bluuebunny/crossref_metadata_2025_split huggingface dataset:
Anti‐platelet and Anti‐DNA IgM in waldenström macroglobulinemia and ITP
Authors: Vartlcovski L. , Pick A. I. , Schattner A. , Shoenfeld Y. 
Published: February 2007

AbstractWe describe a monoclonal IgM that was purified from the serum of a patient with Waldenstrom macroglobulinemia and thrombocytopenia. The binding and idiotypic characteristics of the patient's macroglobulin were similar to those of a human monoclonal IgM secreted by a hybridoma established from peripheal blood lymphocytes of a patient with systemic lupus erythematosus and immune thrombocytepenia.In the absence of other causes for thrombocytopenia in this patient, our results suggest an autoimmune mechanism for destruction of platelets by the monoclonal IgM. This is the first report of a Waldenstrom macroglobulinemia with anti‐platelet activity of monoclonal IgM.Although the study involves a single patient, the results suggest that there may be a common origin for autoantibodies in autoimmune diseases and monoclonal immunoglobulins having autoantibody activity in monoclonal gammopathies.
Hb Q‐Thailand‐Hb H disease in a chinese living in Geneva, Switzerland: Characterization of the variant and identification of the two α‐thalassemic chromosomes
Authors: Beris P. , Huber P. , Miescher P. A. , Wilson J. B. , Kutlar A. , Chen S. S. , Huisman T. H. J. 
Published: February 2007

AbstractData on a 24‐year‐old Chinese male with Hb Q‐Thailand‐Hb H disease are presented. The hemoglobin variant was characterized by fast microprocedures, mainly by reverse‐phase high‐performance liquid chromatography. Gene mapping analyses identified the α‐thalassemia‐2, which is associated with the α‐Q chain, as caused by a 4.2‐kb deletion involving the α2 globin gene, while the α‐thalassemia‐1 anomaly was the common Southeast Asian type in which part of the Ψζ, the Ψα, and the α2 and α1 globin genes are deleted.
Phenotypic changes associated with chemically induced differentiation of a hairy cell leukemia cell line
Authors: Hooper W. Craig , Barth Rolf F. , Minowada Jun 
Published: February 2007

AbstractIn vitro chemically induced differentiation of the JOK‐1 cell line, derived from a patient with hairy cell leukemia, has been studied. Non‐induced JOK‐1 cells expressed sIgM, B‐1, CALLA, Ia, and C3 receptors. Following exposure to 2% DMSO, or 100 ng/ml 12‐0‐tetradecanoyl‐phorbol‐13‐acetate (TPA), the cells underwent a phenotypic change from lymphoblastoid to monocytoid. In the presence of TPA, they became adherent and developed dendritic processes. Associated with these changes was a decrease in sIgM and CALLA and variable expression of C3 receptors. No monocyte/macrophage‐associated antigens were detected. These results suggest that the JOK‐1 cell line may have a limited ability to differentiate but is not mature enough to complete the differentiation process.
Immunological aspects of the anemia of rheumatoid arthritis
Authors: Sugimoto Masakuni , Wakabayashi Yoshihisa , Hirose Shun‐Ichi , Takaku Fumimaro 
Published: February 2007

AbstractIn order to investigate the cause of the anemia concomitant with rheumatoid arthritis (RA), we examined, using the erythroid colony assay of human bone marrow colonyforming units‐erythroid (CFU‐e) and burst‐forming units‐erythroid (BFU‐e), the effects of the patients' serum and peripheral blood T lymphocytes on the CFU‐e‐derived colonies.The counts of erythroid colonies of RA patients were markedly lower than those of human control subjects [CFU‐e: control 152.9 ± 30.6 (n = 19), RA 51.1 ± 13.6 (n = 7), t = 7.66567, p < 0.01; BFU‐e: control 25.2 ± 5.9 (n = 5), RA 12.6 ± 2.6 (n = 7), t = 4.574, p < 0.01]. The serum from two out of seven RA patients slightly inhibited the formation of CFU‐e‐derived colonies of human control subjects (t = 2.31, 0.05 < p < 0.1); however, the serum from the other five RA patients did not significantly inhibit human control erythroid colony formation as compared with human control serum (t = 0.981, 0.3 < p < 0.4). On the other hand, peripheral blood T lymphocytes of the patients markedly inhibited the formation of CFU‐e‐derived colonies of the control subjects (t = 4.24, p > 0.01).The above‐mentioned results suggest that the peripheral blood T lymphocytes of RA patients might play a role as one of the causes of the concomitant anemia of RA patients.
Immunoarchitecture of the bone marrow in neutropenia: Increased HNK‐1 + cells define a subset of neutropenic patients
Authors: Picker Louis J. , Kadin Marshall E. , Furst Annette , Robinson Stephen H. 
Published: February 2007

