B-cell prolymphocytic leukemia

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B-cell prolymphocytic leukemia

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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

Overview

Historical Perspective

Classification

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

Differentiating Acute lymphoblastic leukemia from other Diseases

Epidemiology and Demographics

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Natural History, Complications and Prognosis

Overview

B-cell prolymphocytic leukemia is a more aggressive but still treatable form of leukemia. The malignant B cells are larger than average.

Prognosis

It has a relatively poor prognosis.[5]. But usually has a better prognosis than T-cell prolymphocytic leukemia.[6]

Symptoms

Differential diagnosis

Epidemiology and demographics

  • Age: 65-69 years median age[7]
  • Incidence: ~1% of lymphocytic leukemias
  • Sex: no male or female predominance
  • Survival: 30-50 months median survival

Diagnosis exam

  • Blood chemistry studies
  • Bone marrow aspiration and biopsy
  • Complete blood count (CBC)
  • CT (CAT) scan
  • Cytogenetic analysis
  • Immunophenotyping
  • Peripheral blood smear
  • Physical exam and history (H&P)

Treatment

B-cell prolymphocytic leukemia responds better when combinations of chemotherapy drugs are used. Some combinations that may be used are:[6]

Other chemotherapy drugs (purine analogues) are often used to treat T-cell prolymphocytic leukemia are:

Biological therapy

Monoclonal antibodies are a type of biological therapy that has been effective in treating certain types of leukemias. These drugs may be used alone or in combination with chemotherapy to treat prolymphocytic leukemia.

  • Alemtuzumab seems to be particularly effective in treating T-cell prolymphocytic leukemia. It may be used in people whose lymphoma is no longer responding to chemotherapy drugs like fludarabine.

Splenectomy or radiation therapy to the spleen

Splenectomy and external beam radiation therapy to the spleen may be used in some people with Prolymphocytic leukemia.

Stem cell trasplant

A stem cell transplant is sometimes used to treat people with aggressive prolymphocytic leukemia. Many people with prolymphocytic leukemia are older or may not be in good health, so a stem cell trasplant is often not a suitable option for them. However, it may be an effective option for younger people with prolymphocytic leukemia.

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. 6.0 6.1 "Canadian Cancer Society".
  7. 7.0 7.1 "National cancer institute".
  8. 8.0 8.1 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.

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