Non-Hodgkin lymphoma natural history, complications and prognosis: Difference between revisions

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Latest revision as of 20:44, 21 January 2019

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

Overview

Common complications of non-Hodgkin lymphoma include lymphadenopathy, disseminated intravascular coagulation, superior vena cava (SVC) syndrome, autoimmune hemolytic anemia and infection. The indolent non-Hodgkin lymphoma types are associated with a relatively good prognosis. The 5-year relative survival rate of patients with NHL is 71.4%.

Natural History

Complications

Common complications of non-Hodgkin lymphoma include:^[1]

Lymphadenopathy especially cervical lymphadenopathy
Cytopenias such as neutropenia, anemia and thrombocytopenia secondary to bone marrow infiltration
Autoimmune hemolytic anemia
Bleeding secondary to thrombocytopenia
Disseminated intravascular coagulation (DIC)
Infection secondary to neutropenia
Cardiac problems secondary to large pericardial effusion
Cardiac arrhythmias secondary to cardiac metastases
Respiratory problems secondary to pleural effusion
Superior vena cava (SVC) syndrome secondary to a large mediastinal tumor
Spinal cord compression secondary to vertebral metastases
Neurologic problems secondary to primary CNS lymphoma or lymphomatous meningitis
Gastrointestinal obstruction, perforation, and bleeding in a patient with MALT lymphoma
Pain secondary to tumor invasion
Lymphocytosis in leukemic phase of disease

Prognosis

The 5-year relative survival rate of patients with NHL is 71.4%.^[2]
The survival rate has steadily improved over the last 2 decades, thanks to improvements in medical and nursing care, the advent of novel therapeutic strategies (ie, monoclonal antibodies), validation of biomarkers of response, and the implementation of tailored treatment.
The prognosis for patients with NHL depends on the following factors:^[2]
- Tumor histology
- Tumor stage
- Patient age
- Tumor bulk
- Performance status
- Serum lactate dehydrogenase (LDH) level
- Beta2-microglobulin level

Prognostic Indexes

International Prognostic Index (IPI)

The International Prognostic Index (IPI), which was originally designed as a prognostic factor model for aggressive non Hodgkin lymphoma (NHL) appears to be useful for predicting the outcome of patients with low-grade lymphoma and mantle cell lymphoma.^[2]^[3]
This index is used to identify patients at high risk of relapse, based on specific sites of involvement, including bone marrow, CNS, liver, testis, lung, and spleen.
Clinical features included in the IPI that are independently predictive of survival include the following:
- Age - Younger than 60 years versus older than 60 years
- LDH level - Within the reference range versus elevated
- Performance status - Eastern Cooperative Oncology Group ( ECOG) grade 0-1 versus 2-4
- Ann Arbor stage - Stage I-II versus III-IV
- Number of extranodal sites - Zero to 1 versus more than 1
With this model, relapse-free and overall survival rates at 5 years are as follows:
- 0-1 risk factors - 75%
- 2-3 risk factors - 50%
- 4-5 risk factors - 25%

References

↑ Dehghani M, Haddadi S, Vojdani R (2015). "Signs, Symptoms and Complications of Non-Hodgkin's Lymphoma According to Grade and Stage in South Iran". Asian Pac J Cancer Prev. 16 (8): 3551–7. PMID 25921177.
↑ ^2.0 ^2.1 ^2.2 Abla O, Weitzman S, Blay JY, O'Neill BP, Abrey LE, Neuwelt E; et al. (2011). "Primary CNS lymphoma in children and adolescents: a descriptive analysis from the International Primary CNS Lymphoma Collaborative Group (IPCG)". Clin Cancer Res. 17 (2): 346–52. doi:10.1158/1078-0432.CCR-10-1161. PMC 4058714. PMID 21224370.
↑ Shalabi H, Angiolillo A, Vezina G, Rubenstein JL, Pittaluga S, Raffeld M; et al. (2015). "Prolonged Complete Response in a Pediatric Patient With Primary Peripheral T-Cell Lymphoma of the Central Nervous System". Pediatr Hematol Oncol. 32 (8): 529–34. doi:10.3109/08880018.2015.1074325. PMC 4942274. PMID 26384083.

