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Published on: February 23, 2015
Juvenile myelomonocytic leukemia presenting with facial nerve paresis: a unique presentation
Lorie B Smith1, Yamily Valdes, William E Check
1Department of Pediatrics, George and Marie Backus Children's Hospital, Savannah, GA, USA.
Insights
Juvenile myelomonocytic leukemia (JMML), a childhood cancer, can infiltrate the central nervous system (CNS). This case report details CNS involvement as part of JMML progression.
Area of Science:
- Pediatric Oncology
- Hematology
- Neurology
Background:
- Juvenile myelomonocytic leukemia (JMML) is a rare childhood myeloproliferative neoplasm.
- Typical JMML symptoms include failure to thrive, fever, bleeding, and organ enlargement.
- Central nervous system (CNS) involvement is not typically reported at JMML diagnosis.
Observation:
- A 21-month-old boy presented with right facial paresis.
- Magnetic resonance imaging revealed a brain mass.
- Cerebrospinal fluid analysis confirmed leukemic infiltration of the CNS.
Findings:
- This case demonstrates central nervous system (CNS) infiltration in juvenile myelomonocytic leukemia (JMML).
- CNS involvement can occur as part of the natural progression of JMML.
- Literature review indicates a lack of prior reports on CNS involvement at JMML diagnosis.
Implications:
- Highlights the potential for CNS involvement in JMML, expanding the understanding of its clinical spectrum.
- Suggests the need for vigilance and potential CNS surveillance in pediatric patients with JMML.
- Contributes to the literature on rare manifestations of childhood leukemia.
Abstract:
Juvenile myelomonocytic leukemia (JMML) is a distinct myeloproliferative malignancy of early childhood with a varied clinical presentation that may include failure to thrive, malaise, fever, bleeding, pallor, lymphadenopathy, and hepatosplenomegaly. Skin, pulmonary, and gastrointestinal involvement have also been reported. There are no reports of central nervous system (CNS) involvement at diagnosis of this disease. This is a report of a 21-month old boy who had a right facial paresis at presentation. A brain mass was demonstrated on magnetic resonance imaging and cerebrospinal fluid analysis confirmed CNS leukemic infiltration. We report the presence of CNS infiltration as a part of the natural course of JMML and provide a review of the literature.
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