Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00396.X
C. Danielson
Patients with sickle cell disease have abnormal red blood cells (RBCs). This can cause chronic hemolytic anemia and vaso-occlusion leading to tissue hypoxemia and organ dysfunction. RBC exchange transfusion can, without increasing the whole-blood viscosity, quickly replace abnormal erythrocytes with normal and raise the hematocrit resulting in improved delivery of oxygen to hypoxic tissues. Unfortunately, transfusion can also be associated with complications. This paper reviews the role of transfusion, both simple and exchange, in the treatment and prevention of sickle-related complications. The benefits of exchange versus simple transfusion and transfusion versus alternative therapies are discussed.
{"title":"The role of red blood cell exchange transfusion in the treatment and prevention of complications of sickle cell disease.","authors":"C. Danielson","doi":"10.1046/J.1526-0968.2002.00396.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00396.X","url":null,"abstract":"Patients with sickle cell disease have abnormal red blood cells (RBCs). This can cause chronic hemolytic anemia and vaso-occlusion leading to tissue hypoxemia and organ dysfunction. RBC exchange transfusion can, without increasing the whole-blood viscosity, quickly replace abnormal erythrocytes with normal and raise the hematocrit resulting in improved delivery of oxygen to hypoxic tissues. Unfortunately, transfusion can also be associated with complications. This paper reviews the role of transfusion, both simple and exchange, in the treatment and prevention of sickle-related complications. The benefits of exchange versus simple transfusion and transfusion versus alternative therapies are discussed.","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75362900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00400.X
J. Dominguez, Edward Sha
Removal of cryoglobulins by plasma exchange is now an accepted therapy. Cryoglobulins are circulating complexes that can deposit on small vessels and cause limited or extensive tissue injury. There are 3 major classes of cryoglobulins. Type I cryoglobulins are monoclonal and are detected in a variety of lymphoproliferative disorders. Type II cryoglobulins are mixed containing monoclonal and polyclonal IgG or IgM molecules. Type III cryoglobulins are also mixed and contain polyclonal IgG. Type II cryoglobulins are largely caused by hepatitis C virus infection; hence, they are the most common of the 3 types. In hepatitis C, cryoglobulins are linked to glomerular immune complex injury, often times accompanied by vasculitis of the skin, nerves, and other vital organs. Immediate removal of cryoglobulins by plasma exchange is an effective short-term treatment that can complement more-specific therapies. Plasma exchange has also been used to remove other circulating nephrotoxic agents such as antiglomerular basement antibodies that cause Goodpasture's syndrome, protease inhibitor autoantibodies that cause thrombotic thrombocytopenic purpura, and antiglomerular factors that cause some types of focal glomerulosclerosis.
{"title":"Apheresis in cryoglobulinemia complicating hepatitis C and in other renal diseases.","authors":"J. Dominguez, Edward Sha","doi":"10.1046/J.1526-0968.2002.00400.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00400.X","url":null,"abstract":"Removal of cryoglobulins by plasma exchange is now an accepted therapy. Cryoglobulins are circulating complexes that can deposit on small vessels and cause limited or extensive tissue injury. There are 3 major classes of cryoglobulins. Type I cryoglobulins are monoclonal and are detected in a variety of lymphoproliferative disorders. Type II cryoglobulins are mixed containing monoclonal and polyclonal IgG or IgM molecules. Type III cryoglobulins are also mixed and contain polyclonal IgG. Type II cryoglobulins are largely caused by hepatitis C virus infection; hence, they are the most common of the 3 types. In hepatitis C, cryoglobulins are linked to glomerular immune complex injury, often times accompanied by vasculitis of the skin, nerves, and other vital organs. Immediate removal of cryoglobulins by plasma exchange is an effective short-term treatment that can complement more-specific therapies. Plasma exchange has also been used to remove other circulating nephrotoxic agents such as antiglomerular basement antibodies that cause Goodpasture's syndrome, protease inhibitor autoantibodies that cause thrombotic thrombocytopenic purpura, and antiglomerular factors that cause some types of focal glomerulosclerosis.","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"89975983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00224.X
L. Mccarthy
{"title":"Therapeutic apheresis: current perspectives.","authors":"L. Mccarthy","doi":"10.1046/J.1526-0968.2002.00224.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00224.X","url":null,"abstract":"","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90171642","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00330.X
Changsheng Zhao, Xiaohua Zhang, Y. Yue
Membrane plasma fractionation is an important part of double filtration plasmapheresis. We evaluated a newly developed polyethersulfone (PES) hollow-fiber plasma fractionation membrane, using 3 parameters: abnormal protein ratio (APRR), different protein ratio (DPR), and plasma filtration efficiency, except sieving coefficients. The APRR of the fractionation membrane was over 0.90, and the DPR changed after the plasma fractionation. We also compared the sieving coefficients of the PES hollow membrane with some commercial plasma fractionation membranes. The data were significant for the clinical use of the PES hollow-fiber fractionation membrane.