AbstractAn immunoperoxidase technique was used to examine the distribution of lymphocyte subsets in bone marrow biopsies of 15 patients with neutropenia and seven non‐neutropenic controls. The bone marrow of most patients and controls had similar distributions of immune effector cells characterized by a diffuse array of predominantly cytotoxic/suppressor T‐cells and occasional nodular aggregates of helper T‐cells. Cells displaying the natural killer cell marker HNK‐1 were sparse in controls and most neutropenic patients. However, marked increases in marrow HNK‐1 + cells were identified in four of the 15 patients. Three of these patients had diffuse HNK‐1 + infiltrates associated with increased Leu 4+ (OKT‐3 +) T‐cells while one had a nodular HNK‐1 + infiltrate associated with small B‐cell follicles. Each of these patients had clinical features similar to those described in the large granular lymphocyte (LGL) lymphocytosis (leukemia) syndrome, but only one of four demonstrated persistently increased numbers of LGLs in the peripheral blood. Thus, this study extends the association of neutropenia and increased numbers of cells with a T/NK phenotype to include patients whose bone marrow is the only demonstrable site of involvement. Since morphologic examination of the bone marrow could not identify the bone marrows with increased HNK‐1 + cells, immunulogic techniques are required to detect these cases.
Correction of the bleeding time in treated patients with severe von willebrand disease is not solely dependent on the normal multimeric structure of plasma von willebrand factor
Authors: Mannucci P. M. , Moia M. , Altieri D. , Rebulla P. , Monteagudo J. , Castillo R. 
Published: February 2007

AbstractEven though it is generally held that cryoprecipitate and fraction I‐0 correct the prolonged bleeding time (BT) in patients with von Willebrand disease (VWD), perusal of reported data indicates that the correction is usually short‐lasting and often partial. We decided to do a controlled study of the relationship between the multimeric structure of von Willebrand factor (VWF) and the BT in five patients with severe (type III) VWD after infusion of three plasma concentrates (“wet” cryoprecipitate, lyophilized cryoprecipitate, and fraction I‐0) given in random order. The dosage of concentrates was tailored from in vitro measurements to achieve post‐infusion levels of ristocetin cofactor above the lower normal limit (50 U/dL) for at least 3 hours. The postinfusion BT became transiently normal in only two of five patients treated with wet cryoprecipitate, whereas it remained prolonged in all five patients treated with lyophilized cryoprecipitate or fraction I‐0. For all the concentrates, the proportion of large VWF multimers calculated by scanning the electrophoretic gels were the same as those for normal standard plasmas. An intact multimeric structure was recovered in postinfusion plasmas of patients treated with wet cryoprecipitate, whereas there was a postinfusion loss of large multimers after lyophilized cryoprecipitate and fraction I‐0. These findings indicate that the attainment of a normal BT is the exception rather than the rule after the infusion of three plasma fractions used in the treatment of severe VWD, and that an intact multimeric structure in concentrates and postinfusion plasmas is necessary but not sufficient to sustain a normal BT.
Myeloid lineage specificity and high sensitivity of monoclonal antibody GM 58/8 proves its usefulness as a diagnostic reagent in acute leukemia
Authors: Gupta Amar Das , Dhond Subodh R. , Barbhaya Shakuntala A. , Advani Suresh H. 
Published: February 2007

AbstractMonclonal antibody (MoAb) GM 58/8 was earlier reported to be directed against an antigen expressed by myeloid progenitors (CFU‐GM), myeloid precursors, granulocytes, and monocytes [5]. Immunophenotyping of 216 cases of acute leukemia [acute myeloblastic leukemia (AML) = 147 and acute lymphoblastic leukemia (ALL) = 69] and 18 cases of chronic granulocytic leukemia in blast crisis (CGLBC) with this antibody showed that GM 58/8 reacted with 92% of AML cases (M1‐M5) and 100% of myeloblastic crisis in CGL cases. All cases of ALL, lymphoblastic crisis in CGL, erythroleukemia, and erythroblastic crisis in CGL were unreactive with GM 58/8. The antibody revealed the myeloid phenotype in an additional 15 cases of otherwise unclassifiable acute leukemia and six cases of CGLBC. Eleven cases of acute “mixed lineage” leukemia were also diagnosed with the help of GM 58/8. The high specificity (100%) and sensitivity (92%) of MoAb GM 58/8 for myeloblastic leukemia is unmatched by almost all previously described myeloid MoAb and proves its usefulness as a single diagnostic reagent for AML and myeloblastic crisis in CGL.
Serum from patients with non‐hodgkin's lymphoma(nhl) inhibits T‐cell colony formation
Authors: Hutchison Robert E. , Tice Diane G. , Davey Frederick R. 
Published: February 2007

