Transient Hyperammonemic Encephalopathy in a Child with Relapsed Acute Lymphoblastic Leukemia and Severe Tumor Lysis Syndrome

Tobias Feuchtinger,Esther Maier,Florian Hoffmann,Ilja Dubinski,Moritz Tacke

doi:10.1055/s-0041-1733871

Tobias Feuchtinger, Esther Maier + Show 3 more

Open Access

https://doi.org/10.1055/s-0041-1733871

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Abstract

AbstractIdiopathic hyperammonemia (IHA) is a severe condition, which has been reported in intensive chemotherapy and bone marrow transplantation. This case elucidates the diagnostic dilemma in children undergoing initiation of chemotherapy and developing severe disorders of qualitative and quantitative consciousness in the presence of hyperammonemia (HA) and lactic acidosis. The role of mitoxantrone as a causative agent for IHA in children is elusive. Children undergoing chemotherapy are often in a poor general condition, and the clinical presentation of HA is heterogeneous. This case should be a reminder for clinicians to check for HA in children with tumor lysis syndrome and acute neurological deterioration.

Highlights

Hyperammonemia (HA) is defined as an elevation of plasma ammonia concentrations above the normal range
We describe a unique clinical phenotype of idiopathic hyperammonemia (IHA) associated with marked tumor lysis syndrome (TLS) after the administration of dexamethasone and mitoxantrone
The treatment was started according to the IntReALL-HR protocol (International Study for Treatment of High Risk Childhood Relapsed Acute Lymphoblastic Leukemia 2010, HighRisk group) with dexamethasone (20 mg/m2, intravenously, twice a day) from therapy day 0 to 5 and mitoxantrone (10 mg/m2, intravenously, once a day) on therapy day 0

Summary

Introduction

Hyperammonemia (HA) is defined as an elevation of plasma ammonia concentrations above the normal range. The treatment was started according to the IntReALL-HR protocol (International Study for Treatment of High Risk Childhood Relapsed Acute Lymphoblastic Leukemia 2010, HighRisk group) with dexamethasone (20 mg/m2, intravenously, twice a day) from therapy day 0 to 5 and mitoxantrone (10 mg/m2, intravenously, once a day) on therapy day 0. He received a concomitant intravenous hydration therapy (4,000 mL/m2 per day). Ammonia in serum (with a range from 54 to 80 μmol/L), they returned to normal after 165 hours (►Fig. 2)

Discussion

Conclusion