B-cell prolymphocytic leukemia pathophysiology: Difference between revisions
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{{B-cell prolymphocytic leukemia}} | {{B-cell prolymphocytic leukemia}} | ||
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== Overview == | == 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]]. | 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]]. |
Revision as of 19:17, 2 April 2019
B-cell prolymphocytic leukemia |
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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-cell, 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]
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.
- ↑ Stone RM (April 1990). "Prolymphocytic leukemia". Hematol. Oncol. Clin. North Am. 4 (2): 457–71. PMID 2182602.