bluuebunny/crossref_metadata_2025_split huggingface dataset:
Sequential intermediate‐dose cytosine arabinoside and mitoxantrone for patients with relapsed and refractory acute myelocytic leukemia
Authors: Paciucci Paolo Alberto , Cuttner Janet , Holland James F. 
Published: February 2007

AbstractBased on in vitro evidence of time‐dependent synergistic kill of HL‐60 leukemia cells exposed to Ara‐C and mitoxantrone, 44 patients with relapsed or refractory AML and 3 with blastic CML were treated with a timed sequence of both drugs. There were 25 females and 22 males, with a median age of 53 (range 21–75). Of 31 patients with relapsed AML, 24 had one prior remission, 6 had two and 1 had three. Of these, 15 had failed a second reinduction attempt. Thirteen patients were primarily refractory to induction with Ara‐C plus daunorubicin. Each dose of Ara‐C, 500 mg/m2, was followed after 6 hr by mitoxantrone, 5 mg/m2, and the sequence was repeated four to six times (44–68 hr) in different cohorts of patients. All but two patients (one with blastic CML and one in relapse and refractory) are evaluable for response and toxicity. Of 16 patients in relapse without prior reinduction 7 achieved CR and 3 PR (62% response rate); there were 3 CR in the 14 patients who were in relapse and refractory (21% response rate) and 4 CR and 1 PR (35% response rate) in the 14 patients with primary anthracycline resistance. Five of seven patients previously exposed to mitoxantrone achieved CR. Response lasted from 2 to 42 months, with two patients alive and in continuing remission at 34 and 42 months. Average marrow recovery was seen after 25 days and time to remission was 30 days. Six patients died in induction (four from sepsis and two from the tumor lysis syndrome) and 21 had progessive disease. Chemotherapy was well tolerated with minor nausea and vomiting in 13 patients, moderate in 20, and severe in 2. Most patients did not have evidence of drug‐induced mucositis: it was minor in 9 and moderate in 2. Renal dysfunction was attributable to the use of nephrotoxic antibiotics. Hepatic dysfunction was reversible and was minor in 10 patients, moderate in 13, and severe in 3. Sequential, timed administration of intermediate‐dose Ara‐C and mitoxantrone is an active and well‐tolerated antileukemic regimen.
Alloimmune neonatal thrombocytopenia associated with incidental maternal thrombocytopenia
Authors: Burrows Robert F. , Kelton John G. 
Published: February 2007

AbstractIn this report, we describe a mother with mild incidental thrombocytopenia who delivered a severely thrombocytopenic infant having alloimmune thrombocytopenia. This coincidental association should be considered because the recognition and proper diagnosis of alloimmune neonatal thrombocytopenia is important, not only in the current pregnancy, but also in future pregnancies.
Hemoglobin dhonburi α2β2 126 (H4) Val → Gly: A new unstable β variant producing a β‐thalassemia intermedia phenotype in association with β°‐Thalassemia
Authors: Bardakdjian‐Michau J. , Delanoe‐Garin J. , Lacombe C. , Blouquit Y. , Riou J. , Galacteros F. , Fucharoen S. , Winichagoon P. , Wasi P. , Kister J. 
Published: February 2007

AbstractWhile investigating the mechanism of a β‐thalassemia intermedia phenotype in a 34 year old Thai male, a new Hb variant β 126 Val → Gly named Hb Dhonburi was discovered. Genetic and structural studies revealed the existence of a β°‐thalassemia genotype in association with the β variant. The new variant is unstable but exhibits normal oxygen binding properties. Hb Dhonburi was also discovered in the mother of the propositus in association with Hb E.
Acquired von willebrand disease in multiple myeloma secondary to absorption of von willebrand factor by plasma cells
Authors: Richard C. , Cuadrado M. A. , Prieto M. , Batlle J. , Fernández M. Flópez , Salazar M. L. Rodriguez , Bello C. , Recio M. , Santoro T. , Casares M. T. Gomez , Zubizarreta A. 
Published: February 2007

