B-cell prolymphocytic leukemia pathophysiology: Difference between revisions

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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|B-cell]] [[prolymphocytic leukemia]] arises from mature [[B-cells]], which are [[Hematology|hematologic]] [[white cells]] that are normally involved in the in the [[humoral immunity]] component of the [[adaptive immune system]] by secreting [[antibodies]].


==Pathophysiology==
==Pathophysiology==


===Genetics===
*Genetic mutations like mutation or loss of p53 is thought to play a role.<ref name="pmid9058723">{{cite journal |vauthors=Lens D, De Schouwer PJ, Hamoudi RA, Abdul-Rauf M, Farahat N, Matutes E, Crook T, Dyer MJ, Catovsky D |title=p53 abnormalities in B-cell prolymphocytic leukemia |journal=Blood |volume=89 |issue=6 |pages=2015–23 |date=March 1997 |pmid=9058723 |doi= |url=}}</ref>
*11q23 and 13q14 deletions are associated with B cell prolymphocytic leukemia.<ref name="pmid9595043">{{cite journal |vauthors=Solé F, Woessner S, Espinet B, Lloveras E, Florensa L, Pérez-Losada A, Vilà RM, Besses C, Sans-Sabrafen J |title=Cytogenetic abnormalities in three patients with B-cell prolymphocytic leukemia |journal=Cancer Genet. Cytogenet. |volume=103 |issue=1 |pages=43–5 |date=May 1998 |pmid=9595043 |doi= |url=}}</ref><ref name="pmid10360375">{{cite journal |vauthors=Lens D, Coignet LJ, Brito-Babapulle V, Lima CS, Matutes E, Dyer MJ, Catovsky D |title=B cell prolymphocytic leukaemia (B-PLL) with complex karyotype and concurrent abnormalities of the p53 and c-MYC gene |journal=Leukemia |volume=13 |issue=6 |pages=873–6 |date=June 1999 |pmid=10360375 |doi= |url=}}</ref>
*t(11;14) translocation rembles the mutation of mantle cell lymphoma, which makes it harder for the clinicians to distinguish the two entities.<ref name="pmid15086413">{{cite journal |vauthors=Ruchlemer R, Parry-Jones N, Brito-Babapulle V, Attolico I, Wotherspoon AC, Matutes E, Catovsky D |title=B-prolymphocytic leukaemia with t(11;14) revisited: a splenomegalic form of mantle cell lymphoma evolving with leukaemia |journal=Br. J. Haematol. |volume=125 |issue=3 |pages=330–6 |date=May 2004 |pmid=15086413 |doi=10.1111/j.1365-2141.2004.04913.x |url=}}</ref><ref name="pmid24891323">{{cite journal |vauthors=van der Velden VH, Hoogeveen PG, de Ridder D, Schindler-van der Struijk M, van Zelm MC, Sanders M, Karsch D, Beverloo HB, Lam K, Orfao A, Lugtenburg PJ, Böttcher S, van Dongen JJ, Langerak AW, Kappers-Klunne M, van Lom K |title=B-cell prolymphocytic leukemia: a specific subgroup of mantle cell lymphoma |journal=Blood |volume=124 |issue=3 |pages=412–9 |date=July 2014 |pmid=24891323 |doi=10.1182/blood-2013-10-533869 |url=}}</ref>
*It can involve deletions from [[chromosome 11]] and [[chromosome 13]].<ref name="pmid10720137">{{cite journal |author=Lens D, Matutes E, Catovsky D, Coignet LJ |title=Frequent deletions at 11q23 and 13q14 in B cell prolymphocytic leukemia (B-PLL) |journal=Leukemia |volume=14 |issue=3 |pages=427-30 |year=2000 |pmid=10720137 |doi=}}</ref>


===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|B-cell]] [[prolymphocytic leukemia]] cells are positive for [[B cell|B cell markers]] 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><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>
*[[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 cell|Tumor cells]] express surface [[IgM|IgM proteins]].


===Microscopic pathology===
===Microscopic pathology===
*The originating cell line for B-cell prolymphocytic leukemia is a mature B-cells and are medium sized cells.
*The originating cell line for [[B cell|B-cell]] [[prolymphocytic leukemia]] is a mature [[B cell|B-cells]] and are medium sized cells.
*More than 50 percent of the circulating cells in the peripheral blood are prolymphocytes.  
*More than 50 percent of the circulating cells in the [[peripheral blood]] are [[Prolymphocyte|prolymphocytes]].  
*The nucleus is typically round or oval, and the cytoplasm is usually moderately abundant.  
*The [[Cell nucleus|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.<ref name="pmid2182602">{{cite journal |vauthors=Stone RM |title=Prolymphocytic leukemia |journal=Hematol. Oncol. Clin. North Am. |volume=4 |issue=2 |pages=457–71 |date=April 1990 |pmid=2182602 |doi= |url=}}</ref>
*[[Leukemic|Leukemic cells]] can be found in [[peripheral blood]], [[Lymph node|lymph nodes]], [[bone marrow]], [[spleen]], [[liver]], and [[skin]].<ref name="pmid2182602">{{cite journal |vauthors=Stone RM |title=Prolymphocytic leukemia |journal=Hematol. Oncol. Clin. North Am. |volume=4 |issue=2 |pages=457–71 |date=April 1990 |pmid=2182602 |doi= |url=}}</ref>


==References==
==References==
{{reflist|2}}
{{reflist|2}}


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Latest revision as of 19:22, 8 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

Markers

Microscopic pathology

References

  1. ↑ 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.
  2. ↑ "Pathology". Archived from the original on 7 February 2009. Retrieved 2009-01-31.
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ Stone RM (April 1990). "Prolymphocytic leukemia". Hematol. Oncol. Clin. North Am. 4 (2): 457–71. PMID 2182602.


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