bluuebunny/crossref_metadata_2025_split huggingface dataset:
Rapid improvement of thrombotic thrombocytopenic purpura with vincristine and plasmapheresis
Authors: Welborn Jeanna L. , Emrick Pam , Acevedo Maxine 
Published: February 2007

AbstractMany patients with thrombotic thrombocytopenic purpura (TTP) fail to respond to daily plasmapheresis and the results of alternative treatments have been inconsistent. Vincristine was given weekly with continued plasmapheresis to eight patients who were refractory to plasmapheresis, antiplatelet agents, and/or corticosteroids. A rapid response to the vincristine occurred in all eight patients in 5 days with a marked rise in the platelet count and resolution of symptoms. The results in these patients suggest that early initiation of vincristine therapy in conjunction with plasmapheresis is useful in TTP.
Discordance between phenotype and function of japanese adult T cell leukemia cells
Authors: Imamura Nobutaka , Mtasiwa Deodatus M. , Ota Hiromi , Kuramoto Atsushi 
Published: February 2007

AbstractPhenotypes of cells from 12 patients with ATL were analysed by means of a fluorescence‐activated cell sorter by utilizing a panel of monoclonal antibodies. A majority of the cells from peripheral blood coexpressed the antigens against MAbs CD2, CD3, CD4, CD5, Ti (WT31), CD25, CD38, CD45, and CD29, but did not express the antigens against CD1, CD13, CD14, CD33, CD36, CD10, CD19, CD20, CD21, CD24, CD41, CD42, CD45RA, CD56, and CD57. The expression of antigen for TQ‐1 or Leu8 was variable. Surface immunoglobulins were not detected. Phenotypes of cultured cells established by utilizing recombinant interleukin II were similar to those of the uncultured peripheral blood lymphoid cells except for the lack of expression of CD8. By means of two‐color fluorescence, the ATL cells possessing CD4 in peripheral blood and culture coexpressed CD29, but did not express CD45RA. The suppression of PWM‐induced B‐cell immunoglobulin synthesis by normal T and B cells was found in five cases in the presence of ATL cells. The ATL cells demonstrated helper T‐cell phenotypes (CD4+, CD29+) with suppressor function, paradoxically.We conclude that the phenotype of the ATL cells was CD4+, CD29+, and CD45RA− but that the function of these cells was of suppressor T‐cells. Our results inevitably suggest the possible existence of suppressor T‐cells with CD4+, CD29+ phenotype in persons without evidence of any underlying hematologic disorder.
Th polyagglutination with fatal outcome in a patient with massive intravascular hemolysis and perforated tumor of colon
Authors: Levene N. A. , Sigler E. , Berrebi A. , Merhav H. , Levene C. , Gekker K. 
Published: February 2007

AbstractPolyagglutination is a rare disorder which has been associated with intravascular hemolysis (Levene et al.: Transfus Med Rev 2:176–185, 1988). In this condition cryptantigens exposed on the red blood cell membrane agglutinate with compatible sera and with lectins. A 76 year‐old man with an acute abdomen due to a perforated tumor of the colon and severe fatal intravascular hemolysis is described. Th polyagglutination of the red blood cells was found.
Identification of p70 and p80 Associations With Class II MHC Molecules and Ii
Authors: Sorli Christopher H. , Reisert Patricia S. , Humphreys Robert E. , Welch William J. 
Published: February 2007

AbstractTwo proteins, p70 and p80, were found in chemically crosslinked complexes with class II MHC molecules and Ii after 3–12 hr labelings with [35S]methionine. Two‐dimensional, nonreduced/reduced SDS gel electrophoresis of immunoprecipitated complexes revealed 1) endogenous disulfide linkages between Ii‐Ii and Ii‐p70 and 2) chemically crosslinked, nearest neighbors of α‐β, α‐Ii, Ii‐p70, and α‐p80. Although such nearest neighbors within multimeric complexes were identified as dimers in nonreduced/reduced 2D gels, stoichiometries could not be determined in the high molecular weight complex(es), which included α, β, Ii, p70, and p80, and were not separated in the first dimension. p80 was not the chondroitin‐sulfate form of Ii (Ii‐CS) because it was not electrophoretically heterogeneous and was not sensitive to chondroitinase ABC. p70 was not hsp72/74 detected with C92 or N27 mAbs, and p80 was not BiP detected with its respective mAb. While only these two proteins associated prominently with class II MHC antigens and Ii late after synthesis, their functions are unknown.
Leukocyte depletion filters: A comparison of efficiency
Authors: Bodensteiner David C. 
Published: February 2007

