Posttransplantation thrombotic thrombocytopenic purpura: A single-center experience and a contemporary review

Michelle A. Elliott, William L. Nichols, Elizabeth A. Plumhoff, Stephen Maxted Ansell, Angela Dispenzieri, Dennis A. Gastineau, Morie Gertz, David J. Inwards, Martha Lacy, Ivana Micallef, Ayalew Tefferi, Mark R. Lrrzow

Research output: Contribution to journalArticle

99 Citations (Scopus)

Abstract

Objective: To assess the activity of von Willebrand factor-cleaving protease (vWF-CP) in patients with thrombotic thrombocytopenic purpura (TTP) complicating bone marrow transplantation (BMT) and peripheral blood stem cell transplantation (PBSCT). Patients and Methods: From March 1, 1999, to June 30, 2001, allogeneic and autologous hematopoietic stem cell transplantation was performed in 118 and 400 patients, respectively. We reviewed risk factors for development of posttransplantation TTP and measured vWF-CP activity during active TTP in 10 recipients. Results: The incidence of TTP after allogeneic and autologous transplantation was 6.8% (8/118) and 0.25% (1/400), respectively. Among the allogeneic transplant recipients, the incidence of TTP after nonmyeloablative (NMA) PBSCT, matched unrelated donor BMT, and sibling BMT or PBSCT was 15.4% (2/13), 11.8% (2/17), and 4.5% (4/88), respectively. Of the 10 patients with TTP, 9 (90%) had received extensive prior therapy, including autologous transplantation in both NMA recipients. Acute graft-vs-host disease (GVHD) prophylaxis consisted of cyclosporine and methotrexate in most affected patients. The vWF antigen level was elevated in all patients, and no patients showed evidence of vWF-CP deficiency. During active TTP, 6 patients had grade II-IV acute GVHD, 1 had extensive chronic GVHD, and 4 had cytomegalovirus viremia. Risk factor analysis for development of TTP showed that transplant type (NMA and matched unrelated donor) and source of stem cells (bone marrow vs peripheral blood stem cell) were significant. Conclusions: Posttransplantation TTP was not found to be associated with severe vWF-CP deficiency. The elevated levels of vWF antigen are consistent with diffuse endothelial injury likely because of multiple interacting factors such as extensive prior therapy, GVHD, cyclosporine, and reactivation of cytomegalovirus. The disorder appears to be more frequent among patients with, or at risk for, acute GVHD, suggesting a possible role in the pathogenesis. Nonmyeloablative transplantation does not appear to confer a lesser risk, possibly for these reasons.

Original languageEnglish (US)
Pages (from-to)421-430
Number of pages10
JournalMayo Clinic Proceedings
Volume78
Issue number4
StatePublished - Apr 1 2003

Fingerprint

Thrombotic Thrombocytopenic Purpura
Graft vs Host Disease
Peripheral Blood Stem Cell Transplantation
Bone Marrow Transplantation
Unrelated Donors
Autologous Transplantation
Cytomegalovirus
Cyclosporine
Antigens
Viremia
Hematopoietic Stem Cell Transplantation
Incidence
Homologous Transplantation
Methotrexate
Statistical Factor Analysis
Siblings
Stem Cells
Transplantation
Bone Marrow
Transplants

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Elliott, M. A., Nichols, W. L., Plumhoff, E. A., Ansell, S. M., Dispenzieri, A., Gastineau, D. A., ... Lrrzow, M. R. (2003). Posttransplantation thrombotic thrombocytopenic purpura: A single-center experience and a contemporary review. Mayo Clinic Proceedings, 78(4), 421-430.

Posttransplantation thrombotic thrombocytopenic purpura : A single-center experience and a contemporary review. / Elliott, Michelle A.; Nichols, William L.; Plumhoff, Elizabeth A.; Ansell, Stephen Maxted; Dispenzieri, Angela; Gastineau, Dennis A.; Gertz, Morie; Inwards, David J.; Lacy, Martha; Micallef, Ivana; Tefferi, Ayalew; Lrrzow, Mark R.

In: Mayo Clinic Proceedings, Vol. 78, No. 4, 01.04.2003, p. 421-430.

