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{{B-cell prolymphocytic leukemia}}
{{B-cell prolymphocytic leukemia}}
{{SI}}
{{SI}}
{{CMG}} {{CLG}}
{{CMG}}; {{AE}}{{Qurrat}}, {{CLG}}


{{SK}} B-PLL
{{SK}} B-PLL, Prolymphocytic leukemia, B-cell type


==[[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]]==
==[[B-cell prolymphocytic leukemia causes|Causes]]==


==[[B-cell prolymphocytic leukemia differential diagnosis|Differentiating B-cell prolymphocytic leukemia from other Diseases]]==
==[[B-cell prolymphocytic leukemia differential diagnosis|Differentiating B-cell prolymphocytic leukemia from other Diseases]]==
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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]]==


==Overview==
==Diagnosis==
'''B-cell prolymphocytic leukemia''' is a more aggressive but still treatable form of [[leukemia]]. The malignant [[B cells]] are larger than average.


==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>
 
==Symptoms==
* [[Anemia]]<ref name=B-PLL>{{cite web | title = National cancer institute| url = http://seer.cancer.gov/seertools/hemelymph/51f6cf58e3e27c3994bd53f9/ }}</ref>
* Massive splenomegaly
* Rapidly rising lymphocyte count
* [[Thrombocytopenia]]
* Peripheral lymphadenopathy (minimal)
 
==Differential diagnosis==
* [[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>
* [[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>
 
==Epidemiology and demographics==
* 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>
* Incidence: ~1% of lymphocytic leukemias
* Sex: no male or female predominance
* Survival: 30-50 months median survival
 
==Diagnosis exam==
 
* Blood chemistry studies
* Bone marrow aspiration and biopsy
*Complete blood count (CBC)
*CT (CAT) scan
*Cytogenetic analysis
*Immunophenotyping
*Peripheral blood smear
*Physical exam and history (H&P)


==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]]
 
* CVP – [[Cyclophosphamide]], [[vincristine]] and [[prednisone]].
 
* CHOP – [[Cyclophosphamide]], [[doxorubicin]], [[vincristine]] and [[prednisone]].
 
Other chemotherapy drugs (purine analogues) are often used to treat T-cell prolymphocytic leukemia are:
 
* [[Fludarabine]]
* [[Cladribine]]
* [[Pentostatin]]
 
===Biological therapy===
 
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.
 
* [[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]].
 
===Splenectomy or radiation therapy to the spleen===
 
[[Splenectomy]] and external beam radiation therapy to the spleen may be used in some people with Prolymphocytic leukemia.
 
===Stem cell trasplant===
 
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.


==References==
==References==

Latest revision as of 16:33, 1 April 2019

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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]

Synonyms and keywords: B-PLL, Prolymphocytic leukemia, B-cell type

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating B-cell prolymphocytic leukemia from other Diseases

Epidemiology and Demographics

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History and Symptoms | Physical Examination | Laboratory Findings | Biopsy | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

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