B-cell prolymphocytic leukemia pathophysiology
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B-cell prolymphocytic leukemia |
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Differentiating B-cell prolymphocytic leukemia from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2],Carlos A Lopez, M.D. [3]
Overview
B-cell prolymphocytic leukemia arises from mature B-cells, which are hematologic white cells that are normally involved in the in the humoral immunity component of the adaptive immune system by secreting antibodies.
Pathophysiology
Genetics
- Genetic mutations like mutation or loss of p53 is thought to play a role.[1]
- 11q23 and 13q14 deletions are associated with B cell prolymphocytic leukemia.[2][3]
- t(11;14) translocation rembles the mutation of mantle cell lymphoma, which makes it harder for the clinicians to distinguish the two entities.[4]
- It can involve deletions from chromosome 11 and chromosome 13.[5]
Markers
- B-cell prolymphocytic leukemia cells are positive for B cell maerkers such as CD19, CD20, CD22.[6]
- CD23 is negative but CD5 is expressed in one third tumor cells population.[7]
- Another case was described as CD45+, CD19+, CD20+, CD5+, HLA-DR+, CD10-, CD23+/-, CD38+ and FMC7[8]
- Tumor cells express surface IgM proteins.
Microscopic pathology
- The originating cell line for B-cell prolymphocytic leukemia is a mature B-cells and are medium sized cells.
- More than 50 percent of the circulating cells in the peripheral blood are prolymphocytes.
- The nucleus is typically round or oval, and the cytoplasm is usually moderately abundant.
- Leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver, and skin.[9][9]
References
- ↑ Lens D, De Schouwer PJ, Hamoudi RA, Abdul-Rauf M, Farahat N, Matutes E, Crook T, Dyer MJ, Catovsky D (March 1997). "p53 abnormalities in B-cell prolymphocytic leukemia". Blood. 89 (6): 2015–23. PMID 9058723.
- ↑ Solé F, Woessner S, Espinet B, Lloveras E, Florensa L, Pérez-Losada A, Vilà RM, Besses C, Sans-Sabrafen J (May 1998). "Cytogenetic abnormalities in three patients with B-cell prolymphocytic leukemia". Cancer Genet. Cytogenet. 103 (1): 43–5. PMID 9595043.
- ↑ Lens D, Coignet LJ, Brito-Babapulle V, Lima CS, Matutes E, Dyer MJ, Catovsky D (June 1999). "B cell prolymphocytic leukaemia (B-PLL) with complex karyotype and concurrent abnormalities of the p53 and c-MYC gene". Leukemia. 13 (6): 873–6. PMID 10360375.
- ↑ Ruchlemer R, Parry-Jones N, Brito-Babapulle V, Attolico I, Wotherspoon AC, Matutes E, Catovsky D (May 2004). "B-prolymphocytic leukaemia with t(11;14) revisited: a splenomegalic form of mantle cell lymphoma evolving with leukaemia". Br. J. Haematol. 125 (3): 330–6. doi:10.1111/j.1365-2141.2004.04913.x. PMID 15086413.
- ↑ Lens D, Matutes E, Catovsky D, Coignet LJ (2000). "Frequent deletions at 11q23 and 13q14 in B cell prolymphocytic leukemia (B-PLL)". Leukemia. 14 (3): 427–30. PMID 10720137.
- ↑ Yamamoto K, Hamaguchi H, Nagata K, Shibuya H, Takeuchi H (April 1998). "Splenic irradiation for prolymphocytic leukemia: is it preferable as an initial treatment or not?". Jpn. J. Clin. Oncol. 28 (4): 267–9. doi:10.1093/jjco/28.4.267. PMID 9657013.
- ↑ "Pathology". Archived from the original on 7 February 2009. Retrieved 2009-01-31.
- ↑ Crisostomo RH, Fernandez JA, Caceres W (May 2007). "Complex karyotype including chromosomal translocation (8;14) (q24;q32) in one case with B-cell prolymphocytic leukemia". Leuk. Res. 31 (5): 699–701. doi:10.1016/j.leukres.2006.06.010. PMID 16997373.
- ↑ 9.0 9.1 Stone RM (April 1990). "Prolymphocytic leukemia". Hematol. Oncol. Clin. North Am. 4 (2): 457–71. PMID 2182602.