B-cell prolymphocytic leukemia overview
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B-cell prolymphocytic leukemia |
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Differentiating B-cell prolymphocytic leukemia from other Diseases |
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Diagnosis |
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Treatment |
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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. 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 include complete blood count, 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 and, 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 complete blood count, 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.