bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical utility of marrow cell tissue cultures in acute leukemia of childhood
Authors: Estrov Zeev , Grunberger Tom , Freedman Melvin H. 
Published: February 2007

AbstractTo determine the clinical value of tissue culture techniques in the study of acute leukemia, marrows from 50 children (40 acute lymphoblastic [ALL], 10 acute myeloblastic [AML]) were cultured by the use of an AML blast colony assay and the CFU‐GEMM assay. In the AML assay, AML marrow gave rise to high numbers of blast colonies within 24 to 48 hr; in sharp contrast, 37 of 40 ALL marrows did not yield colonies in this assay. In the CFU‐GEMM assay, AML marrow produced excessive numbers of monocyte‐macrophage CFU‐C colonies, an obvious background of individual macrophages and clusters, and occasional blast colonies. ALL marrow yielded very low numbers of granulocytic CFU‐C colonies, no background of macrophage cells, and no blast colonies. These clear‐cut differences in cellular growth kinetics in vitro between ALL and AML marrows should allow confirmation of the type of acute leukemia within 24 to 48 hr. The colony assays may also be valuable in differentiating ALL from AML in difficult diagnoses when conventional approaches are nondiagnostic.
Porphyria cutanea tarda in a patient with acute leukemia
Authors: Dutcher Janice P. , Fisher Michael , Spivack Morton , Wiernik Peter H. 
Published: February 2007

AbstractA 20‐year‐old woman with acute nonlymphocytic leukemia in remission was treated postremission with intermittent courses of intensive chemotherapy. She required blood and platelet transfusions following therapy, and oral contraceptives were used to suppress her menses while she was thrombocytopenic. One and one‐half years after treatment began, she developed blisters on her hands, hypertrichosis, and dark urine. The diagnosis of porphyria cutanea tarda was made. This report discusses the possible precipitating cause(s) and the subsequent treatment of both her porphyria and her leukemia.
Platelet‐associated immunoglobulins IgG, IgM, IgA and complement C3 in immune and nonimmune thrombocytopenic disorders
Authors: Panzer Simon , Szamait Sabine , Bödeker Rolf‐Hasso , Haas Oskar A. , Haubenstock Alexander , Mueller‐Eckhardt Christian 
Published: February 2007

AbstractA two‐stage radioactive antiglobulin test‐using unlabelled antisera specific for IgG, IgA, IgM and C3 followed by binding of 125I‐staphyloccocal protein A–was applied to determine platelet‐associated immunoglobulins (PAIg) and complement (PAC3) in thrombocytopenias of various etiologies. One hundred and one patients with immune thrombocytopenia (chronic autoimmune, 48; acute autoimmune, 37; Evans syndrome, nine; connective tissue diseases, seven) and 20 patients with presumed nonimmune thrombocytopenia (bone marrow aplasia or malignancy, six; septicemia, five; hypersplenism, five; cirrhosis of liver, three; others, one) were studied.Increased levels of PAIg/C3 were found in 76% of patients with immune thrombocytopenia. PAIgG was raised in 66%, PAIgM in 57%, PAIgA in 44%, and PAC3 in 29%. Isolated elevation of PAIgG and of PAIgM was found in four and three cases, respectively; PAIgA and PAC3 were elevated in one case each. PAIgG was associated with PAIgM in 56%, with PAIgA in 34%, and with PAC3 in 27%. Both patients with Evans' syndrome and patients with connective tissue diseases had significantly higher PAIgM levels than the other patients with immune thrombocytopenia.In patients with nonimmune thrombocytopenia, increased rates of PAIg/C3 were also encountered. Positive test results were found in 88% (PAIgG 88%, PAIgM 47%, PAIgA 35%, and PAC3 24%).In immune‐mediated thrombocytopenia, we observed a significant inverse correlation between platelet counts and PAIgG, PAIgA, and PAC3, but not with PAIgM. In contrast, no such correlation was found in patients with nonimmune thrombocytopenia.Our data indicate that the evaluation of neither parameter alone nor the combination of PAIg/C3 will discriminate between immune and nonimmune thrombocytopenia. Preferential coating with certain immunoglobulins, however, may be present in some subgroups of immune thrombocytopenias.
Spα1/65 hereditary elliptocytosis in North Africa
Authors: Alloisio N. , Guetarni D. , Morlé L. , Pothier B. , Ducluzeau M. T. , Soun A. , Colonna P. , Clerc M. , Philippe N. , Delaunay J. 
Published: February 2007

