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thrombotic microangiopathy
Classification:
- thrombotic thrombocytopenic purpura (TTP)
- hemolytic uremic syndrome
Etiology:
1) infections
a) Escherichia coli 0157-H7
b) Pseudomonas
c) Shigella
d) Campylobacter jejuni
e) Yersinia
f) Salmonella
g) HIV1
h) Mycoplasma pneumoniae
i) Legionella
j) Coxsackie A virus, Coxsakie B virus
2) autoimmune disease
a) scleroderma renal crisis
b) systemic lupus erythematosus
c) antiphospholipid antibody syndrome
3) drugs
a) antineoplastic agents
- mitomycin
- gemcitabine
- cisplatin
- bleomycin
- VEGF inhibitors bevacizumab, sunitinib
b) mTOR inhibitors
- sirolimus, everolimus [1]
c) calcineurin inhibitors
- tacrolimus, cyclosporin A
d) antiplatelet agents
- clopidogrel
- ticlopidine
e) valacyclovir
f) quinine
g) oral contraceptives
4) vaccinations
a) yellow fever vaccine
b) influenza virus vaccine
c) measles-mumps-rubella vaccine
5) hematopoietic stem cell transplantation
6) pregnancy & the post-partum state
7) malignant hypertension
8) neoplasms
9) idiopathic
Pathology:
- disease of small blood vessels & capillaries
- multiple organ involvement
- thrombocytopenia
- microangiopathic hemolytic anemia
- thrombosis
Laboratory:
- complete blood count
a) anemia
b) thrombocytopenia
- peripheral blood smear
- schistocytes (may be rare) [5], evidence of microangiopathic hemolysis
- urinalysis
- urine protein, urine albumin/creatinine, nephrotic syndrome [5]
- serum creatinine: worsening renal function
- serum lactate dehydrogenase increased from hemolysis
- see ARUP consult [2]
Management:
- plasma exchange
Specific
HELLP syndrome
hemolytic uremic syndrome (HUS)
thrombotic thrombocytopenic purpura (TTP)
General
thrombotic disorder
microangiopathy
References
- Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18.
American College of Physicians, Philadelphia 2009, 2012, 2015, 2018.
- ARUP Consult: Thrombotic Microangiopathies - TMA
The Physician's Guide to Laboratory Test Selection & Interpretation
https://www.arupconsult.com/content/thrombotic-microangiopathies
- George JN, Nester CM.
Syndromes of thrombotic microangiopathy.
N Engl J Med. 2014 Aug 14;371(7):654-66. Review.
PMID: 25119611
- George JN, Nester CM.
Syndromes of thrombotic microangiopathy.
N Engl J Med. 2014 Nov 6;371(19):1847-8.
PMID: 25372103 Free Article
- Shatzel JJ, Taylor JA.
Syndromes of Thrombotic Microangiopathy.
Med Clin North Am. 2017 Mar;101(2):395-415. Review.
PMID: 28189178
- NEJM Knowledge+ Nephrology/Urology