B-cell prolymphocytic 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]
Synonyms and keywords: B-PLL, Prolymphocytic leukemia, B-cell type
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
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.
B-cell prolymphocytic leukemia was first described in 1974 by Galton et al.
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.
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 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.
Common risk factors in the development of B-cell prolymphocytic leukemia are age, gender, race and ethnicity.
Screening for B-cell prolymphocytic leukemia is not recommended.
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.
References
Historical Perspective
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
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
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 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.
Pathophysiology
Markers
- B-cell prolymphocytic leukemia cells are positive for B cell markers such as CD19, CD20, CD22.[1]
- CD23 is negative but CD5 is expressed in one third tumor cells population.[2][3]
- Another case was described as CD45+, CD19+, CD20+, CD5+, HLA-DR+, CD10-, CD23+/-, CD38+ and FMC7[4]
- Tumor cells express surface IgM proteins.
Microscopic pathology
- The originating cell line for B-cell prolymphocytic leukemia is a mature B-cells and are medium sized cells.
- More than 50 percent of the circulating cells in the peripheral blood are prolymphocytes.
- The nucleus is typically round or oval, and the cytoplasm is usually moderately abundant.
- Leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver, and skin.[5]
References
- ↑ Yamamoto K, Hamaguchi H, Nagata K, Shibuya H, Takeuchi H (April 1998). “Splenic irradiation for prolymphocytic leukemia: is it preferable as an initial treatment or not?”. Jpn. J. Clin. Oncol. 28 (4): 267–9. doi:10.1093/jjco/28.4.267. PMID 9657013.
- ↑ “Pathology”. Archived from the original on 7 February 2009. Retrieved 2009-01-31.
- ↑ Yi S, Li Z, Wang H, Liu W, Lyu R, Yu Z, Qi J, Qiu L (April 2014). “[The immunophenotypic characteristics of 260 patients with CD5 + B cell lymphoproliferative disorders]”. Zhonghua Xue Ye Xue Za Zhi (in Chinese). 35 (4): 337–41. doi:10.3760/cma.j.issn.0253-2727.2014.04.019. PMID 24759024.
- ↑ Crisostomo RH, Fernandez JA, Caceres W (May 2007). “Complex karyotype including chromosomal translocation (8;14) (q24;q32) in one case with B-cell prolymphocytic leukemia”. Leuk. Res. 31 (5): 699–701. doi:10.1016/j.leukres.2006.06.010. PMID 16997373.
- ↑ Stone RM (April 1990). “Prolymphocytic leukemia”. Hematol. Oncol. Clin. North Am. 4 (2): 457–71. PMID 2182602.
Causes
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
Some mutations and genetic factors are thought to play a role in the pathogenesis of B-cell prolymphocytic leukemia. Some known mutations are: p53 gene mutation, 11q23 and 13q14 deletions and deletions from chromosome 11 and chromosome 13.
Genetics
- Genetic mutations like mutation or loss of p53 is thought to play a role.[1][1]
- 11q23 and 13q14 deletions are associated with B cell prolymphocytic leukemia.[2][3][4]
- t(11;14) translocation rembles the mutation of mantle cell lymphoma, which makes it harder for the clinicians to distinguish the two entities.[5][6][7]
- It can involve deletions from chromosome 11 and chromosome 13.[8]
References
- ↑ 1.0 1.1 Lens D, De Schouwer PJ, Hamoudi RA, Abdul-Rauf M, Farahat N, Matutes E, Crook T, Dyer MJ, Catovsky D (March 1997). “p53 abnormalities in B-cell prolymphocytic leukemia”. Blood. 89 (6): 2015–23. PMID 9058723.
- ↑ Brito-Babapulle V, Ellis J, Matutes E, Oscier D, Khokhar T, MacLennan K, Catovsky D (September 1992). “Translocation t(11;14)(q13;q32) in chronic lymphoid disorders”. Genes Chromosomes Cancer. 5 (2): 158–65. PMID 1381952.
- ↑ Solé F, Woessner S, Espinet B, Lloveras E, Florensa L, Pérez-Losada A, Vilà RM, Besses C, Sans-Sabrafen J (May 1998). “Cytogenetic abnormalities in three patients with B-cell prolymphocytic leukemia”. Cancer Genet. Cytogenet. 103 (1): 43–5. PMID 9595043.
- ↑ Lens D, Coignet LJ, Brito-Babapulle V, Lima CS, Matutes E, Dyer MJ, Catovsky D (June 1999). “B cell prolymphocytic leukaemia (B-PLL) with complex karyotype and concurrent abnormalities of the p53 and c-MYC gene”. Leukemia. 13 (6): 873–6. PMID 10360375.
- ↑ Ruchlemer R, Parry-Jones N, Brito-Babapulle V, Attolico I, Wotherspoon AC, Matutes E, Catovsky D (May 2004). “B-prolymphocytic leukaemia with t(11;14) revisited: a splenomegalic form of mantle cell lymphoma evolving with leukaemia”. Br. J. Haematol. 125 (3): 330–6. doi:10.1111/j.1365-2141.2004.04913.x. PMID 15086413.
- ↑ van der Velden VH, Hoogeveen PG, de Ridder D, Schindler-van der Struijk M, van Zelm MC, Sanders M, Karsch D, Beverloo HB, Lam K, Orfao A, Lugtenburg PJ, Böttcher S, van Dongen JJ, Langerak AW, Kappers-Klunne M, van Lom K (July 2014). “B-cell prolymphocytic leukemia: a specific subgroup of mantle cell lymphoma”. Blood. 124 (3): 412–9. doi:10.1182/blood-2013-10-533869. PMID 24891323.
- ↑ Miao Y, Wang R, Fan L, Qiu H, Wu Y, Chen Y, Xu W, Li J (2015). “Detection of t(12;14)(p13;q32) in a patient with IGH-CCND1 negative mantle cell lymphoma resembling ultra-high risk chronic lymphocytic leukemia”. Int J Clin Exp Pathol. 8 (6): 7494–8. PMC 4525993. PMID 26261659.
- ↑ Lens D, Matutes E, Catovsky D, Coignet LJ (2000). “Frequent deletions at 11q23 and 13q14 in B cell prolymphocytic leukemia (B-PLL)”. Leukemia. 14 (3): 427–30. PMID 10720137.
Differentiating B-cell prolymphocytic leukemia from other Diseases
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
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
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
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
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 | Physical Examination | Laboratory Findings | Biopsy | CT | MRI | Other Imaging Findings | Other Diagnostic Studies
Treatment
Treatment
Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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