AbstractThe Spα1/65 variant of the spectrin has been recently described in black people with hereditary elliptocytosis (HE). The present study reports on a similar Spα1/65 variant in nine North African persons belonging to four unrelated families. The abnormality was associated with a variable degree of elliptocytosis. In one case, red cell morphology was normal. In the nine carriers of the biochemical abnormality, the spectrin dimer self‐association was defective. The association constant was reduced: 0.65 to 1.7 × 105 M−1 (controls: 4.6 × 0.5 × 105 m M−1 (n = 21)); in six cases, there was a higher level of spectrin dimer in the low ionic strength extract at 4°C: 13.0 to 19.7% (controls: 6.4 ± 2.1% (n = 7)). Limited tryptic digests of spectrin from the nine persons revealed a decrease of the 80,000‐dalton α‐1 domain, and the concomitant appearance of a peptide with a molecular weight of 65,000 daltons and an isoelectric point ranging from 5.0 to 5.1. There was a correlation between the proportion of the 65,000‐dalton fragments, the defect of spectrin self‐association, and the extent of morphological alteration. This is the first large series concerning a spectrin abnormality in non‐black persons. In North Africa, cases of HE that are not due to a protein 4.1 defect have turned out so far to be associated with the Spα1/65 variant.
Prednisone stimulation of erythropoiesis in leukemic children during remission
Authors: Amylon Michael D. , Perrlne Susan P. , Glader Bertil E. 
Published: February 2007

AbstractChildren with acute leukemia in remission manifest reticulocytosis and a significant increase in hemoglobin concentration (mean increment of 2.3 ± 1.1 g/d 1) following prednisone pulse therapy. This hemoglobin increment is not associated with changes in serum erythropoietin activity. It is speculated that this stimulation of red cell production may be a direct effect of steroids on erythroid progenitor cells.
Serum bactericidal and opsonic activities in chronic lymphocytic leukemia and multiple myeloma
Authors: Kansu Emin , Akalin Erdal , Civelek Cahid , Tekesin Oktay , Lalell Yahya , Firat Dinçer 
Published: February 2007

AbstractSusceptibility to infection is widely recognized as the major cause of morbidity and mortality in patients with CLL and MM. The present study was designed to investigate the serum bactericidal (SBA) and serum opsonic activities (SOA) in 12 CLL and 12 MM patients, and results were compared to 20 normals. SBA and SOA were measured by a new radiometric assay. SBA was found to be normal in 11 patients with CLL and in all 12 patients with MM. In contrast, SOA was significantly lower in 11 out of 12 patients with CLL. Ten of twelve patients with MM also had significantly lower SOA compared to those of controls. No correlation was detected between the serum immunoglobulin and complement levels of the patients or between the degree of the opsonic defect and the incidence of infection. In mixture experiments, untreated normal serum partially corrected the opsonic activity of CLL and MM serum. The results suggest the presence of a possible inhibitor in the serum of patients with two well‐known B‐lymphocyte‐derived disorders.
Depletion of T lymphocytes from human bone marrow by the use of counterflow elutriation centrifugation
Authors: Takaue Yoichi , Roome Aaron J. , Turpin James A. , Reading Christopher L. 
Published: February 2007