AbstractFour commercially available leukocyte depletion filters were evaluated using a flow cytometric technique to determine the efficiency of each type of filter. The Sepacell R‐500A® and the PALL RC50® were the most efficient in leukocyte depletion having a mean depletion percentage of 99.3% and 99.5%, respectively. The PALL RC100® which is used for two units also had a 99.3% depletion with the first unit but with the second unit the depletion dropped to 94.2%. The Imugard IG‐500® had a 97.3% depletion. Red cell recovery ranged from 87.4% for the PALL RC50® to 92.2% for the Sepacell R‐500A®.
Major prognostic factors of Japanese patients with lymphoma‐type adult T‐cell leukemia
Authors: Shimamoto Yoshinori , Suga Kenji , Nishimura Junji , Nawata Hajime , Yamaguchi Masaya 
Published: February 2007

AbstractFifty‐three Japanese patients with the lymphoma‐type adult T‐cell leukemia (ATL) were analyzed to study the prognostic value of various clinical findings recorded at the time of diagnosis. All patients were positive for human T‐cell leukemia virus type I (HTLV‐I) antibody and demonstrated monoclonal integration of HTLV‐I proviral DNA in their malignant cells. The important individual variables detected in a previous univariate analysis were placed in a multiple regression model to identify the major prognostic factors for survival. This analysis showed that serum lactate dehydrogenase (LDH), calcium, and total protein levels had a strong predictive relationship with the length of survival (in descending order of importance). Among the 53 patients, 46 were dead at the time of analysis. The cause of death in relation to the duration of survival is also reviewed in this article.
Acute myelomonocytic leukemia: An immunoelectron microscopic study
Authors: Saito Nagahito 
Published: February 2007

AbstractAcute myelomonocytic leukemia (M4; French‐American‐British classification) is light microscopically defined as the leukemia constituting leukemic cells in both granulocytic and monocytic lineages. Therefore, the characteristics of M4 have not been fully elucidated. The author previously indicated that normal neutrophilic granulocytes could be ultrastructurally differentiated from normal monocytes by the double staining of lactoferrin and lysozyme. In this investigation, the ultrastructural localization of both proteins was observed in order to make the outline of M4 clear. The leukemic cells in acute myeloid leukemia (M2) were also examined in comparison with those in M4. The leukemic cells in M4 showed the double stainability of lactoferrin and lysozyme, and the positive reactions were localized in the cytoplasmic matrix and in the granules. The staining pattern was similar to that in M2. The coexistance of lactoferrin and lysozyme in the leukemic cells in M4, which has ultrastructurally the monocytic characteristics, implied that the leukemic cells also possess the characteristics of the cells in the granulocytic lineage. This suggests that the presence of the various leukemic cells signifies the diversely abnormal maturations in vivo of the monocytes/granulocytes precursor cell and that M4 consists of not two kinds of distinguishable cells of granulocytic and monocytic lineages but various consecutive cells based on a malignant transformation of the precursor cell.
Developmental and inducible patterns of human θ1‐globin gene expression in embryonic/fetal and adult erythroid cells
Authors: Mamalaki Avgi , Anagnou Nicholas P. , Moschonas Nicholas K. 
Published: February 2007

AbstractHuman theta (θ1)‐globin gene represents a member of the α‐like globin gene family residing on chromosome 16. θ1‐Specific transcripts have been detected so far only in erythroid tissues and in erythroleukemia K562 cells. To investigate systematically its inducible expression and developmental specificity, we analyzed at the RNA level five additional human erythroleukemia cell lines with diverse developmental globin programs, two somatic cell hybrids between K562 and mouse erythroleukemia (MEL) cells, a human fetal liver x MEL somatic cell hybrid, and reticulocytes and bone marrow cells from normal adults. θ1‐Globin gene was expressed in all cell types. Inducible expression (two‐to sixfold) was documented both in HEL and K562 erythroleukemia cells after 5‐azacytidine treatment. Like K562 cells, HEL cells also displayed hemin‐inducible θ1‐globin gene expression. Following transfer of human chromosome 16 from embryonic/fetal K562 to the adult MEL cells, θ1‐globin gene remained active but lost its potential for inducibility, suggesting probably a trans regulation mechanism. Higher levels of θ1 mRNA were found in fetal liver cells compared with trace amounts in reticulocytes and normal adult bone marrow cells. These data clearly show that in contrast to the embryonic and adult patterns of expression of ζ and α‐globin genes, respectively, θ1‐globin gene displays a different profile, being active predominantly during the early stages of ontogeny, switching to lower levels of expression in adulthood.
Massive mediastinal extramedullary hematopoiesis in hereditary spherocytosis: A case report
Authors: Bastion Yves , Coiffier Bertrand , Felman Pascale , Assouline David , Tigaud Jean‐Dominique , Espinouse Daniel , Bryon Paul‐André 
Published: February 2007