{"title":"Performance evaluation of plasma fractionation membrane.","authors":"Changsheng Zhao, Xiaohua Zhang, Y. Yue","doi":"10.1046/J.1526-0968.2002.00330.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00330.X","url":null,"abstract":"Membrane plasma fractionation is an important part of double filtration plasmapheresis. We evaluated a newly developed polyethersulfone (PES) hollow-fiber plasma fractionation membrane, using 3 parameters: abnormal protein ratio (APRR), different protein ratio (DPR), and plasma filtration efficiency, except sieving coefficients. The APRR of the fractionation membrane was over 0.90, and the DPR changed after the plasma fractionation. We also compared the sieving coefficients of the PES hollow membrane with some commercial plasma fractionation membranes. The data were significant for the clinical use of the PES hollow-fiber fractionation membrane.","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"82621344","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00392.X
J. Jansen, James M. Thompson, M. Dugan, P. Nolan, Michael Wiemann, R. Birhiray, P. Jean Henslee‐Downey, Luke P. Akard
Peripheral blood progenitor cells (PBPCs) have become increasingly popular over the last 15 years as the source of hematopoietic stem cells for transplantation. In the early 1990s, PBPCs replaced bone marrow (BM) as the preferred source of autologous stem cells, and recently the same phenomenon is seen in the allogeneic setting. Under steady-state conditions, the concentration of PBPCs (as defined by CFU-GM and/or CD34+ cells) is very low, and techniques were developed to increase markedly this concentration. Such mobilization techniques include daily injections of filgrastim (G-CSF) or a combination of chemotherapy and growth factors. Leukapheresis procedures allow the collection of large numbers of circulating white blood cells (and PBPCs). One or two leukapheresis procedures are often sufficient to obtain the minimum number of CD34+ cells considered necessary for prompt and consistent engraftment (i.e., 2.5-5.0 x 10(6)/kg). As compared to BM, autologous transplants with PBPCs lead to faster hematologic recovery and have few, if any, disadvantages. In the allogeneic arena, PBPCs also result in faster engraftment, but at a somewhat higher cost of chronic graft-versus-host disease (GvHD). This may be a double-edged sword leading to both increased graft-versus-tumor effects and increased morbidity. The rapid advances in the study of hematopoietic, and even earlier, stem cells will continue to shape the future of PBPC transplantation.
在过去的15年中，外周血祖细胞(PBPCs)作为移植用造血干细胞的来源越来越受欢迎。在20世纪90年代早期，PBPCs取代骨髓(BM)成为自体干细胞的首选来源，最近在同种异体环境中也出现了同样的现象。在稳态条件下，pbpc(由CFU-GM和/或CD34+细胞定义)的浓度非常低，并且开发了显着增加该浓度的技术。这种动员技术包括每日注射非格拉西汀(G-CSF)或化疗和生长因子的组合。白细胞分离程序允许收集大量循环白细胞(和pbpc)。一次或两次白细胞分离术通常足以获得被认为是快速和一致移植所需的最小CD34+细胞数量(即2.5-5.0 x 10(6)/kg)。与骨髓移植相比，带有PBPCs的自体移植可以更快地恢复血液学，并且几乎没有缺点。在同种异体领域，pbpc也导致更快的移植，但慢性移植物抗宿主病(GvHD)的成本更高。这可能是一把双刃剑，既增加了移植物抗肿瘤效果，也增加了发病率。造血研究的快速发展，甚至更早，干细胞将继续塑造PBPC移植的未来。
{"title":"Peripheral blood progenitor cell transplantation.","authors":"J. Jansen, James M. Thompson, M. Dugan, P. Nolan, Michael Wiemann, R. Birhiray, P. Jean Henslee‐Downey, Luke P. Akard","doi":"10.1046/J.1526-0968.2002.00392.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00392.X","url":null,"abstract":"Peripheral blood progenitor cells (PBPCs) have become increasingly popular over the last 15 years as the source of hematopoietic stem cells for transplantation. In the early 1990s, PBPCs replaced bone marrow (BM) as the preferred source of autologous stem cells, and recently the same phenomenon is seen in the allogeneic setting. Under steady-state conditions, the concentration of PBPCs (as defined by CFU-GM and/or CD34+ cells) is very low, and techniques were developed to increase markedly this concentration. Such mobilization techniques include daily injections of filgrastim (G-CSF) or a combination of chemotherapy and growth factors. Leukapheresis procedures allow the collection of large numbers of circulating white blood cells (and PBPCs). One or two leukapheresis procedures are often sufficient to obtain the minimum number of CD34+ cells considered necessary for prompt and consistent engraftment (i.e., 2.5-5.0 x 10(6)/kg). As compared to BM, autologous transplants with PBPCs lead to faster hematologic recovery and have few, if any, disadvantages. In the allogeneic arena, PBPCs also result in faster engraftment, but at a somewhat higher cost of chronic graft-versus-host disease (GvHD). This may be a double-edged sword leading to both increased graft-versus-tumor effects and increased morbidity. The rapid advances in the study of hematopoietic, and even earlier, stem cells will continue to shape the future of PBPC transplantation.","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88556202","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00393.X