AbstractNormal donor bone marrow buffy coat (BMBC) cells were fractionated into three subsets by counterflow elutriation centrifugation. Fraction 1 (Fr‐1) was lymphocyteenriched, fraction 2 (Fr‐2) was a mixture of lymphocytes and myelomonocytic cells, and fraction 3 (Fr‐3) was enriched for myelomonocytic cells. The extent of T cell depletion was determined by limiting dilution analysis of the growth of T cells. The cells in each group were stimulated with phytohemagglutinin and cultured in the presence of interleukin‐2. These conditions allowed clonogenic growth of one of three T cells purified from peripheral blood. After a 3‐week culture period, replicate wells were scored for growth and precursor frequency was determined. The frequency of T cells in BMBC was 1/22 (n = 4), 1/16 in FR‐1 (n = 4), and 1/246 in Fr‐2 (n = 2), and in Fr‐3 it ranged from 1/3,000 to 1/10,000 (n = 4). For comparison, depletion of T cells from bone marrow by cytofluorographic sorting with OKT‐11 and twice E‐rosetting yielded 98.1% and 97.4% removal of T cells, respectively. Thus, the clonogenic assay assured that more than 99.3% of the original T cells were depleted by elutriation; however, hematopoietic progenitor assay (CFU‐GM, CFU‐GEMM, and BFU‐E) showed no enrichment in any particular fraction, implying that the progenitors measured in this assay are of diverse sizes. Elutriation represents a useful method for depletion of T cells from human bone marrow. Limiting dilution assay of T cell growth is a sensitive measure to monitor T cell depletion.
Long‐term follow‐up of prosthetic joint replacement in hemophilia
Authors: Rana Nasim A. , Shapiro Gary R. , Green David 
Published: February 2007

AbstractWe evaluated the outcome of seven severe hemophilic patients who underwent four total hip and four total knee arthroplasties since 1976. These patients have been followed at regular intervals over a period of 2.5–9.5 years (mean 5.8). Of the four total hip replacements, one had to be removed because of loosening and secondary infection 3 years after the initial surgery but was salvaged by pseudoarthrosis; the other three are pain‐free and radiologically stable and have an excellent range of motion 2.5, 5, and 7 years postoperatively. Of the four total knee replacements, one had to be removed because of infection but was successfully salvaged by arthrodesis; one patient has loose components, but the prosthesis is still functional; and the final patient with bilateral knee prostheses is pain‐free with limited but functional range of motion. Clotting‐factor replacement therapy was effective in controlling intraoperative bleeding, even in a patient with an inhibitor, and only one procedure was complicated by hematoma formation. We conclude that prosthetic joint replacement may be safely performed in hemophilic patients but should be reserved for those who have limited function because of severe pain, joint destruction, and deformity. Total hip arthroplasty is as successful in these patients as in nonhemophiliacs. Total knee arthroplasty provides relief of pain, reduces the frequency of hemarthroses, and corrects most of the deformity, but it is usually associated with a limited range of motion.
Management of treatment failures in thrombotic thrombocytopenic purpura
Authors: Liu Edison T. , Linker Charles A. , Shuman Marc A. 
Published: February 2007

AbstractThe prognosis and optimal management of patients with thrombotic thrombocytopenic purpura (TTP) who fail initial therapy with plasmapheresis or splenectomy are unclear. We report our experience with eight patients with TTP who did not respond to initial therapy. Seven patients achieved complete remission when alternate therapy was started soon after the recognition of initial treatment failure. One patient who received no alternative therapy died of progressive TTP. Our cases combined with those in the literature indicate a 74% salvage rate for patients who fail initial treatment for TTP. The combination of splenectomy, dextran, and steroids appears to be an effective treatment for patients with TTP who fail to respond adequately to plasmapheresis.
Isolated anterior chamber relapse in acute monoblastic leukemia
Authors: Hertler Andrew A. , Rosenwasser George O. , Gluck W. Larry 
Published: February 2007

