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




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*Peripheral blood smear
*Peripheral blood smear
*Physical exam and history (H&P)
*Physical exam and history (H&P)
==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>
* 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===
===Stem cell trasplant===

Revision as of 16:05, 5 October 2015

B-cell prolymphocytic leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Carlos A Lopez, M.D. [2]

Synonyms and keywords: B-PLL

Overview

Historical Perspective

Classification

Pathophysiology

Differentiating B-cell prolymphocytic leukemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Overview

B-cell prolymphocytic leukemia is a more aggressive but still treatable form of leukemia. The malignant B cells are larger than average.


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)

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.

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