bluuebunny/crossref_metadata_2025_split huggingface dataset:
Effect of histocompatibility factors on pulmonary retention of indium‐111‐labeled granulocytes
Authors: Dutcher Janice P. , Riggs Charles , Fox Jeremiah J. , Johnston Gerald S. , Norris Dottie , Wiernik Peter H. , Schiffer Charles A. 
Published: February 2007

AbstractGranulocyte transfusions are associated with a number of side effects including febrile transfusion reactions and occasionally pulmonary infiltrates. There is evidence that the presence of preformed antibodies may be a cause of these complications. In this study, allogeneic 111lndium‐labeled granulocytes were used to evaluate the pulmonary retention of radioactivity in alloimmunized and non‐alloimmunized patients in an attempt to assess antibody effect on granulocyte migration. After injection of labeled allogeneic granulocytes into neutropenic patients, the ratios of lung to heart activity were calculated for the first 30 min of scanning. There was significantly greater retention of radioactivity from cells in the lungs of patients who were alloimmunized, having both lymphocytotoxic (anti‐HLA) and leukoagglutinating antibodies, compared to the activity in the lungs of non‐alloimmunized patients (P < 0.001) or of patients receiving autologous granulocytes (P < 0.001). This study demonstrates that labeled, mismatched granulocytes may be retained in the lungs for a significantly longer time in patients with preformed antibodies. This implies that transfusion of large numbers of such mismatched granulocytes, i. e., granulocyte transfusions, may also be retained in the lungs of alloimmunized patients, which could lead to pulmonary compromise. Therefore, granulocyte transfusions from random donors should not be given to alloimmunized patients.
No beneficial effect of splenectomy in hereditary high red cell membrane phosphatidylcholine hemolytic anemia: Clinical and membrane studies of 20 patients
Authors: Otsuka Akiyo , Sugihara Takashi , Yawata Yoshihito 
Published: February 2007

AbstractThe clinical features and red cell membrane characteristics of 20 patients with hereditary high red cell membrane phosphatidylcholine hemolytic anemia (HPCHA) were studied in relation to the effect of splenectomy. After splenectomy, anemia worsened and the extent of increased hemolysis in these patients was unchanged, indicating a contraindication for splenectomy. Concomitant with these results in clinical hematology, marked stomatocytic changes, increased red cell phosphatidylcholine content, and enhanced sodium transport, which were observed before splenectomy, were not improved by splenectomy.
Surface light chain phenotype in indolent lymphomas: Lack of prognostic significance
Authors: Vandermolen Louis A. , Duffey Patricia L. , Cossman Jeffrey , Jaffe Elaine S. , Longo Dan L. 
Published: February 2007

AbstractThe indolent follicular and diffuse lymphomas are neoplasms of B‐cell origin. In several other B‐cell neoplastic disorders, including multiple myeloma, hairy cell leukemia, and chronic lymphocytic leukemia, the light chain isotype of the surface immunoglobulin has been reported to have prognostic significance. Patients with tumors expressing λ light chains usually fare more poorly than those with k light chain‐bearing tumors. We analyzed the clinical data and immunologic phenotype of 101 patients with indolent lymphoma. Eighty‐nine of the 101 patients demonstrated surface immunoglobulin of only one light chain type (k‐47, λ‐42). Patients in both groups were matched for known prognostic factors. There were no significant differences in disease‐free survival or overall survival between the two groups or within histologic subtypes. These results indicate that the immunologic light chain phenotype in indolent lymphoma, unlike other B‐cell neoplasms, is not a prognostic indicator of survival.
Neutrophilic myelofibrosis presenting as philadelphia chromosome negative BCR non‐rearranged chronic myeloid leukemia
Authors: Stewart Keith , Carstairs Kelvin C. , Dubé Ian D. , Keating Armand 
Published: February 2007