AbstractA case of acquired von Willebrand disease (AvWD) associated with an IgA lambda multiple myeloma is reported. No form of inhibitor could be detected. SDS‐agarose gel electrophoresis patterns of von Willebrand factor (vWF) both in plasma and platelet lysates were normal but a decrease in all‐sized multimers with a type IA pattern was seen. After 1‐deamino‐8‐D arginine vasopressin (DDAVP) infusion, vWF multimers larger than those seen in the resting state appeared in patient plasma, which were progressively cleared. Indirect immunofluorescence studies with a monoclonal antibody to vWF showed that vWF was selectively absorbed into myelomatous cells. This is the first case of AvWD associated with multiple myeloma resulting from the selective absorption of vWF into abnormal plasma cells.This feature established a new pathophysiological mechanism of AvWD in multiple myeloma and probably in other lymphoproliferative diseases.
Proliferation and differentiation of erythroid progenitors in liquid culture: Analysis of progenitors derived from patients with polycythemia vera
Authors: Fibach Eitan , Rachmilewitz Eliezer A. 
Published: February 2007

AbstractWe have recently described a new two‐phase liquid culture that supports the development of human erythroid progenitors (Fibach et al., Blood 73:100, 1989). The procedure separates the erythroid burst‐forming units (BFUe) from the erythroid colony‐forming units (CFUe) stage and enables quantitation of the proliferation and differentiation of BFUe into CFUe. In the present study we have utilized this system to study erythroid progenitors in polycythemia vera (PV). The abnormality of the erythroid series in PV has been shown to be associated with an increased responsiveness of the progenitors to the hormone erythropoietin (Epo). A basic question in this clonal stem cell disorder is at what developmental stage this abnormality of the PV clone is phenotypically expressed. We have studied this question by comparing the development of Epo‐dependent and Epo‐independent CFUe from peripheral blood BFUe of the PV patient during the BFUe to CFUe transition in the liquid culture. The results indicated that both types of CFUe are generated and that in all cases tested the ratio of Epo‐independent progenitors at both the BFUe and CFUe stage was similar indicating no preferential development of Epoindependent CFUe. These results suggest that the abnormality of the PV erythroid progenitors is expressed only at the CFUe level. Moreover, since the liquid culture did not contain Epo, the results also support the conclusion that BFUe do not require Epo for proliferation or differentiation into CFUe.
Efficacy of the high molecular weight fraction of plasma for the maintenance of pregnancy associated with thrombotic thrombocytopenic purpura
Authors: Koyama Tetsuji , Suehiro Akira , Kakishita Eizo , Taira Shozo , Isojima Shinzo , Norioka Mihoko , Ito Kazuhiko 
Published: February 2007

AbstractWe have investigated the methods for the maintenance of a pregnancy in a patient with thrombotic thrombocytopenic purpura (TTP), said condition, since 1984, having been controlled by a plasma infusion every 3 to 4 weeks. In a preliminary trial it was confirmed that an infusion of the high molecular weight fraction (HMW‐F) of plasma, separated by an Evaflux® 2A fractionator, improved the patient's thrombocytopenia as the plasma infusion, and maintained its beneficial effect for about 2 weeks during early pregnancy. Though an occurrence of a toxemia‐like syndrome responded to repeated plasma infusion, the dose of plasma required to improve the thrombocytopenia gradually increased and reached 5,040 ml by the 20th week of pregnancy. Thus, instead of periodic infusions of whole plasma, periodic infusions of the HMW‐F of plasma were used. Under this regimen the platelet count remained above 10.0 × 104/μl during late pregnancy, and the total dose (2,600 ml) of HMW‐F of plasma that was administered until delivery at full term was less than the dosage of whole plasma that was used during early pregnancy. In this manner we were able to obtain a healthy baby by controlling the patient's TTP during pregnancy. This method of preventing thrombocytopenia appears to be safer with respect to volume loading during pregnancy in the TTP patient.
Disseminated intravascular coagulation and hemorrhage in hemophilia B following elective surgery
Authors: Conlan Maureen G. , Hoots W. Keith 
Published: February 2007

AbstractTwo patients with hemophilia B are described in whom disseminated intravascular coagulation (DIC) developed following infusion of repeated doses of Factor IX concentrate in the perioperative period. In both cases the surgery was elective, Factor IX survival studies had been done to assure proper dosing, and Factor IX levels were monitored daily. Neither patient had clinically significant liver disease. The DIC manifested itself as excessive blood loss from surgical drains without documented thrombosis and was accompanied by prolonged coagulation times, increased fibrin split products and decreased fibrinogen and platelets. In both patients the process was quickly reversed with administration of fresh frozen plasma, cryoprecipitate, and the addition of heparin to the Factor IX concentrate. These cases highlight the difficulty in managing patients with hemophilia B undergoing surgical procedures due to the potential thrombogenicity of the currently available concentrates, and the importance of differentiating the bleeding associated with DIC from underdosing with Factor IX. Furthermore, the potential complications associated with the presently available Factor IX concentrates stress the need for the development of purer, safer Factor IX concentrates.
Familial case of may‐hegglin anomaly associated with familial spastic paraplegia
Authors: Fujita Yukihisa , Fujii Tsuneo , Nishio Akira , Tuboi Kazuhiko , Tsuji Katuhiko , Nakamura Mituo 
Published: February 2007