Research output: Contribution to journalArticle

Elliott, Michelle A. ; Nichols, William L. ; Plumhoff, Elizabeth A. ; Ansell, Stephen Maxted ; Dispenzieri, Angela ; Gastineau, Dennis A. ; Gertz, Morie ; Inwards, David J. ; Lacy, Martha ; Micallef, Ivana ; Tefferi, Ayalew ; Lrrzow, Mark R. / Posttransplantation thrombotic thrombocytopenic purpura : A single-center experience and a contemporary review. In: Mayo Clinic Proceedings. 2003 ; Vol. 78, No. 4. pp. 421-430.
@article{d26dc8e61f95425a8c8edfe898f388c9,
title = "Posttransplantation thrombotic thrombocytopenic purpura: A single-center experience and a contemporary review",
abstract = "Objective: To assess the activity of von Willebrand factor-cleaving protease (vWF-CP) in patients with thrombotic thrombocytopenic purpura (TTP) complicating bone marrow transplantation (BMT) and peripheral blood stem cell transplantation (PBSCT). Patients and Methods: From March 1, 1999, to June 30, 2001, allogeneic and autologous hematopoietic stem cell transplantation was performed in 118 and 400 patients, respectively. We reviewed risk factors for development of posttransplantation TTP and measured vWF-CP activity during active TTP in 10 recipients. Results: The incidence of TTP after allogeneic and autologous transplantation was 6.8{\%} (8/118) and 0.25{\%} (1/400), respectively. Among the allogeneic transplant recipients, the incidence of TTP after nonmyeloablative (NMA) PBSCT, matched unrelated donor BMT, and sibling BMT or PBSCT was 15.4{\%} (2/13), 11.8{\%} (2/17), and 4.5{\%} (4/88), respectively. Of the 10 patients with TTP, 9 (90{\%}) had received extensive prior therapy, including autologous transplantation in both NMA recipients. Acute graft-vs-host disease (GVHD) prophylaxis consisted of cyclosporine and methotrexate in most affected patients. The vWF antigen level was elevated in all patients, and no patients showed evidence of vWF-CP deficiency. During active TTP, 6 patients had grade II-IV acute GVHD, 1 had extensive chronic GVHD, and 4 had cytomegalovirus viremia. Risk factor analysis for development of TTP showed that transplant type (NMA and matched unrelated donor) and source of stem cells (bone marrow vs peripheral blood stem cell) were significant. Conclusions: Posttransplantation TTP was not found to be associated with severe vWF-CP deficiency. The elevated levels of vWF antigen are consistent with diffuse endothelial injury likely because of multiple interacting factors such as extensive prior therapy, GVHD, cyclosporine, and reactivation of cytomegalovirus. The disorder appears to be more frequent among patients with, or at risk for, acute GVHD, suggesting a possible role in the pathogenesis. Nonmyeloablative transplantation does not appear to confer a lesser risk, possibly for these reasons.",
author = "Elliott, {Michelle A.} and Nichols, {William L.} and Plumhoff, {Elizabeth A.} and Ansell, {Stephen Maxted} and Angela Dispenzieri and Gastineau, {Dennis A.} and Morie Gertz and Inwards, {David J.} and Martha Lacy and Ivana Micallef and Ayalew Tefferi and Lrrzow, {Mark R.}",
year = "2003",
month = "4",
day = "1",
language = "English (US)",
volume = "78",
pages = "421--430",
journal = "Mayo Clinic Proceedings",
issn = "0025-6196",
publisher = "Elsevier Science",
number = "4",

}

TY - JOUR

T1 - Posttransplantation thrombotic thrombocytopenic purpura

T2 - A single-center experience and a contemporary review

AU - Elliott, Michelle A.

AU - Nichols, William L.

AU - Plumhoff, Elizabeth A.

AU - Ansell, Stephen Maxted

AU - Dispenzieri, Angela

AU - Gastineau, Dennis A.

AU - Gertz, Morie

AU - Inwards, David J.

AU - Lacy, Martha

AU - Micallef, Ivana

AU - Tefferi, Ayalew

AU - Lrrzow, Mark R.

