B-cell prolymphocytic leukemia overview
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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.
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]
Overview
B-cell prolymphocytic leukemia was first described in 1974 by Galton David Abraham Goitein.
B-cell prolymphocytic leukemia
B-cell prolymphocytic leukemia initially, when first described by Galton David Abraham Goitein. in 1974 was considered a variant of chronic lymphoid leukemia.[1] In 1995 Ciril Rozman and Emilio Montserrat described the etilogy, classification and natural history of B-cell prolymphocytic leukemia in detail.[2]
References
- ↑ Galton DA, Goldman JM, Wiltshaw E, Catovsky D, Henry K, Goldenberg GJ (May 1974). “Prolymphocytic leukaemia”. Br. J. Haematol. 27 (1): 7–23. PMID 4137136.
- ↑ Rozman C, Montserrat E (October 1995). “Chronic lymphocytic leukemia”. N. Engl. J. Med. 333 (16): 1052–7. doi:10.1056/NEJM199510193331606. PMID 7675049.
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.
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]
Overview
There is classification sysytem for B-cell prolymphocytic leukemia.
B-cell prolymphocytic leukemia
No criteria for the classification of B-cell prolymphocytic leukemia has emerged. [1][2]
References
- ↑ “International Classification of Diseases for Oncology”. Invalid parameter “prolymphocytic” in
<ref>tag. The supported parameters are: dir, follow, group, name. - ↑ Bennett JM, Catovsky D, Daniel MT, Flandrin G, Galton DA, Gralnick HR, Sultan C (June 1989). “Proposals for the classification of chronic (mature) B and T lymphoid leukaemias. French-American-British (FAB) Cooperative Group”. J. Clin. Pathol. 42 (6): 567–84. PMC 1141984. PMID 2738163.
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.
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]
Overview
B-cell prolymphocytic leukemia must be differentiated from other diseases such as T-cell prolymphocytic leukemia, chronic lymphocytic leukemia, and hairy cell leukemia.
Differential diagnosis
Other conditions resembling B-cell prolymphocytic leukemia are:[1]
- Hairy cell leukemia
- Waldenström macroglobulinemia
- T-cell prolymphocytic leukemia
- Chronic lymphocytic leukemia
- Mantle cell lymphoma
- Lymphoplasmacytic lymphoma
- Hairy cell leukemia
- Splenic marginal zone lymphoma
References
- ↑ Nakashima H, Saito B, Ariizumi H, Matsuda I, Nakamaki T, Tomoyasu S (December 2008). “Splenic irradiation as a successful treatment for an elderly patient with B-cell prolymphocytic leukemia”. Rinsho Ketsueki. 49 (12): 1619–22. doi:10.11406/rinketsu.49.1619. PMID 19110524.
Epidemiology and Demographics
The incidence of B-cell prolymphocytic leukemia is 1% of lymphocytic leukemias. B-cell prolymphocytic leukemia affects men and women equally.
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]
Overview
The incidence of B-cell prolymphocytic leukemia is 1% of lymphocytic leukemias. B-cell prolymphocytic leukemia affects males and females equally.
Epidemiology and demographics
Age
- The median age for the development of B-cell prolymphocytic leukemia is around 65-69 years of age.[1][2]
Incidence
- The incidence of B-cell prolymphocytic leukemia is 1% of lymphocytic leukemias.[3]
Gender
Survival
- The duration of median survival for this disease is 30-50 months.
References
- ↑ “National cancer institute”.
- ↑ Melo JV, Catovsky D, Galton DA (June 1986). “The relationship between chronic lymphocytic leukaemia and prolymphocytic leukaemia. I. Clinical and laboratory features of 300 patients and characterization of an intermediate group”. Br. J. Haematol. 63 (2): 377–87. PMID 3487341.
- ↑ 3.0 3.1 Yamamoto JF, Goodman MT (May 2008). “Patterns of leukemia incidence in the United States by subtype and demographic characteristics, 1997-2002”. Cancer Causes Control. 19 (4): 379–90. doi:10.1007/s10552-007-9097-2. PMID 18064533.
Risk Factors
Common risk factors in the development of B-cell prolymphocytic leukemia are age, gender, race and ethnicity.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Carlos A Lopez, M.D. [2]
Overview
Common risk factors in the development of B-cell prolymphocytic leukemia are age, gender, race, and ethnicity.
B-cell prolymphocytic leukemia risk factors
B-cell prolymphocytic leukemia risk factors may include the following:
- Age
- Gender: Men are five times more likely to develop hairy cell leukemia than women.
- Race
- Ethnicity: B-cell prolymphocytic leukemia is more common in white race and Ashkenazi jewish males.
References
Screening
Screening for B-cell prolymphocytic leukemia is not recommended.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Carlos A Lopez, M.D. [2]
Overview
Screening for B-cell prolymphocytic leukemia is not recommended.
B-cell prolymphocytic leukemia
Screening for B-cell prolymphocytic leukemia is not recommended.
References
Natural History, Complications and Prognosis
The prognosis for B-cell prolymphocytic leukemia is generally poor.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Carlos A Lopez, M.D. [2]
Overview
The prognosis for B-cell prolymphocytic leukemia is generally poor.
Prognosis
The prognosis for B-cell prolymphocytic leukemia is generally poor. However, it usually has a better prognosis than T-cell prolymphocytic leukemia.[1][2]
References
- ↑ Del Giudice I, Davis Z, Matutes E; et al. (2006). “IgVH genes mutation and usage, ZAP-70 and CD38 expression provide new insights on B-cell prolymphocytic leukemia (B-PLL)”. Leukemia. 20 (7): 1231–7. doi:10.1038/sj.leu.2404238. PMID 16642047.
- ↑ “Canadian Cancer Society”.
Diagnosis
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
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
Medical Therapy
Chemotherapy, biological therapy, and splenectomy or radiation therapy to the spleen are indicated in the treatment of B-cell prolymphocytic leukemia.
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