AbstractChronic myeloid leukemia consists of Philadelphia chromosome positive disease in 90% of cases, and a further 5%, although Philadelphia chromosome negative, exhibit bcr gene rearrangements consistent with the disease. The remaining 5% of cases have a heterogeneous clinical picture with a course unlike that of classical chronic myeloid leukemia, and may belong to different pathologic entities.We report five cases belonging to the latter group, initially identified as Philadelphia chromosome negative, bcr non‐rearranged chronic myeloid leukemia, that developed progressive leucocytosis, absolute monocytosis, myelodysplasia, extramedullary hematopoiesis, and had evidence of myelofibrosis. These cases may represent a distinct clinical entity characterized by neutrophilic myelofibrosis, which can be identified prospectively by clinical and pathologic criteria. Standard therapy for treating chronic myeloid leukemia or idiopathic myelofibrosis may not be appropriate for this group.
Patient with pyruvate kinase deficiency developed acute myelogenous leukemia
Authors: Leki Ryuji , Miwa Shiro , Fujii Hisaichi , Kudoh Shouji , Kimura Hitoshi , Takaku Fumimaro 
Published: February 2007

AbstractA 44‐year‐old previously healthy male was diagnosed as anemic and treated at a nearby hospital. A year later, he was admitted to our hospital for precise examination of the progression of refractory anemia. Blood examination showed hemolytic anemia and more detailed examination of this hemolysis revealed pyruvate kinase deficiency. The activity was 15.1% of a normal control. Pyruvate kinase activities of his family members were normal or slightly decreased. Ten months after admission to our hospital, 2% of blast cells with Auer's body in the peripheral blood were noted which increased progressively. The bone marrow contained 17.3% of blast cells with a subsequent diagnosis of acute myelogenous leukemia being made.Although intensive chemotherapy was performed, the patient achieved a brief remission and died 2 years after admission to our hospital.
Diagnosis of cobalamin deficiency I: Usefulness of serum methylmalonic acid and total homocysteine concentrations
Authors: Allen Robert H. , Stabler Sally P. , Savage David G. , Lindenbaum John 
Published: February 2007

AbstractThe serum cobalamin assay is the primary diagnostic test for cobalamin deficiency. It appears to be an excellent screening test since most patients with clinically confirmed cobalamin deficiency have low levels. Recent studies indicate that the clinical picture of cobalamin deficiency is much more diverse than previously believed. It is also apparent that many patients with low serum cobalamin concentrations are not cobalamin deficient. Thus, there is a need for additional diagnostic tests to further distinguish patients with low serum cobalamin levels who are actually cobalamin deficient and will benefit from lifetime treatment from those who are not deficient and will not benefit. Serum levels of methylmalonic acid and total homocysteine have been shown to be markedly elevated in most patients with cobalamin deficiency, and total homocysteine concentrations are markedly elevated in most patients with folate deficiency. The levels of these metabolites fall to normal if these patients are treated with the appropriate vitamin but remain essentially unchanged if the wrong vitamin is administered. These observations demonstrate that serum methylmalonic acid and total homocysteine levels are useful in diagnosing patients with cobalamin and folate deficiency and in distinguishing between these two vitamin deficiencies.
Subtle and atypical cobalamin deficiency states
Authors: Carmel Ralph 
Published: February 2007

AbstractEvidence for cobalamin (vitamin B12) deficiency usually involves some combination of low serum cobalamin levels, clinical abnormalities (classically, megaloblastic anemia and neurologic defects), metabolic abnormalities, and response to therapy. However, cobalamin deficiency may often display few of the expected clinical findings. Identification of the underlying cause is also important in the diagnosis of deficiency, and its value may be particularly great when the expression of deficiency is subtle. The cause of cobalamin deficiency is usually malabsorptive, but may sometimes be limited to malabsorption of food cobalamin while free cobalamin is absorbed normally. Nongastroenterologic entities may sometimes also be found. All of these considerations allow the proposal of four patterns of cobalamin deficiency. The first type is classical deficiency; typical megaloblastic anemia with or without neurologic dysfunction occurs because of classical cobalamin malabsorption such as lack of intrinsic factor (pernicious anemia). The second type consists of classical cobalamin malabsorption in which the cobalamin deficiency is expressed subtly rather than in classical fashion. There is no megaloblastic anemia and sometimes the only evidence of deficiency may be metabolic. In the third type, cobalamin deficiency is expressed classically but is attributable to a subtle or atypical cause, such as food‐cobalamin malabsorption. In the fourth type, deficiency is both expressed subtly and arises from subtle or atypical causes. Such presentations require further investigation but are a challenging expansion of our understanding and recognition of cobalamin deficiency.
Function of vitamin B12 in the central nervous system as revealed by congenital defects
Authors: Hall Charles A. 
Published: February 2007