PY - 2003/4/1

Y1 - 2003/4/1

N2 - Objective: To assess the activity of von Willebrand factor-cleaving protease (vWF-CP) in patients with thrombotic thrombocytopenic purpura (TTP) complicating bone marrow transplantation (BMT) and peripheral blood stem cell transplantation (PBSCT). Patients and Methods: From March 1, 1999, to June 30, 2001, allogeneic and autologous hematopoietic stem cell transplantation was performed in 118 and 400 patients, respectively. We reviewed risk factors for development of posttransplantation TTP and measured vWF-CP activity during active TTP in 10 recipients. Results: The incidence of TTP after allogeneic and autologous transplantation was 6.8% (8/118) and 0.25% (1/400), respectively. Among the allogeneic transplant recipients, the incidence of TTP after nonmyeloablative (NMA) PBSCT, matched unrelated donor BMT, and sibling BMT or PBSCT was 15.4% (2/13), 11.8% (2/17), and 4.5% (4/88), respectively. Of the 10 patients with TTP, 9 (90%) had received extensive prior therapy, including autologous transplantation in both NMA recipients. Acute graft-vs-host disease (GVHD) prophylaxis consisted of cyclosporine and methotrexate in most affected patients. The vWF antigen level was elevated in all patients, and no patients showed evidence of vWF-CP deficiency. During active TTP, 6 patients had grade II-IV acute GVHD, 1 had extensive chronic GVHD, and 4 had cytomegalovirus viremia. Risk factor analysis for development of TTP showed that transplant type (NMA and matched unrelated donor) and source of stem cells (bone marrow vs peripheral blood stem cell) were significant. Conclusions: Posttransplantation TTP was not found to be associated with severe vWF-CP deficiency. The elevated levels of vWF antigen are consistent with diffuse endothelial injury likely because of multiple interacting factors such as extensive prior therapy, GVHD, cyclosporine, and reactivation of cytomegalovirus. The disorder appears to be more frequent among patients with, or at risk for, acute GVHD, suggesting a possible role in the pathogenesis. Nonmyeloablative transplantation does not appear to confer a lesser risk, possibly for these reasons.

AB - Objective: To assess the activity of von Willebrand factor-cleaving protease (vWF-CP) in patients with thrombotic thrombocytopenic purpura (TTP) complicating bone marrow transplantation (BMT) and peripheral blood stem cell transplantation (PBSCT). Patients and Methods: From March 1, 1999, to June 30, 2001, allogeneic and autologous hematopoietic stem cell transplantation was performed in 118 and 400 patients, respectively. We reviewed risk factors for development of posttransplantation TTP and measured vWF-CP activity during active TTP in 10 recipients. Results: The incidence of TTP after allogeneic and autologous transplantation was 6.8% (8/118) and 0.25% (1/400), respectively. Among the allogeneic transplant recipients, the incidence of TTP after nonmyeloablative (NMA) PBSCT, matched unrelated donor BMT, and sibling BMT or PBSCT was 15.4% (2/13), 11.8% (2/17), and 4.5% (4/88), respectively. Of the 10 patients with TTP, 9 (90%) had received extensive prior therapy, including autologous transplantation in both NMA recipients. Acute graft-vs-host disease (GVHD) prophylaxis consisted of cyclosporine and methotrexate in most affected patients. The vWF antigen level was elevated in all patients, and no patients showed evidence of vWF-CP deficiency. During active TTP, 6 patients had grade II-IV acute GVHD, 1 had extensive chronic GVHD, and 4 had cytomegalovirus viremia. Risk factor analysis for development of TTP showed that transplant type (NMA and matched unrelated donor) and source of stem cells (bone marrow vs peripheral blood stem cell) were significant. Conclusions: Posttransplantation TTP was not found to be associated with severe vWF-CP deficiency. The elevated levels of vWF antigen are consistent with diffuse endothelial injury likely because of multiple interacting factors such as extensive prior therapy, GVHD, cyclosporine, and reactivation of cytomegalovirus. The disorder appears to be more frequent among patients with, or at risk for, acute GVHD, suggesting a possible role in the pathogenesis. Nonmyeloablative transplantation does not appear to confer a lesser risk, possibly for these reasons.

UR - http://www.scopus.com/inward/record.url?scp=10744228488&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=10744228488&partnerID=8YFLogxK

M3 - Article

C2 - 12683694

AN - SCOPUS:10744228488

VL - 78

SP - 421

EP - 430

JO - Mayo Clinic Proceedings

JF - Mayo Clinic Proceedings

SN - 0025-6196

IS - 4

ER -