AbstractA case of extramedullary hematopoiesis (EMH) with a massive mediastinal mass in a 72‐year‐old woman with hereditary spherocytosis is reported. Several cases of EMH have been described in nonsplenectomized adults with hereditary spherocytosis, and it is thought to be a consequence of long‐term stimulation of erythropoiesis. The exact mechanism remains controversial. EMH should be considered in the differential diagnosis of an asymptomatic paravertebral mass.
Extranodal T‐cell lymphoma mimicking malignant histiocytosis
Authors: Sun Tsieh , Brody Judith , Susin Myron , Boss Eleonore , Moskowitz Lisa , Lichtman Stuart 
Published: February 2007

AbstractWe report a case of extranodal T‐cell lymphoma with fever, hepatosplenomegaly, pancytopenia, and diffuse sinusoidal infiltration of the spleen, liver, and bone marrow by the tumor cells, mimicking malignant histiocytosis. This is the second case of T‐γ (T‐cell suppressor) lymphoma resembling the case reported by Kadin et al. [N Engl J Med 304:648, 1981]. The lack of lymph node involvement in this case supports the theory that this type of lymphoma arises in the spleen. This paper draws attention to the extranodal T‐cell lymphoma groups that mimic malignant histiocytosis and to the need of immunophenotyping for a correct diagnosis. The causes for the absence of T‐cell receptor gene rearrangement in T‐cell tumors are discussed.
Evaluation of an automated bleeding time device in the newborn
Authors: Andrew Maureen , Paes Bosco , Bowker Jan , Vegh Patsy 
Published: February 2007

AbstractSick infants frequently develop impaired hemostasis as a secondary event. Abnormal platelet‐vessel wall interaction contributes to the hemorrhagic side effects. Currently, the bleeding time is the best measure of in vivo platelet participation in small vessel hemostasis. Bleeding times in the newborn have been previously determined with an automated device designed for the adult or with nonautomated devices. The automated bleeding time device for the adult is not suitable for the infant (length 5 mm, depth 1 mm). We evaluated the performance of an automated bleeding time device designed specifically for the newborn making a cut 2.5 mm in length and 0.5 mm in depth. The results with the automated device showed a strong correlation with the modified template bleeding time device previously used for the newborn. The normal range was shorter and the sensitivity to abnormal vessel wall interaction was greater with the automated device. As well, the ease of determining the bleeding time with the automated device was considerably improved over the template device.
Induction of alkaline phosphatase activity in chronic myeloid leukemia cells: In vitro studies and speculative hypotheses
Authors: De Renzo A. , Micera V. , Vaglio S. , Luciano L. , Selleri C. , Rotoli B. 
Published: February 2007

AbstractThree patients with Ph chromosome + chronic myeloid leukemia (CML) in chronic phase suffered from intercurrent pleuritis of undefined origin. At that time, leucocyte alkaline phosphatase activity (LAPA) score was low for circulating neutrophils, but high for those from pleural effusion. LAP negative circulating CML granulocytes were incubated with the pleural liquid: after 40–70 hr, almost all were intensely LAP positive. This finding suggests that the low LAPA score in resting CML neutrophils is attributable to the absence of appropriate stimuli rather than to an incapacity to synthesize the enzyme.
A fatal case of low titer anti‐PR cold agglutinin disease
Authors: Rousey Steven R. , Smith Roy E. 
Published: February 2007

AbstractCold agglutinin disease typically produces a low‐grade hemolytic anemia. We describe herein the unusual occurrence of an anti‐PR cold agglutinin with high thermal amplitude which caused significant and ultimately fatal hemolytic anemia at very low titers.
Circulating hyaluronan in the myelofibrosis/osteomyelosclerosis syndrome and other myeloproliferative disorders
Authors: Hasselbalch H. , Junker P. , Lisse I. , Lindqvist U. , Engstr Laurent A. Öm 
Published: February 2007

