Chronic lymphocytic leukemia natural history, complications and prognosis: Difference between revisions
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* [[Beta-2-microglobulin]] (higher levels imply a worse prognosis). | * [[Beta-2-microglobulin]] (higher levels imply a worse prognosis). | ||
* Lymphocyte doubling time (doubling of the white blood cell count in excess of 1 year implies a favorable prognosis). | * Lymphocyte doubling time (doubling of the white blood cell count in excess of 1 year implies a favorable prognosis). | ||
* Bone marrow histology - worse if histology id diffuse. | |||
Survival varies from 5 years to more than 25 years. | Survival varies from 5 years to more than 25 years. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]
Overview
Natural History, Complications and Prognosis
Natural History
The clinical course of chronic lymphocytic leukemia varies widely from patient to patient. Some patients die early because of complications, but most patients survive for 5 - 10 yrs. The course of the disease is benign initially but followed by terminal, progressive and resistant phase which lasts for 1 - 2 yrs.
Complications
- Hypogammaglobulinemia leading to recurrent infection.
- Autoimmune hemolytic anemia - IgG type
- Transformation to high grade lymphoma
- Richter's transformation
- Gastrointestinal (GI) involvement - intussusception, small intestinal bacterial contamination, colitis
- Increased risk of other cancers - melanoma, lung and gastrointestinal cancers
Prognosis
The prognosis of chronic lymphocytic leukemia depends on:
- The stage of chronic lymphocytic leukemia, whether lymphocytes are spread throughout the bone marrow, whether the chronic lymphocytic leukemia progresses to lymphoma or prolymphocytic leukemia.
- Treatment response: Whether the chronic lymphocytic leukemia patient gets better with treatment or has come back.
- The patient's general health.
- Beta-2-microglobulin (higher levels imply a worse prognosis).
- Lymphocyte doubling time (doubling of the white blood cell count in excess of 1 year implies a favorable prognosis).
- Bone marrow histology - worse if histology id diffuse.
Survival varies from 5 years to more than 25 years.
Gene mutation status
Recent publications suggest that two[1] or three[2] prognostic groups of CLL exist based on the maturational state of the cell. This distinction is based on the maturity of the lymphocytes as discerned by the immunoglobulin variable-region heavy chain (IgVH) gene mutation status.[3] High risk patients have an immature cell pattern with few mutations in the DNA in the IgVH antibody gene region whereas low risk patients show considerable mutations of the DNA in the antibody gene region indicating mature lymphocytes.
Since assessment of the IgVH antibody DNA changes is difficult to perform, the presence of either cluster of differentiation 38 (CD38) or Z-chain–associated protein kinase-70 (ZAP-70) may be surrogate markers of high risk subtype of CLL.[3] Their expression correlates with a more immature cellular state and a more rapid disease course. Unmutated IgVH survive worse than mutated and are associated with aggressive CLL. The ZAP70 (AKA Zeta-Associated Protein) presence on the CLL cell correlates with unmutated immunoglobulin genes and a poor prognosis. Conversely, its absence indicates the presence of mutated genes and a good clinical outcome. Patients positive for ZAP70 have a CLL more aggressive in nature and more refractory to treatment. They are more likely to evolve to more unfavorable cytogenetic abnormalitites.
References
- ↑ Rosenwald A, Alizadeh AA, Widhopf G; et al. (2001). "Relation of gene expression phenotype to immunoglobulin mutation genotype in B cell chronic lymphocytic leukemia". J. Exp. Med. 194 (11): 1639–47. PMID 11733578.
- ↑ Ghia P, Guida G, Stella S; et al. (2003). "The pattern of CD38 expression defines a distinct subset of chronic lymphocytic leukemia (CLL) patients at risk of disease progression". Blood. 101 (4): 1262–9. doi:10.1182/blood-2002-06-1801. PMID 12406914.
- ↑ 3.0 3.1 Shanafelt TD, Byrd JC, Call TG, Zent CS, Kay NE (2006). "Narrative review: initial management of newly diagnosed, early-stage chronic lymphocytic leukemia". Ann. Intern. Med. 145 (6): 435–47. PMID 16983131.