Chronic lymphocytic leukemia natural history, complications and prognosis: Difference between revisions

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Revision as of 00:05, 14 October 2015

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, and colitis
Increased risk of other cancers - melanoma, lung, and gastrointestinal cancers

Prognosis

The table below lists prognostic factors for chronic lymphocytic leukemia:^[1]^[2]


Prognostic Factor	Description

Age	Older age of diagnosis is associated with a worse prognosis.
Gender	Males are associated with a worse prognosis.
Performance status	Patient's poor performance status is associated with a worse prognosis.
Stage	Binet stages B and C or Rai stages II–IV 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 is 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 levels of LDH are associated with a worse prognosis.
β2-microglobulin level	Elevated levels of β2-microglobulin level are associated with a worse prognosis.
Lymphocyte surface marker	Positive expression of CD38 is associated with a worse prognosis.
Immunoglobulin (Ig)VH gene	The absence of VH 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 poor prognosis.

References

↑ Nabhan C, Rosen ST (2014). "Chronic lymphocytic leukemia: a clinical review". JAMA. 312 (21): 2265–76. doi:10.1001/jama.2014.14553. PMID 25461996.
↑ Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011

Template:Hematology

Template:WikiDoc Sources

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

[2] Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011

[1]

[2]

@@ Line 20: / Line 20: @@
 ===Prognosis===
-The prognosis of chronic lymphocytic leukemia depends on:
+* The table below lists prognostic factors for chronic lymphocytic leukemia:<ref name="pmid25461996">{{cite journal| author=Nabhan C, Rosen ST| title=Chronic lymphocytic leukemia: a clinical review. | journal=JAMA | year= 2014 | volume= 312 | issue= 21 | pages= 2265-76 | pmid=25461996 | doi=10.1001/jama.2014.14553 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25461996  }} </ref><ref>Hoffbrand V, Moss P. Essential Haematology. John Wiley & Sons; 2011</ref>
-* 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.
+{| style="border: 0px; font-size: 90%; margin: 3px; width: 1000px"
-* Treatment response: Whether the chronic lymphocytic leukemia patient gets better with treatment or has come back.
+|valign=top|
-* The patient's general health.
+|+
-* [[Beta-2-microglobulin]] (higher levels imply a worse prognosis).
+! style="background: #4479BA; width: 200px; color: #FFFFFF;"|'''Prognostic Factor'''
-* 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 is diffuse.
-Survival varies from 5 years to more than 25 years.
+! style="background: #4479BA; width: 600px; color: #FFFFFF;"|'''Description'''
-====Gene mutation status====
+|-
-Recent publications suggest that two<ref name="pmid11733578">{{cite journal |author=Rosenwald A, Alizadeh AA, Widhopf G, ''et al'' |title=Relation of gene expression phenotype to immunoglobulin mutation genotype in B cell chronic lymphocytic leukemia |journal=J. Exp. Med. |volume=194 |issue=11 |pages=1639-47 |year=2001 |pmid=11733578 |doi=}}</ref> or three<ref name="pmid12406914">{{cite journal |author=Ghia P, Guida G, Stella S, ''et al'' |title=The pattern of CD38 expression defines a distinct subset of chronic lymphocytic leukemia (CLL) patients at risk of disease progression |journal=Blood |volume=101 |issue=4 |pages=1262-9 |year=2003 |pmid=12406914 |doi=10.1182/blood-2002-06-1801}}</ref> 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]] (IgV<sub>H</sub>) gene mutation status.<ref name="pmid16983131">{{cite journal |author=Shanafelt TD, Byrd JC, Call TG, Zent CS, Kay NE |title=Narrative review: initial management of newly diagnosed, early-stage chronic lymphocytic leukemia |journal=Ann. Intern. Med. |volume=145 |issue=6 |pages=435-47 |year=2006 |pmid=16983131 |doi=|url=http://www.annals.org/cgi/content/full/145/6/435}}</ref>  High risk patients have an immature cell pattern with few mutations in the DNA in the IgV<sub>H</sub> 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 IgV<sub>H</sub> antibody DNA changes is difficult to perform, the presence of either [[cluster of differentiation]] [[CD38|38]] ([[CD38]]) or Z-chain–associated protein kinase-70 ([[ZAP-70]]) may be surrogate markers of high risk subtype of CLL.<ref name="pmid16983131"/> Their expression correlates with a more immature cellular state and a more rapid disease course.  Unmutated IgV<sub>H</sub> 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.
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Age'''|| style="padding: 5px 5px; background: #F5F5F5;" |
+:* Older age of diagnosis is associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Gender'''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:* Males are associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Performance status'''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:* Patient's poor performance status is associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Stage'''|| style="padding: 5px 5px; background: #F5F5F5;" |
+:*Binet stages B and C or Rai stages II–IV are associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Lymphocyte doubling time'''|| style="padding: 5px 5px; background: #F5F5F5;" |
+:*A rapid lymphocyte doubling time is associated with a worse prognosis
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Genetic mutations'''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:*Deletion of chromosome 17 short arm is associated with a worse prognosis.
+|-
+|style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Prolymphocytes percent'''|| style="padding: 5px 5px; background: #F5F5F5;" |
+:*An increased percentage of prolymphocytes is associated with a worse prongnosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Histological analysis'''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:*Diffuse histology on bone marrow aspiration is associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Lactate dehydrogenase (LDH) level'''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:*Elevated levels of LDH are associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''β2-microglobulin level '''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:*Elevated levels of β2-microglobulin level are associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Lymphocyte surface marker'''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:*Positive expression of CD38 is associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Immunoglobulin (Ig)VH gene'''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:*The absence of VH gene mutation is associated with a worse prognosis.
+|-
+| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold" align=center | '''Membrane-bound proteins'''|| style="padding: 5px 5px; background: #F5F5F5;"|
+:*The expression of Zeta-chain-associated protein kinase 70 (ZAP) is associated with a poor prognosis.
+|}
 ==References==

v t e Myeloid Hematological malignancy/leukemia histology (ICD-O 9590-9989, C81-C96, 200-208)
CFU-GM/ and other granulocytes	Template:Navbox subgroup
MEP	Template:Navbox subgroup
CFU-Mast	Mastocytoma (Mast cell leukemia, Mast cell sarcoma, Systemic mastocytosis)
Multiple/unknown	AML (Acute panmyelosis with myelofibrosis, Myeloid sarcoma) · MP (Myelofibrosis) · Acute biphenotypic leukaemia
See also hematology, lymphoid malignancy