摘要:

AbstractPlatelet‐associated IgG (PAIgG) can be measured on intact platelets or following platelet lysis. Measurement of PAIgG following platelet lysis may provide different or additional information compared to PAIgG measured on intact platelets. The PAIgG of lysed platelets represents “total” PAIgG, ie, IgG on the surface of platelets plus any IgG that was inside the platelet. To investigate the clinical relevance of the two types of PAIgG assay we performed a prospective study on washed platelets collected from 47 patients with idiopathic thrombocytopenic purpura (ITP). The PAIgG was measured on intact and lysed platelets using an immunoradiometric assay. Platelet‐associated IgG was 2–3 times higher when measured on lysed platelets from healthy controls or patients with ITP compared to PAIgG measured on the same intact platelets. The higher level of PAIgG observed following platelet lysis was not due to the reactions not achieving equilibrium. Using lysed platelets, PAIgG was elevated on 29 of 47 samples from different ITP patients and elevated in 31 samples when measured on intact platelets.The PAIgG is invariably higher when measured following platelet lysis compared measurements made on intact platelets. Neither technique offers a diagnostic advantage over

ISSN:0361-8609    

DOI:10.1002/ajh.2830180102

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractFour patients with idiopathic hemochromatosis were treated with intensive phlebotomy therapy. In 1 to 2 years, 8.8–16.7 g iron was removed. In three out of four patients hemoglobin levels fell at the end of therapy. Serum ferritin was continuously measured during therapy. The greatly elevated serum ferritin levels normalized or decreased to subnormal levels in all patients after therapy. Despite some fluctuations in the first phase of therapy, the fall in serum ferritin was regular with halving of the ferritin levels after about 50% of the excess iron was removed. The normalization of serum ferritin occurred in advance of the hemoglobin decrease at the end of therapy, indicating that in the later stages of therapy the normal iron stores are also depleted. It is emphasized that serum ferritin measurements are useful for monitoring of intensive phlebotomy therapy, and in particular to indicate the end of therapy before anemia develop

ISSN:0361-8609    

DOI:10.1002/ajh.2830180103

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractBecause there is little available data to demonstrate whether demand therapy is adequate to prevent progression of hemophilic arthropathy, a cohort of 64 patients with severe Hemophilia A (Factor VIII level<1%) and no inhibitor were studied with respect to the progression of knee arthropathy over a period of 6 years. Both degree of disease in flexion and extension and progression of disease were rated on an arbitrary scale. At the outset of the study, the majority of knees were either not restricted or mildy restricted as to motion. Of those knees that were not restricted at the outset, 96% remained not restricted. Of the knees that were mildy restricted, only 58% remained stable or improved over the 6 years, and of the knees that were moderately to severely restricted, 75% improved or remained stable. It thus appears that a demand therapy program can maintain normal range of motion in a knee joint. However, if the patient's joint has progressed to mild disease, the ability to maintain or improve the joint with demand therapy appears to be decreased.

ISSN:0361-8609    

DOI:10.1002/ajh.2830180104

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractBy sodium dodecyl sulfate‐polyacrylamide gel electrophoresis (SDS‐PAGE), the protein profile and autophosphorylation of plasma membranes of red cells from 3‐, 9‐, 18‐, and 30‐month‐old rats was examined. No major changes in SDS‐PAGE protein profiles were noticeable. However, endogenous (auto) and exogenous phosphorylation in the presence of (γ−32P)ATP showed an age‐dependent increase in32P utake. pH optimum was between 8.0 and 9.0 for all age groups. Cyclic AMP was without effect on either endogenous or exogenous phosphorylation. Further analysis of the autophosphorylation reaction by quantification of autoradiograms obtained from SDS‐PAGE gels displayed an age‐dependent increase in phosphorylation of bands 2, 3, 4.1, and 4.5 [nomenclature of Fairbanks et al.Biochemistry10:2606, 1971] and of several other minor bands. The data imply that alteration in red cell membranes may occur during aging of rats causing an enhancement in endogenous kinase activity and possibly in the number of phosphat

ISSN:0361-8609    

DOI:10.1002/ajh.2830180105

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractLabeling red blood cells with Na251CrO4enabled us to study certain aspects of red cell survival and sequestration from the circulation. As a random labeling procedure, however, the51Cr method has certain limitations. Therefore, we developed a cohort labeling method using75Se‐methionine as a two‐rat procedure. This gives us a clear pulse‐labeled population of rat red cells to study the dynamics of sequestration. With this labeling procedure, it was possible to demonstrate that 1) there is an increase in the density of red cells with age, 2) a significant sequestration of red cells from the circulation is apparent at the end of 48 days and essentially is complete at the end of 60 days, 3) there is a corresponding uptake of senescent red cells in the spleen, which peaks at 55 days, and 4) the 60‐day end point is sharper and is more definitive when the “specific activity” (cpm per red blood cell) of the labeled red cells in the spleen is compared to that of the red cells still in the circulation. Asialo red cells, obtained by removal of sialic acid with sialidase, frequently have been used as a model for the study of sequestration of senescent red cells. With the technique herein described, it was possible to show that while asialo red cells will inhibit the uptake of labeled asialo red cells, they have no effect on the sequestration of senescent red cells. Presumably, different sites and mechanisms of sequestration a

