B-cell prolymphocytic leukemia: Difference between revisions
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==Overview== | ==Overview== | ||
'''B-cell prolymphocytic leukemia''' is a more aggressive, but still treatable, form of [[chronic lymphocytic leukemia]]. The malignant [[B cells]] are larger than average. The name is commonly abbreviated B-PLL. | '''B-cell prolymphocytic leukemia''' is a more aggressive, but still treatable, form of [[chronic lymphocytic leukemia]]. The malignant [[B cells]] are larger than average. The name is commonly abbreviated B-PLL. | ||
==Pathophysiology== | ==Pathophysiology== | ||
==Genetics== | ===Genetics=== | ||
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=== | ||
*A case has been described as [[CD20]]+, [[CD22]]+, and [[CD5 (protein)|CD5]]-.<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> | *A case has been described as [[CD20]]+, [[CD22]]+, and [[CD5 (protein)|CD5]]-.<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> | ||
*It can also be CD5+.<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> | *It can also be CD5+.<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> | ||
*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> | ||
==Prognosis== | |||
It has a relatively poor prognosis.<ref name="pmid16642047">{{cite journal |author=Del Giudice I, Davis Z, Matutes E, ''et al'' |title=IgVH genes mutation and usage, [[ZAP-70]] and [[CD38]] expression provide new insights on B-cell prolymphocytic leukemia (B-PLL) |journal=Leukemia |volume=20 |issue=7 |pages=1231-7 |year=2006 |pmid=16642047 |doi=10.1038/sj.leu.2404238}}</ref> | |||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
Revision as of 19:54, 22 August 2015
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
B-cell prolymphocytic leukemia is a more aggressive, but still treatable, form of chronic lymphocytic leukemia. The malignant B cells are larger than average. The name is commonly abbreviated B-PLL.
Pathophysiology
Genetics
It can involve deletions from chromosome 11 and chromosome 13.[1]
Markers
- A case has been described as CD20+, CD22+, and CD5-.[2]
- It can also be CD5+.[3]
- Another case was described as CD45+, CD19+, CD20+, CD5+, HLA-DR+, CD10-, CD23+/-, CD38+ and FMC7-.[4]
Prognosis
It has a relatively poor prognosis.[5]
Differential Diagnosis
- Hairy cell leukemia
- Waldenström macroglobulinemia
Treatment
- Splenic irradiation has been used in the treatment.[6]
- Cladribine (2-chlorodeoxyadenosine) appears to be an active agent (60% complete remission rate) for patients with de novo B-cell prolymphocytic leukemia.[7]
References
- ↑ 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.
- ↑ Del Giudice I, Davis Z, Matutes E; et al. (2006). "IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL)". Leukemia. 20 (7): 1231–7. doi:10.1038/sj.leu.2404238. PMID 16642047.
- ↑ Nakashima H, Saito B, Ariizumi H, Matsuda I, Nakamaki T, Tomoyasu S (December 2008). "Splenic irradiation as a successful treatment for an elderly patient with B-cell prolymphocytic leukemia". Rinsho Ketsueki. 49 (12): 1619–22. doi:10.11406/rinketsu.49.1619. PMID 19110524.
- ↑ "National Cancer Institute".