bluuebunny/crossref_metadata_2025_split huggingface dataset:
Abnormalities in lymphocyte profile and specificity repertoire of patients with Waldenstrom's macroglobulinemia, multiple myeloma, and IgM monoclonal gammopathy of undetermined significance
Authors: Pilarski Linda M. , Andrews E. Joy , Serra Horacio M. , Ledbetter Jeffrey A. , Ruether Bernard A. , Mant Michael J, 
Published: February 2007

AbstractThe characteristics of T and B lymphocyte profile and B lymphocyte specificity repertoire were compared in patients with Waidenstrom's macroglobulinemia (WM), IgM monoclonal gammopathy of undetermined significance (IgM MGUS), multiple myeloma (MM), and age‐matched normal subjects. Patients with MM had both significantly reduced frequency and number of sIg+ (surface Ig) B cells, whereas patients with WM and IgM MGUS had a reduced frequency but normal numbers of sIg+ B cells in circulation as detected in a capping assay. WM was distinguished by the large numbers of cells in the peripheral blood lymphocyte (PBL) pool that expressed CDS (BA‐2) and CD24 (BA‐1) and were monoclonal, based on light chain analysis using flow cytometry. The profile of T lineage cells showed that the ratio of CD4:CD8 was significantly reduced in both MM and WM due to a reduction in the CD4 set. The CD4+ cells were qualitatively abnormal as well, with an enriched proportion of the 4B44 (CDw29) subset and decreased proportion of the Lp220+ (CD45R) subset. This appeared to be an effect of the disease process on the relatively immature Lp220+ set. From clonal analysis, those patients with WM or IgM MGUS (unlike MM patients) did not exhibit enhanced reactivity with auto‐lg determinants, and most WM patients (7/8) and half of the IgM MGUS patients (3/6) did not have enriched proportions of B cells reactive to tetanus toxoid (TT). The TT‐specific B cells in both WM and IgM MGUS, in contrast to MM, appeared fulfy functional in secretion of anti‐TT IgM in vivo. We speculate that the more severe immunodeficiency in MM may be controlled or exacerbated by the presence of an anti‐Ig network. The absence of this network in WM allows a relatively more effective immune response, but the immunodeficency that is observed in these patients involves some abnormality in normal lymphocyte differentiation (is also present in MM).
Studies of the proliferation and differentiation of immature myeloid cells in vitro. I. chronic myelogenous leukemia
Authors: Wang Zique , Gao Xue Zhi , Preisler Harvey D. 
Published: February 2007

AbstractImmature cells prepared from chronic myelogenous leukemic patients in the chronic or blastic phase of their disease were placed into suspension culture in vitro and studied over a 2‐week period. Regardless of the status of the disease, the immature cells proliferated and maintained good viability. In contrast, evolution of the disease toward blastic crisis was associated with a progressive loss in the ability of the immature cells to differentiate. Cell proliferation and differentiation were independent of added growth factors.
Diffuse panbronchiolitis as a pulmonary complication in patients with adult T‐cell leukemia
Authors: Ono Kazutoshi , Shimamoto Yoshinori , Matsuzaki Miwako , Sano Masayuki , Yamaguchi Tsuneko , Kato Osamu , Yamada Hozumi , Yamaguchi Masaya 
Published: February 2007

AbstractForty‐three cases of adult T‐cell leukemia (ATL) admitted to our hospital between 1982 and 1987 were studied. Three of those were found to be complicated with diffuse panbronchiolitis (DPB). The incidence of DPB is considered to be significantly higher in patients with ATL. The three DPB‐complicated cases composed one case each of the smoldering, chronic, and acute type of ATL. In each type, DPB preceded overt ATL and Candida afbicans was found in sputa following detection for bacteria. The DPB complication apparently worsened the prognosis of the ATL patients. We have discussed a possible relationship between ATL and DPB.
Advanced Hodgkin disease (clinical stages IIIB and IV): Low relapse rate after brief chemotherapy followed by high‐dose total lymphoid irradiation
Authors: Colonna P. , Andrieu J. M. , Ghouadni R. , Zouaoui‐Benhadji Z. , Afiane M. , Kubisz P. , Tourani J. M. , Belhadj‐Merzoug K. , Krisch C. , Laugier A. 
Published: February 2007

