Chronic lymphocytic leukemia natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]

Overview

Most patients with chronic lymphocytic leukemia are initially asymptomatic. If left untreated, patients with chronic lymphocytic leukemia may progress to develop weight loss, fever, and lymph nodes swelling. Common complications of chronic lymphocytic leukemia include immunodeficiency, warm autoimmune hemolytic anemia, and Richter’s transformation. Prognosis is generally good, and the 5-year survival rate of patients with chronic lymphocytic leukemia is 81.7%.

Natural History, Complications and Prognosis

Natural History

Most patients with chronic lymphocytic leukemia are initially asymptomatic.
If left untreated, patients with chronic lymphocytic leukemia may progress to develop weight loss, fever, and lymph nodes swelling.

Complications

Common complications of chronic lymphocytic leukemia include:^[1]^[2]^[3]^[4]

Richter’s transformation
Recurrent infections
Anemia
- Autoimmune thrombocytopenia
- Red cell aplasia
- Chemotherapy-induced anemia
Immune thrombocytopenia
Colitis
Intussusception
Small intestinal bacterial contamination
Tumor lysis syndrome
Constrictive pericarditis

Prognosis

Prognosis is generally good, and the 5-year survival rate of patients with chronic lymphocytic leukemia is 81.7%.^[5]
Common prognostic factors for chronic lymphocytic leukemia patients include age, tumor stage, and the presence of certain genetic mutations.^[6]
The table below lists prognostic factors for chronic lymphocytic leukemia patients:^[7]^[8]^[1]


Prognostic Factor	Description

Age	Older age at the time of diagnosis is associated with a worse prognosis.
Gender	Males are associated with a worse prognosis when compared to females.
Performance status	Patient’s poor performance status is associated with a worse prognosis.
Stage	Binet stages B and C or Rai stages 2-4 are associated with a worse prognosis.
Lymphocyte doubling time	A rapid lymphocyte doubling time is associated with a worse prognosis.
Genetic mutations	Deletion of chromosome 17 short arm and chromosome 11 long arm are associated with a worse prognosis.
Prolymphocytes percent	An increased percentage of prolymphocytes is associated with a worse prongnosis.
Histological analysis	Diffuse histology on bone marrow aspiration is associated with a worse prognosis.
Lactate dehydrogenase (LDH) level	Elevated level of LDH is associated with a worse prognosis.
β2-microglobulin level	Elevated level of β2-microglobulin is associated with a worse prognosis.
Lymphocyte surface markers	Over expression of CD38 is associated with a worse prognosis.
Immunoglobulin (Ig)VH gene	The absence of IgVH gene mutation is associated with a worse prognosis.
Membrane-bound proteins	The expression of zeta-chain-associated protein kinase 70 (ZAP) is associated with a worse prognosis.

References

↑ ^1.0 ^1.1 Nabhan C, Rosen ST (2014). “Chronic lymphocytic leukemia: a clinical review”. JAMA. 312 (21): 2265–76. doi:10.1001/jama.2014.14553. PMID 25461996.
↑ Mauro FR, Foa R, Cerretti R, Giannarelli D, Coluzzi S, Mandelli F, Girelli G (May 2000). “Autoimmune hemolytic anemia in chronic lymphocytic leukemia: clinical, therapeutic, and prognostic features”. Blood. 95 (9): 2786–92. PMID 10779422.
↑ Robertson LE, Pugh W, O’Brien S, Kantarjian H, Hirsch-Ginsberg C, Cork A, McLaughlin P, Cabanillas F, Keating MJ (October 1993). “Richter’s syndrome: a report on 39 patients”. J. Clin. Oncol. 11 (10): 1985–9. doi:10.1200/JCO.1993.11.10.1985. PMID 8410123.
↑ Visco C, Ruggeri M, Laura Evangelista M, Stasi R, Zanotti R, Giaretta I, Ambrosetti A, Madeo D, Pizzolo G, Rodeghiero F (February 2008). “Impact of immune thrombocytopenia on the clinical course of chronic lymphocytic leukemia”. Blood. 111 (3): 1110–6. doi:10.1182/blood-2007-09-111492. PMID 17986663.
↑ SEER Stat Fact Sheets: Chronic Lymphocytic Leukemia. National Cancer Institute (2015) http://seer.cancer.gov/statfacts/html/clyl.html Accessed on October, 12 2015
↑ Chen C, Puvvada S (February 2016). “Prognostic Factors for Chronic Lymphocytic Leukemia”. Curr Hematol Malig Rep. 11 (1): 37–42. doi:10.1007/s11899-015-0294-x. PMID 26748932.
↑ Shanafelt TD, Geyer SM, Kay NE (February 2004). “Prognosis at diagnosis: integrating molecular biologic insights into clinical practice for patients with CLL”. Blood. 103 (4): 1202–10. doi:10.1182/blood-2003-07-2281. PMID 14576043.
↑ Chen C, Puvvada S (February 2016). “Prognostic Factors for Chronic Lymphocytic Leukemia”. Curr Hematol Malig Rep. 11 (1): 37–42. doi:10.1007/s11899-015-0294-x. PMID 26748932.

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[pmid25461996-1] 1.0 ^1.1 Nabhan C, Rosen ST (2014). “Chronic lymphocytic leukemia: a clinical review”. JAMA. 312 (21): 2265–76. doi:10.1001/jama.2014.14553. PMID 25461996.

[pmid107794222-2] Mauro FR, Foa R, Cerretti R, Giannarelli D, Coluzzi S, Mandelli F, Girelli G (May 2000). “Autoimmune hemolytic anemia in chronic lymphocytic leukemia: clinical, therapeutic, and prognostic features”. Blood. 95 (9): 2786–92. PMID 10779422.

[pmid8410123-3] Robertson LE, Pugh W, O’Brien S, Kantarjian H, Hirsch-Ginsberg C, Cork A, McLaughlin P, Cabanillas F, Keating MJ (October 1993). “Richter’s syndrome: a report on 39 patients”. J. Clin. Oncol. 11 (10): 1985–9. doi:10.1200/JCO.1993.11.10.1985. PMID 8410123.

[pmid17986663-4] Visco C, Ruggeri M, Laura Evangelista M, Stasi R, Zanotti R, Giaretta I, Ambrosetti A, Madeo D, Pizzolo G, Rodeghiero F (February 2008). “Impact of immune thrombocytopenia on the clinical course of chronic lymphocytic leukemia”. Blood. 111 (3): 1110–6. doi:10.1182/blood-2007-09-111492. PMID 17986663.

[seer-5] SEER Stat Fact Sheets: Chronic Lymphocytic Leukemia. National Cancer Institute (2015) http://seer.cancer.gov/statfacts/html/clyl.html Accessed on October, 12 2015

[pmid267489322-6] Chen C, Puvvada S (February 2016). “Prognostic Factors for Chronic Lymphocytic Leukemia”. Curr Hematol Malig Rep. 11 (1): 37–42. doi:10.1007/s11899-015-0294-x. PMID 26748932.

[pmid14576043-7] Shanafelt TD, Geyer SM, Kay NE (February 2004). “Prognosis at diagnosis: integrating molecular biologic insights into clinical practice for patients with CLL”. Blood. 103 (4): 1202–10. doi:10.1182/blood-2003-07-2281. PMID 14576043.

[pmid26748932-8] Chen C, Puvvada S (February 2016). “Prognostic Factors for Chronic Lymphocytic Leukemia”. Curr Hematol Malig Rep. 11 (1): 37–42. doi:10.1007/s11899-015-0294-x. PMID 26748932.

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