M. J. Drew
The dysproteinemias consist of a broad range of serious disease states with the common thread of excessive production of an abnormal, or para-protein. Various clinical syndromes may arise, either from the underlying disease process, the excess paraprotein, or both. Clinical presentation depends upon the organ system(s) affected by the abnormal protein. Diseases included under the classification of dysproteinemias include cryoprotein-related diseases, Waldenström's macroglobulinemia, hyperviscosity syndrome, monoclonal gammopathy, multiple myeloma, light chain disease, and amyloidosis. Plasmapheresis, often in conjunction with other therapies, has been widely used to treat the dysproteinemias and their resulting clinical syndromes. Automated plasmapheresis, which separates plasma from the cellular blood elements by centrifugation, is used most commonly in the United States. Membrane separation and immunoadsorption techniques are more commonly used in Europe and Japan. In automated plasmapheresis, the plasma is removed from the patient's circulation and replaced with a protein-based fluid such as 5% human albumin solution or plasma protein fraction or with fresh frozen plasma. Membrane separation and immunoadsorption allow the offending proteins to be removed more selectively from the patient's plasma prior to the plasma being returned to the patient. This review article presents a description of each disease, the rationale for plasmapheresis therapy, recommended schedules of plasmapheresis, and the use of adjunctive therapies. Results of published studies, case reports, and the author's experience in treating these diseases will serve as the foundation for discussion.
{"title":"Plasmapheresis in the dysproteinemias.","authors":"M. J. Drew","doi":"10.1046/J.1526-0968.2002.00393.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00393.X","url":null,"abstract":"The dysproteinemias consist of a broad range of serious disease states with the common thread of excessive production of an abnormal, or para-protein. Various clinical syndromes may arise, either from the underlying disease process, the excess paraprotein, or both. Clinical presentation depends upon the organ system(s) affected by the abnormal protein. Diseases included under the classification of dysproteinemias include cryoprotein-related diseases, Waldenström's macroglobulinemia, hyperviscosity syndrome, monoclonal gammopathy, multiple myeloma, light chain disease, and amyloidosis. Plasmapheresis, often in conjunction with other therapies, has been widely used to treat the dysproteinemias and their resulting clinical syndromes. Automated plasmapheresis, which separates plasma from the cellular blood elements by centrifugation, is used most commonly in the United States. Membrane separation and immunoadsorption techniques are more commonly used in Europe and Japan. In automated plasmapheresis, the plasma is removed from the patient's circulation and replaced with a protein-based fluid such as 5% human albumin solution or plasma protein fraction or with fresh frozen plasma. Membrane separation and immunoadsorption allow the offending proteins to be removed more selectively from the patient's plasma prior to the plasma being returned to the patient. This review article presents a description of each disease, the rationale for plasmapheresis therapy, recommended schedules of plasmapheresis, and the use of adjunctive therapies. Results of published studies, case reports, and the author's experience in treating these diseases will serve as the foundation for discussion.","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88064702","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00401.X
J. Kincaid
The inflammatory demyelinating peripheral neuropathies are recognizable treatable entities within the broad field of diseases of the peripheral nervous system. Most peripheral nerve diseases are still of undetermined causation and have no direct disease-modifying treatment. This paper reviews the diagnostic features of the inflammatory neuropathies, presumed mechanisms of pathogenesis, and the role of apheresis, along with other immunomodulating therapies, in the management of these diseases.
