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| {{B-cell prolymphocytic leukemia}} | | {{B-cell prolymphocytic leukemia}} |
| {{SI}} | | {{SI}} |
| {{CMG}} {{CLG}} | | {{CMG}}; {{AE}}{{Qurrat}}, {{CLG}} |
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| {{SK}} B-PLL | | {{SK}} B-PLL, Prolymphocytic leukemia, B-cell type |
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| ==[[B-cell prolymphocytic leukemia overview|Overview]]== | | ==[[B-cell prolymphocytic leukemia overview|Overview]]== |
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| ==[[B-cell prolymphocytic leukemia pathophysiology|Pathophysiology]]== | | ==[[B-cell prolymphocytic leukemia pathophysiology|Pathophysiology]]== |
| It is postulated that the originating cell line for this disease is a mature B-cell. Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver, skin.
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| ===Genetics=== | | ==[[B-cell prolymphocytic leukemia causes|Causes]]== |
| 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>
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| ===Markers=== | | ==[[B-cell prolymphocytic leukemia differential diagnosis|Differentiating B-cell prolymphocytic leukemia from other Diseases]]== |
| *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>
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| *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>
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| *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>
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| ==[[B-cell prolymphocytic leukemia differential diagnosis|Differentiating Acute lymphoblastic leukemia from other Diseases]]==
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| ==[[B-cell prolymphocytic leukemia epidemiology and demographics|Epidemiology and Demographics]]== | | ==[[B-cell prolymphocytic leukemia epidemiology and demographics|Epidemiology and Demographics]]== |
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| ==[[B-cell prolymphocytic leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== | | ==[[B-cell prolymphocytic leukemia natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Overview== | | ==Diagnosis== |
| '''B-cell prolymphocytic leukemia''' is a more aggressive but still treatable form of [[leukemia]]. The malignant [[B cells]] are larger than average.
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| ==Prognosis==
| | [[B-cell prolymphocytic leukemia history and symptoms|History and Symptoms ]] | [[ B-cell prolymphocytic leukemia physical examination|Physical Examination]] | [[B-cell prolymphocytic leukemia laboratory findings|Laboratory Findings]] | [[ B-cell prolymphocytic leukemia biopsy|Biopsy]] | [[B-cell prolymphocytic leukemia CT|CT ]] | [[B-cell prolymphocytic leukemia MRI|MRI]] | [[B-cell prolymphocytic leukemia other imaging findings|Other Imaging Findings]] | [[B-cell prolymphocytic leukemia other diagnostic studies|Other Diagnostic Studies]] |
| 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>. But usually has a better prognosis than T-cell prolymphocytic leukemia.<ref name=T-PLL>{{cite web | title = Canadian Cancer Society| url =http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/prolymphocytic-leukemias/?region=on }}</ref>
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| ==Symptoms==
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| * [[Anemia]]<ref name=B-PLL>{{cite web | title = National cancer institute| url = http://seer.cancer.gov/seertools/hemelymph/51f6cf58e3e27c3994bd53f9/ }}</ref>
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| * Massive splenomegaly
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| * Rapidly rising lymphocyte count
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| * [[Thrombocytopenia]]
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| * Peripheral lymphadenopathy (minimal)
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| ==Differential diagnosis==
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| * [[Hairy cell leukemia]]<ref name="pmid19110524">{{cite journal |author=Nakashima H, Saito B, Ariizumi H, Matsuda I, Nakamaki T, Tomoyasu S |title=Splenic irradiation as a successful treatment for an elderly patient with B-cell prolymphocytic leukemia |journal=Rinsho Ketsueki |volume=49 |issue=12 |pages=1619–22 |date=December 2008 |pmid=19110524 |doi=10.11406/rinketsu.49.1619}}</ref>
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| * [[Waldenström macroglobulinemia]]<ref name="pmid19110524">{{cite journal |author=Nakashima H, Saito B, Ariizumi H, Matsuda I, Nakamaki T, Tomoyasu S |title=Splenic irradiation as a successful treatment for an elderly patient with B-cell prolymphocytic leukemia |journal=Rinsho Ketsueki |volume=49 |issue=12 |pages=1619–22 |date=December 2008 |pmid=19110524 |doi=10.11406/rinketsu.49.1619}}</ref>
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| ==Epidemiology and demographics==
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| * Age: 65-69 years median age<ref name=B-PLL>{{cite web | title = National cancer institute| url = http://seer.cancer.gov/seertools/hemelymph/51f6cf58e3e27c3994bd53f9/ }}</ref>
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| * Incidence: ~1% of lymphocytic leukemias
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| * Sex: no male or female predominance
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| * Survival: 30-50 months median survival
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| ==Diagnosis exam==
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| * Blood chemistry studies
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| * Bone marrow aspiration and biopsy
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| *Complete blood count (CBC)
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| *CT (CAT) scan
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| *Cytogenetic analysis
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| *Immunophenotyping
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| *Peripheral blood smear
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| *Physical exam and history (H&P)
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| ==Treatment== | | ==Treatment== |
| B-cell prolymphocytic leukemia responds better when combinations of [[chemotherapy]] drugs are used. Some combinations that may be used are:<ref name=T-PLL>{{cite web | title = Canadian Cancer Society| url =http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/prolymphocytic-leukemias/?region=on }}</ref> | | [[B-cell prolymphocytic leukemia medical therapy|Medical Therapy]] | [[B-cell prolymphocytic leukemia surgery|Surgery]] | [[B-cell prolymphocytic leukemia primary prevention|Primary Prevention]] | [[B-cell prolymphocytic leukemia secondary prevention|Secondary Prevention]] | [[B-cell prolymphocytic leukemia cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[B-cell prolymphocytic leukemia future or investigational therapies|Future or Investigational Therapies]] |
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| * CVP – [[Cyclophosphamide]], [[vincristine]] and [[prednisone]].
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| * CHOP – [[Cyclophosphamide]], [[doxorubicin]], [[vincristine]] and [[prednisone]].
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| Other chemotherapy drugs (purine analogues) are often used to treat T-cell prolymphocytic leukemia are:
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| * [[Fludarabine]]
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| * [[Cladribine]]
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| * [[Pentostatin]]
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| ===Biological therapy===
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| Monoclonal antibodies are a type of [[biological therapy]] that has been effective in treating certain types of [[leukemias]]. These drugs may be used alone or in combination with chemotherapy to treat prolymphocytic leukemia.
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| * [[Alemtuzumab]] seems to be particularly effective in treating T-cell prolymphocytic leukemia. It may be used in people whose [[lymphoma]] is no longer responding to chemotherapy drugs like [[fludarabine]].
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| ===Splenectomy or radiation therapy to the spleen===
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| [[Splenectomy]] and external beam radiation therapy to the spleen may be used in some people with Prolymphocytic leukemia.
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| ===Stem cell trasplant===
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| A [[stem cell transplant]] is sometimes used to treat people with aggressive prolymphocytic leukemia. Many people with prolymphocytic leukemia are older or may not be in good health, so a stem cell trasplant is often not a suitable option for them. However, it may be an effective option for younger people with prolymphocytic leukemia.
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| ==References== | | ==References== |