bluuebunny/crossref_metadata_2025_split huggingface dataset:
Quinine‐induced immune thrombocytopenia with hemolytic uremic syndrome: Clinical and serological findings in nine patients and review of literature
Authors: Gottschall Jerome L. , Neahring Betsy , McFarland Janice G. , Wu Guo‐Guang , Weitekamp Lee Ann , Aster Richard H. 
Published: February 2007

AbstractQuinine‐induced immune thrombocytopenia with hemolytic uremic syndrome (HUS) is a recently defined clinical entity. In this paper we have attempted to characterize the natural history and laboratory abnormalities typical of quinine‐induced immune thrombocytopenia associated with hemolytic uremic syndrome in nine patients experiencing ten episodes of the disease. In addition, review of other reported cases of probable quinine‐induced HUS is presented. The disease was characterized by the onset of chills, diapheresis, nausea and vomiting, abdominal pain, decreased urine output, and petechiae following quinine exposure. All patients experience significant anemia, severe thrombocytopenia, increased lactate dehydrogenase, elevated serum creatinine, and oliguria. Quinine‐dependent platelet‐reactive antibodies were identified in eight of nine using flow cytometry. Unexpectedly, drug‐dependent antibodies reactive with red cells and granulocytes were identified in four and eight patients, respectively. All patients were treated with plasma exchange (range 1–12 procedures), and seven required hemodialysis. All survive without residual abnormality. Our experience with nine patients with quinine‐induced HUS and the nine additional cases reported by others and reviewed in this paper establishes this condition as a distinct clinical entity. Adult patients presenting with HUS should routinely be asked about exposure to quinine in the form of medication or beverages. The mechanism by which quinine‐dependent antibodies produce renal failure is uncertain, but preliminary studies (described elsewhere) suggest that drug‐induced antibodies reactive with endothelial cells and possibly margination of granulocytes in renal glomeruli may be responsible for this complication. The prognosis in quinine‐induced HUS is better than in other forms of adult HUS. © 1994 Wiley‐Liss, Inc.
Sensitivity of myeloid progenitors to NK‐cell depletion
Authors: Fardoun‐Joalland D. , Texeira‐Lebrun G. , Jouan‐Beades F. , Lenormand B. , Dzondo‐Gadet M. , Vannier J. P. 
Published: February 2007

AbstractTo gain additional informations on the role played by Natural Killer cells (NK) in the differentiation of human hematopoietic precursors, we have studied the effect of NK‐cell depletion on the in vitro proliferation of hematopoietic cells. NK cells were depleted from blood mononuclear cells by FACS using anti CD3 and anti CD56 monoclonal antibodies. Depletion of NK cells suppressed CFU‐GM up to 69% (P < 0,01), while no significant effect on either BFU‐E and CFU‐Mix growth was observed. To define the threshold of CD56+ cells required to support CFU‐GM formation, NK cells were added to NK‐depleted cells in a titrated fashion. Enhancement of CFU‐GM colony growth was observed at NK/NK ‐ depleted cells ratio of 0.15/1. A dose dependent suppression of CFU growth was observed at ratios ranging from 0.25/1 to 0.5/1. Addition of neutralizing antibodies against IL3 and GM‐CSF abrogate the stimulating effect of NK cells.Our results suggest that cells with LGL morphology and NK markers play an important role in differentiation of myeloid precursors and exert a moderate influence on erythroid progenitors. The modulatory effect on hematopietic progenitors depend on the number of NK cells present in the mixed culture. © 1994 Wiley‐Liss, Inc.
Apoptosis induction with three nucleoside analogs on freshly isolated B‐chronic lymphocytic leukemia cells
Authors: Zinzani P. L. , Tosi P. , Visani G. , Martinelli G. , Farabegoli P. , Buzzi M. , Ottaviani E. , Salvucci M. , Bendandi M. , Zaccaria A. , Tura S. 
Published: February 2007

