B-cell prolymphocytic leukemia
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B-cell prolymphocytic leukemia |
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Differentiating B-cell prolymphocytic leukemia from other Diseases |
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Diagnosis |
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Treatment |
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B-cell prolymphocytic leukemia On the Web |
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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
- A case has been described as CD20+, CD22+, and CD5-.[2]
- It can also be CD5+.[3]
- Another case was described as CD45+, CD19+, CD20+, CD5+, HLA-DR+, CD10-, CD23+/-, CD38+ and FMC7-.[4]
Differentiating Acute lymphoblastic leukemia from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
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
- Anemia[7]
- Massive splenomegaly
- Rapidly rising lymphocyte count
- Thrombocytopenia
- Peripheral lymphadenopathy (minimal)
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]
- CVP – Cyclophosphamide, vincristine and prednisone.
- CHOP – Cyclophosphamide, doxorubicin, vincristine and prednisone.
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
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 6.0 6.1 "Canadian Cancer Society".
- ↑ 7.0 7.1 "National cancer institute".
- ↑ 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.