AbstractThis study compared T‐cell colony formation in soft agar of lymphocytes from the peripheral blood and lymph nodes of patients with non‐Hodgkin's lymphoma (NHL) with T‐cell colony formation of peripheral blood lymphocytes from normal individuals. Mononuclear cells were separated from other blood and lymph node elements on density gradient columns, phenotyped for T‐ and B‐cell antigens using monoclonal antibodies, and then plated in soft agar cultures. Lymphocytes from peripheral blood and lymph nodes of patients with NHL exhibited less T‐cell colony formation (p < 0.01) than did lymphocytes from normal individuals. This decrease in T‐cell colony formation was not the result of the number of T cells or null cells plated, or differences in proportions of T helper and T suppressor cells. However, when sera from patients with NHL were incubated with normal lymphocytes before plating in soft agar, a decrease in number of T‐cell colonies was observed (p < 0.01). We conclude that peripheral blood and lymph node mononuclear cells from patients with NHL have a decreased ability to form T‐cell colonies in soft agar cultures and that this decrease is related, at least in part, to the presence of serum factor(s).
Erythroblast transferrin receptors and transferrin kinetics in iron deficiency and various anemias
Authors: Muta Koichiro , Nishimura Junji , Ideguchi Hiroshi , Umemura Tsukuru , Ibayashi Hiroshi 
Published: February 2007

AbstractTo clarify the role of transferrin receptors in cases of altered iron metabolism in clinical pathological conditions, we studied: 1 number of binding sites; 2 affinity; and 3 recycling kinetics of transferrin receptors on human erythroblasts. Since transferrin receptors are mainly present on erythroblasts, the number of surface transferrin receptors was determined by assay of binding of 125I‐transferrin and the percentage of erythroblasts in bone marrow mononuclear cells. 1 The number of binding sites on erythroblasts from patients with an iron deficiency anemia was significantly greater than in normal subjects (p < 0.01). Among those with an aplastic anemia, hemolytic anemia, myelodysplastic syndrome, and polycythemia vera compared to normal subjects, there were no considerable differences in the numbers of binding sites. 2 The dissociation constants (Kd) were measured using Scatchard analysis. The apparent Kd was unchanged (about 10 nmol/L in patients and normal subjects. 3 The kinetics of endocytosis and exocytosis of 125I‐transferrin, examined by acid treatment, revealed no variations in recycling kinetics among the patients and normal subjects. These data suggest that iron uptake is regulated by modulation of the number of surface transferrin receptors, thereby reflecting the iron demand of the erythroblast.
Phosphoglycerate kinase San Francisco: A new variant associated with hemolytic anemia but not with neuromuscular manifestations
Authors: Guis M. S. , Karadsheh N. , Mentzer W. C. 
Published: February 2007

AbstractPhosphoglycerate kinase deficiency is a rare, x‐linked glycolytic defect that, when severe, can be associated with hemolytic anemia, rhabdomyolysis, or neurological disorders. We report here a new phosphoglycerate kinase variant discovered in a boy with severe hemolytic anemia but no evidence of neuromuscular disease or developmental delay. The biochemical properties of the variant enzyme (greatly increased kmATP and km3‐phosphoglycerate; normal pH optimum, electrophoretic mobility, and substrate specificity; resistance to heat inactivation) establish its uniqueness. Separation of light and dense red cells by centrifugation showed no greater loss of phosphoglycerate kinase activity in dense (“old”) variant cells than in normal cells. We postulate that the striking stability of the variant enzyme allows cells capable of protein synthesis to accumulate sufficient enzyme to limit neuromuscular sequelae.
The spectrum of human immunodeficiency virus infection in patients with factor IX deficiency (Christmas Disease)
Authors: Goldsmith J. M. , Variakojis D. , Phair J. P. , Green D. 
Published: February 2007

