Adult T-cell leukemia 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: Haytham Allaham, M.D. [2]; Grammar Reviewer: Natalie Harpenau, B.S.[3]

Overview

The natural history of adult T-cell leukemia varies between the different sub-types of the disease. