Pathophysiology and treatment of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS).

Abstract:

:The central pathogenic feature of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS) is the formation of platelet aggregates, perhaps by damaged endothelial cells. The evidence for endothelial cell damage has been supported by multiple findings, including the harmful effects of TTP/HUS plasma, which induces endothelial cell apoptosis. Ultra-large multimers of von Willebrand factor (vWf), which activates platelets, are found in TTP/HUS patients, presumably after being released into circulation from damaged endothelial cells. It has been reported that loss or dysfunction of the vWf-cleaving protease is related to the development of acute or chronic TTP/HUS. Detection of platelet activation in TTP/HUS patients, which previously had been difficult, is now possible with a particle-counting technique using light scattering. TTP/HUS occurring after bone marrow transplantation (BMT), similar to that in classic TTP/HUS, appears to represent a separate facet of the disease. We observed that BMT-TTP/HUS might be predicted at an early stage by determining any increase in plasma interleukin-12 at the time of leukocyte recovery. It is known that plasma treatment is effective for TTP/HUS patients; we found that a high-molecular-weight fraction (HMW-F) of plasma is effective in treating chronic TTP/HUS patients. HMW-F of plasma may contain the main factor necessary for improvement of TTP/HUS syndrome.

journal_name

Int J Hematol

authors

Kakishita E

subject

Has Abstract

pub_date

2000-06-01 00:00:00

pages

320-7

issue

4

eissn

0925-5710

issn

1865-3774

journal_volume

71

pub_type

杂志文章,评审
  • CD34+ progenitor cell transplantation from two HLA-mismatched healthy fathers to two infants with severe aplastic anemia.

    abstract::Pluripotent stem cells of hematopoiesis are included among CD34+ cells in the blood and bone marrow. After granulocyte-colony stimulating factor (G-CSF) mobilization, 1-2% of the mononuclear cells in the blood are CD34+ cells, which can be obtained by leukapheresis. We performed CD34+ progenitor cell transplantation i...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1016/s0925-5710(97)00088-1

    authors: Yasui M,Park YD,Okamura T,Chayama K,Yoshimoto T,Inoue M,Yagi K,Kawa K

    更新日期:1998-01-01 00:00:00

  • Neutropenic enterocolitis in adult leukemias.

    abstract::Neutropenic enterocolitis is a frequent autopsy finding in adult patients with acute leukemias. The predisposing factors other than neutropenia are not clear. There are also contradictions about treatment. Therefore, this entity still presents a diagnostic and therapeutic dilemma for clinicians. This retrospective stu...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:

    authors: Büyükaşik Y,Ozcebe OI,Haznedaroğlu IC,Sayinalp N,Soylu AR,Ozdemir O,Dündar S

    更新日期:1997-07-01 00:00:00

  • Excision repair cross-complementing group 2/Xeroderma pigmentousm complementation group D (ERCC2/XPD) genetic variations and susceptibility to diffuse large B cell lymphoma in Egypt.

    abstract::Diffuse large B-cell lymphoma (DLBCL) is a genetically heterogeneous neoplasm. Although several genetic and environmental factors have been postulated, no obvious risk factors have been emerged for DLBCL in the general population. DNA repair systems are responsible for maintaining the integrity of the genome and prote...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-013-1462-1

    authors: El-Din MA,Khorshied MM,El-Saadany ZA,El-Banna MA,Reda Khorshid OM

    更新日期:2013-12-01 00:00:00

  • Transforming growth factor beta, pleiotropic regulator of hematopoietic stem cells: potential physiological and clinical relevance.

    abstract::Transforming growth factor beta (TGF-beta) is a pleiotropic regulator of all stages of hematopoieis. Depending on the differentiation stage of the target cell, the local environment, and the concentration of TGF-beta, TGF-beta can be proproliferative or antiproliferative, proapoptotic or antiapoptotic, and/or prodiffe...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/BF02982545

    authors: Ruscetti FW,Bartelmez SH

    更新日期:2001-07-01 00:00:00

  • Molecular basis of clonal evolution in multiple myeloma.

    abstract::The treatment outcome of multiple myeloma (MM) is worse than expected from the average numbers of non-synonymous mutations, which are roughly correlated with the prognosis of cancer patients. The refractoriness of MM may be ascribed to the complex genomic architecture and clonal behavior of the disease. In MM, disease...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/s12185-020-02829-6

    authors: Furukawa Y,Kikuchi J

    更新日期:2020-04-01 00:00:00

  • A prospective cohort study comparing achieved anti-factor Xa peak levels in pregnant and non-pregnant patients receiving therapeutic-dose low-molecular-weight heparin.

    abstract::Venous thromboembolism (VTE) is a leading cause of morbidity and mortality in pregnant women. Enoxaparin is a low-molecular-weight heparin used during pregnancy to treat or prevent VTE. In this study, we compare anti-factor Xa peak levels in pregnant and non-pregnant women, and explore the association between anti-fac...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-020-02873-2

    authors: Aleidan FAS,Aljarba GA,Aldakhil AA,Allehyani BI,Yahia MA,Alghtani NE,Badri M,Alaklabi AA,Alsuhaibani A,Crowther MA

    更新日期:2020-07-01 00:00:00

  • Stable response after administration of stem cell factor combined with granulocyte colony-stimulating factor in aplastic anemia.

    abstract::We report successful treatment with 25 microg/kg of recombinant methionyl human stem cell factor (SCF) combined with 400 microg/m2 of recombinant human granulocyte colony-stimulating factor (G-CSF) in 2 patients with aplastic anemia refractory to immunosuppressive therapy. In one patient, hemoglobin levels increased f...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1532/IJH97.05098

    authors: Usuki K,Iki S,Arai S,Iijima K,Takaku F,Urabe A

    更新日期:2006-06-01 00:00:00

  • Dominant beta-thalassemia with hemoglobin Hradec Kralove: enhanced hemolysis in the spleen.

    abstract::We describe a 6-year-old girl and her mother with dominant beta-thalassemia due to hemoglobin Hradec Kralove (Hb HK). Both patients presented microcytic anemia, jaundice, splenomegaly, cholelithiasis, and recurrent hemolytic bouts. Osmotic resistance tests using saline and coiled planet centrifugation revealed the inc...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/BF02983557

    authors: Ohga S,Nomura A,Takada H,Kato J,Ideguchi H,Hattori Y,Suda M,Suita S,Hara T

    更新日期:2003-11-01 00:00:00

  • Methylation status of nine tumor suppressor genes in multiple myeloma.

    abstract::Aberrant methylation in promoter-associated CpG islands has been recognized as a major mechanism for tumor suppressor gene silencing in several malignancies. We determined the methylation status of nine tumor suppressor genes in 68 newly diagnosed MM patients by methylation-specific PCR. The frequency of promoter hype...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-009-0459-2

    authors: Braggio E,Maiolino A,Gouveia ME,Magalhães R,Souto Filho JT,Garnica M,Nucci M,Renault IZ

    更新日期:2010-01-01 00:00:00

  • Gene-marking studies of hematopoietic cells.

    abstract::Gene-marking studies were the first approved clinical protocols introducing exogenous genetic material into human cells. Such studies were never intended to provide direct therapeutic benefit. Instead, they were expected to provide information about normal cell biology and disease pathogenesis that could not be obtain...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/BF02981898

    authors: Bollard CM,Heslop HE,Brenner MK

    更新日期:2001-01-01 00:00:00

  • Successful treatment with bortezomib in type-1 cryoglobulinemic vasculitis patient after rituximab failure: a case report and literature review.

    abstract::Type-1 cryoglobulinemic vasculitis (CV) and mixed CV differ in their pathophysiology, clinical expression and treatment response. We report one patient with type-1 cryoglobulinemic vasculitis and skin ulcers that had remained active despite treatment with a variety of immunomodulating drugs including rituximab. The pa...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/s12185-013-1323-y

    authors: Besada E,Vik A,Koldingsnes W,Nossent JC

    更新日期:2013-06-01 00:00:00

  • Multicenter, prospective study to evaluate the efficacy of biweekly romiplostim administration in patients with immune thrombocytopenia.

    abstract::Multicenter, prospective study was conducted to evaluate the efficacy of biweekly romiplostim in maintaining platelet ≥ 30 × 10(9)/L for at least 4 weeks. Treatment was started with a weekly injection (1 mcg/kg), and the dose was escalated until a titrated dose was achieved that maintained a platelet 50-200 × 10(9)/L ...

    journal_title:International journal of hematology

    pub_type: 杂志文章,多中心研究

    doi:10.1007/s12185-015-1889-7

    authors: Park S,Yoon SS,Lee JH,Park JS,Jang JH,Lee JW

    更新日期:2016-01-01 00:00:00

  • Effects of cytokines on stem cell engraftment depends on time of evaluation post-marrow-infusion.

    abstract::In vitro treatment of bone marrow cells to expand stem cells may lead to impaired hematopoietic long-term reconstitution. Here we report on studies that show that cytokine-treated cells maintain short-term reconstitution, but lose that potential with time posttransplantation. Hematopoietic progenitors assayed in vitro...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:

    authors: Peters SO,Habibian HK,Vergilis K,Quesenberry PJ

    更新日期:1999-08-01 00:00:00

  • Current status of ex vivo gene therapy for hematological disorders: a review of clinical trials in Japan around the world.

    abstract::Gene therapies are classified into two major categories, namely, in vivo and ex vivo. Clinical trials of human gene therapy began with the ex vivo techniques. Based on the initial successes of gene-therapy clinical trials, these approaches have spread worldwide. The number of gene therapy trials approved worldwide inc...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/s12185-016-2030-2

    authors: Tani K

    更新日期:2016-07-01 00:00:00

  • Application of natural killer T-cells to posttransplantation immunotherapy.

    abstract::Graft-versus-host disease (GVHD) and graft-versus leukemia (GVL) effects are closely related to each other after allogeneic stem cell transplantation. This association exists because of the extensive and complicated interaction between cellular donor components and recipient components concomitant with cytokine storms...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/BF02983991

    authors: Fujii S

    更新日期:2005-01-01 00:00:00

  • Recent advances in the study of immunodeficiency and DNA damage response.

    abstract::DNA breaks can be induced by exogenous stimuli or by endogenous stress, but are also generated during recombination of V, D, and J genes (V(D)J recombination), immunoglobulin class switch recombination (CSR). Among various DNA breaks generated, DNA double strand break (DSB) is the most deleterious one. DNA damage resp...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/s12185-017-2263-8

    authors: Morio T

    更新日期:2017-09-01 00:00:00

  • Involvement of the BCL3 gene in two patients with chronic lymphocytic leukemia.

    abstract::The t(14;19)(q32;q13) is a recurring translocation found in some patients with chronic lymphocytic leukemia (CLL), and the t(14;19) juxtaposes the BCL3 gene on chromosome 19 with the immunoglobulin heavy chain gene (IGH) locus on chromosome 14. Genomic DNAs from 49 patients with chronic B-cell leukemia and the related...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:

    authors: Yabumoto K,Ohno H,Doi S,Edamura S,Arita Y,Akasaka T,Matsumoto J,Kadowaki N,Fukuhara S,Okuma M

    更新日期:1994-04-01 00:00:00

  • Significance of immature platelet fraction and CD41-positive cells at birth in early onset neonatal thrombocytopenia.

    abstract::Early thrombocytopenia is a common hematological abnormality in sick neonates. Here, we examined the relationship between early thrombocytopenia in neonates and parameters associated with thrombopoiesis to identify predictive factors at birth. Two hundred and forty-four neonates admitted to the neonatal intensive care...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-009-0482-3

    authors: Kihara H,Ohno N,Karakawa S,Mizoguchi Y,Fukuhara R,Hayashidani M,Nomura S,Nakamura K,Kobayashi M

