Prolymphocytic leukemia
| Classification according to ICD-10 | |
|---|---|
| C91.3 | B-cell type prolymphocytic leukemia |
| C91.6 | T-cell type prolymphocyte leukemia |
| ICD-10 online (WHO version 2019) | |
The prolymphocytic ( PLL ) is a very rare malignant disease of the lymphatic system . It is counted as a non-Hodgkin lymphoma (NHL). Depending on whether the cells have a B-line immunophenotype or a T-line immunophenotype , one speaks of B-PLL or T-PLL. The B-PLL is much more common than the T-PLL.
The disease occurs practically only in older age (> 60 years) and more often in men. The disease is similar in some aspects to the (much more common) chronic lymphocytic leukemia (CLL), but the PLL usually shows a much more aggressive course. It is often characterized by very high numbers of lymphocytes in the peripheral blood (up to several 100,000 / µl, normal value: approx. 4,000 / µl). Morphologically , more than 50 percent of the cells (mostly> 90%) are immature lymphocyte precursors, so-called prolymphocytes . Lymph node swellings are less common than in CLL. There is often a pronounced enlargement of the spleen ( splenomegaly ).
The prognosis of the disease is much less favorable than that of CLL and the disease usually requires more intensive treatment than CLL. Complete cure can not be achieved (if only because of the advanced age of the patient ). The aim of treatment is to stop or slow down the progression of the disease as far as possible.