AbstractThe cytotoxic effects and the induction of programmed cell death (apoptosis) by Fludarabine (FLU), 2‐chlorodeoxyadenosine (2‐CdA), and deoxycoformycin (DCF) with/without α‐interferon (α‐IFN) were evaluated in vitro against freshly isolated B‐chronic lymphocytic leukemia (B‐CLL) cells. Cytotoxicity was evaluated according to the soluble tetrazolium/formazan assay. Regarding the cytotoxicity, FLU, 2‐CdA, and DCF showed a mean antitumor activity of 45% ± 3.39 (mean ± S.D.), 55% ± 4.72, and 20% ± 3.16, respectively. α‐IFN alone showed a mean cytotoxic activity of 10% ± 2.72. The cytotoxicity of these purine analogues in combination with α‐IFN was 52% ± 2.97, 75% ± 3.41, and 26% ± 7.09, respectively. We observed a statistically significant increase of cytotoxicity compared to controls in FLU alone (P < 0.05), 2‐CdA (P < 0.05), and their combination with α‐IFN (P < 0.05). Apoptosis was evaluated by electrophoresis gel of DNA oligonucleosomal fragments and by a cytofluorimetric method. Only FLU and 2‐CdA activated the apoptosis and DCF showed a minor apoptotic pathway amount. These apoptosis data were confirmed by both gel electrophoresis of DNA and by propidium iodide cytofluorimetric method. FLU and 2‐CdA show activity in B‐CLL cells by direct cytotoxic action and the induction of cell death by apoptosis; in the future, it would be interesting to utilize these in vitro assays in monitoring chemosensitivity and predicting response for the clinical use. © 1994 Wiley‐Liss, Inc.
Acute promyelocytic leukaemia (M3): Relapse with acute myeloblastic leukaemia (M2) and dic(5;17) (q11; p11)
Authors: Hatzis Theodore , Standen Graham R. , Howell Rodney T. , Savill Catherine , Wagstaff Michael , Scott Geoffrey L. 
Published: February 2007

AbstractSecondary leukaemia following treatment of M3 acute promyelocytic leukaemia (APL) is a rare event. We describe a patient in remission following chemotherapy for APL who relapsed with M2 acute non‐lymphoblastic leukaemia (ANLL). The original t(15;17) (q22; q21) chromosome translocation was lost and replaced by a clone containing a dic(5;17) (q11; p11) abnormality. Southern genomic analysis demonstrated re‐arrangements of the retinoic acid receptor α (RARα) and PML genes in the APL blasts at presentation but not in the M2 ANLL marrow at relapse. The significance of unbalanced 5;17 translocations as markers for therapy‐related secondary leukaemia is discussed. ©1995 Wiley‐Liss, Inc.
α‐2a interferon therapy and antibody formation in patients with essential thrombocythemia and polycythemia vera with thrombocytosis
Authors: Törnebohm‐Roche E. , Merup M. , Lockner D. , Paul C. 
Published: February 2007

AbstractIn ten patients with essential thrombocythemia and polycythemia Vera with thrombocytosis we have investigated the therapeutic effect of recombinant α‐2a interferon (Roceron A®) given subcutaneously in a maintenance dosage of 3 million units three times weekly. The aim was to normalize the platelet count (⩽ 400 × 109/L). One of the secondary aims was to study platelet activity measured as β‐thromboglobulin (β‐TG) in urine.All but one patient could administer the injections and in all patients a significant reduction in platelet values was seen. The treatment was discontinued in three patients due to side effects of interferon, two because of hair loss (one with irreversible alopecia), and one because of depression. Three patients developed antibodies to α‐2a interferon and a concomitant rise in the platelet level; in one patient therapy was switched to leukocyte α‐interferon with an excellent response. The initial levels of β‐TG were elevated in 9/10 patients and were significantly reduced at 6 months in 4/5 patients not developing antibodies. Six patient are still on α‐interferon therapy with a long‐term follow‐up of 3‐3.5 years. We conclude that α‐interferon therapy may be an alternative in patients with thrombocytosis and/or complications necessitating treatment. ©1995 Wiley‐Liss, Inc.
Gender and haplotype effects upon hematological manifestations of adult sickle cell anemia
Authors: Steinberg M. H. , Hsu H. , Nagel R. L. , Milner P. F. , Adams J. G. , Benjamin L. , Fryd S. , Gillette P. , Gilman J. , Josifovska O. , Hellman‐Erlingsson S. , Safaya S. , Huey L. , Rieder R. F. 
Published: February 2007

AbstractIn Africa, the β‐globin gene cluster haplotype may be associated with variation of Hb F levels in subjects with sickle cell anemia (SS). These observations have not yet been conclusively confirmed in SS out of Africa, perhaps because of small sample sizes, the predominance of haplotype heterozygotes, and diverse influences, including gender, upon Hb F levels.We studied 384 adult African‐American SS patients (mean age, 31 years) and explored the relationship of gender, β‐globin gene cluster haplotype, and α thalassemia to hematological values and Hb F levels. Both haplotype and gender influenced Hb F concentration. In the total sample, Hb F was higher in females than in males (8.2 vs. 6.5%). In 35 males who were either homozygous for the Senegal chromosome or had the Senegal/Benin haplotype, the mean percent Hb F (8.0%) was equivalent to the Hb F level in females with Benin and Bantu haplotypes (‐7.5%). Both females and males homozygous for the Senegal haplotype chromosome or with the Senegal/Benin combination had a significant increase in Hb F compared to other groups. In 44 Senegal/Senegal or Senegal/Benin females the Hb F was 10.9%, or 1.0 g/dl, the highest value observed in all primary analysis groups. Preliminary analyses suggested that the presence of a Bantu chromosome blunted the gender‐associated difference in Hb F, but Hb F differences between females with the Senegal/Benin haplotype (11.2%) and the Senegal/Bantu haplotype (8.8%) were not statistically significant. Hemoglobin concentrations were higher in males than in females except in subjects with at least one Senegal haplotype chromosome, where hemoglobin levels were equal. As expected, α thalassemia reduced the MCV, increased hemoglobin concentration, and lowered reticulocyte counts, regardless of haplotype. Hb F levels were not affected by the presence of α thalassemia in any group.We conclude that gender and β‐globin gene cluster haplotype interact significantly in the modulation of Hb F and anemia in adults with SS. ©1995 Wiley‐Liss, Inc.
A new myeloproliferative syndrome
Authors: Schofield Jill R. , Robinson William A. 
Published: February 2007

AbstractWe report here two cases of a previously undescribed myeloprollferative disorder. Both were young adult males who presented with generalized lymphadenopathy, splenomegaly, leukocytosis, polycythemia, and persistent thrombocytopenia. The leukocyte alkaline phosphatase (LAP) score was low in both cases, and the bone marrow was hypercellular without dysplasia or fibrosis, but lacked the Philadelphia chromosome, BCR gene rearrangement, or other karyotypic abnormalities. The clinical course was indolent in each case. One patient died from an unusual ‘blast crisis’ after 12 years, while the second patient remains in a complete hematologic remission on hydroxyurea and alpha interferon 4 years from diagnosis. Interestingly, changes in therapy in this patient have consistently resulted in precise and concerted fluctuations in his blood counts, with the red and white cells cycling together and the platelets and mean corpuscular volume (MCV) changing concomitantly but in the opposite direction. This unique myeloproliferative disorder is distinguishable from all previously described forms of chronic myeloid leukemia and other myeloproliferative syndromes. ©1995 Wiley‐Liss, Inc.
Bone marrow biopsy imprints (touch preparations) for assessment of iron stores
Authors: Pasquale Donald , Chikkappa G. 
Published: February 2007

AbstractNoninvasive evaluation for iron deficiency is compromised in many individuals due to the presence of chronic inflammatory processes and/or malignancy, thus necessitating bone marrow examination for definitive diagnosis. However, bone marrow aspiration is not obtainable or is inadequate for interpretation (dry tap) in some individuals, and decalcified bone marrow biopsies require 24‐48 hr to prepare, and may falsely indicate absence of iron. We evaluated the accuracy of bone marrow biopsy imprints (touch preparations) compared with aspirate particle smears for semiquantitation of bone marrow iron stores. Results indicate that Prussian blue‐stained bone marrow biopsy imprints accurately reflect the quantity of iron, compared with bone marrow aspirate particle smears, allowing for rapid determination of iron stores in individuals in whom a bone marrow aspirate cannot be obtained. © 1995 Wiley‐Liss, Inc.
Biochemical indices of vitamin B12 nutrition in pregnant patients with subnormal serum vitamin B12 levels
Authors: Metz Jack , McGrath Katherine , Bennett Michael , Hyland Keith , Bottiglieri Teodoro 
Published: February 2007

AbstractTo determine the significance of the commonly observed fall in serum vitamin B12 levels during pregnancy, serum levels of the B12 metabolites methylmalonic acid (MMA) and homocysteine (Hcy) were measured in a group of 50 pregnant patients with subnormal serum B12 (range 45‐199 pg/ml) and the results compared with those of 25 pregnant controls (serum B12208‐580 pg/ml). Mean values for serum MMA and total Hcy in the subnormal B12 group were 445.4 nmol/L and 7.03 μmol/L, respectively, which were not significantly different from the mean MMA of 440.5 nmol/L and Hcy of 6.88 nmol/L in the controls. For the total group of patients, neither serum MMA nor serum Hcy levels correlated with serum B12. One‐third of pregnant patients showed elevated serum MMA values, independent of B12 status. Significant elevation of serum Hcy was detected in only two patients, both with subnormal serum B12 and hematological evidence of B12 deficiency. We conclude that the usual fall in serum B12 concentration in pregnancy does not reflect B12 deficiency at the biochemical level. In establishing true B12 deficiency in pregnancy, the serum Hcy level (in the absence of folate deficiency) but not serum MMA, is of value. © 1995 Wiley‐Liss, Inc.
Bone marrow transplantation in a young child with sickle cell anemia
Authors: Kalinyak Karen A. , Morris Christopher , Ball William S. , Ris M. Douglas , Harris Richard , Rucknagel Donald 
Published: February 2007

AbstractBone marrow transplantation (BMT) is the only curative therapy available for hemoglobinopathies. BMT was performed on a young child with sickle cell anemia (SCA) after approximately 9 months of transfuslon therapy following her initial stroke. The patient received a matched sibling donor (sickle trait) BMT. The conditioning regimen consisted of busulfan 4 mg/kg/day × 4, cyclophosphamide 50 mg/kg/day × 4. Graft vs. host disease prophylaxis was daily cyclosporine for 6 months. There were no significant complications during BMT. Engraftment occurred on day + 17 and the patient was transfusion independent since day +45. Pre‐BMT cerebral arteriography showed multiple stenotic cerebral vessels and a moya‐moya pattern. Perfusion MRI demonstrated reduced capillary perfusion. Approximately 170 days after BMT the patient experienced episodes of transient left‐sided weakness and speech problems. Neuroimaging revealed progression of large vessel pathology by angiography despite significant improvement in cortical perfusion (MR perfusion scan). Molecular analysis by PCR and DNA fingerprinting confirmed absence of mixed mosaicism. Rheologic evaluation showed normal corrected bulk viscosity. It is possible that progression of large vessel pathology and return of clinical symptoms in the face of normal rheologic parameters may be due to worsening of the already damaged cerebral vessels by the BMT conditioning regimen. Further evaluations of patients with SCA undergoing BMT after a stroke are needed to answer this question. © 1995 Wiley‐Liss, Inc.
High‐dose methylprednisolone is an alternative treatment for adults with autoimmune thrombocytopenic purpura refractory to intravenous immunoglobulins and oral corticosteroids
Authors: Godeau Bertrand , Zini Jean‐Marc , Schaeffer Annette , Bierling Philippe 
Published: February 2007