AbstractThe 13 cases of methylcobalamin (MeCbl) deficiency presenting in early infancy have all been developmentally delayed, and the majority have had seizures, hypotonia, lethargy, and microcephaly. The CNS injury appears to occur during the first 6 months of postnatal life. The same symptoms are seen in acquired cobalamin (Cbl) deficiency in the same age group. MRI performed at age 18–19 months and after 13–14 months of large amounts of Cbl, in two cases showed delayed myelination, most pronounced in the cerebrum. Isolated MeCbl deficiency is the consequence of cblE and G mutations where the lesion is of a single Cbl‐dependent enzyme, the methyltransferase. One effect of a deficiency of MeCbl, and of the associated failure of the methionine synthase reaction, is, therefore, an impairment of myelination of the brain of the newborn. The slow, but usually incomplete, improvement in psychomotor status after years of treatment with Cbl may be related to the eventual myelination. However, the hypotonia, lethargy, and impaired responsiveness react to treatment with Cbl within 24–48 hours, which suggests an expression of MeCbl deficiency on the CNS distinct from the delayed myelination. Although there is much to be learned, it is now clear that a normally functioning Cbl‐dependent methyl transferase is required for development and function of the human brain.
Low holotranscobalamin II is the earliest serum marker for subnormal vitamin B12 (cobalamin) absorption in patients with AIDS
Authors: Herbert Victor , Fong Warren , Gulle Veronica , Stopler Tracy 
Published: February 2007

AbstractIn AIDS, as previously found in pernicious anemia (PA), the earliest serum marker of subnormal vitamin B12 (cobalamin) absorption, and therefore of negative B12 balance, is low serum holotranscobalamin II (holo‐TC II; B12‐TC II) despite normal total serum B12 level, normal serum homocysteine, and normal classic (oral free radio‐B12) Schilling test. This may be accompanied by subtle and insidious damage to hematopoietic, immunologic, neuropsychiatric, nutritional and alimentary systems, confirmed by correction on therapeutic trial with B12 therapy. Our studies suggest such selective B12 deficiency occurs in about half of the HIV‐1 infected, in part due to frequent depression of B12 absorption by HIV‐1 attack on the gastric mucosa and/or opportunistic infection attack on the small bowel, and in part due to a telescoping of the continuum of the stages of negative B12 balance in relation to damage to B12 delivery by the infective and/or systemic disease process. In AIDS, when total serum B12 is normal despite tissue depletion of B12, if the classic Schilling test does not reveal subnormal food B12 absorption, the food Schilling test does. We hypothesize that DNA‐synthesizing cells of the hematopoietic, immunologic, neurologic and other systems which have surface receptors solely for holo‐TC II, and which have low B12 stores, rapidly become dysfunctional due to B12 deficiency when holo‐TC II is low, while cells (such as liver cells) which also have surface receptors for holohaptocorrin (B12‐haptocorrin) remain B12‐replete. We believe this to be another example of the concept of selective nutrient deficiency in one cell line but not another.
Application of the avidin‐biotin‐peroxidase complex technique for ultraimmunocytochemical characterization of leukemic cells
Authors: Yano Takahiro , Ohtake Ken‐Ichi , Kameda Kazuo , Kizaki Masahiro , Ogawa Tetsuhei 
Published: February 2007

