Severe platelet dysfunction in hairy cell leukemia with improvement after splenectomy

Area of Science:

• Hematology
• Oncology
• Platelet Biology

Background:

• Hairy cell leukemia (HCL) is a rare B-cell malignancy.
• Bleeding complications in HCL are typically attributed to thrombocytopenia.
• This case explores purpura in HCL not explained by low platelet counts.

Observation:

• A patient with HCL presented with purpura despite normal platelet counts.
• Platelet studies revealed reduced aggregation, impaired serotonin uptake, and depleted dense granules.
• Electron microscopy showed platelet degranulation.

Findings:

• Qualitative platelet function defects were identified in this HCL patient.
• These defects included impaired aggregation, reduced malondialdehyde production, and serotonin uptake.
• Splenectomy led to significant improvement in platelet morphology and function.

Implications:

• Qualitative platelet dysfunction in HCL can cause clinically significant bleeding.
• Splenectomy may be an effective treatment for bleeding diathesis in HCL patients with platelet defects.
• This highlights the importance of evaluating platelet function in HCL patients with unexplained bleeding.