PB0990 - Acquired FXI deficiency (aFXIdef) in a HIV + patient with SLE. Alternative evolution to lupus Anticoagulant (LA) during immunosuppressive therapy.

Tuesday, July 12, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: In-Person

Co-Author(s)

CS

Cristian M. Seehaus

Hematologist doctor
Department of Hematology, Hospital Italiano de Buenos Aires, Argentina
Ciudad Autónoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina

Presenting Author(s)

Marina S. Lopez, Specialist

Biochemist
Hematology and hemostasis division, Central Laboratory,Hospital Italiano de Buenos Aires, Argentina.
Ciudad Autonoma de buenos aires, Ciudad Autonoma de Buenos Aires, Argentina

Co-Author(s)

LB

Luis H. Barrera

Director Central Laboratory
Hospital Italiano de Buenos Aires
CIUDAD AUTONOMA DE BUENOS AIRES, Buenos Aires, Argentina
PQ

Paulina Quiñones Maffassanti

Biochemist
Central Laboratory,Hospital Italiano de Buenos Aires, Argentina.
Ciudad Autónoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
LF

Leandro G. Ferreyra Garrott

Rheumatologist
Department of Rheumatology, Hospital Italiano de Buenos Aires, Argentina
Ciudad Autónoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
Veronica Privitera

MD
Hospital Italiano de Buenos Aires
Ciudad Autónoma de Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
CF

Chuliber Fernando, MD

MD Hematologist
Grupo CAHT
Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
DP

Diana Penchasky

MD
Hospital Italiano de Buenos Aires
Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
EV

Estela Viñuales

MD
Hospital Italiano de Buenos Aires
Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
JA

Jorge Arbelbide

MD
Hospital Italiano de Buenos Aires
Buenos Aires, Ciudad Autonoma de Buenos Aires, Argentina
MM

Marta E. Martinuzzo

Biochemist Supervisor Hemostasis Central Laboratory
Hospital Italiano de Buenos Aires
CIUDAD AUTONOMA DE BUENOS AIRES, Buenos Aires, Argentina

Background: Few cases with non-neutralizing inhibitors causing aFXIdef, but frequently LAs are described in Systemic Lupus Erythematosus (SLE).

Aims: To describe a clinical case of an HIV+ patient with SLE who presented an aFXIdef, including FXI and LA evolution during treatment.

Methods: Not applicable, clinical case report.

Results: A 42 y.o. HIV+ female patient with a recent diagnosis of SLE was referred to Haematology Service in 2017 because of prolonged APTT and eventual renal biopsy requirement (Glomerular microhematuria and non-nephrotic proteinuria). Prolonged APTT, low FXI,3U/dL and normal FVIII, IX and XII were detected. APTT, Silica Clotting time (SCT) and FXI presented negative mixing studies, SCT confirm was negative and Diluted Russell Viper Venom Time (DRVVT) normal, indicating LA not present. Anticardiolipin and anti β2glycoprotein I presented low positive results as well as ANA, Anti dsDNA and Anti-Ro (SSA). Normal APTT until 2015 and 65 sec with negative mixing study in 2016, confirmed aFXIdef.
Three fresh frozen plasma units’ infusion resulted in a moderate, but transient FXI increased up to 22 IU/dL at 2 hours, demonstrating abnormally fast half live falling to 5 IU/dL at 12 hours.
Meprednisone and hydroxychloroquine treatment produced any improvement on FXI and renal manifestations. Mycophenolate was started, with several treatment changes according to clinical evolution. Renal biopsy was suspended. Clinical, treatment and laboratory evolution are shown in figures 1 and 2. At the present time, aFXIdef disappear and LA is present. (figure 2)
During aFXIdef periods without exposition to bleeding challenges, not bleeding was observed. Recently, while LA + and normal FXI levels, an inguinal hernioplasty and a annexhysterectomy for cervical intraepithelial neoplasia were performed without bleeding complications.

Conclusion(s): Proper coagulation laboratory diagnosis during evolution of SLE patients, when the coagulation disorders change alternatively according to immunosuppressive treatment, is important to take clinical decisions as exposition to bleeding challenges.