A novel association of acquired ADAMTS13 inhibitor and acute dengue virus infection.
BACKGROUND
Dengue is a mosquito-borne viral disease with an increasing incidence worldwide. Thrombocytopenia is a common finding in dengue virus (DV) infection; however, the underlying mechanisms remain unknown.


METHODS
Here we provide the first evidence of a case of antibody formation against ADAMTS13 (ADAMTS13 inhibitor) in the course of a severe acute DV infection resulting in thrombotic microangiopathy (TMA). The patient presented with classical dengue symptoms (positive epidemiology, high fever, myalgia, predominantly in the lower limbs and lumbar region for 1 week) and, after 11 days of initial symptoms, developed TMA. Clinical and laboratorial investigation of dengue and TMA was performed.


RESULTS
The patient presented with ADAMTS13 inhibitor (IgG) during the acute phase of the disease, without anti-platelet antibodies detectable. Dengue infection had laboratorial confirmation. There were excellent clinical and laboratory responses to 11 serial plasma exchanges. Anti-ADAMTS13 inhibitor disappeared after remission of TMA and dengue resolution. No recurrence of TMA symptoms was observed after 2-year follow-up.


CONCLUSIONS
Although the real incidence of dengue-related TMA is unknown, this case provides the basis for future epidemiologic studies on acquired ADAMTS13 deficiency in DV infection. The prompt clinical recognition of this complication and early installment of specific therapy with plasma exchange are likely to improve the outcome of severe cases of dengue.
Hematology and Hemotherapy Center, State University of Campinas, UNICAMP, Campinas, Brazil.
Fernanda C
FC
Rossi
Rodrigo N
RN
Angerami
Erich V
EV
de Paula
Fernanda L
FL
Orsi
Dezhi
D
Shang
Vânia M
VM
del Guercio
Mariângela R
MR
Resende
Joyce M
JM
Annichino-Bizzacchi
Luiz J
LJ
da Silva
X Long
XL
Zheng
Vagner
V
Castro
D051722:ADAM Proteins / Q000276:immunology*; D000071120:ADAMTS13 Protein; D000208:Acute Disease; D001323:Autoantibodies / Q000097:blood*; D001792:Blood Platelets / Q000276:immunology; D003715:Dengue / Q000097:blood / Q000150:complications / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D057049:Thrombotic Microangiopathies / Q000209:etiology / Q000276:immunology* / Q000628:therapy
D001323:Autoantibodies; D007074:Immunoglobulin G; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human