AbstractA case of acute monoblastic leukemia relapsing in the anterior chamber of the eye is described. Despite rigorous evaluation, no systemic or central nervous system involvement was detected. All previously reported cases of anterior chamber infiltrates in AML have had coexistent systemic or central nervous system disease.
Effects of a 4‐week freshwater fish (Trout) diet on platelet aggregation, platelet fatty acids, serum lipids, and coagulation factors
Authors: Atkinson P. M. , Wheeler M. C. , Mendelsohn D. , Pienaar N. , Chetty N. 
Published: February 2007

AbstractEight healthy subjects consumed a diet in which all animal products were replaced by 750 g/day of freshwater trout. Platelet eicosapentaenoic acid (EPA) as a percentage of total platelet fatty acids rose from a prediet level of 0.2±0.4% to 3.4±1.6% after 4 weeks on the diet. Platelet arachidonic acid remained unchanged. Platelets became more hyperaggregable to collagen (p<0.025) but became hypoaggregable to arachidonic acid (p< 0.05). The Ivy bleeding time became prolonged rising from a mean of 148 seconds before the diet to 168 seconds at 2 weeks and 202 seconds at 4 weeks. Serum lipids, coagulation profiles, and blood pressure remained unchanged. The changes induced by this diet were much less marked than changes induced by marine fish diets and diets supplemented by marine fish oil extracts, despite the fact that an equivalent amount of EPA was consumed and incorporated into platelets. These findings suggest that other substances, in addition to n‐3 unsaturated fatty acids, may be responsible for the antithrombotic properties of marine fish.
Clinical efficacy of desmopressin acetate for hemostatic control in patients with primary platelet disorders undergoing surgery
Authors: Kentro T. B. , Lottenberg R. , Kitchens C. S. 
Published: February 2007

AbstractDesmopressin acetate (DDAVP) is efficacious in patients with von Willebrand's disease. It additionally appears to have value in patients with uremic or aspirininduced platelet dysfunction. We report here three patients with primary platelet defects who had previously experienced grossly inadequate hemostasis to whom we administered DDAVP. Each successfully underwent surgical procedures with DDAVP as the only hemostatic agent. Although the mechanism of these salutary effects is unclear, DDAVP may exert an influence directly on the endothelium independent of correcting abnormalities of the factor VIII: von Willebrand complex associated with von Willebrand's disease.
Platelet dysfunction associated with wilms tumor and hyaluronic acid
Authors: Bracey Arthur W. , Wu Alan H. B. , Aceves Javier , Chow Thomas , Carlile Shirley , Hoots W. Keith 
Published: February 2007

AbstractAcquired von Willebrand disease (AVWD) has been described in two cases of nephroblastoma. We studied a patient with nephroblastoma who presented with a coagulopathy suggestive of AVWD. The subject had undetectable levels of F. VIIIR: Ag, diminished F. VIIIR: WF (16.3%), F. VIII: C activity (37%), and lack of platelet aggregation to ADP, epinephrine, collagen, and arachidonic acid. These results were associated with abnormally high serum levels (850 mg/dl) of hyaluronic acid (HA), which made the patient's serum hyperviscous. Examination of the neoplasm revealed HA in the tumor matrix. All abnormalities of coagulation resolved after chemotherapy and extirpation of the neoplasm, which produced normal serum HA levels.To study the effects of HA on platelet function, we added HA to normal platelet‐poor plasma (NPP), which rendered F. VIIIR: Ag undetectable; treatment of HA with hyaluronidase eliminated F. VIIIR: Ag assay interference caused by HA. F. VIII: C activity decreased in vitro when HA was mixed with normal platelet‐poor plasma (NPP). HA reduced the initial slope of normal platelet aggregation. Partial correction of platelet aggregation occurred after hyaluronidase treatment of HA‐spiked PRP. Experiments in rabbits exposed to HA (serum level 400 mg/dl) demonstrated abnormalities similar to those noted in the patient. Shear rate studies of whole blood containing HA (500 mg/dl) yielded high shear stress, 27‐136 dynes/cm2 over shear rates of 10‐216 sec‐1. We conclude that the coagulopathy demonstrated in this case is secondary to hyperviscosity produced by elevated levels of HA, which interferes with the assay for F. VIIIR: Ag. Thus the acquired coagulopathy associated with other cases of nephroblastoma may present as spurious von Willebrand disease.
A variant of type II von willebrand disease with an abnormal triplet structure and discordant effects of protease inhibitors on plasma and platelet von willebrand factor structure
Authors: Gralnick Harvey R. , Williams Sybil B. , McCkeown Laurie P. , Maisonneuve Pascal , Jenneau Christine , Sultan Yvette 
Published: February 2007

