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Heintz, Nicholas H.; Howard, Phillip L.
doi: 10.1002/ajh.2830300102pmid: 2910077
Hemoglobin Casper is characterized by the substitution of cytidine for thymidine in condon 106 of the beta globin gene. This substitution results in the creation of a new restriction site for Msp I but not for the isoschizimer Hpa II. The restriction pattern following digestion with Msp I reveals a 9.9‐kb fragment not seen in normal individuals or following digestion of Casper DNA with Hpa II. This finding confirms the predicted base mutation and indicates that the cytidine in the newly acquired CpG site is methylated.
Elliott, William L.; Sorli, Christopher H.; Reisert, Patricia S.; Humphreys, Robert E.
doi: 10.1002/ajh.2830300103pmid: 2642651
Antibodies to either Ii or class II major histocompatibility complex (MHC) antigens did not recognize cell surface forms of Ii in immunoprecipitates of cells that had been radioiodinated by the lactoperoxidase method, whereas they bound (35S)methionine metabolically labeled molecules. N‐hydroxysuccinimidobiotin (NHS‐B) and biotin hydrazide (B‐H) were used to react more generally with cell surface proteins via amino groups and nitrene coupling, respectively. Each of these latter compounds labeled α and β chains of class II MHC antigens as seen in Western‐blotted, electrophoresed immunoprecipitates probed with 125I‐labeled streptavidin but not Ii or its associated forms. Although tyrosine residues might have been inaccessible to radioiodination in carbohydratederivatized forms of Ii, the lack of Ii biotinylation in these controled, sensitive studies was consistent with the view that Ii forms were not surface expressed, with the possible exception of the chondroitin sulfate‐derivatized forms of Ii (Ii‐CS).
Takahashi, Hoyu; Tatewaki, Wataru; Nakamura, Tadao; Hanano, Masaharu; Wada, Ken; Shibata, Akira
doi: 10.1002/ajh.2830300104pmid: 2521276
The profile of blood coagulation and fibrinolysis was studied in detail in eight patients with acute thrombotic thrombocytopenic purpura (TTP). In the majority of the patients, fibrinogen, factor XIII, antithrombin III, α2‐plasmin inhibitor, plasminogen, and α2‐macroglobulin were normal, whereas FDP, plasmin‐α2‐plasmin inhibitor complex, and tissuetype plasminogen activator antigen were marginally or moderately elevated. Low fibronectin values were observed in four patients. Protein C and C4b‐binding protein were nearly normal, whereas total protein S and free protein S were reduced in five and six patients, respectively. A positive correlation was found between total protein S and C4 and between free protein S and C3. von Willebrand factor antigen (vWf:Ag) and ristocetin cofactor (RCof) were either normal or elevated, but RCof/vWf:Ag ratio was decreased in seven patients. Crossed immunoelectrophoresis and sodium dodecyl sulfate (SDS)‐agarose gel electrophoresis revealed that the large vWf multimers were either absent from or relatively decreased in all patients except one. In addition, one patient had unusually large vWf multimers, and a low‐molecular‐weight vWf fragment was apparently observed in three patients. These findings indicate that the intravascular generation of thrombin and plasmin was minimal in TTP and suggest that the alterations of the vWf molecule were caused not only by consumption through its participation in platelet thrombus formation but also by accelerated proteolysis. Low protein S values would be related to the immunological abnormalities underlying TTP.
Schwartz, Richard S.; Ewing, Nadia P.; Gorenc, Toni J.; Spero, Joel A.
doi: 10.1002/ajh.2830300105pmid: 2491929
To determine if heat‐treated factor IX complex concentrate is as effective as nonheated factor IX complex concentrate for treatment of subjects with hemophilia A and antifactor VIII antibodies (inhibitor patients), we have retrospectively reviewed consecutive home treatment records of ten inhibitor patients who had been receiving nonheat‐treated factor IX complex concentrate (NHT‐Konyne) and subsequently converted to heat‐treated factor complex concentrate (Konyne‐HT) when it was licensed in late 1984. Overall, 162 of 284 (57%) separate bleeding episodes treated with NHT‐Konyne and 53 of 80 (66.3%) separate bleeding episodes treated with Konyne‐HT required only one treatment course of ∼60‐75 U/kg. The distribution of bleeding sites and the absolute factor IX unitage required per treatment episode were similar for both preparations. These data suggest that the percentage of hemophilic inhibitor patients responding to factor IX complex concentrate remains at least 50%, as was reported several years ago in a controlled study, and that inhibitor bypass activity has not altered by heat treatment.
