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.

Pathophysiology

It is postulated that the originating cell line for this disease is a mature B-cell. Due to the systemic nature of this disease, leukemic cells can be found in peripheral blood, lymph nodes, bone marrow, spleen, liver, skin.

Genetics

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

Markers

Prognosis

It has a relatively poor prognosis.[5]

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. 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.
  3. "Pathology". Archived from the original on 7 February 2009. Retrieved 2009-01-31.
  4. 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.
  5. 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.
  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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