Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy

G. Ardissino; S. Salardi; S. Berra; G. Colussi; M. Cugno; M. Zecca; F. Giglio; J. Peccatori; E. Diral; F. Tel; A. Clivio; S. Tedeschi

doi:S1083-8791(17)30460-3 [pii]

Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy

G. Ardissino, S. Salardi, S. Berra, G. Colussi, M. Cugno, M. Zecca, F. Giglio, J. Peccatori, E. Diral, F. Tel, A. Clivio, S. Tedeschi

Research output: Contribution to journal › Article › peer-review

Abstract

Hematopoietic stem cell transplant-related thrombotic microangiopathy (HSCT-TMA) is a severe complication whose pathophysiology is unknown. We describe 6 patients in which the disease was associated with complement regulatory gene abnormalities received from their respective donors. It is suggested that mutated and transplanted monocyte-derived cells are responsible for production of abnormal proteins, complement dysregulation, and, ultimately, for the disease. This observation might have important drawbacks as far as HSCT-TMA pathophysiology and treatment are concerned.

Original language	English
Pages (from-to)	1580-1582
Number of pages	3
Journal	Biology of Blood and Marrow Transplantation
Volume	23
Issue number	9
DOIs	https://doi.org/S1083-8791(17)30460-3 [pii]
Publication status	Published - Sep 1 2017

Keywords

Complement
Complement gene mutations
Hematopoietic stem cell transplantation (HSCT)
Hemolytic uremic syndrome
Thrombotic microangiopathy (TMA)

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S1083-8791(17)30460-3 [pii]

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Ardissino, G., Salardi, S., Berra, S., Colussi, G., Cugno, M., Zecca, M., Giglio, F., Peccatori, J., Diral, E., Tel, F., Clivio, A., & Tedeschi, S. (2017). Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy. Biology of Blood and Marrow Transplantation, 23(9), 1580-1582. https://doi.org/S1083-8791(17)30460-3 [pii]

Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy. / Ardissino, G.; Salardi, S.; Berra, S.; Colussi, G.; Cugno, M.; Zecca, M.; Giglio, F.; Peccatori, J.; Diral, E.; Tel, F.; Clivio, A.; Tedeschi, S.

In: Biology of Blood and Marrow Transplantation, Vol. 23, No. 9, 01.09.2017, p. 1580-1582.

Research output: Contribution to journal › Article › peer-review

Ardissino, G, Salardi, S, Berra, S, Colussi, G, Cugno, M , Zecca, M, Giglio, F, Peccatori, J, Diral, E, Tel, F, Clivio, A & Tedeschi, S 2017, 'Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy', Biology of Blood and Marrow Transplantation, vol. 23, no. 9, pp. 1580-1582. https://doi.org/S1083-8791(17)30460-3 [pii]

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abstract = "Hematopoietic stem cell transplant-related thrombotic microangiopathy (HSCT-TMA) is a severe complication whose pathophysiology is unknown. We describe 6 patients in which the disease was associated with complement regulatory gene abnormalities received from their respective donors. It is suggested that mutated and transplanted monocyte-derived cells are responsible for production of abnormal proteins, complement dysregulation, and, ultimately, for the disease. This observation might have important drawbacks as far as HSCT-TMA pathophysiology and treatment are concerned.",

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AU - Ardissino, G.

AU - Salardi, S.

AU - Berra, S.

AU - Colussi, G.

AU - Cugno, M.

AU - Zecca, M.

AU - Giglio, F.

AU - Peccatori, J.

AU - Diral, E.

AU - Tel, F.

AU - Clivio, A.

AU - Tedeschi, S.

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N2 - Hematopoietic stem cell transplant-related thrombotic microangiopathy (HSCT-TMA) is a severe complication whose pathophysiology is unknown. We describe 6 patients in which the disease was associated with complement regulatory gene abnormalities received from their respective donors. It is suggested that mutated and transplanted monocyte-derived cells are responsible for production of abnormal proteins, complement dysregulation, and, ultimately, for the disease. This observation might have important drawbacks as far as HSCT-TMA pathophysiology and treatment are concerned.

AB - Hematopoietic stem cell transplant-related thrombotic microangiopathy (HSCT-TMA) is a severe complication whose pathophysiology is unknown. We describe 6 patients in which the disease was associated with complement regulatory gene abnormalities received from their respective donors. It is suggested that mutated and transplanted monocyte-derived cells are responsible for production of abnormal proteins, complement dysregulation, and, ultimately, for the disease. This observation might have important drawbacks as far as HSCT-TMA pathophysiology and treatment are concerned.

KW - Complement

KW - Complement gene mutations

KW - Hematopoietic stem cell transplantation (HSCT)

KW - Hemolytic uremic syndrome

KW - Thrombotic microangiopathy (TMA)

U2 - S1083-8791(17)30460-3 [pii]

DO - S1083-8791(17)30460-3 [pii]

M3 - Article

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JO - Biology of Blood and Marrow Transplantation

JF - Biology of Blood and Marrow Transplantation

SN - 1083-8791

IS - 9

ER -

Acquired Complement Regulatory Gene Mutations and Hematopoietic Stem Cell Transplant-Related Thrombotic Microangiopathy

Abstract

Keywords

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