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Extensive Extramedullary Involvement at Presentation in B-Cell Acute Lymphoblastic Leukemia
Siddharth Sharma1, Vijay Singh, Manish Ora
1From the Department of Nuclear Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Clinical Nuclear Medicine
|February 17, 2023
Summary
Extensive extramedullary involvement is rare in pediatric B-cell acute lymphoblastic leukemia. This case highlights widespread organ involvement beyond the bone marrow at diagnosis, including salivary glands.
Area of Science:
- Pediatric Oncology
- Hematology
- Medical Imaging
Background:
- Pediatric B-cell acute lymphoblastic leukemia (B-ALL) typically presents with bone marrow involvement.
- Extramedullary disease at initial diagnosis is uncommon in pediatric B-ALL.
Observation:
- A 7-year-old boy presented with painless parotid gland enlargement, pancytopenia, and elevated lactate dehydrogenase.
- Fine-needle aspiration cytology, flow cytometry, and bone marrow biopsy confirmed B-cell acute lymphoblastic leukemia.
- 18F-FDG PET/CT imaging revealed extensive bone marrow disease and infiltration of multiple organs.
Findings:
- The patient exhibited widespread extramedullary disease involving the spleen, pancreas, kidneys, parotid, submandibular, and lacrimal glands.
- Negligible physiological brain uptake was noted on PET/CT scan.
- This presentation underscores an unusual pattern of disease spread in pediatric B-ALL.
Implications:
- This case emphasizes the importance of considering extensive extramedullary disease in the differential diagnosis of pediatric B-ALL with unusual presentations.
- Comprehensive staging with imaging modalities like 18F-FDG PET/CT is crucial for identifying the full extent of disease.
- Understanding rare presentations aids in tailoring treatment strategies for pediatric leukemia patients.
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