Prolymphocytic Leukemia (PLL)
Pathogenesis
The offending cell line is either the peripheral B (postgerminal) or T (post-thymic) cell (?).
Clinical signs
- very rare disease, affecting mostly elderly patients (60 – 70 years old)
- splenomegaly without lymphadenopathy
- leukocytosis and lymphocytosis
- anemia
- thrombocytopenia
- prognosis: not good, the disease often does not respond to therapy, median
survival time is short (less than 1 year).
Histology
Prolymphocytes — middle-sized cells with round nuclei, dispersed chromatin,
prominent nucleoli and a narrow rim of slightly basophilic cytoplasm.
Imunophenotype: B lineage (CD20 a CD79a+) nebo T lineage (CD3+), CD23-