Large granular lymphocytic leukemia
Large granular lymphocytic leukemia
Updated: 08/30/2024
© Jun Wang, MD, PhD
General features
- Heterogeneous group
- Persistent increased peripheral blood large granular lymphocytes (LGL)
- May coexist with other hematopoietic neoplasms
Clinical presentations
- Associated with autoimmune disorders, most common rheumatoid arthritis
Key Laboratory findings
- Lymphocytosis
- Often severe neutropenia with or without anemia
- Aplastic anemia, thrombocytopenia, splenomegaly, etc
Key morphological features
- Large lymphocytes with azurophilic granules
- Either T cell or NK cell
Genetic abnormalities
- Activating mutation of STAT3
Diagnosis
- Persistent (more than 6 months) increase of peripheral blood large granular lymphocytes, usually 2-20 x 10⁹
- T cell with coexpression of NK cell associated antigens (CD16, CD56 or CD57) and decreased CD2, CD5 or CD7 expression
- Proof of T cell clonality by molecular or flow cytometry study
- STAT3 or STAT5b mutation
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