AbstractThe serum concentration of hyaluronan (HYA) was determined in 59 patients with various myeloproliferative disorders, including 33 patients with idiopathic myelofibrosis. In 18 patients the serum concentration of the aminoterminal propeptide of type III procollagen (PIIINP) was measured concomitantly. Raised serum HYA levels were seen in patients with active disease compared with age‐matched healthy subjects, whereas no significant difference in serum HYA was seen between patients with stable disease and age‐matched controls. Serum HYA concentrations correlated significantly with the leukocyte count (rho = 0.38; P < 0.02) and with the serum concentration of PIIINP (rho = 0.50; P < 0.001). During cytotoxic treatment, the serum HYA and PIIINP concentrations decreased in concert with declining leukocyte counts. These findings suggest that clonal expansion is accompanied by a bone marrow stromal reaction similar to the repair processes following injury to soft connective tissues. The relatively modest changes in serum HYA with frequent overlaps between patient categories and healthy subjects imply that the clinical utility of single determinations of serum HYA in the present disease groups is restrained. On the other hand, sequential measurements of HYA may provide a reflection of the myeloproliferative process in individual patients.
Impaired production of burst promoting activity by blood mononuclear cells from chronic uremic patients
Authors: Kawano Yoshifumi , Takaue Yoichi , Minakuchi Jun , Abe Takanori , Matsunaga Keiko , Hirao Atsushi , Watanabe Tsutomu , Hirose Masao , Ninomiya Tsuneo , Kawashima Shuh , Kuroda Yasuhiro 
Published: February 2007

AbstractThe ability of blood mononuclear cells (MNC) to produce burst promoting activity (BPA) was evaluated in 31 patients with chronic renal failure. The BPA of cells from uremic patients, with or without hemodialysis, was consistently lower than that of 17 normal donors (mean 64%, P < 0.01). Coculture of MNC with recombinant erythropoietin (rEpo) in vitro did not increase BPA production. Five of 31 patients received in vivo treatment with rEpo (1,500 units × 3/week) and showed therapeutic benefit, but in all patients the BPA production remained low. On the other hand, in four patients who were on a hemodialysis protocol and subsequently underwent renal transplantation, impaired BPA production was resolved quickly, and at the same time the number of circulating BFU‐E and the hemoglobin level increased toward normal ranges. Furthermore, such impaired BPA production was not observed in patients receiving continuous ambulatory peritoneal dialysis. These observations suggest that decreased production of BPA may play a role in the development of anemia associated with chronic uremic patients, and the correction of BPA production by the improvement of hemodialysis procedure may result in more effective therapy with rEpo for those patients.
Superwarfarin ingestion and detection
Authors: Routh Charles R. , Triplett Douglas A. , Murphy Michael J. , Felice Larry J. , Sadowski James A. , Bovill E. G. Ted 
Published: February 2007

AbstractBecause of the emergence of warfarin resistance, new potent long‐acting anticoagulants are now readily available in several over‐the‐counter rodenticide products. The availability of these “superwarfarin” compounds has led to accidental and purposeful human ingestions, one of which has resulted in a death. We summarize the prior case reports and report a second death. In addition, we report the availability of an assay to detect the presence of brodifacoum (a superwarfarin compound) in human plasma and tissues.
Acquired selective factor X deficiency in acute nonlymphocytic leukemia
Authors: Caimi M. T. , Redaelli R. , Cattarieo D. , Nosari A. M. , Baudo F. , De Cataldo F. 
Published: February 2007

AbstractA case of acute nonlymphocytic leukemia (ANLL) and selective acquired FX deficiency at presentation is reported. The deficiency was not corrected by the infusion of fresh frozen plasma, but the patient's plasma had no neutralizing activity in vitro. The cause of the deficiency is unknown.
Hiv‐associated Hodgkin disease: A clinical study of 18 cases and review of the literature
Authors: Gold Jay E. , Altarac David , Ree H. J. , Khan Amjad , Sordillo Peter P. , Zalusky Ralph 
Published: February 2007

