ESPN 50th Annual Meeting

ESPN 2017


 
Expecting the (un)expected - nephrotic syndrome in a child with a history of acute myeloid leukemia
ANNE SCHIJVENS 1 NATASJA DORS 2 NICOLE VAN DE KAR 1 MICHIEL SCHREUDER 1

1- DEPARTMENT OF PEDIATRIC NEPHROLOGY, RADBOUDUMC AMALIA CHILDREN’S HOSPITAL, NIJMEGEN, THE NETHERLANDS
2- DEPARTMENT OF PEDIATRIC ONCOLOGY, RADBOUDUMC AMALIA CHILDREN’S HOSPITAL, NIJMEGEN, THE NETHERLANDS
 
Introduction:

Both nephrotic syndrome and acute myeloid leukemia (AML) are rare diseases in childhood. In most children the cause of nephrotic syndrome is minimal change nephropathy, however nephrotic syndrome is also a recognized manifestation of neoplastic diseases. Nephrotic syndrome associated with malignancy can appear prior to onset, concurrently, or after diagnosis of malignancy. Although rare, the association of nephrotic syndrome and AML has been reported previously. In this report, we describe a patient with a history of AML who developed nephrotic syndrome.

Material and methods:

A 5-year-old boy presented at our hospital with edema, abdominal distension and persistent proteinuria. He had a history of AML at 13 months of age, for which he was successfully treated with chemotherapy conform the Dutch-Belgian pediatric AML protocol and so far remained in complete remission. Initial laboratory evaluation in combination with edema showed the triad of nephrotic syndrome. In order to exclude recurrence of AML or other malignancies an invasive and extensive work-up was done prior to the start of nephrotic syndrome treatment to avoid masking malignancy and thereby a delay in final diagnosis.

Results:

Complete blood count and white blood cell differential showed no abnormalities. Bone marrow biopsy and lumbar puncture indicated no signs of AML recurrence. PET-CT scan excluded other malignant causes. Renal biopsy was consistent with minimal change nephropathy. After excluding malignancy as secondary cause of nephrotic syndrome, standard oral prednisolone treatment was started and the patient went into remission within 3 weeks. At follow-up 1.5 years later, our patient is doing well, has remained in remission and shows no signs of recurrence of AML.

Conclusions:

We report a child with two unrelated rare diseases in childhood. It is important to rule out various causes of nephrotic syndrome, especially in children with a history of malignancy, but it may turn out to be an idiopathic nephrotic syndrome.