Barr, Ronald D.; Koekebakker, Marijke
doi: 10.1002/ajh.2830300106pmid: 2910078
Quantitation of small amounts (2–3 μg) of hemoglobin (Hb) was achieved by each of three spectrophotometric techniques. An assay employing benzidine base consistently underestimated Hb at this level and gave a wide scatter of results at higher values. The cyanmethemoglobin method was at the limit of its sensitivity and suffers from the disadvantage of having high optical densities in the blank samples. By contrast, pyridine hemochromogen can be detected accurately with 200 ng of Hb (equivalent of 6,000 mature erythrocytes), and the regression line with this technique virtually passes through the origin. Furthermore, the extinction coefficient of pyridine hemochromogen in the Soret band is 13.5‐fold greater than that of cyanmethemoglobin. In normal human erythroid clones, generated in vitro from bone marrow cells, mean cell hemoglobin (MCH) values were determined after various intervals of culture. The MCH after 5, 7, 10, and 14 days were 11.8, 15.8, 26.6, and 34.4 pg, respectively, by the pyridine hemochromogen method. Reaction product was also identified in granulocyte‐macrophage clones, presumably reflecting the content of other heme proteins such as catalase and cytochromes. Account must be taken of this non‐Hb material in computing true MCH values for erythroid cells.
Leebeek, Frank W. G.; Knot, Eduard A. R.; Cate, Jan Wouter Ten; Traas, Daan W.
doi: 10.1002/ajh.2830300107pmid: 2491930
We report a 45‐year‐old female patient with recurrent spontaneous deep vein thrombosis associated with an isolated hypoplasminogenemia (plasminogen activity and antigen level of 42% and 37%, respectively). The plasminogen molecule was normal as demonstrated by a normal activation by tissue plasminogen activator, electrophoretic mobility on crossed immunoelectrophoresis, molecular weight, and binding to lysine sepharose. All other hemostatic parameters predisposing to recurrent thrombosis were normal. A stimulation test with desmopressin acetate (DDAVP) showed a normal plasma rise of both tissue plasminogen activator and factor VIIIR:WF. This isolated plasminogen deficiency apparently is due to a decreased synthesis of a normal plasminogen molecule and is associated with a severe thrombotic tendency.
Kerrigan, Daniel P.; Foucar, Kathy; Dressler, Lynn
doi: 10.1002/ajh.2830300108pmid: 2535611
Evolution of low‐grade Non‐Hodgkin Lymphoma (NHL) into a more aggressive neoplasm is a common, well‐documented event in NHL. The reverse process, in which a less aggressive component becomes evident during the course of treatment for a highergrade NHL, has only recently been recognized. This lymphoma “downgrading” has been reported at the time of relapse in both radiation‐ and chemotherapy‐treated patients who initially presented with high‐ or intermediate‐grade lymphoma. The etiology of this unusual transformation has not yet been determined. We present the clinical, morphologic, immunologic, and flow‐cytometric features of a patient with diffuse immunoblastic lymphoma who achieved a complete response to chemotherapy and then relapsed with follicular small‐cleaved‐cell lymphoma 3 years later. Morphologic and immunophenotypic findings suggest that both immunoblasts and small cleaved cells were present in the initial biopsy. DNA content analysis of the initial and relapse biopsies suggests that the immunoblastic component was more susceptible than the small cleaved cells to the chemotherapy that the patient received. Successful eradication of the rapidly proliferating immunoblasts with survival of less rapidly proliferating small cleaved cells may account for the unusual histologic transformation seen in this case.
Heaton, David C.; Duff, Gerald B.
doi: 10.1002/ajh.2830300109pmid: 2910079
Bilateral ovarian relapse in a young woman with acute lymphoblastic leukaemia (ALL) was treated by oophorectomy and intensive chemotherapy. She remains well off therapy 97 months following diagnosis and 45 months from ovarian relapse.
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