Thrombotic thrombocytopenic purpura in a patient on long-term alpha-interferon therapy for essential thrombocythemia: a case report.
Alpha interferon
Case report
Essential thrombocythemia
Plasma exchange
Thrombotic thrombocytopenic purpura
Journal
BMC nephrology
ISSN: 1471-2369
Titre abrégé: BMC Nephrol
Pays: England
ID NLM: 100967793
Informations de publication
Date de publication:
23 05 2023
23 05 2023
Historique:
received:
06
09
2022
accepted:
12
05
2023
medline:
25
5
2023
pubmed:
24
5
2023
entrez:
23
5
2023
Statut:
epublish
Résumé
Thrombotic thrombocytopenic purpura (TTP) is rare and severe thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, and renal dysfunction. In contrast, essential thrombocythemia (ET) is a myeloproliferative disease associated with an abnormal increase in platelet numbers. Previous studies reported several cases of the development of ET in patients with TTP. However, the case of an ET patient complicated with TTP has not been previously reported. In this case study, we present a patient with TTP who was previously diagnosed with ET. Therefore, to the best of our knowledge, this is the first report of TTP in ET. A 31-year-old Chinese female who was previously diagnosed with ET presented with anemia and renal dysfunction. The patient had been on long-term treatment with hydroxyurea, aspirin, and alpha interferon (INF-α) for ten years. The diagnosis of TTP was confirmed by clinical features, schistocytes noted on the peripheral blood smear, and lower ADAMTS13 activity (8.5%), together with the renal biopsy results. INF-α was discontinued, and the patient was then treated with plasma exchange and corticosteroids. After one year of follow-up, the patient had a normal hemoglobin level and platelet numbers, and her ADAMTS13 activity had improved. However, the patient's renal function remains impaired. We report a case of an ET patient complicated with TTP that was possibly due to INF-α, highlighting the potential complications associated with long-term ET therapy. The case also highlights the importance of considering TTP in patients with pre-existing ET who present with anemia and renal dysfunction, extending the spectrum of known studies.
Sections du résumé
BACKGROUND
Thrombotic thrombocytopenic purpura (TTP) is rare and severe thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, and renal dysfunction. In contrast, essential thrombocythemia (ET) is a myeloproliferative disease associated with an abnormal increase in platelet numbers. Previous studies reported several cases of the development of ET in patients with TTP. However, the case of an ET patient complicated with TTP has not been previously reported. In this case study, we present a patient with TTP who was previously diagnosed with ET. Therefore, to the best of our knowledge, this is the first report of TTP in ET.
CASE PRESENTATION
A 31-year-old Chinese female who was previously diagnosed with ET presented with anemia and renal dysfunction. The patient had been on long-term treatment with hydroxyurea, aspirin, and alpha interferon (INF-α) for ten years. The diagnosis of TTP was confirmed by clinical features, schistocytes noted on the peripheral blood smear, and lower ADAMTS13 activity (8.5%), together with the renal biopsy results. INF-α was discontinued, and the patient was then treated with plasma exchange and corticosteroids. After one year of follow-up, the patient had a normal hemoglobin level and platelet numbers, and her ADAMTS13 activity had improved. However, the patient's renal function remains impaired.
CONCLUSIONS
We report a case of an ET patient complicated with TTP that was possibly due to INF-α, highlighting the potential complications associated with long-term ET therapy. The case also highlights the importance of considering TTP in patients with pre-existing ET who present with anemia and renal dysfunction, extending the spectrum of known studies.
Identifiants
pubmed: 37221468
doi: 10.1186/s12882-023-03200-7
pii: 10.1186/s12882-023-03200-7
pmc: PMC10207803
doi:
Substances chimiques
Interferon-alpha
0
Types de publication
Case Reports
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
143Informations de copyright
© 2023. The Author(s).
Références
BMC Nephrol. 2022 Dec 13;23(1):400
pubmed: 36513992
Acta Haematol. 2000;102(3):160-2
pubmed: 10692682
Nat Rev Nephrol. 2012 Nov;8(11):622-33
pubmed: 22986360
Eur J Pediatr. 2016 Apr;175(4):465-73
pubmed: 26498648
N Engl J Med. 2014 Aug 14;371(7):654-66
pubmed: 25119611
Blood. 2017 May 25;129(21):2847-2856
pubmed: 28416508
South Med J. 1990 Jan;83(1):57-9
pubmed: 2300836
Medicine (Baltimore). 2002 Jul;81(4):321-31
pubmed: 12169887
Nat Rev Drug Discov. 2007 Dec;6(12):975-90
pubmed: 18049472
J Med Case Rep. 2012 Apr 03;6:93
pubmed: 22472269
Am J Hematol. 2020 Dec;95(12):1599-1613
pubmed: 32974939
Leuk Lymphoma. 1996 Oct;23(3-4):247-52
pubmed: 9031105
Blood. 2002 Jul 15;100(2):710-3
pubmed: 12091372
Blood Cancer J. 2018 Feb 9;8(2):15
pubmed: 29426921
Blood. 2010 Feb 25;115(8):1500-11; quiz 1662
pubmed: 20032506
Pediatr Blood Cancer. 2013 Oct;60(10):1676-82
pubmed: 23729372
Clin Pharmacol Ther. 1981 Aug;30(2):239-45
pubmed: 7249508
Acta Haematol. 1997;97(3):184-6
pubmed: 9066715
Haematologica. 2006 Aug;91(8 Suppl):ECR34
pubmed: 16923518
J Cancer Res Clin Oncol. 2005 Mar;131(3):169-78
pubmed: 15662525
Turk J Haematol. 2020 Jul 23;37(4):302-304
pubmed: 32702948
J Natl Compr Canc Netw. 2017 Jun;15(6):757-760
pubmed: 28596255
Nat Rev Gastroenterol Hepatol. 2011 May;8(5):257-64
pubmed: 21468124
N Engl J Med. 1991 Aug 8;325(6):393-7
pubmed: 2062330
J Thromb Haemost. 2020 Oct;18(10):2496-2502
pubmed: 32914526
J Thromb Haemost. 2005 Jul;3(7):1432-6
pubmed: 15978100
Thromb Res. 2020 Sep;193:53-59
pubmed: 32521335
Lancet Haematol. 2016 May;3(5):e237-45
pubmed: 27132698
Blood. 2017 May 25;129(21):2836-2846
pubmed: 28416507
Curr Opin Hematol. 2001 Sep;8(5):286-93
pubmed: 11604563
Nephron. 1982;32(1):73-4
pubmed: 7177281
Blood. 2008 Jul 1;112(1):141-9
pubmed: 18451306