections as compared to the no treatment group. A minority of patients who had had partial response to chemotherapy obtained complete remission while on therapy with IFNα. Toxicity was mild and patient compliance was excellent. We conclude that IFNα may have a role as maintenance therapy in CLL for patients responding to chemotherapy. © 1992 Wiley‐Liss, Inc.
Coexistence of congenital afibrinogenemia and protein c deficiency in a patient
Authors: Hanano Masaharu , Takahashi Hoyu , Shibata Akira , Itoh Masakazu 
Published: February 2007

AbstractA rare association of congenital afibrinogenemia and hereditary protein C deficiency is described in a 37‐year‐old female who suffered from ischemic necrosis in the left first toe. The diagnosis of afibrinogenemia was assessed by the absence of fibrinogen in clotting and immunological assays. The diagnosis of hereditary heterozygous type I protein C deficiency was based on the evidence of proportional decreases of activity and antigen of plasma protein C in the propositus, her mother, and two maternal aunts. © 1992 Wiley‐Liss, Inc.