AbstractFrom January 1980 to September 1986, 50 patients with Hodgkin disease, clinical stages (CS) IIIB (26 cases) and IVB (24 cases) were treated by three cycles of mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) chemotherapy, followed by high‐dose (40 Gy) (sub)total lymphoid irradiation, including the spleen. Ten patients (2 CS IIIB, 8 CS IVB) were in failure, and seven (4 CS IIIB, 3 CS IVB) died during their first complete remission (2 from treatment‐related complications, 1 from unknown cause, 4 from insufficient supportive care and/or a shortage of health supplies); three patients (CS IIIB) relapsed (2 alive in second complete remission, 1 deceased). After 7 years, actuarial survival and relapse‐free duration were, respectively, 64% for the 50 patients and 89% for the 40 patients in complete remission. Unfavourable outcome was observed in patients with pelvic nodal involvement. The low relapse rate (none in CS IVB) was the most striking result after brief chemotherapy followed by total lymphoid irradiation.
Splenic irradiation as primary therapy for prolymphocytic leukemia
Authors: Kiss A. , Haubenstock A. , Bognar H. , Scheiderbauer R. , Ai‐Mobarak M. , Base W. 
Published: February 2007

AbstractA patient with prolymphocytic leukemia (PLL), a lymphoproliferative disorder that carries a poor prognosis, is presented. The disease was diagnosed at an early stage and treatment could be delayed for four years. When massive, painful splenomegaly developed, splenic irradiation (SI) was chosen as the primary form of therapy and an excellent systemic response could be achieved. Our observation is in agreement with preliminary studies, which advocate SI as the primary form of therapy in PLL. Furthermore, it is emphasized that an early diagnosis of PLL is necessary to establish its true course and that the prognosis may be better than originally thought.
Morphologic and cytochemical characteristics of acute promyelocytic leukemia
Authors: Davey F. R. , Davis R. B. , Maccallum J. M. , Nelson D. A. , Mayer R. J. , Ball E. D. , Griffin J. D. , Schiffer C. A. , Bloomfield C. D. 
Published: February 2007

AbstractThe existence of two distinct subtypes of acute promyelocytic leukemia was confirmed and characterized based on morphologic features of leukemic cells in a series of 63 patients studied by the Cancer and Leukemia Group B (CALGB). Seventeen patients (27%) had microgranular leukemic cells (M3V), and 46 patients (73%) had hypergranular leukemic cells (M3). These patient cohorts were studied for other laboratory and clinical features. Leukemic cells from M3V patients stained less frequently than leukemic cells from M3 patients for myeloperoxidase (median, 93% vs. 99%; P=.006), periodic acid‐Schiff (median, 57% vs. 92%; P=.0001), ASD‐chloroacetate esterase (median, 45% vs. 87%; P<.0001), and alpha‐naphthyl acetate esterase (0% vs. 37%; P=.003). Patients with M3V had a higher platelet count (median, 50 vs. 30 × 109/L; P=.01) and tended to have a higher leukocyte count (median, 7.4 vs. 2.2 × 109/L; P=.06) than M3 patients. The patients with M3V morphology were more likely to be nonwhite (29% vs. 7%; P=.03), female (71% vs. 37%; P=.02), and to be infected at the time of presentation (71% vs. 35%; P=.02). No differences in the frequency of the t(15;17) karyotype or the immunopheno‐typic expression of the leukemic cells were noted in the two morphologic subtypes of acute promyelocytic leukemia. Fewer patients with M3V tended to enter complete remission (65% vs. 80%; P=.20), but no significant differences were found in the duration of complete remission (P=.81; 1 year rate, 50% vs. 85%), or probability of survival (P=.67; 1 year rate, 49% vs. 68%).
Reversal of bone marrow fibrosis and subsequent development of polycythemia in patients with myeloproliferative disorders
Authors: Talarico Lilia , Wolf Barbara C. , Kumar Aruna , Weintraub Lewis R. 
Published: February 2007

AbstractBone marrow fibrosis is a characteristic finding in agnogenic myeloid metaplasia and in the spent phase of polycythemia vera. It is commonly believed that the reticulin deposition is irreversible. However, we report four patients who demonstrated clinical and laboratory evidence of transition from myelofibrosis to polycythemia. The transition was documented by improvement in the hemoglobin concentration and by determination of the Cr51 red blood cell mass, accompanied by a resolution of the fibrosis on serial bone marrow biopsies. Two of the patients had been treated with alkylating agents and splenectomy, one with myelosuppressive therapy without splenectomy, and one with splenectomy alone. These findings indicate that bone marrow fibrosis in the chronic myeloproliferative disorders is not always an irreversible phenomenon. Pathogenetic implications will be discussed.
Cyclosporin A in the treatment of severe aplastic anemia: Description of a case complicated by the development of tubercular pericarditis during treatment
Authors: Pogliani E. M. , Cortellaro M. , Foa P. , Lurlo A. , Polli E. E. 
Published: February 2007

