ESPN 50th Annual Meeting

ESPN 2017


 
UNEXPLAINED THROMBOCYTOPENIA AS THE FIRST MANIFESTATION OF A SYSTEMIC DISEASE – A CASE REPORT
EKATERINA POPIK 1 INêS MAIO 1 RAQUEL OLIVEIRA 1 EMíLIA COSTA 2 MARGARIDA GUEDES 2 VASCO MIRANDA 3 LIANE CORREIA-COSTA 1 LILIANA ROCHA 1 TERESA COSTA 1 MARIA SAMEIRO FARIA 1 PAULA MATOS 1 CONCEIÇãO MOTA 1 ALBERTO CALDAS AFONSO 1

1- 1- PAEDIATRIC NEPHROLOGY UNIT, CENTRO MATERNO-INFANTIL DO NORTE, CENTRO HOSPITALAR E UNIVERSITáRIO DO PORTO
2- 2- PAEDIATRIC DEPARTMENT, CENTRO MATERNO-INFANTIL DO NORTE, CENTRO HOSPITALAR E UNIVERSITáRIO DO PORTO
3- 3- OPHTHALMOLOGY DEPARTMENT, HOSPITAL DE SANTO ANTóNIO, CENTRO HOSPITALAR E UNIVERSITáRIO DO PORTO
 
Introduction:

Immune thrombocytopenia (ITP) can be triggered by a viral infection or another immunologic mechanism. The management and study of this pathology might reveal an association with several underlying systemic diseases.

Material and methods:

NA.

Results:

A previously healthy, nine-year old boy, son of a non-consanguineous healthy couple, presented to the emergency department with intermittent fever over the course of seven days, petechial rash and bruises on his legs. The platelet count was 63000/µL, with normal hemoglobin and leucocyte count. Investigation showed frankly positive direct antiglobulin test, elevated erythrocyte sedimentation rate and positive antinuclear antibodies (ANA), lupus anticoagulant test, anticardiolipin antibodies and anti-beta2-glycoprotein-I. These tests continued to be positive 12 weeks after the presentation. During follow-up, he continued to present sporadic but spontaneously resolving petechial rash. Four years after presentation, he was found to have cutaneous vasculitis on his hand and a positive anti-double-stranded deoxyribonucleic acid antibody, thus meeting enough criteria for the diagnosis of systemic lupus eritematosus (SLE) with antiphospholipid syndrome. Treatment with hydroxychloroquine (6mg/kg/day) and aspirin was initiated. Two years after SLE diagnosis, he was started on oral prednisone due to the finding of active urinalysis and elevated serum creatinine (1,07 mg/dL). These parameters normalized and no renal flare occurred since then. Renal biopsy revealed mesangial proliferative lupus nephritis (class II). Recently on ophthalmologic examination a retinopathy in bull´s eye was observed and hydroxychloroquine was timely interrupted.

Conclusions:

In 15% of cases, ITP might be the first manifestation of SLE, antedating this diagnosis. The authors emphasize the importance of monitoring closely children with positive ANA and ITP, in order to allow the early identification of SLE manifestations. Bull´s eye retinopathy is rare and its risk increases with high cumulative dosis of hydroxychloroquine. Nonetheless, in this patient case, it is not totally clear if it represents another manifestation of SLE rather than an iatrogenic consequence.