B-cell prolymphocytic leukemia pathophysiology: Difference between revisions

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===Markers===
===Markers===
*B-cell prolymphocytic leukemia cells are positive for B cell maerkers such as [[CD19]], [[CD20]], [[CD22]].<ref name="pmid9657013">{{cite journal |author=Yamamoto K, Hamaguchi H, Nagata K, Shibuya H, Takeuchi H |title=Splenic irradiation for prolymphocytic leukemia: is it preferable as an initial treatment or not? |journal=Jpn. J. Clin. Oncol. |volume=28 |issue=4 |pages=267–9 |date=April 1998 |pmid=9657013 |doi= 10.1093/jjco/28.4.267|url=http://jjco.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=9657013}}</ref>
*B-cell prolymphocytic leukemia cells are positive for B cell maerkers such as [[CD19]], [[CD20]], [[CD22]].<ref name="pmid9657013">{{cite journal |author=Yamamoto K, Hamaguchi H, Nagata K, Shibuya H, Takeuchi H |title=Splenic irradiation for prolymphocytic leukemia: is it preferable as an initial treatment or not? |journal=Jpn. J. Clin. Oncol. |volume=28 |issue=4 |pages=267–9 |date=April 1998 |pmid=9657013 |doi= 10.1093/jjco/28.4.267|url=http://jjco.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=9657013}}</ref>
*[[CD23]] is negative but [[CD5]] is expressed in one third tumor cells population.<ref name="urlPathology">{{cite web |url=http://www.med-ed.virginia.edu/courses/path/innes/wcd/lympleuk.cfm |title=Pathology |format= |work= |accessdate=2009-01-31| archiveurl= http://web.archive.org/web/20090207235133/http://www.med-ed.virginia.edu/courses/path/innes/wcd/lympleuk.cfm| archivedate= 7 February 2009 <!--DASHBot-->| deadurl= no}}</ref>
*[[CD23]] is negative but [[CD5]] is expressed in one third tumor cells population.<ref name="urlPathology">{{cite web |url=http://www.med-ed.virginia.edu/courses/path/innes/wcd/lympleuk.cfm |title=Pathology |format= |work= |accessdate=2009-01-31| archiveurl= http://web.archive.org/web/20090207235133/http://www.med-ed.virginia.edu/courses/path/innes/wcd/lympleuk.cfm| archivedate= 7 February 2009 <!--DASHBot-->| deadurl= no}}</ref><ref name="pmid24759024">{{cite journal |vauthors=Yi S, Li Z, Wang H, Liu W, Lyu R, Yu Z, Qi J, Qiu L |title=[The immunophenotypic characteristics of 260 patients with CD5 + B cell lymphoproliferative disorders] |language=Chinese |journal=Zhonghua Xue Ye Xue Za Zhi |volume=35 |issue=4 |pages=337–41 |date=April 2014 |pmid=24759024 |doi=10.3760/cma.j.issn.0253-2727.2014.04.019 |url=}}</ref>
*Another case was described as [[CD45]]+, [[CD19]]+, [[CD20]]+, [[CD5]]+, [[HLA-DR]]+, [[CD10]]-, [[CD23]]+/-, [[CD38]]+ and [[FMC7]]<ref name="pmid16997373">{{cite journal |author=Crisostomo RH, Fernandez JA, Caceres W |title=Complex karyotype including chromosomal translocation (8;14) (q24;q32) in one case with B-cell prolymphocytic leukemia |journal=Leuk. Res. |volume=31 |issue=5 |pages=699–701 |date=May 2007 |pmid=16997373 |doi=10.1016/j.leukres.2006.06.010 |url=http://linkinghub.elsevier.com/retrieve/pii/S0145-2126(06)00218-9}}</ref>
*Another case was described as [[CD45]]+, [[CD19]]+, [[CD20]]+, [[CD5]]+, [[HLA-DR]]+, [[CD10]]-, [[CD23]]+/-, [[CD38]]+ and [[FMC7]]<ref name="pmid16997373">{{cite journal |author=Crisostomo RH, Fernandez JA, Caceres W |title=Complex karyotype including chromosomal translocation (8;14) (q24;q32) in one case with B-cell prolymphocytic leukemia |journal=Leuk. Res. |volume=31 |issue=5 |pages=699–701 |date=May 2007 |pmid=16997373 |doi=10.1016/j.leukres.2006.06.010 |url=http://linkinghub.elsevier.com/retrieve/pii/S0145-2126(06)00218-9}}</ref>
*Tumor cells express surface IgM proteins.
*Tumor cells express surface IgM proteins.

Revision as of 19:25, 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

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.[10]

References

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. 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.
  7. "Pathology". Archived from the original on 7 February 2009. Retrieved 2009-01-31.
  8. Yi S, Li Z, Wang H, Liu W, Lyu R, Yu Z, Qi J, Qiu L (April 2014). "[The immunophenotypic characteristics of 260 patients with CD5 + B cell lymphoproliferative disorders]". Zhonghua Xue Ye Xue Za Zhi (in Chinese). 35 (4): 337–41. doi:10.3760/cma.j.issn.0253-2727.2014.04.019. PMID 24759024.
  9. 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.
  10. Stone RM (April 1990). "Prolymphocytic leukemia". Hematol. Oncol. Clin. North Am. 4 (2): 457–71. PMID 2182602.