AbstractWe report on a 62 year‐old woman who developed a severe aplastic anemia requiring frequent packed red cell and platelet transfusions. Since conventional pharmacological therapy was ineffective, treatment with Cyclosporin A was started, resulting in a significant increase of leukocyte, platelet, and red cell counts. However, 5 months after the beginning of Cyclosporin A treatment, despite a past medical history not significant for clinical tuberculosis, the patient developed tubercular pericarditis. This report underlines the need for a careful follow‐up of Cyclosporin A‐treated patients aimed at a prompt diagnosis of possible infectious complications.
Ring‐shaped nuclei in human neutrophilic leukocytes of healthy individuals: Evidence of their occurrence and characteristics
Authors: Cabral Humberto R. A. , Robert Gabriela B. 
Published: February 2007

AbstractIn our previous work we found the presence of ring‐shaped nuclei in circulating neutrophils of healthy individuals whose nuclei were stained only by means of the Feulgen reaction [1]. In our present work, we performed another study on the blood of 52 healthy human donors and stained their blood films with hematoxylin. In all cases, ring‐shaped nuclei were revealed. In this series, the number of this type of nuclei was high: 15.61% ± SD 7.18%. The ring nuclei of healthy humans had thin segments, such as we had previously found by means of Feulgen method.
Reversible agranulocytosis related to azidothymidine therapy
Authors: Goldsmith Jonathan C. , Irvine Wendy 
Published: February 2007

AbstractHematopoietic toxicity from Zidovudine (AZT) is fairly common, resulting in a requirement for red cell transfusions in up to 25% of patients. Reversible agranulocytosis occurred following approximately 1 week of AZT therapy in a man with AIDS. He recovered from the episode without incident. There was no evidence for underlying bone marrow dysfunction or an adverse drug reaction. Careful monitoring of AZT therapy continues to be of great importance.
In vitro DNA synthesis by megaloblastic bone marrow: Effect of folates and cobalamins on thymidine incorporation and de novo thymidylate synthesis
Authors: Das Kshitish C. , Herbert Victor 
Published: February 2007

AbstractThe de novo pathway of thymidylate synthesis (i.e., methylation of dUMP to dTMP) is directly folate dependent and indirectly vitamin B12 (cobalamins) dependent. In deficiency of these vitamins, this pathway is impaired, and exogenous deoxyuridine (dU) fails to suppress adequately in vitro incorporation of [3H]thymidine (3H‐TdR) into DNA via the salvage pathway (i.e., abnormal dU suppression). This abnormality is corrected by the addition of folate compounds (analogues) and/or vitamin B12 depending on the nature of the underlying deficiency. We studied the effects of addition of PteGlu, 5‐methyl THF (5‐CH3‐FH4), 5‐formyl‐THF (5‐CHO‐FH4), and hydroxy‐cobalamin (OH‐cbl) on 3H‐TdR incorporation into DNA and thymidine kinase activity (salvage pathway), and on [3H]deoxyuridine (3H‐dU) incorporation and dU suppression values (de novo pathway) in cultures of normal and megaloblastic bone marrows. The results showed that 3H‐TdR incorporation into DNA and the salvage enzyme, thymidine kinase, activity were greater and 3H‐dU incorporation into DNA less in megaloblastic cells as compared with normal cells. The addition of folates significantly reduced 3H‐TdR incorporation and thymidine kinase activity and enhanced 3H‐dU incorporation in folate and vitamin B12‐deficient cells except that 5‐CH3‐FH4 had no effect on vitamin B12‐deficient cells. None of these additives had any significant effect on normal cells. This study also showed that the addition of the deficient vitamin(s) to the “control tubes” in the dU suppression test is inappropriate, as these vitamins may at least partially correct the defect in cellular DNA synthesis caused by the deficiences of these vitamins and may mask these deficiencies in the results of the in vitro correction of the dU suppression abnormalities in mild cases of megaloblastic anemia.
Characterization of von willebrand factor in factor VIII concentrates
Authors: Fricke William A. , Yu Mei‐Ying Wong 
Published: February 2007

