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Hairy cell leukemia presenting as a peripancreatic mass: cytomorphology and radiographic correlates
A P Subhawong1, T K Subhawong, S Z Ali
1Department of Pathology, Johns Hopkins School of Medicine, Baltimore, MD, USA.
Insights
Hairy cell leukemia (HCL), a rare B-cell malignancy, can present unusually. This case highlights HCL manifesting as a peripancreatic mass, emphasizing diagnostic vigilance.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Hairy cell leukemia (HCL) is a chronic lymphoproliferative disorder typically characterized by peripheral cytopenias, bone marrow infiltration, and splenomegaly.
- Lymphadenopathy and distinct mass lesions are uncommon presentations of HCL.
Observation:
- A patient with a history of HCL, treated years prior, presented with weight loss, fatigue, and a large, metabolically active peripancreatic mass.
- Radiological findings suggested an infiltrative lesion, initially raising suspicion for a different primary malignancy.
Findings:
- Fine-needle aspiration revealed discohesive lymphoid cells with characteristic morphology.
- Flow cytometry confirmed a clonal B-cell population expressing CD20, CD25, and CD103, confirming HCL.
Implications:
- This case represents the first documented instance of HCL presenting as a peripancreatic mass.
- It underscores the critical need to correlate imaging findings with clinical history and cytopathological analysis for accurate HCL diagnosis.
- Awareness of rare presentations is crucial for timely and appropriate patient management in hematologic malignancies.
Abstract:
Hairy cell leukemia (HCL) usually presents with peripheral cytopenias, diffuse marrow infiltration, and splenomegaly. This chronic lymphoproliferative disorder is not typically associated with lymphadenopathy or mass lesions. We report a case of HCL first treated by splenectomy, followed by several years of interferon therapy. Twenty-five years later, the patient presented with weight loss, fatigue, and a large PET-avid mass surrounding the head of the pancreas. Fine-needle aspiration was pursued to investigate the unusual and infiltrative appearance of the lesion, which was suggestive of another primary malignancy. Cytology smears showed discohesive lymphoid cells with round nuclei and delicate cytoplasmic projections. Flow cytometry confirmed the presence of a clonal B-cell population with bright expression of CD20 as well as CD25 and CD103, diagnostic of HCL. This is the first report of HCL presenting as a peripancreatic mass. The importance of correlation with radiology and clinical history is emphasized when evaluating such lesions.