ISSN:0361-8609    

DOI:10.1002/ajh.2830180106

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractThe effect of glucocorticoid administration on the nuclear segmentation of circulating granulocytes was studied in 13 patients with hematologic disorders and in two normal volunteers. In seventeen of eighteen courses, the granulocyte nuclear lobe count increased from 3.24 ± 0.12 (SD) to 3.41 ± 0.12 by the third day of treatment (P<.001). Nuclear segmentation increased even further with continued therapy during a week of observation. Thus, like deficiencies of folate and of vitamin B12, glucocorticoid therapy causes neutrophil hypersegmentation. The cause of this effect is not know

ISSN:0361-8609    

DOI:10.1002/ajh.2830180107

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractAfter initial French‐American‐British (FAB) diagnosis by a multiinstitutional Southwest Oncology Group panel, slides of acute leukemia cases were recirculated to panel members for second review. The reproducibility of the FAB classification is analyzed. The classification is reproducible in the 70% range in panel reviewer hands and allows remarkable reproducibility in the morphologic and cytochemical distinction of acute lymphoid leukemia (ALL) from acute myeloid leukemia (AML). The limitations of a morphologic and cytochemical classification of acute leukemia are discussed. A simplification of the FAB system, merging M1, M2, and M4 as M7, is proposed; this simplification improves the system's reproducibil

ISSN:0361-8609    

DOI:10.1002/ajh.2830180108

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractCongenital dysgranulopietic neutropenia (CDN) is a recently proposed entity that describes a small subgroup of children with clinically severe neutropenia. We followed and studied a 3‐year‐old girl with neutropenia (<500/mm3) and recurrent severe infections in whom repeated marrow evaluations revealed large (30‐50 μm) multinucleated promyelocytes to polymorphonuclear cells with as many as 4 to 16 nuclei or nuclear lobes, respectively. In addition to the nuclear endoreduplication, ultrastructural and cytochemical evaluation of these cells demonstrated abnormalities in granule genesis and centriole structure. Concomitantly. immunoperoxidase staining indicated that many of the granules were devoid of lactoferrin but not lysozyme. In vitro proliferation studies revealed normal to increased thymidine labeling, normal numbers of colony‐forming cells, and normal colony‐stimulating activity from blood and marrow mononuclear cells, findings consistent with ineffective myelopoiesis. However, serum folate, B12, and lysozyme levels were normal. The nuclear and cytoplasmic abnormalities in this patient result in an extreme example of CDN, distinct from previously descr

ISSN:0361-8609    

DOI:10.1002/ajh.2830180109

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractLymphomas are an uncommon complication of solid organ transplantation and rarely occur after marrow transplantation. When post‐marrow transplant lymphomas have occurred, they have been of donor cell origin and when sought, Epstein‐Barr virus DNA has been found in the tumor. A 21‐year‐old woman developed a poorly differentiated lymphocytic lymphoma 6 months after bone marrow transplantation for acute myeloid leukemia in remission. Cyclosporin A had been used as an immunosuppressant. A chromosomal polymorphism demonstrated that the tumor was of host origin and contained a monoclonal tumor marker, 46, XX INV 4 (p16q12). The tumor did not contain the DNA of the Epstein‐B

ISSN:0361-8609    

DOI:10.1002/ajh.2830180110

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY

摘要:

AbstractTwo patients with immune thrombocytopenic purpura (ITP) refractory to conventional management with steroids and splenectomy were treated with 5‐day courses of intravenous gamma globulin (Gamimune‐Cutter), 400 mg/kg/day. Improvement was observed in both cases even while steroids were being tapered. In each case when thrombocytopenia subsequently recurred or worsened, further therapy with IV gamma globulin without concomitant steroids was followed by definite increase in platelet numbers. These observations confirm that the Cutter preparation of IV gamma globulin is efficacious in the management of refractory ITP and suggest that in this clinical setting response to IV gamma globulins can occur without concomitant administration of corticostero

ISSN:0361-8609    

DOI:10.1002/ajh.2830180111

出版商:Wiley Subscription Services, Inc., A Wiley Company    

年代:1985

数据来源: WILEY