AbstractWe report a family with May‐Hegglin anomaly associated with familial spastic paraplegia. The propositus was a 39 year old male. His peripheral blood showed a Döhle‐like inclusion bodies in WBC, giant platelets, and thrombocytopenia. He had been suffering from progressive gait disturbance of spastic paraplegia since 20 years old.He was in a state of chronic renal failure and showed sensory hearing impairment. His two children showed similar hematological abnormalities and spastic gait. As far as we know, this is the first case of May‐Hegglin anomaly associated with familial spastic paraplegia in the literature.
Dry tap bone marrow aspiration: Clinical significance
Authors: Humphries John E. 
Published: February 2007

AbstractFailure to obtain bone marrow on attempted marrow aspiration, “dry tap”, has commonly been ascribed to faulty technique. All reports of simultaneous marrow aspirations and biopsies performed at the University of Virginia between January 1, 1983, and July 1, 1989, were reviewed to determine the frequency of dry taps, the diagnoses and pathologic findings in these cases, and the associated laboratory findings. Among 2,235 simultaneous bone marrow aspirations and biopsies, 87 were dry taps (3.9%). Of these 87 dry taps, only six (6.9%) showed normal marrow biopsies, whereas the majority showed significant marrow pathology, usually associated with fibrosis, or hypercellularity, or both. These conditions most likely account for the inability to aspirate marrow. The most frequent diagnoses were metastatic carcinoma (17.2%), chronic myelogenous leukemia (14.9%), idiopathic myelofibrosis (13.8%), and hairy cell leukemia (10.3%). The presence of peripheral blood nucleated red blood cells, thrombocytopenia, and elevation of the serum lactate dehydrogenase were frequent findings in patients who experienced dry taps. Methods to obtain sufficient marrow for rapid diagnosis in these cases are discussed.
Recurrent disseminated intravascular coagulation and fulminant intra hepatic thrombosis in a patient with the anti‐phospholipid syndrome
Authors: Stinson J. , Tomkin G. , McDonald G. , Jackson F. , Harrison M. , Murray A. , Feighery C. , Jackson J. 
Published: February 2007

AbstractWe describe a patient with the lupus anti‐coagulant who had recurrent episodes, over a 2 year period, of a severe and disseminated intravascular coagulopathy. This patient also had positive serological assays for syphilis and anti‐cardiolipin antibodies. Associated with the coagulopathy were co‐expressed episodes of liver disease, ultimately terminating in fulminant liver failure. At autopsy the features were characteristic of the Budd‐Chiari syndrome. This is the first report to document how consumptive coagulopathy may present as a dominant feature of the anti‐phospholipid syndrome. It also clearly describes an immune mediated thrombotic mechanism as a cause of hepatic veno‐occlusive disorders. Furthermore, this case highlights the varied clinical spectrum of the anti‐phospholipid syndrome and suggests that a high index of suspicion is required to ensure its diagnosis.
Antithrombin III‐Amiens: A new family with an Arg47 → cys inherited variant of antithrombin iii with impaired heparin cofactor activity
Authors: Roussel B. , Dieval J. , Delobel J. , Fernandez‐Rachubinski F. , Eng B. , Rachubinski R. A. , Blajchman M. A. 
Published: February 2007

AbstractA family with an antithrombin III variant (AT‐III‐Amiens) demonstrating abnormal heparin cofactor activity is described. Amplification and direct sequencing of genomic DNA by the polymerase chain reaction procedure permitted the identification of an Arg47 Cys mutation in exon 2 of the variant antithrombin III gene.
Clinical pharmacokinetics of a placenta‐derived factor XIII concentrate in type I and type II factor XIII deficiency
Authors: Rodeghiero Francesco , Tosetto Alberto , Bona Eros Di , Castaman Giancarlo 
Published: February 2007