    更新日期:2010-03-01 00:00:00

  • Hematopoiesis and angiogenesis.

    abstract::Hematopoiesis is closely linked with angiogenesis, because they interact with each other and have common ancestors: hemangioblasts or hematogenic endothelial cells. The relationship is reasonable, because vascular and hematopoietic systems must develop together in order to establish the body's oxygen-delivery system d...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:

    authors: Suda T,Takakura N,Oike Y

    更新日期:2000-02-01 00:00:00

  • An unusual case of transient dermatological reaction to bortezomib in AL amyloidosis.

    abstract::We report an unusual dermatological reaction to bortezomib in a 61-year-old man with AL amyloidosis. Systemic AL amyloidosis is a rare complication of monoclonal gammopathy or myeloma in which abnormally unstable free light chains cause fibrillary deposits in organs leading to multisystem disease. The treatment of AL ...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-009-0460-9

    authors: Shah AD,Watts AJ,Mehta AB,Wechalekar AD

    更新日期:2010-01-01 00:00:00

  • Bone marrow, peripheral blood and cord blood stem cell transplantation in children: ten years' experience at Siriraj Hospital.

    abstract::Stem cell transplantations were performed in 69 children at Siriraj Hospital over a ten year period. The source of stem cells was bone marrow (60), peripheral blood (3), or cord blood (6). The diseases treated included 35 thalassemias, 11 Burkitt's lymphoma, five non-Hodgkin's lymphoma, five aplastic anemia, eight acu...

    journal_title:International journal of hematology

    pub_type: 临床试验,杂志文章

    doi:10.1016/s0925-5710(98)00083-8

    authors: Suvatte V,Tanphaichitr VS,Visuthisakchai S,Mahasandana C,Veerakul G,Chongkolwatana V,Chandanayingyong D,Issaragrisil S

    更新日期:1998-12-01 00:00:00

  • Prognostic value of early response to treatment combined with conventional risk factors in pediatric acute lymphoblastic leukemia.

    abstract::To determine useful prognostic factors in treating childhood acute lymphoblastic leukemia (ALL), we correlated conventional risk factors and bone marrow response 14 days after induction chemotherapy. Our study included 116 precursor B-cell (n = 104) and T-cell (n = 12) ALL patients treated with our protocol between 19...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1532/ijh97.04114

    authors: Morimoto A,Kuriyama K,Hibi S,Todo S,Yoshihara T,Kuroda H,Imashuku S

    更新日期:2005-04-01 00:00:00

  • Proposed diagnostic criteria, disease severity classification and treatment strategy for TAFRO syndrome, 2015 version.

    abstract::TAFRO syndrome is a systemic inflammatory disorder characterized by thrombocytopenia, anasarca including pleural effusion and ascites, fever, renal insufficiency, and organomegaly including hepatosplenomegaly and lymphadenopathy. Its onset may be acute or sub-acute, but its etiology is undetermined. Although several c...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-016-1979-1

    authors: Masaki Y,Kawabata H,Takai K,Kojima M,Tsukamoto N,Ishigaki Y,Kurose N,Ide M,Murakami J,Nara K,Yamamoto H,Ozawa Y,Takahashi H,Miura K,Miyauchi T,Yoshida S,Momoi A,Awano N,Ikushima S,Ohta Y,Furuta N,Fujimoto S,Ka

    更新日期:2016-06-01 00:00:00

  • EBV-positive PEL-like lymphoma that developed in the course of antisynthetase syndrome treated with tacrolimus.