{"title":"Apheresis in treatment of the inflammatory demyelinating peripheral neuropathies.","authors":"J. Kincaid","doi":"10.1046/J.1526-0968.2002.00401.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00401.X","url":null,"abstract":"The inflammatory demyelinating peripheral neuropathies are recognizable treatable entities within the broad field of diseases of the peripheral nervous system. Most peripheral nerve diseases are still of undetermined causation and have no direct disease-modifying treatment. This paper reviews the diagnostic features of the inflammatory neuropathies, presumed mechanisms of pathogenesis, and the role of apheresis, along with other immunomodulating therapies, in the management of these diseases.","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90344911","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00319.X
S. Kutlay, O. Ilhan, O. Arslan, M. Beksaç
The aim of this study was to evaluate the in vitro activation in platelet suspensions collected with CS 3000 Plus and Cobe Spectra cell separators using platelet storage containers and the role of white blood cell (WBC) concentration of the suspension in this activation. Seventy-seven donors were subjected to automated platelet donations with 1 type of equipment (37 with Cobe Spectra and 40 with CS 3000 Plus). Blood samples were obtained immediately after separation and on the third day of storage at 22 degrees C in constant agitation. The WBC concentrations of these samples were studied before storage. Paraformaldehyde-fixed platelets were incubated with 2 murine monoclonal antibodies: CD42b and CD62. Murine monoclonal antibody immunoglobulin G was used as a negative isotypic control. Bound antibody was then quantitated by flow cytometry. On the third day of storage, a significant increase in CD62 expression rate was observed in platelet suspensions collected with both kinds of equipment. Mean expression rates for Cobe Spectra on Day 0 and Day 3 were 25.6 +/- 6.2% and 69.2 +/- 9.7%, respectively. Mean expression rates for CS 3000 Plus on Day 0 and Day 3 were 23.4 +/- 8.2% and 67.0 +/- 8.2%, respectively. The mean results for both devices were 22.8 +/- 4.56% for Day 0 and 68.7 +/- 13.2% for Day 3. There was no difference between CD42b mean fluorescence intensity on Days 0 and 3 for the 2 devices (p > 0.5). Mean WBC concentrations in the platelet suspensions for Cobe Spectra and CS 3000 Plus were 0.37 x 10(3)/microl and 0.42 x 10(3)/microl, respectively, and there was no relation between WBC concentration and increase in CD62 expression. Both kinds of equipment were found to be similar according to in vitro activation markers.
{"title":"Influence of storage time on activation of platelets collected with CS 3000 Plus and Cobe Spectra using platelet storage containers.","authors":"S. Kutlay, O. Ilhan, O. Arslan, M. Beksaç","doi":"10.1046/J.1526-0968.2002.00319.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00319.X","url":null,"abstract":"The aim of this study was to evaluate the in vitro activation in platelet suspensions collected with CS 3000 Plus and Cobe Spectra cell separators using platelet storage containers and the role of white blood cell (WBC) concentration of the suspension in this activation. Seventy-seven donors were subjected to automated platelet donations with 1 type of equipment (37 with Cobe Spectra and 40 with CS 3000 Plus). Blood samples were obtained immediately after separation and on the third day of storage at 22 degrees C in constant agitation. The WBC concentrations of these samples were studied before storage. Paraformaldehyde-fixed platelets were incubated with 2 murine monoclonal antibodies: CD42b and CD62. Murine monoclonal antibody immunoglobulin G was used as a negative isotypic control. Bound antibody was then quantitated by flow cytometry. On the third day of storage, a significant increase in CD62 expression rate was observed in platelet suspensions collected with both kinds of equipment. Mean expression rates for Cobe Spectra on Day 0 and Day 3 were 25.6 +/- 6.2% and 69.2 +/- 9.7%, respectively. Mean expression rates for CS 3000 Plus on Day 0 and Day 3 were 23.4 +/- 8.2% and 67.0 +/- 8.2%, respectively. The mean results for both devices were 22.8 +/- 4.56% for Day 0 and 68.7 +/- 13.2% for Day 3. There was no difference between CD42b mean fluorescence intensity on Days 0 and 3 for the 2 devices (p > 0.5). Mean WBC concentrations in the platelet suspensions for Cobe Spectra and CS 3000 Plus were 0.37 x 10(3)/microl and 0.42 x 10(3)/microl, respectively, and there was no relation between WBC concentration and increase in CD62 expression. Both kinds of equipment were found to be similar according to in vitro activation markers.","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"88706674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00394.X
A. Greist
An elevated platelet count is now a common finding in both hospitalized and ambulatory patients with the advent of automated complete blood cell counters. Clinicians may be called upon to make a distinction between a reactive process and a primary hematologic disorder as the cause of a thrombocytosis and to determine whether treatment is indicated. Essential thrombocythemia and other myeloproliferative disorders may present with marked increases in the platelet counts and may be associated with thrombohemorrhagic complications. Reactive thrombocytosis can be caused by iron deficiency and a variety of inflammatory conditions, infections, malignancy, bleeding or hemolysis, splenectomy, and drugs. Acute therapy for all of these disorders has included blood component removal, specifically plateletpheresis. The role of plateletpheresis in current management of thrombocytosis is considered, based on current knowledge of pathophysiology and a review of the literature.
