Anaplastic large cell lymphoma natural history, complications and prognosis

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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]

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

Complications

  • Common complications of anaplastic large cell lymphoma include:
    • Organ failure due metastasis
    • Immuno-suppression thus causing infection
    • CNS involvement causing:
    • Anemia due to bleeding
    • Stroke due to vascular occlusion from leukemic cells

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

  1. 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): 533–540. doi:10.4103/IJPM.IJPM_778_15. PMID 29323068.
  2. "ALK− 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".
  3. "Comparison of four prognostic scores in peripheral T-cell lymphoma".

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