AbstractEight patients with severe chronic autoimmune thrombocytopenic purpura (AITP) refractory to high‐dose intravenous immunoglobulin (IVIgG) and/or oral prednisone were treated with one to three infusions of high‐dose methylprednisolone (HDMP) (15 mg/kg/day). The mean platelet count before treatment was 12 ± 10 × 109/L. HDMP therapy led to a safe platelet count (>50 × 109/L) after 2‐5 days in five patients, and a minimal platelet increase (34 × 109/L) able to stop bleeding in a sixth patient. The effect of HDMP was, however, translent in four of the five responders. No side effects were observed, even in the four patients older than 70 years. HDMP thus appears to be a good alternative in emergency situations or prior to surgery for patients with AITP refractory to conventional therapy.
Oxidized LDL induces serotonin release from blood platelets
Authors: Zhao B. , Dierichs R. , Harrach‐Ruprecht B. , Winterhorff H. 
Published: February 2007

AbstractOxidized low‐density lipoprotein (LDL) induces a release of serotonin from morphologically resting platelets and shape changed platelets. This suggests that oxidized LDL, a newly reported weak agonist, contributes to atherogenesis and thrombogenesis by stimulating platelets. © 1995 Wiley‐Liss, Inc.
New variant of type II von willebrand's disease with structural abnormality of plasma von willebrand factor in a patient with very mild bleeding history
Authors: Baillod Peter , Gaucher Christine , Affolter Beat , Mazurier Claudine , Pflugshaupt Rolf 
Published: February 2007

AbstractA new variant of von Willebrand's disease has been discovered in 2 members of a Macedonian family of 6. The proband, an 8‐year‐old boy, showed a prolonged bleeding episode on 1 occasion. Ristocetin‐induced platelet aggregation and bleeding time were normal. In plasma, ristocetin cofactor activity (RCo) and von Willebrand factor (vWf) antigen were reduced to the same clearly low level. The determination of vWf antigen of platelets resulted in borderline values, while RCo was clearly reduced. Low‐ and intermediate‐resolution agarose gel electrophoresis showed absence of the largest multimers in plasma vWf, and slight reduction in platelet vWf. High‐resolution gels revealed abnormal multimeric structure only in plasma vWf. The smaller multimers could be resolved in a broad central band flanked by 4 fainter satellite bands; however, satellite bands close to the central band were more intense, and more distant ones were fainter, compared to normal plasma. The central band of the fastest‐moving multimer was markedly intensified, and the mobility of the whole quintuplet was slightly reduced. Heredity seems to be autosomal‐dominant. No mutation was found in exon 28 of the vWf gene.Because there was only 1 mild bleeding episode in the family, this structural variation seems to have only little clinical consequence. We conclude that this vWf abnormality is different from those observed in other type II variants previously described. Based on the revised classification by the International Society on Thrombosis and Haemostasis, we proposed designation type 2A‐Bern for this new subtype. © 1995 Wiley‐Liss, Inc.
Recombinant hirudin (HBW 023): Biological data of ten patients with severe venous thrombo‐embolism
Authors: Bridey F. , Dreyfus M. , Parent F. , Bros A. , Fischer A. M. , Camez A. , Simonneau G. , Duroux P. , Meyera D. 
Published: February 2007