AbstractLimited data are available about the pharmacokinetics of placenta‐derived factor XIII (FXIII) concentrate in patients with FXIII deficiency. This concentrate contains only the active subunit A but not the carrier subunit B of the factor, and perplexities have been raised about its clinical use. Moreover, no data are available on its use in the rare patients completely lacking both subunit A and subunit B. Therefore, we evaluated the pharmacokinetics of a commercial placenta concentrate in three patients with FXIII deficiency: two lacking subunit A (type II) and one lacking both subunits (type I).The elimination half‐life of the infused placenta subunit A in the three patients was very similar (280, 283, and 272 hr) and was also consistent with the previously reported data for plasma‐derived FXIII. No thrombin‐independent activity was observed in our concentrate batches. The recovery was significantly lower in the type I patient, in whom infusion of subunit A was not able to elicit a monthly increment of subunit B, as usually observed in type II patients. Monthly infusions of placenta concentrate (at higher dosage in type I patient) have been administered to our patients for two to three years and no evidence of inhibitor against factor XIII activity has been observed.We conclude that placenta concentrates may be as effective as plasma derivatives in replacement therapy of factor XIII deficiency, even in patients who lack subunit B.
Humoral immune responses to gag and env proteins from human immunodeficiency virus type 1 in hemophiliac patients
Authors: Kinney Janet S. , Conway James H. , Hilgartner Margaret W. , Clayman Barbara , Mayur Kumudini , Yolken Robert H. , Viscidi Raphael P. 
Published: February 2007

AbstractSolid‐phase enzyme immunoassays using recombinant gag and env proteins were developed to study humoral immune responses to HIV infection in a cohort of 105 hemophiliac patients. Thirteen patients with ARC or AIDS and 92 asymptomatic patients were studied. A cross‐sectional study showed a wide range of antibody responses to gag and env proteins; however, the differences between the ARC/AIDS and asymptomatic patients were statistically significant for both antigens (P < .0004). In a longitudinal study, antibody levels in sera from 11 asymptomatic patients with gag antibody log units ≦ 1.5 were compared to levels in sera from 10 ARC/AIDS patients and 8 asymptomatic patients with gag antibody >1.5. These patient groups were followed for comparable periods of time (67.1‐71.7 mo). The asymptomatic patients with low gag antibody and the ARC/AIDS patients showed a similar pattern of antibody response to gag protein overtime. In hemophiliac patients with HIV‐1 infection a low titer of antibody to gag protein is not invariably associated with clinical deterioration and is not a useful serologic marker of impending progression to AIDS.
Neutrophil alkaline phosphatase activity in the urinary neutrophils of a patient with chronic myelogenous leukemia
Authors: Janković Gradimir , Petrović Milan , Čolović Milica , Žilić Mirjana , Dotlić Rajko 
Published: February 2007

AbstractWe report here a patient with Philadelphia chromosome (Ph)‐positive chronic myelogenous leukemia (CML) in chronic phase in whom the alkaline phosphatase activity of neutrophils in the peripheral blood was low while at the same time the alkaline phosphatase content of neutrophils present in the urine was elevated. This observation provides independent clinical support for the recent experimental finding that an extrinsic factor (granulocyte colony‐stimulating factor) controls alkaline phosphatase expression in human neutrophils.
Antiplatelet antibodies in childhood idiopathic thrombocytopenic purpura
Authors: Özsoylu Şlinasi , Karabent Adnan , Irken Gülersu , Tuncer Murat 
Published: February 2007

AbstractAntiplatelet antibodies were shown by the Handin and Stossel method in the sera of all 103 patients with acute ideopathic thrombocytopenic purpura (ITP) and in 100 cases following recovery from it. These antibodies were also shown in the sera of all 46 patients with chronic ITP and 32 cases after recovery. The decrease in level of antiplatelet antibodies was significant in all these children following recovery (P < 0.001 for acute ITP, P < 0.05 for chronic ITP). Antiplatelet antibodies could be determined in 67 acute and 21 chronic ITP cases in thrombocytopenic phase and following recovery, which showed very significant decreases in levels in each case in a later period. Antiplatelet antibody levels corresponding to the thrombocytopenic phase and recovery in acute and chronic ITP were significantly higher than normal and thrombocytopenic control values (P < 0.001 for each).
T‐lymphoid blast crisis in chronic myeloid leukemia
Authors: Advani S. H. , Malhotra H. , Kadam P. R. , Iyer R. S. , Nanjangud G. , Balsara B. , Saikia T. , Gopal R. , Nair C. N. 
Published: February 2007