AbstractCommercial concentrates of factor VIII (FVIII) were analyzed in order to 1) determine the effects of viral inactivation on von Willebrand factor (vWF); 2) evaluate the vWF content of the new, immunopurified concentrates; and 3) assess their potential for correcting the long bleeding time of von Willebrand disease (vWD). Included in our study were products that had been treated to inactivate viruses; older, untreated products; and the new, immunopurified concentrates. We measured von Willebrand factor antigen (vWF:Ag), ristocetin cofactor activity (RCoF), and vWF multimeric and subunit composition. A newly developed radioimmunoassay (RIA) was used to quantitate vWF:Ag. The vWF:Ag content varied from 0.083 μg/IU FVIII:C for Hemofil M to 32.2 μg/IU FVIII:C for Humate‐P, whereas pooled normal human plasma (NHP) contained 6.3 μg/IU FVIII:C. The RCoF varied from 0.0007 to 2.09 U/IU FVIII:C, with the immunopurified concentrates having the lowest values and Humate‐P the highest. The ratio of RCoF to vWF:Ag ranged from 11 to 96 U/mg, as compared to a ratio of 160 for NHP. All of the concentrates lacked the largest vWF multimers, and all had abnormal triplet patterns. Modest differences between some untreated concentrates and their treated counterparts were noted. As expected, the immunopurified concentrates had much lower levels of all vWF activities than the conventionally prepared products. Our data suggest that none of the concentrates have as great a capacity as NHP to correct the prolonged bleeding time of von Willebrand disease.
Pituitary abscess after autologous bone marrow transplantation
Authors: Leff Richard S. , Martino Roy L. , Pollock W. James , Knight William A. 
Published: February 2007

AbstractThe first case of pituitary abscess arising in a patient during recovery from autologous bone marrow transplantation is reported. A 31‐year‐old man with a 9 month history of T‐cell lymphoma died suddenly more than 60 days after successful treatment with high‐dose cyclophosphamide, total body irradiation, and autologous bone marrow infusion. Autopsy revealed a pituitary abscess associated with clinically silent sphenoid sinusitis. Unique aspects of this case are presented and clinical and pathologic features of pituitary abscess are reviewed. Although rare, pituitary abscess may complicate recovery from bone marrow transplantation.
Age‐dependent variation in the cytosol/membrane distribution of red cell protein kinase‐C
Authors: Ramachandran M. , Abraham E. C. 
Published: February 2007

AbstractAn age‐dependent increase in membrane association of protein kinase‐c and a decrease in the cytosolic enzyme, especially in the densest fraction rich in sensescent red cells was observed in Stractan‐gradient‐separated normal erythrocytes.
Controlled prospective study of factor IX concentrate therapy and immunodeficiency
Authors: Hultin Mae B. , Dattwyler Raymond J. , Lipton Richard A. 
Published: February 2007

AbstractIn 1983, six patients who were exclusively treated with factor IX (FIX) concentrate (>40,000 units/yr) were prospectively matched for age and dosage to six patients treated exclusively with factor VIII (FVIII) concentrate and to six normal male controls. At baseline evaluation between October 1983 and May 1984, both groups had significantly decreased absolute T helper cell counts (mean of 452/μl and 505/μl for FIX‐ and FVIII‐treated groups, respectively) compared to normal (1,157/μl). By August 1988, three of the six FIX‐treated group have developed AIDS and two are seropositive for antibody to the human immunodeficiency virus (HIV). Four of the six FVIII‐treated group have HIV seropositivity or disease. The other three patients (1/6 for FIX and 2/6 for FVIII) declined HIV antibody testing. Our results support other studies showing a dose‐related risk of HIV exposure for FIX concentrate‐treated patients and do not support the view that FIX concentrate was intrinsically safer than FVIII concentrate.
Relative iron deficiency in hereditary spherocytosis
Authors: Zanella A. , Barosi G. , Berzuini A. , Colombo M. B. , Quaglini S. , Volpes D. , Sirchia G. 
Published: February 2007

