Absence of thrombocytopaenia and/or microangiopathic haemolytic anaemia does not reliably exclude recurrence of atypical haemolytic uraemic syndrome after kidney transplantation

Anoushka R. Krishnan, Brian Siva, Aron Chakera, Germaine Wong, Daniel Wong, Wai H. Lim

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

A 54-year-old man was diagnosed with atypical haemolytic uraemic syndrome (aHUS) with confirmed complement H mutation in 2012, requiring ongoing dialysis. He was commenced on eculizumab in 2014 once the pharmaceutical board approved this drug. After 4months, he received a live unrelated donor renal transplant from his wife and continued eculizumab post-transplant. Three months later, there was a rise in his creatinine with no laboratory features of haemolysis and a kidney biopsy confirmed rejection, which was treated with increased immunosuppression. After completing 12months of treatment with eculizumab, he opted for close monitoring rather than continuation with therapy. Five months post-cessation of the drug, there was a rise in creatinine, and once again, haematological parameters remained within reference range; however, his kidney biopsy showed features consistent with recurrence of aHUS; hence, eculizumab was recommenced with good effect. While there was no evidence of haemolysis, there was a gradual rise in LDH level and a drop in platelet count, although the parameters remained within the normal range. This suggests that aHUS can recur in the allograft in the absence of haematological abnormalities. Clinicians should have a low threshold for allograft biopsy if haematological parameters are not just outside the reference range, but possibly also if there are changes of at least >25% from baseline in platelet count and LDH levels, particularly in those patients who are no longer eligible for eculizumab.

Original languageEnglish
Pages (from-to)28-31
Number of pages4
JournalNephrology
Volume22
Issue numberSupp. 1
DOIs
Publication statusPublished - 7 Feb 2017

Cite this

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title = "Absence of thrombocytopaenia and/or microangiopathic haemolytic anaemia does not reliably exclude recurrence of atypical haemolytic uraemic syndrome after kidney transplantation",
abstract = "A 54-year-old man was diagnosed with atypical haemolytic uraemic syndrome (aHUS) with confirmed complement H mutation in 2012, requiring ongoing dialysis. He was commenced on eculizumab in 2014 once the pharmaceutical board approved this drug. After 4months, he received a live unrelated donor renal transplant from his wife and continued eculizumab post-transplant. Three months later, there was a rise in his creatinine with no laboratory features of haemolysis and a kidney biopsy confirmed rejection, which was treated with increased immunosuppression. After completing 12months of treatment with eculizumab, he opted for close monitoring rather than continuation with therapy. Five months post-cessation of the drug, there was a rise in creatinine, and once again, haematological parameters remained within reference range; however, his kidney biopsy showed features consistent with recurrence of aHUS; hence, eculizumab was recommenced with good effect. While there was no evidence of haemolysis, there was a gradual rise in LDH level and a drop in platelet count, although the parameters remained within the normal range. This suggests that aHUS can recur in the allograft in the absence of haematological abnormalities. Clinicians should have a low threshold for allograft biopsy if haematological parameters are not just outside the reference range, but possibly also if there are changes of at least >25{\%} from baseline in platelet count and LDH levels, particularly in those patients who are no longer eligible for eculizumab.",
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author = "Krishnan, {Anoushka R.} and Brian Siva and Aron Chakera and Germaine Wong and Daniel Wong and Lim, {Wai H.}",
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Absence of thrombocytopaenia and/or microangiopathic haemolytic anaemia does not reliably exclude recurrence of atypical haemolytic uraemic syndrome after kidney transplantation. / Krishnan, Anoushka R.; Siva, Brian; Chakera, Aron; Wong, Germaine; Wong, Daniel; Lim, Wai H.

In: Nephrology, Vol. 22, No. Supp. 1, 07.02.2017, p. 28-31.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Absence of thrombocytopaenia and/or microangiopathic haemolytic anaemia does not reliably exclude recurrence of atypical haemolytic uraemic syndrome after kidney transplantation

AU - Krishnan, Anoushka R.

AU - Siva, Brian

AU - Chakera, Aron

AU - Wong, Germaine

AU - Wong, Daniel

AU - Lim, Wai H.

PY - 2017/2/7

Y1 - 2017/2/7

N2 - A 54-year-old man was diagnosed with atypical haemolytic uraemic syndrome (aHUS) with confirmed complement H mutation in 2012, requiring ongoing dialysis. He was commenced on eculizumab in 2014 once the pharmaceutical board approved this drug. After 4months, he received a live unrelated donor renal transplant from his wife and continued eculizumab post-transplant. Three months later, there was a rise in his creatinine with no laboratory features of haemolysis and a kidney biopsy confirmed rejection, which was treated with increased immunosuppression. After completing 12months of treatment with eculizumab, he opted for close monitoring rather than continuation with therapy. Five months post-cessation of the drug, there was a rise in creatinine, and once again, haematological parameters remained within reference range; however, his kidney biopsy showed features consistent with recurrence of aHUS; hence, eculizumab was recommenced with good effect. While there was no evidence of haemolysis, there was a gradual rise in LDH level and a drop in platelet count, although the parameters remained within the normal range. This suggests that aHUS can recur in the allograft in the absence of haematological abnormalities. Clinicians should have a low threshold for allograft biopsy if haematological parameters are not just outside the reference range, but possibly also if there are changes of at least >25% from baseline in platelet count and LDH levels, particularly in those patients who are no longer eligible for eculizumab.

AB - A 54-year-old man was diagnosed with atypical haemolytic uraemic syndrome (aHUS) with confirmed complement H mutation in 2012, requiring ongoing dialysis. He was commenced on eculizumab in 2014 once the pharmaceutical board approved this drug. After 4months, he received a live unrelated donor renal transplant from his wife and continued eculizumab post-transplant. Three months later, there was a rise in his creatinine with no laboratory features of haemolysis and a kidney biopsy confirmed rejection, which was treated with increased immunosuppression. After completing 12months of treatment with eculizumab, he opted for close monitoring rather than continuation with therapy. Five months post-cessation of the drug, there was a rise in creatinine, and once again, haematological parameters remained within reference range; however, his kidney biopsy showed features consistent with recurrence of aHUS; hence, eculizumab was recommenced with good effect. While there was no evidence of haemolysis, there was a gradual rise in LDH level and a drop in platelet count, although the parameters remained within the normal range. This suggests that aHUS can recur in the allograft in the absence of haematological abnormalities. Clinicians should have a low threshold for allograft biopsy if haematological parameters are not just outside the reference range, but possibly also if there are changes of at least >25% from baseline in platelet count and LDH levels, particularly in those patients who are no longer eligible for eculizumab.

KW - absence of thrombocytopenia

KW - aHUS recurrence

KW - anaemia

KW - eculizumab

KW - renal biopsy

KW - renal transplant

KW - COMPLEMENT

KW - ABNORMALITIES

KW - ECULIZUMAB

KW - CHILDREN

KW - IMPACT

KW - GENES

U2 - 10.1111/nep.12937

DO - 10.1111/nep.12937

M3 - Article

VL - 22

SP - 28

EP - 31

JO - Nephrology (Carlton, Vic.)

JF - Nephrology (Carlton, Vic.)

SN - 1320-5358

IS - Supp. 1

ER -