Dados do Trabalho

Título

Acute promyelocytic leukemia with persistently positive central nervous system involvement: a case report

Apresentação do caso

A 17-year-old female sought medical attention for epistaxis and bruises. The initial workout showed
anemia, thrombocytopenia, leukocytosis (94.000 - 82% blasts) and coagulopathy. Due to the
suspicion of APL, ATRA was started. A subsequent bone marrow analysis confirmed the diagnosis.
The patient evolved with hemodynamic instability, disseminated intravascular coagulation, an
intraparenchymal bleeding in the CNS and a subdural hematoma. On day 45 of ATRA she achieved
clinical stability that enabled the performance of a lumbar puncture. The analysis of the cerebrospinal
fluid (CSF) revealed the presence of 69% blasts, and it was decided to start intrathecal chemotherapy
twice a week. Even though morphological and molecular remission of APL was achieved after
consolidation I of the CLEHOP protocol without ATO, the CSF remained with positive oncotic
cytology. An alternative chemotherapy regimen with high dose cytarabine and doubled dose of
intrathecal cytarabine was chosen for follow up treatment. The CSF was cleared of blasts after 3
weeks and the patient was switched to the CLEHOP protocol with ATO for consolidation.

Discussão

Even though the best way to treat CNS involvement in APL is not well-established, the use of
systemic treatment with high dose cytarabine and an optimized intrathecal chemotherapy with
doubled dose cytarabine may be an option.

Comentários Finais

Central nervous system (CNS) involvement in acute promyelocytic leukemia (APL) is rare and occurs
mainly in relapses. Risk factors include high leukocyte count and CNS bleeding at diagnosis. This
report seeks to describe the case of an adolescent with APL and a persistently positive CNS
involvement.