[pmid25921177-1] Dehghani M, Haddadi S, Vojdani R (2015). "Signs, Symptoms and Complications of Non-Hodgkin's Lymphoma According to Grade and Stage in South Iran". Asian Pac J Cancer Prev. 16 (8): 3551–7. PMID 25921177.

[pmid21224370-2] 2.0 ^2.1 ^2.2 Abla O, Weitzman S, Blay JY, O'Neill BP, Abrey LE, Neuwelt E; et al. (2011). "Primary CNS lymphoma in children and adolescents: a descriptive analysis from the International Primary CNS Lymphoma Collaborative Group (IPCG)". Clin Cancer Res. 17 (2): 346–52. doi:10.1158/1078-0432.CCR-10-1161. PMC 4058714. PMID 21224370.

[pmid26384083-3] Shalabi H, Angiolillo A, Vezina G, Rubenstein JL, Pittaluga S, Raffeld M; et al. (2015). "Prolonged Complete Response in a Pediatric Patient With Primary Peripheral T-Cell Lymphoma of the Central Nervous System". Pediatr Hematol Oncol. 32 (8): 529–34. doi:10.3109/08880018.2015.1074325. PMC 4942274. PMID 26384083.

[1]

[2]

[3]

@@ Line 1: / Line 1: @@
+__NOTOC__
 {{Non-Hodgkin lymphoma}}
-{{CMG}}
+{{CMG}} {{AE}} {{Preeti}}
 ==Overview==
-Common complications of Non-Hodgkin lymphoma include [[autoimmune hemolytic anemia]] and [[infection]]. The indolent Non-Hodgkin lymphoma types is associated with relatively good prognosis.
+Common complications of non-Hodgkin lymphoma include lymphadenopathy, disseminated intravascular coagulation, superior vena cava (SVC) syndrome, [[autoimmune hemolytic anemia]] and [[infection]]. The indolent non-Hodgkin lymphoma types are associated with a relatively good prognosis. The 5-year relative survival rate of patients with NHL is 71.4%.
+== Natural History ==
 ==Complications==
-Common complications of Non-Hodgkin lymphoma include:<ref> Non-Hodgkin lymphoma. medline plus. https://www.nlm.nih.gov/medlineplus/ency/article/000581.htm Accessed on September 22, 2015</ref>
+Common complications of non-Hodgkin lymphoma include:<ref name="pmid25921177">{{cite journal| author=Dehghani M, Haddadi S, Vojdani R| title=Signs, Symptoms and Complications of Non-Hodgkin's Lymphoma According to Grade and Stage in South Iran. | journal=Asian Pac J Cancer Prev | year= 2015 | volume= 16 | issue= 8 | pages= 3551-7 | pmid=25921177 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25921177  }} </ref>
-* [[Autoimmune hemolytic anemia]]
+*[[Lymphadenopathy]] especially cervical [[lymphadenopathy]]
-* [[Infection]]
+*Cytopenias such as [[neutropenia]], [[anemia]] and [[thrombocytopenia]] secondary to bone marrow infiltration
+*[[Autoimmune hemolytic anemia]]
+*Bleeding secondary to [[thrombocytopenia]]
+*[[Disseminated intravascular coagulation (patient information)|Disseminated intravascular coagulation (DIC)]]
+*Infection secondary to [[neutropenia]]
+*Cardiac problems secondary to large [[pericardial effusion]]
+*[[Cardiac arrhythmias]] secondary to cardiac metastases
+*Respiratory problems secondary to [[pleural effusion]]
+*[[Superior vena cava (SVC) syndrome]] secondary to a large mediastinal tumor
+*Spinal cord compression secondary to vertebral metastases
+*Neurologic problems secondary to [[primary CNS lymphoma]] or lymphomatous [[meningitis]]
+*Gastrointestinal obstruction, perforation, and bleeding in a patient with [[MALT lymphoma]]
+*Pain secondary to [[tumor]] invasion
+*[[Lymphocytosis]] in leukemic phase of disease
 ==Prognosis==
-* The Non-Hodgkin lymphoma can be divided into two prognostic groups: the indolent lymphomas and the aggressive lymphomas.<ref name=”cancergov”>National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/publications/pdq</ref>
+*The 5-year relative survival rate of patients with NHL is 71.4%.<ref name="pmid21224370">{{cite journal| author=Abla O, Weitzman S, Blay JY, O'Neill BP, Abrey LE, Neuwelt E et al.| title=Primary CNS lymphoma in children and adolescents: a descriptive analysis from the International Primary CNS Lymphoma Collaborative Group (IPCG). | journal=Clin Cancer Res | year= 2011 | volume= 17 | issue= 2 | pages= 346-52 | pmid=21224370 | doi=10.1158/1078-0432.CCR-10-1161 | pmc=4058714 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21224370  }} </ref>
