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Classic hairy cell leukemia with an aggressive presentation
Tyler Fugere1, Susan Harley2, Abhijit Godbole3
1Department of Internal Medicine, University of Arkansas for Medical Sciences , Little Rock , Arkansas.
Insights
Hairy cell leukemia, a rare B cell cancer, can present with severe cytopenias and splenomegaly. Early diagnosis is crucial for effective treatment with purine analogs, leading to excellent patient outcomes.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Hairy cell leukemia (HCL) is a rare, indolent B-cell neoplasm characterized by specific cytologic and immunophenotypic features.
- Typical presentation involves older adults with pancytopenia and splenomegaly.
Observation:
- A patient presented with severe anemia, thrombocytopenia, leukocytosis, and splenomegaly.
- The patient experienced a complicated clinical course including transfusion-refractory pancytopenia, airway compromise due to bleeding, subdural hematoma, and Staphylococcus aureus bacteremia.
Findings:
- This case underscores the diverse and severe clinical manifestations of HCL.
- The patient's complex presentation highlights the diagnostic challenges HCL can pose.
Implications:
- Hairy cell leukemia should be considered in the differential diagnosis for patients presenting with cytopenia and splenomegaly.
- Prompt diagnosis and treatment with purine analogs are associated with excellent response rates in HCL.
Abstract:
Hairy cell leukemia is an indolent neoplasm involving mature B cells with distinct cytologic and immunophenotypic features. It typically presents in older adults with pancytopenia and splenomegaly. We describe a patient who presented with severe anemia and thrombocytopenia, leukocytosis, and splenomegaly. He had an unusually complicated clinical course including pancytopenia refractory to multiple transfusions, oropharyngeal bleeding requiring intubation for airway protection, subdural hematoma after minor trauma, and sepsis due to Staphylococcus aureus bacteremia. This case highlights the importance of considering hairy cell leukemia in the differential diagnosis of all patients with cytopenia and splenomegaly, as this is a disease that has excellent response to treatment with purine analogs.
