
Health Dictionary
Reliable, easy-to-read patient information on common medical conditions β written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
Anaplastic large cell lymphoma natural history, complications and prognosis
Introduction
Overview
Overview
The ALK-positive anaplastic large cell lymphoma is associated with the most favorable prognosis. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 70-80%. ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 30-40%.
Natural History
- If left untreated, patients with anaplastic large cell lymphoma may progress to develop anemia, intestinal obstruction, and immuno-suppression thus causing infection.
Complications
- Common complications of anaplastic large cell lymphoma include:
- Organ failure due metastasis
- Immuno-suppression thus causing infection
- CNS involvement causing:
- Meningitis
- Visual disturbance
- Facial numbness
- Anemia due to bleeding
- Stroke due to vascular occlusion from leukemic cells
Prognosis
Prognosis
- Those with ALK positivity have a better prognosis. [1]
- ALK-negative anaplastic large cell lymphomas represent other T-cell lymphomas in a final common pathway of disease progression.
- Overall better prognosis than other aggressive lymphomas.
- ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 70-80%.
- ALK-positive anaplastic large cell lymphoma is associated with a 5 year survival rate of 30-40%.
- The International Prognostic Iindex (IPI) is used to estimate the prognosis of patients.
- The IPI takes into account 5 variables:
- Patient’s age (>60 years)
- Elevated serum lactate dehydrogenase (LDH)
- Eastern Cooperative Oncology Group (ECOG) performance status
- Ann Arbor clinical stage III or IV
- Number of involved extra nodal sites > 1
- If any of this criteria is met, one point is awarded for the IPI. The interpretation of the total score is as follows:
- 0 to 1: Low risk
- 2: Low-intermediate risk
- 3: High-intermediate risk
- 4 to 5: High risk
- According to the International Peripheral T cell Lymphoma Project, the estimated 5-years survival for each of the IPI stages are as follows:[2]
- Low risk (IPI 0-1): 90%
- Low-intermediate risk (IPI 2): 68%
- High-intermediate risk (IPI 3): 23%
- High risk (IPI 4-5): 33%
- However, more recently, the International peripheral T-cell lymphoma Project score (IPTCLP) has demonstrated to be the most accurate score to predict overall survival in patients.[3] This score includes:
- Age
- Eastern Cooperative Oncology Group (ECOG) performance status
- Platelet count
References
References
- β Agrawal K, Shet T, Sridhar E, Dhende S, Sengar M, Arora B; et al. (2017). “Story of survival in anaplastic large cell lymphoma – sometimes more than the anaplastic lymphoma kinase status: An evaluation of pathologic prognostic factors in 102 cases”. Indian J Pathol Microbiol. 60 (4): 533β540. doi:10.4103/IJPM.IJPM_778_15. PMIDΒ 29323068.
- β “ALKβ anaplastic large-cell lymphoma is clinically and immunophenotypically different from both ALK+ ALCL and peripheral T-cell lymphoma, not otherwise specified: report from the International Peripheral T-Cell Lymphoma Project”.
- β “Comparison of four prognostic scores in peripheral T-cell lymphoma”.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2], Kamal Akbar, M.D.[3]; Grammar Reviewer: Natalie Harpenau, B.S.[4]
