• Generally, prolymphocyte proportion exceeds 90% Minimal or absence of lymphadenopathy - abnormalities in size, number or consistency of lymph nodes Splenomegaly - Abnormal enlargement of the spleen High white blood cell count B-symptoms - Fever, night sweats and/or weight loss Similar to other leukemias, B-cell prolymphocytic leukemia is often asymptomatic. (wikipedia.org)
  • It is postulated that the originating cell line for this disease is a mature (post-thymic) T-cell. (wikipedia.org)
  • T-PLL has the immunophenotype of a mature (post-thymic) T-lymphocyte, and the neoplastic cells are typically positive for pan-T antigens CD2, CD3, and CD7 and negative for TdT and CD1a. (wikipedia.org)
  • People affected by T-cell prolymphocytic leukemia typically have systemic disease at presentation, including enlargement of the liver and spleen, widespread enlargement of the lymph nodes, and skin infiltrates. (wikipedia.org)
  • In the spleen, the leukemic cell infiltrate both the red pulp and white pulp, and lymph node involvement is typically diffuse through the paracortex. (wikipedia.org)
  • 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. (wikipedia.org)
  • Most patients with T-cell prolymphocytic leukemia require immediate treatment. (wikipedia.org)
  • Some patients who successfully respond to treatment also undergo stem cell transplantation to consolidate the response. (wikipedia.org)
  • This type of leukemia is characterized by: More than 55% of circulating cells in peripheral blood (red blood cells, white blood cells and platelets collectively) are prolymphocytes. (wikipedia.org)
  • B-prolymphocytes are characterized by: Large size - approximately twice the size of a normal small lymphocyte Round or oval-shaped nuclei Single prominent nucleolus Moderately condensed nuclear chromatin High nuclear-cytoplasmic ratio - indicates more abundant cytoplasm This technique is used to study proteins expressed in cells using immunologic markers. (wikipedia.org)
  • This is the highest incidence among all sub-types of B-cell malignancies. (wikipedia.org)
  • B-PLL represents less than 1% of all leukemia cases worldwide, mainly affecting the elderly population with a mean age of presentation between 65-70 years. (wikipedia.org)
  • Immunophenotyping helps distinguish B-PLL from similar diseases, one of its key identifiers is the absence in expression of the surface antigens CD10, CD11c, CD25, CD103 and cyclin D1 - an important regulator of cell-cycle progression. (wikipedia.org)
  • High white blood cell counts - greater than 100 x 109/L - are also indicative of B-PLL. (wikipedia.org)
  • TP53 is an important transcriptional activator of genes involved in the regulation of the G1 checkpoint of the cell cycle as well as certain genes responsible for programmed-cell death (apoptosis). (wikipedia.org)
  • A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases. (wikipedia.org)
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