AbstractThis study reports on the biological data of ten patients with acute venous thromboembolism. They were treated for 5 days with continuous intravenous infusion of a fixed dose (0.05 mg/kg/hr) of a recombinant hirudin (r‐H HBW 023 Behringwerke, Germany). The plasma level of r‐H (HBW 023), assessed by an anti‐factor Ila amidolytic activity, was stable after Day 2 and showed considerable individual variations. It correlated with APTT ratio, suggesting that this test is a reliable tool to monitor therapy. In contrast, thrombin time was constantly over 120 sec (control 15 sec) and consequently was not a useful parameter. Prothrombin time showed a slight, but significant, prolongation, which was correlated with the increase of APTT ratio. There was no bleeding time prolongation, platelet count, or ATIII level decrease. Levels of thrombin‐antithrombin III complexes, and D‐dimers, which were high in all patients on admission, decreased during the course of the treatment but remained abnormal on Day 5, showing an ongoing hemostasis and fibrinolysis activation: this is consistent with the delayed, but only slightly decreased thrombin generation evidenced by thrombin generation test performed on Day 3. These results suggest that thrombin inhibition by rH‐hirudin at this dosage is only partial, which allows the generation of traces of thrombin needed for the feed‐back thrombin production generated by factor V and VIII activation. © 1995 Wiley‐Llss, Inc.
Decreased production of TNF and IL‐6 in whole blood of CLL patients
Authors: Oahlke E. , Schlag R. , Langenmayer I. , Frankenberger M. , Käfferlein E. , Subkowski T. , Emmerich B. , Ziegler‐Heitbrock H. W. L. 
Published: February 2007

AbstractMonocyte derived cytokines, tumor necrosis factor (TNF) and interleukin‐6 (IL‐6), were determined in cell free plasma after stimulation of heparinized whole blood from chronic lymphocytic leukemia (CLL) patients with lipopolysaccharide (LPS) at 1 μg/ml for 6 hr. Compared to control donors (390 U/ml), CLL patients in average had eight‐fold lower levels of TNF bioactivity (50 U/ml). The depressed levels were observed over a wide range of LPS concentrations (0.01 to 10 μg/ml). Furthermore, after stimulation with S. aureus bacteria, CLL samples gave three‐fold lower levels, as well. TNF levels were not decreased because of defective bioactivity of TNF, since strongly reduced levels of TNF protein were also detected in an immunoassay. Finally, interleukin‐6 levels after LPS stimulation were decreased threefold.Flow cytometry analysis with CD14 antibodies demonstrated comparable numbers of monocytes for control donors and CLL patients (698 ± 802 and 427 ± 267, respectively). This suggests that deficient cytokine production was not due to a reduction in monocyte number, but rather to a functional impairment.The deficiency in cytokine production observed after ex vivo stimulation of whole blood from CLL patients suggests that in vivo during bacterial infection, CLL patients will exhibit an inappropriate response as well. © 1995 witey‐Liss, Inc.
Hb Lepore (Pylos)/Hb S compounds heterozygosity in two Greek families
Authors: Voskaridou E. , Konstantopoulos K. , Kollia P. , Papadakis M. , Loukopoulos D. 
Published: February 2007

AbstractWe have studied three compound heterozygotes for Hb Lepore (“Pylos”)/HbS hemoglobin, a combination quite uncommon in the literature. It is of interest that while two of these cases are clinically similar to those thus far reported, the third one is free of symptoms and the diagnosis was put incidentally. This mild condition may be due to the high level of fetal hemoglobin produced. © 1995 Wiley‐Liss, Inc.
Transformation of chronic myelomonocytic leukemia to acute lymphoblastic leukemia: Case report and review of the literature of lymphoblastic transformation of myelodysplastic syndrome
Authors: Kouides Peter A. , Bennett John M. 
Published: February 2007

AbstractIf chronic myelomonocytic leukemia (CMML) transforms into an acute leukemic phase, the blast crisis is invariably myeloid. Occasionally, the other subtypes of myelodysplastic syndrome (MDS) (refractory anemia, refractory anemia with ringed sideroblasts, refractory anemia with excess blasts, refractory anemia with excess blasts in transformation) have been noted to transform into acute lymphoblastic leukemia (ALL). We now report a case of CMML that transformed into ALL and we review the literature of 13 other cases of MDS with ALL transformation. Such cases provide suggestive clinical evidence that MDS can involve a pluripotent stem cell. © 1955 Wiley‐Liss, Inc.