AbstractIntermediate‐ and high‐grade B‐cell non‐Hodgkin lymphoma (NHL) occurring in a human immunodeficiency virus (HIV)‐infected patient is considered diagnostic of the acquired immunodeficiency syndrome (AIDS). Other neoplasms (both hematopoietic and nonhematopoietic) have also been reported in patients with HIV infection, although none except Kaposi sarcoma carries the same diagnosis of AIDS as B‐cell NHL in an HIV‐infected host. There have been previous reports in the literature of Hodgkin disease (HD) in HIV‐infected patients. We describe our clinical and pathological experience with HD from 1984–1989, in 18 patients with documented HIV infection and also review the literature on HD in HIV‐infected patients. Almost all patients described herein presented with advanced disease and mixed cellularity histology and did very poorly despite some good initial responses to therapy. By statistical analysis, we found that the patients with HIV‐associated HD had a strong tendency to be outside the age range seen in non‐HIV‐associated HD (P <0.005). We also discuss the possible relationship between HIV and HD and consider whether HIV‐associated HD, like B‐cell NHL, is a manifestation of AIDS.
Relationship between CD45 antigen expression and putative stages of differentiation in B‐cell malignancies
Authors: Caldwell Charles W. , Patterson William P. 
Published: February 2007

AbstractThe cell‐surface antigen CD45 is a complex family of high‐molecular‐weight glycoproteins expressed on all lymphohematopoietic cells, but not in the same molecular isoform. This antigen complex is known to exhibit protein tyrosine phosphatase (PTPase) activity and appears to have a role in regulation of cell differentiation. In that CD45 expression parallels stages of differentiation in normal bone marrow B cells, it was of interest to evaluate this process in malignant B cells. Monoclonal antibodies (MoAbs) were used to investigate the quantitative expression of CD45 and CD45RA on the B cells of lymphoid leukemias. Employing standardized flow cytometric methods, it was found that the fluorescence intensity (FI) of immunostained malignant B cells, as a reflection of the antigen content, demonstrated correlations with the putative stage of cell differentiation for malignancies at the earlier stages, but at the later stages, a progressive loss of CD45 was observed. Since this antigen family has been found to display PTPase activity, further investigation of CD45 alterations in malignancies may provide insight into potential regulatory disturbances.
Sickle cell anemia with few painful crises is characterized by decreased red cell deformability and increased number of dense cells
Authors: Ballas Samir K. 
Published: February 2007

AbstractThe frequency and severity of the painful sickle cell crisis vary greatly among affected patients. Aside from a high level of Hb F(>20%) there is no established parameter which may modulate the clinical severity of the disease. In this paper we describe two groups of adult patients with homozygous SS and present their characteristics. The division into these two groups was on the basis of relatively low RBC deformability (≤37% of control) and high RBC deformability (>65% of control) in the steady state. None of the patients had α‐gene deletion and all had Hb F level <6.0%. Each patient was followed for a minimum of 3 years. The number of dense cells was quantitated by centrifugation on discontinuous Stractan gradient. RBC deformability index in isotonic medium (DI 290) was determined by ektacytometry and expressed as % of control. The patients with low RBC deformability had significantly less painful crises and more leg ulcers than those patients with high RBC deformability. The average number of dense cells was 22.2% and 9.8% of total circulating cells in the first and second group respectively. Moreover, the group with high red cell deformability had 33% mortality during the study period whereas no deaths occurred in the group with low RBC deformability.The data indicate that there is a subset of patients with SS who have relatively few painful crises despite low Hb F level. We wish to designate these by the acronym MIDDD syndrome: Mild disease as far as painful crises are concerned, Increased number of Dense cells, and Decreased red cell Deformability. In addition these patients have high incidence of leg ulcers, have low incidence of urinary tract infection, and less mortality. Cellular factors seem to contribute to the incidence of painful crises.
Blood zinc protoporphyrin is elevated only in sickle cell patients with low fetal hemoglobin
Authors: Hirsch Rhoda Elison , Pulakhandam Usha Rao , Billett Henny H. , Nagel Ronald L. 
Published: February 2007

AbstractIncreased levels of various porphyrin species have been reported in sickle cell anemia (SS) patients in the absence of lead poisoning and iron deficiency anemia, but conflicting data remain. Suspecting that SS patients may be heterogenous for this abnormality, we have studied zinc protoporphyrin (ZPP) and protoporphyrin IX (PPIX) blood levels and find abnormally elevated levels of ZPP in those with low peripheral fetal hemoglobin (%HbF) levels. Two groups exist: one with <9% HbF and elevated ZPP, and one with ≥9% HbF and normal ZPP levels (P < 8.1 × 10−4). There is a strong negative correlation of ZPP levels with %Hb F (r = –0.83, P < 8.0 × 10−5), and a moderate one with total hemoglobin levels (r = –0.55, P < 0.05). These results suggest that ZPP may indeed contribute to the pathophysiology of the disease and/or serve as a marker of the severity of the disease.