    abstract::Primary effusion lymphoma (PEL) is a rare type of extranodal lymphoma, typically of a B-cell origin, which presents as lymphomatous effusion with no nodal enlargement or tumor masses. The development PEL is universally associated with human herpes virus-8 (HHV-8) infection. Cases of HHV-8-negative primary lymphomatous...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-018-2426-2

    authors: Okada K,Asakura S,Yano T,Kishimoto T

    更新日期:2018-09-01 00:00:00

  • Detection of red blood cell-bound immunoglobulin G by flow cytometry and its application in the diagnosis of autoimmune hemolytic anemia.

    abstract::Detection of autoantibodies to erythrocytes is of fundamental importance in the diagnosis of autoimmune hemolytic anemia (AIHA). The routinely used direct antiglobulin test (DAT) has the disadvantage of low sensitivity. In this study, we investigated the optimal test conditions of measurement of red blood cell (RBC)-b...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/BF02981936

    authors: Wang Z,Shi J,Zhou Y,Ruan C

    更新日期:2001-02-01 00:00:00

  • Increase in plasma thrombin-activatable fibrinolysis inhibitor may not contribute to thrombotic tendency in antiphospholipid syndrome because of inhibitory potential of antiphospholipid antibodies toward TAFI activation.

    abstract::The causes of thrombosis in antiphospholipid syndrome (APS) remain unknown, though several hypotheses in regard to hypofibrinolysis have been proposed. To clarify the mechanism, we measured plasma levels of thrombin-activatable fibrinolysis inhibitor (TAFI) in APS patients. Both the TAFI antigen (TAFI:Ag) level measur...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-010-0590-0

    authors: Ieko M,Yoshida M,Naito S,Nakabayashi T,Kanazawa K,Mizukami K,Mukai M,Atsumi T,Koike T

    更新日期:2010-06-01 00:00:00

  • Unique association of Waldenström macroglobulinemia with optic neuritis and monoclonal T cell expansion.

    abstract::Waldenström macroglobulinemia is a lymphoplasmacytic lymphoma characterized by production of the immunoglobulin M (IgM) monoclonal protein. Commonly involved sites are the bone marrow, lymph nodes, and spleen. Lymphoplasmacytic infiltration of the central nervous system (CNS), in contrast, is referred to as Bing-Neel ...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1007/s12185-013-1372-2

    authors: Morita K,Yoshimi A,Masuda A,Ichikawa M,Yatomi Y,Kurokawa M

    更新日期:2013-08-01 00:00:00

  • In vitro development of hematopoietic system from mouse embryonic stem cells: a new approach for embryonic hematopoiesis.

    abstract::We developed an efficient differentiation induction system (OP9 system) from mouse embryonic stem (ES) cell to blood cells by coculture on a novel stromal cell line, OP9. This stromal cell line does not produce functional macrophage colony-stimulating factor (M-CSF) and presumably the deficiency of M-CSF production of...

    journal_title:International journal of hematology

    pub_type: 杂志文章

    doi:10.1016/s0925-5710(96)00531-2

    authors: Nakano T

    更新日期:1996-12-01 00:00:00

  • Immune pathophysiology of aplastic anemia.

    abstract::Acquired aplastic anemia (AA) is considered an immune-mediated disease because approximately 70% of AA patients improve with immunosuppressive therapy. However, little is known about the inciting antigens or the mechanisms responsible for the destruction of hematopoietic stem cells by immune system attack. Recent adva...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1532/IJH97.05116

    authors: Nakao S,Feng X,Sugimori C

    更新日期:2005-10-01 00:00:00

  • The relationship of aplastic anemia and PNH.

    abstract::Bone marrow failure has been regarded as one of the triad of clinical manifestations of paroxysmal noctumal hemoglobinuria (PNH), and PNH in turn has been described as a late clonal disease evolving in patients recovering from aplastic anemia. Better understanding of the pathophysiology of both diseases and improved t...

    journal_title:International journal of hematology

    pub_type: 杂志文章,评审

    doi:10.1007/BF03165111

    authors: Young NS,Maciejewski JP,Sloand E,Chen G,Zeng W,Risitano A,Miyazato A

    更新日期:2002-08-01 00:00:00