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==[[B-cell prolymphocytic leukemia overview|Overview]]== | ==[[B-cell prolymphocytic leukemia overview|Overview]]== | ||
'''B-cell prolymphocytic leukemia''' is a form of [[leukemia]] or [[hematological malignancy|cancer of the white blood cells]]. Prolymphocytic leukemia is classified in [[B-cell prolymphocytic leukemia]] and [[T-cell prolymphocytic leukemia]], B-cell prolymphocytic leukemia is part of a subclassification of | '''B-cell prolymphocytic leukemia''' is a form of [[leukemia]] or [[hematological malignancy|cancer of the white blood cells]]. [[Prolymphocytic leukemia]] is classified in [[B-cell prolymphocytic leukemia]] and [[T-cell prolymphocytic leukemia]], [[B cell|B-cell]] [[prolymphocytic leukemia]] is part of a subclassification of [[Leukemias|prolymphocytic leukemias]]. Arises from mature [[B cell|B-cells]], which are [[hematologic]] [[White blood cells|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]] must be differentiated from other diseases such as [[T-cell prolymphocytic leukemia]], [[chronic lymphocytic leukemia]] and [[hairy cell leukemia]]. The incidence of [[B cell|B-cell]] [[prolymphocytic leukemia]] is 1% of [[Leukemias|lymphocytic leukemias]]. [[B-cell]] [[prolymphocytic leukemia]] affects men and women equally. The [[prognosis]] is generally poor. [[Symptom|Symptoms]] of [[B cell|B-cell]] [[prolymphocytic leukemia]] include generalised [[Muscle weakness|weakness]] and, [[anemia]],massive [[splenomegaly]], [[weight loss]], [[loss of appetite]], [[thrombocytopenia]] and [[Lymphadenopathy|peripheral lymphadenopathy]]. Common physical examination findings include [[tachycardia]], [[pallor]], [[splenomegaly]] and [[lymphadenopathy]]. [[Laboratory findings template|Laboratory finding]]<nowiki/>s consistent with the diagnosis of [[B-cell]] [[prolymphocytic leukemia]] include [[anemia]], [[lymphocytosis]], abnormal [[Blood chemistry tests|blood chemistry studies]] and abnormal [[blood]] [[peripheral smear]]. [[B cell|B-cell]] [[prolymphocytic leukemia]] [[bone marrow biopsy]] is considered a definitive diagnosis. Other diagnostic studies include [[immunophenotyping]]. | ||
==[[B-cell prolymphocytic leukemia historical perspective|Historical Perspective]]== | ==[[B-cell prolymphocytic leukemia historical perspective|Historical Perspective]]== | ||
B-cell prolymphocytic leukemia was first described in 1974 by Galton et al. | [[B cell|B-cell]] [[prolymphocytic leukemia]] was first described in 1974 by Galton et al. | ||
==[[B-cell prolymphocytic leukemia classification|Classification]]== | ==[[B-cell prolymphocytic leukemia classification|Classification]]== | ||
Prolymphocytic leukemia is classified in [[B-cell prolymphocytic leukemia]] and [[T-cell prolymphocytic leukemia]]. B-cell prolymphocytic leukemia is part of a subclassification of | [[Prolymphocytic leukemia]] is classified in [[B-cell prolymphocytic leukemia]] and [[T-cell prolymphocytic leukemia]]. [[B cell|B-cell]] [[prolymphocytic leukemia]] is part of a subclassification of [[Leukemias|prolymphocytic leukemias]]. | ||
==[[B-cell prolymphocytic leukemia pathophysiology|Pathophysiology]]== | ==[[B-cell prolymphocytic leukemia pathophysiology|Pathophysiology]]== | ||
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 cell|B-cells]], which are [[Hematology|hematologic]] [[White blood cells|white cells]] that are normally involved in the in the [[humoral immunity]] component of the [[adaptive immune system]] by secreting [[antibodies]]. | ||
==[[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]]== | ||
B-cell prolymphocytic leukemia must be differentiated from other diseases such as [[T-cell prolymphocytic leukemia]], [[chronic lymphocytic leukemia]] and [[hairy cell leukemia]]. | [[B-cell]] [[prolymphocytic leukemia]] must be differentiated from other [[Disease|diseases]] such as [[T-cell prolymphocytic leukemia]], [[chronic lymphocytic leukemia]] and [[hairy cell leukemia]]. | ||
