322.

DISORDERS OF COAGULATION OR FIBRINOLYSIS | NOVEMBER 13, 2019

"a Needle in a Haystack". Let´s Talk

about Acquired Hemophilia in Pediatrics
*,1 *,2 *,3 *,4
German Alejandro Detarsio, Sr. BSc, Mariana Raviola, Mauro Davoli, MD, Maria Fabiana Garcia
1
Rosario National University, Rosario Hemophilia Foundation, Ciudad De Galvez, Argentina
2
Rosario Haemophilia Foundation, Rosario, ARG
3
Rosario Hemophilia Foundation, Rosario, ARG
4
Hospital Italiano de Rosario, Rosario, Argentina

bloodjournal Blood blood (2019) 134 (Supplement_1) : 4936.

http://doi.org/10.1182/blood-2019-125977

Introduction

Acquired hemophilia (AH) is a rare disorder due to the production of autoantibodies against factor VIII or
IX procoagulant function. The development of these autoantibodies results in hemorrhagic symptoms in
patients without previous coagulopathy. The typical clinical manifestations of the acquired hemophilia are:
extensive cutaneous purpura and internal hemorrhages while hemarthrosis are not common. Acquired
hemophilia is less frequent than the inherited form of the disease, with an estimated incidence of 2 to 4
cases/million inhabitants/year, value that increases with age, but is extremely infrequent in children (0.045
cases/million/year). It affects all ethnic groups and has a biphasic distribution with a peak incidence in
subjects between 20 and 30 years and another peak between 60 and 80 years.

Objective

To report a case of AH in a 2-year-old child. Clinical data: Hematoma in the left iliac flank, forearm and
legs. Personal history: Otherwise healthy 2-year-old boy with a twin brother and both parents without a
history of bleeding disorders. Prior to the onset of bruising, the patient suffered an important trauma on
one foot without bleeding complications.

A short time after this episode he presented a hematoma in the gluteus that was interpreted as result of
a slight trauma due to a fall. Subsequently, he presented multiple spontaneous hematomas that motivate
the consultation.
• Laboratory upon admission: Hemoglobin: 12.8g/dL; PT: 11" (Control: 11"); APTT:>180" (Control: 30").
Plt: 543000/mm3

• Clinical evolution: With the suspicion of bleeding reactivation, he was referred to the Rosario
Hemophilia Foundation to be evaluated. Reactivation of bleeding was ruled out and imaging had shown
subcutaneous bleeding without muscle involvement. The presence of an inhibitor to Factor VIII was
detected. The patient was commenced on Prednisolone 1mg/kg/day to eradicate the acquired antibody.

• Laboratory Results: Hb: 12.6 g/dL, Plt: 575000/mm3, PT: 12" (11") (Innovin), APTT: 76" (30") (Actin
FSL) which does not normalize after addition of normal plasma and potentiate when was incubated 2
hours at 37°C, TT: 14"(14") (Trombin), FVIII <1%, FIX 45%, FXI 80% (Siemens), the dilution curves of
FVIII and FIX rule out the presence of an inhibitor of interference. Lupus Anticoagulant was negative. The
inhibitor titer was quantified by the Nijmegen-Bethesda Assay and was 32 UNB/mL.

Conclusions

AH is a rare autoimmune disease first described in 1940 and is extremely infrequent in children younger
than 16 years. Although it is usually associated with other autoimmune disorders, in our patient, we
have not found other autoimmunity markers. We should suspect this pathology in all patients who begin
with cutaneo-mucosal bruising without a previous history of bleeding, with prolonged APTT that does
not correct with the addition of normal plasma and whose prolongation potentiates after incubating
the mixture for 2 hours at 37#C. These results are usually associated with PT and normal platelets
count. The FVIII inhibitor can be quantified by the Bethesda-Nijmegen method. Although the title of the
Inhibitor does not correlate well with the clinical severity of the patient, its value is useful in monitoring the
immunosuppressive therapeutic response. Finally, although AH is unusual in pediatrics, it must be taken
into account among the differential diagnoses.

Disclosures
No relevant conflicts of interest to declare.

Author notes
*Asterisk with author names denotes non-ASH members.

© 2019 by the American Society of Hematology