B-cell prolymphocytic leukemia

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

Overview

B-cell prolymphocytic leukemia is a more aggressive, but still treatable, form of chronic lymphocytic leukemia. The malignant B cells are larger than average. The name is commonly abbreviated B-PLL.

It can involve deletions from chromosome 11 and chromosome 13.[1]

It has a relatively poor prognosis.[2]

Pathophysiology

Genetics

Markers

Differential Diagnosis

  • Hairy cell leukemia
  • Waldenström macroglobulinemia

Treatment

  • Splenic irradiation has been used in the treatment.[6]
  • Cladribine (2-chlorodeoxyadenosine) appears to be an active agent (60% complete remission rate) for patients with de novo B-cell prolymphocytic leukemia.[7]

References

  1. 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.
  2. 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.
  3. 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.
  4. "Pathology". Archived from the original on 7 February 2009. Retrieved 2009-01-31.
  5. 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.
  6. 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.
  7. "National Cancer Institute".

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