-* Indolent Non-Hodgkin lymphoma types have a relatively good prognosis with a median survival as long as 20 years, but they usually are not curable in advanced clinical stages. Early-stage (stage I and stage II) indolent Non-Hodgkin lymphoma can be effectively treated with radiation therapy alone. Most of the indolent types are nodular (or follicular) in morphology.
+*The survival rate has steadily improved over the last 2 decades, thanks to improvements in medical and nursing care, the advent of novel therapeutic strategies (ie, [[monoclonal antibodies]]), validation of biomarkers of response, and the implementation of tailored treatment.
-* The aggressive type of Non-Hodgkin lymphoma has a shorter natural history, but a significant number of these patients can be cured with intensive combination chemotherapy regimens.
+*The prognosis for patients with NHL depends on the following factors:<ref name="pmid21224370">{{cite journal| author=Abla O, Weitzman S, Blay JY, O'Neill BP, Abrey LE, Neuwelt E et al.| title=Primary CNS lymphoma in children and adolescents: a descriptive analysis from the International Primary CNS Lymphoma Collaborative Group (IPCG). | journal=Clin Cancer Res | year= 2011 | volume= 17 | issue= 2 | pages= 346-52 | pmid=21224370 | doi=10.1158/1078-0432.CCR-10-1161 | pmc=4058714 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21224370  }} </ref>
-* In general, with modern treatment of patients with Non-Hodgkin lymphoma, overall survival at 5 years is over 60%. Of patients with aggressive Non-Hodgkin lymphoma, more than 50% can be cured. The vast majority of relapses occur in the first 2 years after therapy. The risk of late relapse is higher in patients who manifest both indolent and aggressive histologies.
+**Tumor histology
-* While indolent Non-Hodgkin lymphoma is responsive to [[immunotherapy]], [[radiation therapy]], and [[chemotherapy]], a continuous rate of relapse is usually seen in advanced stages. Patients, however, can often be re-treated with considerable success as long as the disease histology remains low grade. Patients who present with or convert to aggressive forms of Non-Hodgkin lymphoma may have sustained complete remissions with combination chemotherapy regimens or aggressive consolidation with marrow or stem cell support.
+**Tumor stage
+**Patient age
+**Tumor bulk
+**Performance status
+**[[Serum lactate dehydrogenase]] (LDH) level
+**[[Beta2-microglobulin]] level
 ===Prognostic Indexes===
 ====International Prognostic Index (IPI)====
-The [[International Prognostic Index]], or IPI, is the most widely used prognostic system for Non-Hodgkin's lymphoma. This system uses 5 factors:
+*The International Prognostic Index (IPI), which was originally designed as a prognostic factor model for aggressive non Hodgkin lymphoma (NHL) appears to be useful for predicting the outcome of patients with low-grade lymphoma and [[mantle cell lymphoma]].<ref name="pmid21224370">{{cite journal| author=Abla O, Weitzman S, Blay JY, O'Neill BP, Abrey LE, Neuwelt E et al.| title=Primary CNS lymphoma in children and adolescents: a descriptive analysis from the International Primary CNS Lymphoma Collaborative Group (IPCG). | journal=Clin Cancer Res | year= 2011 | volume= 17 | issue= 2 | pages= 346-52 | pmid=21224370 | doi=10.1158/1078-0432.CCR-10-1161 | pmc=4058714 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21224370  }} </ref><ref name="pmid26384083">{{cite journal| author=Shalabi H, Angiolillo A, Vezina G, Rubenstein JL, Pittaluga S, Raffeld M et al.| title=Prolonged Complete Response in a Pediatric Patient With Primary Peripheral T-Cell Lymphoma of the Central Nervous System. | journal=Pediatr Hematol Oncol | year= 2015 | volume= 32 | issue= 8 | pages= 529-34 | pmid=26384083 | doi=10.3109/08880018.2015.1074325 | pmc=4942274 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26384083  }} </ref>
-:*Age
+*This index is used to identify patients at high risk of relapse, based on specific sites of involvement, including [[Bone marrow abnormality|bone marrow]], [[Brain abscess|CNS]], liver, testis, lung, and spleen.
-:*[[Lactate dehydrogenase]] level (a blood test)
+*Clinical features included in the IPI that are independently predictive of survival include the following:
-:*Performance status
+**Age - Younger than 60 years versus older than 60 years
