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

• 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

• Those with ALK positivity have a better prognosis.
• 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:

• 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. This score includes:

• Age
• Eastern Cooperative Oncology Group (ECOG) performance status
• Platelet count