AbstractChronic myeloid leukemia (CML) is considered to be a pleuripotential stem cell disorder with the capacity to differentiate into myeloid, erythroid, megakaryocytic, and lymphoid cell lines. Consequently, blast crisis (BC) involving each of the above lineages has been well described. Among lymphoblastic crises, differentiation frequently occurs along B‐cell lineage. We report four patients of CML who terminated in T‐cell extramedullary BC in lymph nodes after a variable duration of chronic phase. The T‐lineage was established by characteristic cytochemical staining and reactivity with a panel of anti‐T‐cell monoclonal antibodies. All four cases were Philadelphia (Ph) chromosome positive and demonstrated the Ph chromosome and associated anomalies (extra Ph, +19) in the lymph nodes. Our data adds to the growing evidence that CML is a disorder of the common stem cell from which T, B, and myeloid precursors originate.
Erythropoietin life span in rats with hypoplastic and hyperplastic bone marrows
Authors: Piroso Ettore , Erslev Allan J. , Flaharty Kristen K. , Caro Jaime 
Published: February 2007

AbstractThe metabolic fate of erythropoietin (EPO) remains unknown. Urinary excretion does not appear to play a major role and liver catabolism has been shown to occur only after terminal sugars on the hormone have been removed. However, it has been proposed that EPO is eliminated by consumption in the bone marrow. In order to examine the extent of such consumption we measured the half‐life of radioidinated recombinant EPO injected intravenously (IV) to rats with bone marrows suppressed by cyclophosphamide or hypertransfusion and marrows stimulated by phenylhydrazine or bleeding. The mean half‐life or erythropoietin in normal rats was 179 ± 16 min, with similar half‐lives found in the other rats regardléss of decreased or increased bone marrow activity. The results indicate that it is unlikely that erythroid activity determines EPO life span and catabolism.
Abnormal proteolytic degradation of von willebrand factor after desmopressin infusion in a new subtype of von willebrand disease (ID)
Authors: Lopez‐Fernandez M. F. , Gonzalez‐Boullosa R. , Blanco‐Lopez M. J. , Perez M. , Batlle J. 
Published: February 2007

AbstractWe describe two members of a single family, father and son, with mild factor XII deficiency associated to von Willebrand disease (vWD) with aberrant structure in whom distinct multimeric abnormalities and an abnormal proteolytic processing of von Willebrand factor (vWF) after desmopressin (DDAVP) administration were present. They had a mild bleeding history, low levels of vWF‐related activities, and a prolonged bleeding time. Low‐resolution agarose‐ gel electrophoresis showed a vWF with all size multimers in plasma and platelets. Higher‐resolution agarose gels demonstrated that the main band was present, but the relative proportion of the satellite bands was markedly reduced. The smallest oligomer was not increased. After the infusion of DDAVP to the father, a transient increase in the relative proportion of the satellite bands was seen, as described in normal individuals. No difference in the structure of vWF was observed when blood was collected with proteinase inhibitors. The analysis of native subunits of vWF and their proteolytic derived fragments, after DDAVP administration, showed a temporary augmentation of the 176 kDa fragment, as seen in normal subjects, as well as an increase of the 189 kDa fragment. This finding had not been reported previously either in normal individuals or in patients with vWD.
High‐dose dexamethasone for refractory or relapsing multiple myeloma
Authors: Friedenberg William R. , Kyle Robert A. , Knospe William H. , Bennett John M. , Tsiatis Anastasios A. , Oken Martin M. 
Published: February 2007

AbstractIn order to assess the efficacy and toxicity of dexamethasone as a single agent without the concomitant infusion of Adriamycin and vincristine (VAD), an ECOG pilot study was initiated using 40 mg by mouth daily for 4 days every week for 8 weeks. Patients who responded were then maintained on the same treatment, but at 2 week intervals. Of the 32 patients evaluable for response, three were completely refractory to all prior therapy. All patients had advanced disease and 26 had received multiple prior treatments. There were 13/32 (40%) objective responses by ECOG criteria. Of the 28 patients evaluable for subjective response, i.e., significant decrease in performance status and/or bone pain, eight (28.5%) responded. Of the 34 patients evaluable for toxicity, 19 patients (55%) had moderate to severe side effects, including nine who had central nervous system effects, three who had gastrointestinal bleeding, two who had pulmonary emboli, one with psychosis, and four who had serious infections with one death. Median survival for the entire group was 19 weeks, with 31 weeks in the responders and 9 weeks in the non‐responders.Although high‐dose dexamethasone is capable of producing a significant number of partial responses (40%), it is associated with excessive toxicity. Less frequent administration of the dexamethasone at 2 week intervals was well tolerated in the maintenance of partial response, but has not been studied for efficacy in induction of remission.