AbstractEarly reports suggested that hemophiliacs with factor IX deficiency (Christmas Disease) may be at less risk for developing the acquired immunodeficiency syndrome (AIDS) than patients with classic hemophilia. We evaluated 12 factor IX deficient patients for clinical and immunologic abnormalities related to infection with the human immunodeficiency virus (HIV). Antibody to HIV was not detected in these patients prior to 1982. By 1985, 66 percent (eight of 12) patients were seropositive. All three concentrates available commercially before 1985 were associated with seropositivity. Furthermore, seropositive hemophiliacs had received on average significantly more factor IX concentrate than seronegative hemophiliacs (27,825 ± 17,976 (S.D.) versus 1,250 ± 1,500 factor units/year, (p < 0.02). Half of the seropositive individuals had generalized lymphadenopathy with splenomegaly. Two seropositive patients have developed AIDS, one with cryptococcal meningitis and another with a large cell immunoblastic lymphoma. Infection with HIV has occurred with high frequency in hemophiliacs who received unmodified factor IX concentrates.
Systemic lupus erythematosus and sickle hemoglobinopathies: A report of two cases and review of the literature
Authors: Katsanis Emmanuel , Hsu Elizabeth , Luke Koon‐Hung , McKee James A. 
Published: February 2007

AbstractTwo adolescent black females with sickle cell disease who developed systemic lupus erythematosus (SLE) are presented. The recognition of SLE was delayed because all new symptoms were initially attributed to their underlying sickle cell disease. Nine similar cases have been previously reported. The hypothesis that a deficiency of the alternative complement pathway in some patients with sickle hemoglobinopathies predisposes them toward immune complex disorders was not confirmed in our study. Our first patient had normal and our second increased activity of the alternative pathway of complement activation.
High‐dose intravenous IgG therapy in a seven‐week‐old infant with chronic autoimmune hemolytic anemia
Authors: Sasaki Hiroshi , Akutagawa Hiroshi , Kuwakado Katsuji , Uemura Masaru , Emi Isamu 
Published: February 2007

AbstractA 7‐week‐old male infant tested positive for autoimmune hemolytic anemia. Serological studies revealed a positive Coombs test and a strong antibody with relative anti‐E specificity.Despite corticosteroid therapy, he frequently suffered severe hemolytic crises. During the course of the disease he had pneumonia, subcutaneous abscess, and sepsis, which were thought to be caused by his immunosuppressive state. His clinical course was chronic and refractory.Recently, high‐dose intravenous IgG therapy for the treatment of chronic AIHA has been reported, but it has not been successful in the management of adult AIHA. We attempted this therapy in our patient, and successful management was obtained.
A distinct subtype of idiopathic myelofibrosis with bone marrow features mimicking hairy cell leukemia: Evidence of an autoimmune pathogenesis
Authors: Hasselbalch Hans , Jans Henning , Nielsen Preben Løvgreen 
Published: February 2007

AbstractA young female patient presented with severe anemia and myelofibrosis. The spleen was not enlarged on clinical examination and no leukoerythroblastosis or tear‐drop poikilocytosis was found. A bone marrow biopsy disclosed severe myelofibrosis and many megakaryocytes, which appeared morphologically normal. A striking feature was a heavy spongy infiltration by large lymphocytic cells, which were tartrateresistant acid phosphatase negative and without hairy projections. This bone marrow appearance highly resembled the clear cell appearance of the hairy cell infiltrate in hairy cell leukemia. A diagnosis of acute autoimmune myelofibrosis was made. During high‐dose glucocorticoid therapy the bone marrow fibrosis completely resolved and there was an improvement of peripheral blood values. The disorder is supposed to feature a distinct clinicopathological entity within the syndrome of idiopathic myelofibrosis. The disease should be considered in the differential diagnosis of patients with myelofibrosing disorders, including acute myelofibrosis and hairy cell leukemia.
Maturation of B‐lineage blast crisis cells in chronic myeloid leukemia
Authors: Ng C. S. , Arnold M. , Chan John K. C. 
Published: February 2007

AbstractA 44 year‐old Chinese male presented with lymphoid blast crisis of chronic myeloid leukemia, in which the blast cells exhibited a pre‐pre‐B cell phenotype (B4+ B1− J5+ 12+smIg−). There was a phenotypic transition during the course of the disease to a more mature B cell phenotype (B4+ B1− J5+ 12+smIgGλ+). The maturation of blast crisis cells may be related to the chemotherapeutic agents used in the treatment of the disease.
Involvement of von willebrand factor in thrombosis following asparaginase‐prednisone‐vincristine therapy for leukemia
Authors: Pui Ching‐Hon , Jackson Carl W. , Chesney Carolyn M. , Abildgaard Charles F. 
Published: February 2007