AbstractSeventy‐three patients with hereditary spherocytosis (HS) (58 nonsplenectomized, 15 splenectomized) were studied to evaluate iron status and the adequacy of iron availability for erythropoiesis. Splenectomized patients, who had hemoglobin levels in the normal or upper normal range, had higher levels of serum iron, transferrin saturation, and serum ferritin than normal matched controls and normal zinc protoporphyrin (ZnPP) levels. On the contrary, nonsplenectomized patients presenting with mild to severe anemia had higher red cell ZnPP concentrations than both splenectomized subjects and matched normal controls. ZnPP in nonsplenectomized patients correlated inversely with Hb concentration, mean corpuscular volume (MCV), mean red cell hemoglobin concentration (MCHC), transferrin saturation, and serum iron, and directly with reticulocyte count. At multiple regression analysis only Hb concentration was a significant explanatory variable for high ZnPP. The authors conclude that a number of nonsplenectomized HS patients have relative iron deficiency primarily because of expansion of erythropoiesis caused by anemia.
Net renal tubular reabsorption of zinc in healthy man and impaired handling in sickle cell anemia
Authors: Yuzbasiyan‐Gurkan Vilma A. , Brewer George J. , Vander Arthur J. , Guenther Michael J. , Prasad Ananda S. 
Published: February 2007

AbstractZinc deficiency is a significant clinical finding in sickle cell anemia (SCA) and abnormalities of zinc handling such as hyperzincuria are present. The cause of increased urinary zinc excretion in SCA is not clear. To define the renal handling of zinc in SCA and in healthy subjects, we measured zinc (total and ultrafilterable plasma zinc, urine zinc) and creatinine clearance in eight healthy and seven SCA subjects. Ultrafilterable zinc in plasma was assessed by equilibration of plasma with 65Zn followed by filtration through Amicon Cetriflo CF25 cones. While the mean filtered load of zinc was not significantly different between the two groups, the mean zinc excretion rate was approximately threefold higher in patients (1.73 ± 0.96 vs. 0.63 ± 0.39 μg/min, P<.05). In controls, zinc excreted was significantly less than zinc filtered (P<.005), the fractional excretion of zinc averaging 0.49 ± 0.31, indicating net reabsorption. This was not the case for the SCA patients. We conclude that there is impaired renal tubular handling of zinc in SCA.
Frequency of congenital anomalies in patients with histiocytosis X
Authors: Sheils Catherine , Dover George J. 
Published: February 2007

AbstractThe frequency of congenital anomalies in 39 cases of histiocytosis × collected over a 30 year period was 23%. Through a retrospective chart review of these cases and two control populations (children with bone tumors and children with suspected child abuse) we sought to assess the significance of this finding as well as any special characteristics of those histiocytic patients who had congenital anomalies. The frequency of congenital anomalies in the histiocytosis × group (23%) was greater than the frequency found in our control groups, the bone tumor group (13%), and the child advocacy control group (15%). Considering only major congenital anomalies, the histiocytic population had an increased frequency (18%) relative to both control groups (3% and 8%, respectively). Only one patient with unifocal eosinophilic granuloma had congenital anomalies. Patients with histiocytosis × and congenital anomalies were more likely to have histiocytosis X involving organ dysfunction (lung, liver, hematopoietic). Through this retrospective study we observe an increased frequency of congenital anomalies in patients with histiocytosis X. This observation does not seem to apply to patients with unifocal eosinophilic granuloma. The presence of congenital anomalies, especially multiple congenital anomalies, seemed to be a prognostic indicator of organ dysfunction in our histiocytic population.
Myelodysplastic syndrome with trisomy 11 associated with polycythemia vera
Authors: Ohyashiki Kazuma , Nagasu Masaya , Hojo Hitoshi , Iwabuchi Atsuhiro , Ohyashiki Junko H. , Toyama Keisuke 
Published: February 2007

AbstractA 52‐year‐old male with myelodysplastic syndrome (MDS) who had a prior history of polycythemia vera is reported. Chromosome analysis revealed that the bone marrow and blood cells at the MDS phase contained trisomy of chromosome 11 as the sole cytogenetic change. Trisomy 11 is rarely found in hematologic neoplasia, and all of the reported cases with trisomy 11 were diagnosed as having nonlymphocytic neoplasia. In this report, a correlation between the chromosome change and leukemia/MDS developed in polycythemia vera is discussed.
Acute nonlymphocytic leukemia with basophilic differentiation and t(9,11)(p22,q23) in a child
Authors: Kubota Masaru , Akiyama Yuichi , Tabata Yasuyuki , Yoshigi Masaaki , Mikawa Haruki 
Published: February 2007

AbstractA 20‐month‐old child was treated for acute nonlymphocytic leukemia (ANLL) with basophilic differentiation. His leukemic cells also had the cytogenetic abnormality of t(9,11)(p22,q23). Although immature blasts responded well to induction therapy with etoposide, the leukemic cells that were more differentiated toward basophils were quite refractory to the drug. However, complete remission was finally achieved with a conventional multidrug regimen.