AbstractFor simultaneous demonstration of cellular ultrastructure, myeloperoxidase activity, and presence of a membrane‐bound antigen in a given blood cell, we examined three different fixatives: periodate‐lysine‐paraformaldehyde (PLP) and paraformaldehyde and glutaraldehyde for their applicability to preembedding electron microscopic immunocytochemistry using monoclonal antibodies and the avidin‐biotin‐peroxidase complex (ABC) technique. This procedure was examined in samples from 3 normal volunteers and 29 patients with acute lymphoblastic leukemia (ALL), chronic lymphocytic leukemia (CLL), lymphosarcoma cell leukemia (LSCL), blastic phase of chronic myelogenous leukemia (CML‐BC), or other unclassified leukemias. PLP fixation preserved the immunoreactivity of surface glycoproteins as well as immunoglobulins to the most satisfactory extent. Leukemic cells fixed with PLP maintained their fine structural details, so that we could identify their cytoplasmic organelles, although glutaraldehyde produced the best preservation of cellular ultrastructure. In three patients with ALL, our method revealed that a significant portion of blasts possessed both lymphoid surface antigens and peroxidase‐positive cytoplasmic granules. Our method was also useful in identifying the lineage of peroxidase‐negative leukemic cells, including monoblastic leukemia and megakaryoblastic leukemia cells. Ultraimmunocytochemistry using PLP fixation and the ABC technique may be a promising strategy for determining the nature of blastic cells that remain unclear after a conventional work‐up, for characterizing leukemic cells in patients with a relatively low blast cell count in the bone marrow or peripheral blood, and for estimating the presence and frequency of leukemia with multilineage expression.
Properties of the exchangeable splenic platelets released into the circulation during exercise‐induced thrombocytosis
Authors: Chamberlain Kevin G. , Tong Michael , Penington David G. 
Published: February 2007

AbstractThe human spleen normally retains about one‐third of the body's platelets in an exchangeable pool which can be released into the circulation by α‐adrenergic stimulation. Some previous investigators concluded that the splenic platelet population was enriched in a subpopulation of large, young, dense platelets (megathrombocytes) but more recent research suggests that platelet size, age, and density are largely independent variables. In this investigation the properties of the splenic platelets were studied after their release into the circulation by acute strenuous exercise in 11 normal subjects. The exercise caused a rise in mean platelet count from 245 ± 49 to 328 ± 71 × 109/L—a net increase of 24 ± 6% after correction for haemoconcentration. The mean platelet volume (MPV) of citrated platelets increased from 6.38 ± 0.78 to 6.59 ± 0.68 fL after exercise (P< 0.01)—a rise of 3.7 ± 4.1% suggesting that the MPV of the splenic platelet population was about 20% greater than that of the normal circulating population. The age distribution of the platelets was studied by measuring the platelet monoamine oxidase (MAO) activity several days after irreversible inhibition by tranylcypromine, when the young platelets had normal MAO activity but the older platelets had only 20% of normal activity. The mean platelet MAO activity did not change after exercise, indicating that the age distributions of the circulating and splenic populations were very similar. The platelet contents of several putative markers of platelet age (sialic acid, serotonin, β‐thromboglobulin, β‐N‐acetylglucosaminidase) were also unchanged after exercise. Modal platelet density decreased slightly but not significantly after exercise. The splenic platelet population has a larger MPV but appears to have similar age and density distributions to the basal circulating population.
Effectiveness of the cryosupernatant fraction of plasma in the treatment of refractory thrombotic thrombocytopenic purpura
Authors: Byrnes John J. , Moake Joel L. , Klug Panpit , Periman Phillip 
Published: February 2007

AbstractMany patients with thrombotic thrombocytopenic purpura (TTP) satisfactorily respond to plasma therapy (plasmapheresis and/or plasma infusion). Some, however, respond either not at all or only transiently and incompletely. Evidence indicates that platelets and endothelial cell‐derived unusually large von Willebrand factor (ULvWF) multimers, as well as the largest vWF multimers in plasma, form thrombi which are deposited in the microvascular circulation. Accordingly, platelet transfusions are avoided unless there is intra‐cranial or other life‐threatening hemorrhage. The largest plasma multimers of vWF are, however, replenished by the infusion of large volumes of whole plasma. We postulated that under conditions of massive plasma replacement, plasma depleted of the largest multimers of vWF might be preferable for the treatment of TTP episodes. The largest vWF multimers sediment in the cryoprecipitate, and the cryoprecipitate‐poor fraction of plasma (cryosupernatant) is depleted of these forms. Seven patients responding inadequately to intensive plasma therapy were switched to receive cryosupernatant in place of whole plasma. All patients improved quickly following this change in therapy, and the TTP syndrome resolved in all seven.
Bone marrow in vitro growth and cytogenetic studies in patients with fab‐classified primary myelodysplastic syndromes
Authors: Swolin Birgitta , Rödjer Stig , Westin Jan 
Published: February 2007