AbstractTo determine if factor VIII‐von Willebrand factor (vWF) complex is involved in the thrombosis associated with asparaginase‐prednisone‐vincristine induction therapy for acute lymphoblastic leukemia, plasma vWF was analyzed by sodium dodecyl sulfateagarose gel electrophoresis and crossed immunoelectrophoresis. Five patients with cerebral thrombosis were studied; all had a decreased platelet count following the complication. Sequential studies of three patients disclosed changes in plasma vWF multimer pattern. One patient who was studied serially from 2 days before to 1 day after the event, had an increase in unusually large plasma vWF multimers that disappeared after the complication. The other two patients who were studied at presentation subsequently showed a decrease in plasma large vWF multimers, especially remarkable in the patient having the sharpest decrease in platelet count. No appreciable difference in vWF multimer pattern, when compared to normal pooled plasma, was found in the remaining two patients who were only studied at presentation, or in the seven controls who received the same treatment but did not develop thrombosis. Crossed immunoelectrophoretic analyses of two patients tested disclosed a right shift of immunoprecipitin line in one and a left shift in the other. Our findings suggest that the thrombotic complications resulted from platelet agglutination by plasma vWF.
Platelet IgG Fc receptor
Authors: Kelton John G. , Smith James W. , Santos Aurelio V. , Murphy William G. , Horsewood Peter 
Published: February 2007

AbstractThe glycoprotein localization of the platelet binding site for the Fc IgG has been the subject of debate. We attempted to resolve this issue by relating the binding of radiolabeled IgG immune complexes composed of heat‐aggregated IgG to platelets from 1) healthy individuals; 2) an individual with Bernard‐Soulier syndrome lacking glycoproteins Ib and IX; and 3) a patient with Glanzmann's thrombasthenia lacking glycoproteins IIb and IIIa. The binding of IgG complexes to platelets was determined by measuring the specific binding of radiolabeled heat‐aggregated IgG to washed platelets in a plasma‐free mileu. 125I aggregated IgG bound to normal platelets in a saturable and concentration‐dependent fashion. Specific binding could be inhibited by a 50‐fold excess of purified Fc, but not by F(ab')2. Identical binding curves were obtained by using platelets from a patient with Glanzmann's thrombasthenia and a patient with Bernard‐Soulier syndrome, indicating that the platelet Fc receptor is not carried on glycoproteins Ib, IIb, IIIa, or IX. We then measured the binding of radiolabeled detergent‐solubilized platelets to IgG fixed to a solid matrix. A 40‐kD platelet fragment bound to the immobilized IgG following passage across a density gradient. Confirmation of the Fc specificity of the interaction was shown by inhibition of platelet glycoprotein binding by excess IgG or purified Fc but not F(ab')2. The electrophoretic mobility decreased slightly after reduction, which indicated the existence of at least one intrachain disulfide bond. Treatment with high salt solutions or urea did not solubilize the receptor, which indicated that it was an integral protein. Enzyme studies showed that the platelet Fc receptor was not digested by neuraminidase, but neuraminidase treatment altered mobility by about 3 %. In addition, treatment of platelets with trypsin or pronase did not affect its function as measured by the binding of 125I‐IgG aggregates to enzyme‐treated platelets, but did prevent its detection when using radioimmunoprecipitation studies. The platelet Fc receptor is a 40‐kD, integral protein without interchain disulfide bonds.
Chronic lymphocytic leukemia in china
Authors: Boggs Dane R. , Chen Shu Chang , Zhang Zhi‐Nan , Zhang An 
Published: February 2007

AbstractChronic lymphocytic leukemia (CLL) is a very common form of leukemia among middle‐aged and elderly persons in Western countries. In the Japanese, or in those with Japanese ancestry born and living in the USA, CLL is rather rare. It is also rare in China, but convincing data to support this statement are found primarily in the Chinese literature. We have reviewed previously unpublished data from Peking Union Medical College (PUMC) concerning the frequency of CLL among adult patients with the four common types of leukemia. Of 4,174 such patients (1952–1986), 4.6% had CLL; 22.9% chronic myeloid leukemia (CML); 50.3% acute myeloid leukemia (AML); and 22.3% acute lymphoblastic leukemia (ALL). This low frequency of CLL is none the less higher than that found in a multiinstitutional collection of Chinese patients with leukemia. Various clinical and laboratory aspects of a carefully studied, previously published group of patients with CLL from PUMC were compared to similar published findings in patients from the University of Utah. No differences in the overall nature of the disease were detected between these groups. However, not unexpectedly, the duration of symptoms before the diagnosis was made longer in Chinese than in USA patients and the proportion diagnosed at a time when no CLL related symptoms had been noted was less in Chinese. Neither differences in life span in the two populations nor an excess of CML, AML, and ALL at the expense of CLL were viable hypotheses to explain the rarity of CLL in China. We suggest that the dearth of CLL in Chinese is on a genetic basis, as it is thought to be in the Japanese.