-:*Clinical stage
+**LDH level - Within the reference range versus elevated
-:*Sites of extranodal disease
+**Performance status - Eastern Cooperative Oncology Group ( ECOG) grade 0-1 versus 2-4
+**Ann Arbor stage - Stage I-II versus III-IV
-However, it should be noted that the IPI was developed prior to the introduction of [[rituximab]]. As [[rituximab]] has become a standard part of therapy for [[B-cell]] Non-Hodgkin lymphoma, the impact on the prognostic value of the IPI is unclear.
+**Number of extranodal sites - Zero to 1 versus more than 1
+*With this model, relapse-free and overall survival rates at 5 years are as follows:
-====Follicular Lymphoma International Prognostic Index (FLIPI)====
+**0-1 risk factors - 75%
-For the subtype of Non-Hodgkin lymphoma known as [[follicular lymphoma]], a modified version of the IPI called the FLIPI ([[International Prognostic Index|follicular lymphoma international prognostic index]]) has been developed. The factors which figure into the FLIPI are age, clinical stage, [[lactate dehydrogenase]] level, [[hemoglobin]] level, and number of nodal sites involved. As with the IPI, the FLIPI was developed and validated prior to the widespread use of [[rituximab]], so the same caveats apply as were mentioned with the IPI above.
+**2-3 risk factors - 50%
+**4-5 risk factors - 25%
-===5-Year Survival===
-* Between 2004 and 2010, the 5-year relative survival of patients with Non-Hodgkin lymphoma was 71.4%.<ref name="SEER">Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.</ref>
-* When stratified by age, the 5-year relative survival of patients with Non-Hodgkin lymphoma was 77.2% and 60.9% for patients <65 and ≥ 65 years of age respectively.<ref name="SEER">Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.</ref>
-* The survival of patients with Non-Hodgkin lymphoma varies with the stage of the disease.  Shown below is a table depicting the 5-year relative survival by the stage of Non-Hodgkin lymphoma:<ref name="SEER">Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.</ref>
-{| style="cellpadding=0; cellspacing= 0; width: 600px;"
-|-
-|style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align=center |'''Stage'''|| style="padding: 0 5px; font-size: 100%; background: #4682B4; color: #FFFFFF; width: 10%" align=center | '''5-year relative survival (%), (2004-2010)'''
-|-
-| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''All stages'''|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |69.3%
-|-
-| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Localized'''|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left  |81.6%
-|-
-| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |'''Regional'''|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |72.9%
-|-
-| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left  |'''Distant'''|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left  |61.6%
-|-
-| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left  |'''Unstaged'''|| style="font-size: 100; padding: 0 5px; background: #B8B8B8" align=left |67%
-|}
-* Shown below is an image depicting the 5-year conditional relative survival (probability of surviving in the next 5-years given the cohort has already survived 0, 1, 3 years) between 1998 and 2010 of Non-Hodgkin lymphoma by stage at diagnosis according to [[SEER]]. These graphs are adapted from [[SEER]]: The Surveillance, Epidemiology, and End Results Program of the National Cancer Institute.<ref name="SEER">Howlader N, Noone AM, Krapcho M, Garshell J, Miller D, Altekruse SF, Kosary CL, Yu M, Ruhl J, Tatalovich Z,Mariotto A, Lewis DR, Chen HS, Feuer EJ, Cronin KA (eds). SEER Cancer Statistics Review, 1975-2011, National Cancer Institute. Bethesda, MD, http://seer.cancer.gov/csr/1975_2011/, based on November 2013 SEER data submission, posted to the SEER web site, April 2014.</ref>
-[[Image:5-year survival in non Hodgkin lymphoma in USA.PNG|5-year conditional relative survival (probability of surviving in the next 5-years given the cohort has already survived 0, 1, 3 years) between 1998 and 2010 of non Hodgkin lymphoma by stage at diagnosis according to SEER]]
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