AbstractWe have characterized the plasma and platelet von Willebrand factor (vWf) multimeric structure of a patient with von Willebrand disease (vWd) as having a long bleeding time, no aggregation of her platelet‐rich plasma (PRP) to ristocetin, and very low plasma and platelet von Willebrand antigen (vWf Ag) and vWf activity. The abnormalities of the plasma and platelet vWf have not been previously described. In particular, the patient's plasma and platelet vWf lacked the intermediate and largest vWf multimers and the slowest migrating minor band (band 1) of the triplet was markedly diminished compared to the major band (band 2) and the fastest migrating band of the triplet (band 3). A similar multimeric structure was seen in the platelet vWf. Collection of the patient's blood in protease inhibitors prior to the analysis of the platelet and plasma vWf structure revealed that the plasma vWf multimeric structure did not change, while the patient's platelet vWf showed a marked change with the appearance of the intermediate and large multimers and almost total disappearance of the abnormal multimeric structure. Direct comparison of this patient's plasma with previously reported cases of type IIA, IIC, and IID revealed marked differences in their multimeric organization compared to our patient. Employing the presently accepted convention, we have designated this variant type of vWd type IIG.
An acquired inhibitor to factor VIIIC in a non‐hemophiliac: Twenty years of observation and characterization
Authors: Coots Macie C. , Glueck Helen I. 
Published: February 2007

AbstractA non‐hemophilic patient with an acquired inhibitor to factor VIIIC was initially diagnosed in this laboratory 20 years ago at age 63. Following its initial appearance in 1963, the inhibitor was detectable on two other occasions. We believe that this is the longest duration such an inhibitor has persisted. No sample remained from his initial admission; however, samples were available from the last 8 years of his life for retrospective study. Preparative isofocusing, affinity chromatography, and immunoglobulin subtyping were used to determine the similarities and/or differences in the inhibitor over these 8 years.The following similarities and differences were observed in both the 1975 and 1983 plasmas. Isofocusing showed that both plasmas contained peaks of inhibitory activity with pIs of 7.25, 8.26, and 8.88; the 1975 sample in addition contained two peaks with pIs of 7.77 and 9.45, whereas two other peaks with pIs of 7.64 and 7.85 were found in the 1983 sample.For the final characterization, each isofocused inhibitory peak was eluted from Protein A Sepharose and incubated with antisera to determine the immunoglobulin subtype. Each peak consisted of mixtures of IgG1 and IgG4 with both kappa and lambda light chains.It was concluded that the inhibitor was polyclonal, based on the presence of inhibitory peaks with different pIs and immunoglobulin subtypes. These findings support the conclusion that the development of and changes in the inhibitor was a dynamic process with some inhibitors (antibodies) persisting, while at the same time others with different characteristics were being formed.
Isolated leukemia cutis—A case report
Authors: Haubenstock A. , Zalusky R. , Ghali V. S. , Mernick M. H. , Malamud S. C. , Stein J. J. 
Published: February 2007