AbstractThirty‐eight consecutive patients with a FAB‐classified primary myelodysplastic syndrome (MDS) were investigated for in vitro growth of colony‐forming units for granulocyte‐macrophage precursors (CFU‐GM) and cytogenetic analysis of bone marrow cells. Abnormal CFU‐GM growth was found in 30 patients (79%), and clonal chromosome abnormalities were found in 13 patients (34%). The eight patients who showed normal CFU‐GM growth were either cytogenetically normal (n = 5), or had a 5q‐ deletion (n = 3) as single or dominating karyotypic abnormality. Among the 30 patients with reduced or no colony growth, ten patients had a clonal chromosome abnormality.Leukemia developed in eight patients. None of them grew any CFU‐GM colonies, and three of them were cytogenetically abnormal at the time of diagnosis of MDS.Analysis of the bone marrow in vitro growth for CFU‐GM and the karyotype in patients with MDS emphasizes the close relationship between these disorders and manifest acute leukemia. Subgroups of MDS may be defined by a cytogenetic classification (e.g., the 5q‐ syndrome), and the CFU‐GM growth pattern can be of value for predicting leukemic transformation.
Development of 5q‐ myelodysplasia in a patient with sarcoidosis
Authors: Tunkel Allan R. , Sebastianelli Kenneth J. , Pandit Niraj , Brody Jerome I. 
Published: February 2007

AbstractSarcoidosis has been observed in association with numerous blood dyscrasias including lymphoma, leukemia, and multiple myeloma. This report describes a patient with sarcoidosis and a refractory anemia whose bone marrow karyotype showed deletion of the long arm of chromosome 5, consistent with a myelodysplastic syndrome. Concurrent sarcoidosis and myelodysplasia may relate to the continued availability of cytokines as a consequence of repeated macrophage, T‐cell, and B‐cell interactions, with evolution to the 5q‐ abnormality. This association may merit specific attention in the future approach to the diagnostic evaluation in certain patients with sarcoidosis.
Desferrioxamine improves neutrophil phagocytosis in thalassemia major
Authors: Cantinieaux Brigitte , Hariga Chantal , Fondu Pierre , Ferster Alina , Toppet Michèle 
Published: February 2007

AbstractThe aims of the present study are: first, to assess the toxic role of serum from thalassemic patients in phagocytosis of PMN from healthy controls, and second, to seek to determine whether serum and cellular disturbances of polymorphonuclear neutrophils (PMN) phagocytosis, observed in thalassemic patients, can be prevented and/or corrected by use of desferrioxamine (DFX).Two kinds of in vitro incubations–‐without or with DFX–‐were performed. PMN or serum from thalassemic patients or from healthy controls was used. First, a phagocytosis defect of 3 different bacteria species was induced in PMN from healthy controls by incubation in thalassemic serum. Second, DFX could prevent, already at 1 μM, the phagocytic defect induced in normal PMN by the incubation with thalassemic serum, with disappearance of the toxic role of thalassemic serum at higher concentrations. Third, improvement of the phagocytosis defect of PMN from thalassemic patients was also observed at 1 μM of DFX for the 3 bacteria species. Normalization was obtained at higher concentrations for gram‐negative bacteria.In vivo studies revealed, after a 3 hr subcutaneous infusion of DFX into 3 thalassemic patients, an improvement of the phagocytosis results and a decrease of the Prussian Blue reactivity of the PMN.It is concluded first that an iron‐mediated defect in phagocytosis can be induced in normal neutrophils by incubation in serum from thalassemic patients, and second that a precautious and intensive chelation therapy seems to be advantageous for increasing PMN defense against infectious agents. Special care must nevertheless be taken in order to detect rapidly opportunistic (such as Yersinia) infections.