==[[B-cell prolymphocytic leukemia epidemiology and demographics|Epidemiology and Demographics]]== | ==[[B-cell prolymphocytic leukemia epidemiology and demographics|Epidemiology and Demographics]]== | ||
The incidence of B-cell prolymphocytic leukemia is 1% of lymphocytic leukemias. B-cell prolymphocytic leukemia affects men and women equally. | The [[incidence]] of [[B cell|B-cell]] [[prolymphocytic leukemia]] is 1% of [[Leukemias|lymphocytic leukemias]]. [[B cell|B-cell]] [[prolymphocytic leukemia]] affects men and women equally. | ||
==[[B-cell prolymphocytic leukemia risk factors|Risk Factors]]== | ==[[B-cell prolymphocytic leukemia risk factors|Risk Factors]]== | ||
Common risk factors in the development of B-cell prolymphocytic leukemia are [[age]], gender, [[race]] and ethnicity. | Common [[Risk factor|risk factors]] in the development of [[B cell|B-cell]] [[prolymphocytic leukemia]] are [[age]], gender, [[race]] and [[Ethnicity and health|ethnicity]]. | ||
==[[B-cell prolymphocytic leukemia screening|Screening]]== | ==[[B-cell prolymphocytic leukemia screening|Screening]]== | ||
Screening for B-cell prolymphocytic leukemia is not recommended. | Screening for [[B-cell]] [[prolymphocytic leukemia]] is not recommended. | ||
==[[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]]== | ||
The prognosis for B-cell prolymphocytic leukemia is generally poor. | The [[prognosis]] for [[B cell|B-cell]] [[prolymphocytic leukemia]] is generally poor. | ||
==Diagnosis== | ==Diagnosis== | ||
===History and Symptoms=== | ===History and Symptoms=== | ||
Symptoms of B-cell prolymphocytic leukemia include generalised weakness, [[anemia]], massive splenomegaly, [[weight loss]], loss of appetite, [[thrombocytopenia]] and | [[Symptom|Symptoms]] of [[B cell|B-cell]] [[prolymphocytic leukemia]] include [[Muscle weakness|generalised weakness]], [[anemia]], [[Splenomegaly|massive splenomegaly]], [[weight loss]], [[loss of appetite]], [[thrombocytopenia]] and [[Lymphadenopathy|peripheral lymphadenopathy]]. | ||
peripheral lymphadenopathy. | |||
===Physical Examination=== | ===Physical Examination=== | ||
Common physical examination findings of [[B-cell prolymphocytic leukemia]] include [[tachycardia]], [[pallor]], [[splenomegaly]] and [[lymphadenopathy]]. | Common [[Physical examination|physical examination findings]] of [[B-cell prolymphocytic leukemia]] include [[tachycardia]], [[pallor]], [[splenomegaly]] and [[lymphadenopathy]]. | ||
===Laboratory Findings=== | ===Laboratory Findings=== | ||
Laboratory findings consistent with the diagnosis of B-cell prolymphocytic leukemia include [[anemia]], [[lymphocytosis]], abnormal blood chemistry studies and abnormal blood peripheral smear. | [[Laboratory findings template|Laboratory findings]] consistent with the diagnosis of [[B cell|B-cell]] [[prolymphocytic leukemia]] include [[anemia]], [[lymphocytosis]], abnormal [[Blood chemistry tests|blood chemistry studies]] and abnormal [[Peripheral smear|blood peripheral smear]]. | ||
==Biopsy== | ==Biopsy== | ||
B-cell prolymphocytic leukemia bone marrow biopsy is considered a definitive diagnosis. | [[B cell|B-cell]] [[prolymphocytic leukemia]] [[bone marrow biopsy]] is considered a definitive diagnosis. | ||
===Other Diagnostic Studies=== | ===Other Diagnostic Studies=== | ||
Other diagnostic studies for B-cell prolymphocytic leukemia include [[immunophenotyping]]. | Other [[Diagnostic study of choice|diagnostic studies]] for [[B cell|B-cell]] [[prolymphocytic leukemia]] include [[immunophenotyping]]. | ||
==Medical Therapy== | ==Medical Therapy== | ||