AbstractA 67‐year‐old woman with acute myelomonocytic leukemia had a clinical course characterized by the initial appearance of leukemia cutis without bone marrow involvement. When marrow involvement subsequently occurred, induction chemotherapy cleared all manifestations of the illness. Shortly thereafter, while blood and bone marrow remained in remission, the skin lesions reappeared. The introduction of 6‐thioguanine, as part of the maintenance treatment protocol, resulted in the rapid and permanent disappearence of the leukemic skin infiltrates.
Inhibitory effects of TTP sera on binding of anti‐platelet glycoprotein IIb‐IIIa monoclonal antibodies to human vascular endothelial cells
Authors: Nakajima Tokuo , Koyama Tetsuji , Kakishita Eizo , Nagai Kiyoyasu 
Published: February 2007

AbstractThe binding of anti‐human platelet glycoprotein (GP) IIbIIIa monoclonal antibodies to human umbilical vein endothelial cells (HUVE) was studied. Scatchard analysis using 125I‐anti‐platelet GPIIb‐IIIa monoclonal antibody showed that the maximum binding capacity (B max) was 8 × 104/cell and Kd was 40.2 nM. The binding rate of 125I‐anti‐platelet GPIIbIIIa monoclonal antibodies to HUVE treated with TTP sera was 11.6 ± 2.8%, compared with the 24.0 ± 7.5% observed for HUVE treated with normal sera. These findings showed that there are GPIIb‐IIIa on HUVE and that TTP sera may contain anti‐platelet GPIIbIIIa antibodies.
Acute non‐lymphocytic leukemia (ANLL) following treatment with dacarbazine for malignant melanoma
Authors: Carey Robert W. , Kunz Victoria S. 
Published: February 2007

AbstractANLL followed a brief period of aplastic anemia in a man treated intensively 4 years and 3 months previously with dacarbazine as “adjuvant” therapy for malignant melanoma. This is the first reported instance in which the latency between drug exposure and onset of leukemia strongly implicates dacarbazine as a leukemogenic agent.
Pocked erythrocyte counts in patients with hereditary spherocytosis before and after splenectomy
Authors: Buchanan George R. , Holtkamp Christine A. 
Published: February 2007

AbstractThe pocked (pitted or vacuolated) erythrocyte count has become increasingly utilized as a simple inexpensive test of splenic reticuloendothelial function. Values are less than 2.0% in normal subjects and 20 to 70% following splenectomy. Because scant and conflicting data are available about pocked erythrocyte measurements in hemolytic anemias other than the hemoglobinopathies, we performed pocked erythrocyte counts in 27 patients with hereditary spherocytosis. Prior to splenectomy patients often had elevated values (mean 4.9%). This unexpected observation suggests that hemolytic anemia may result in congestion of the red pulp and/or induce mild splenic reticuloendothelial blockade. As expected, but contrary to a previous report, pocked erythrocyte values following splenectomy were markedly increased (mean 54.9%).
Persistent remission of adult chronic autoimmune thrombocytopenic purpura after treatment with high‐dose intravenous immunoglobulin
Authors: Bierling Philippe , Divine Marine , Farcet Jean‐Pierre , Wallet Patrick , Duedari Najib 
Published: February 2007

AbstractTransient efficacy of high‐dose intravenous immunoglobulin infusions (HDI) is widely demonstrated in autoimmune thrombocytopenic purpura (AITP). A trial involving repeated injections of HDI was conducted in two adult patients with chronic AITP in order to evaluate their long‐term efficacy in refractory forms and their ability to obviate splenectomy. Both patients received 2 g/kg body weight of polyvalent intact intravenous immunoglobulin followed by seven and eight boosters (0.8 to 1.2 g/kg body weight) when the platelet count decreased. It was found that the injections could be spaced progressively. The platelet count remained normal 200 and 800 days after the end of the treatment. The demonstration of the possible long‐term efficacy of repeated injections of intravenous immunoglobulin encourages us to recommend this safe therapy for patients with chronic AITP.