{"title":"The role of blood component removal in essential and reactive thrombocytosis.","authors":"A. Greist","doi":"10.1046/J.1526-0968.2002.00394.X","DOIUrl":"https://doi.org/10.1046/J.1526-0968.2002.00394.X","url":null,"abstract":"An elevated platelet count is now a common finding in both hospitalized and ambulatory patients with the advent of automated complete blood cell counters. Clinicians may be called upon to make a distinction between a reactive process and a primary hematologic disorder as the cause of a thrombocytosis and to determine whether treatment is indicated. Essential thrombocythemia and other myeloproliferative disorders may present with marked increases in the platelet counts and may be associated with thrombohemorrhagic complications. Reactive thrombocytosis can be caused by iron deficiency and a variety of inflammatory conditions, infections, malignancy, bleeding or hemolysis, splenectomy, and drugs. Acute therapy for all of these disorders has included blood component removal, specifically plateletpheresis. The role of plateletpheresis in current management of thrombocytosis is considered, based on current knowledge of pathophysiology and a review of the literature.","PeriodicalId":79755,"journal":{"name":"Therapeutic apheresis : official journal of the International Society for Apheresis and the Japanese Society for Apheresis","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2002-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90700856","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2002-02-01DOI: 10.1046/J.1526-0968.2002.00402.X
P. Porcu, Sherif Farag, G. Marcucci, S. Cataland, M. Kennedy, M. Bissell
Both in children and adults, acute leukemia may present with extremely high blast counts; a phenomenon known as hyperleukocytosis. Respiratory failure, intracranial bleeding, and severe metabolic abnormalities frequently occur in acute hyperleukocytic leukemias (AHLs) and are the primary determinants of the high early mortality (20% to 40%) observed. The process leading to these complications has long been known as leukostasis, but the biological mechanisms underlying its development and progression have remained unclear. Traditionally, leukostasis has been attributed to overcrowding of leukemic blasts in the microcirculation, and its treatment has focused on prompt leukocytoreduction. However, it is becoming increasingly evident that leukostasis results from the adhesive interactions between leukemic blasts and the endothelium; a mechanism that none of the current therapies directly addresses. The endothelial damage associated with leukostasis is likely to be mediated by cytokines released in situ and by subsequent migration of leukemic blasts in the perivascular space. The adhesion molecules displayed by the leukemic blasts and their chemotactic response to the cytokines in the vascular microenvironment are probably more important in causing leukostasis than the cell number. This may explain why leukostasis may develop in some patients with AHL and not in others, and why some patients with acute leukemia without hyperleukocytosis (<50,000 blasts/mm(3)) develop leukostasis and respond to leukocytoreduction. Leukapheresis effectively reduces the blast count in many patients with AHL and is routinely used for immediate leukocytoreduction. However, the most appropriate use of leukapheresis in acute leukemia remains unclear, and the procedure may not prevent early death more efficiently than fluid therapy, hydroxyurea, and prompt induction chemotherapy. The use of cranial irradiation remains very controversial and is not generally recommended. The identification of the adhesion molecules, soluble cytokines, and chemotactic ligand-receptor pairs mediating endothelial cell damage in AHL should become a priority if better outcomes are desired.
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