[[Chemotherapy]], [[biological therapy]], and [[splenectomy]] or radiation therapy to the spleen are indicated in the treatment of B-cell prolymphocytic leukemia. | [[Chemotherapy]], [[biological therapy]], and [[splenectomy]] or [[radiation therapy]] to the [[spleen]] are indicated in the treatment of [[B cell|B-cell]] [[prolymphocytic leukemia]]. | ||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
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Latest revision as of 19:19, 8 April 2019
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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, Prolymphocytic leukemia, B-cell type
Overview
B-cell prolymphocytic leukemia is a form of leukemia or cancer of the white blood cells. Prolymphocytic leukemia is classified in B-cell prolymphocytic leukemia and T-cell prolymphocytic leukemia, B-cell prolymphocytic leukemia is part of a subclassification of prolymphocytic leukemias. 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 prolymphocytic leukemia must be differentiated from other diseases such as T-cell prolymphocytic leukemia, chronic lymphocytic leukemia and hairy cell leukemia. The incidence of B-cell prolymphocytic leukemia is 1% of lymphocytic leukemias. B-cell prolymphocytic leukemia affects men and women equally. The prognosis is generally poor. Symptoms of B-cell prolymphocytic leukemia include generalised weakness and, anemia,massive splenomegaly, weight loss, loss of appetite, thrombocytopenia and peripheral lymphadenopathy. Common physical examination findings include tachycardia, pallor, splenomegaly and lymphadenopathy. Laboratory findings consistent with the diagnosis of B-cell prolymphocytic leukemia include anemia, lymphocytosis, abnormal blood chemistry studies and abnormal blood peripheral smear. B-cell prolymphocytic leukemia bone marrow biopsy is considered a definitive diagnosis. Other diagnostic studies include immunophenotyping.
Historical Perspective
B-cell prolymphocytic leukemia was first described in 1974 by Galton et al.
Classification
Prolymphocytic leukemia is classified in B-cell prolymphocytic leukemia and T-cell prolymphocytic leukemia. B-cell prolymphocytic leukemia is part of a subclassification of prolymphocytic leukemias.
Pathophysiology
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.
Differentiating B-cell prolymphocytic leukemia from other Diseases
B-cell prolymphocytic leukemia must be differentiated from other diseases such as T-cell prolymphocytic leukemia, chronic lymphocytic leukemia and hairy cell leukemia.
Epidemiology and Demographics
The incidence of B-cell prolymphocytic leukemia is 1% of lymphocytic leukemias. B-cell prolymphocytic leukemia affects men and women equally.
Risk Factors
Common risk factors in the development of B-cell prolymphocytic leukemia are age, gender, race and ethnicity.
Screening
Screening for B-cell prolymphocytic leukemia is not recommended.
Natural History, Complications and Prognosis
The prognosis for B-cell prolymphocytic leukemia is generally poor.
Diagnosis
History and Symptoms
Symptoms of B-cell prolymphocytic leukemia include generalised weakness, anemia, massive splenomegaly, weight loss, loss of appetite, thrombocytopenia and peripheral lymphadenopathy.
Physical Examination
Common physical examination findings of B-cell prolymphocytic leukemia include tachycardia, pallor, splenomegaly and lymphadenopathy.
Laboratory Findings
Laboratory findings consistent with the diagnosis of B-cell prolymphocytic leukemia include anemia, lymphocytosis, abnormal blood chemistry studies and abnormal blood peripheral smear.
Biopsy
B-cell prolymphocytic leukemia bone marrow biopsy is considered a definitive diagnosis.
Other Diagnostic Studies
Other diagnostic studies for B-cell prolymphocytic leukemia include immunophenotyping.
Medical Therapy
Chemotherapy, biological therapy, and splenectomy or radiation therapy to the spleen are indicated in the treatment of B-cell prolymphocytic leukemia.