T-cell prolymphocytic leukemia
| T-cell-prolymphocytic leukemia | |
|---|---|
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| Prolymphocyte | |
| Classification and external resources | |
| ICD-O | M9834/3 |
| MeSH | D015461 |
T-cell-prolymphocytic leukemia (T-PLL) is a mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement. T-PLL is a very rare leukemia, primarily affecting adults over the age of 30. It represents 2% of all small lymphocytic leukemias in adults. Other names include T-cell chronic lymphocytic leukemia, "knobby" type of T-cell leukemia, and T-prolymphocytic leukemia/T-cell lymphocytic leukemia.
It is postulated that the originating cell line for this disease is a mature (post-thymic) T-cell.
Patients typically have systemic disease at presentation, including hepatosplenomegaly, generalized lymphadenopathy, and skin infiltrates.
Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver, skin. A high lymphocyte count (> 100 x 109/L) along with anemia and thrombocytopenia are common findings. HTLV-1 serologies are negative, and serum immunoglobins are within normal limits with no paraproteins present.
In the peripheral blood, T-PLL consists of medium-sized lymphocytes with single nucleoli and basophilic cytoplasm with occasional blebs or projections. The nuclei are usually round to oval in shape, with occasional patients having cells with a more irregular nuclear outline that is similar to the cerebriform nuclear shape seen in Sézary syndrome. A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases.
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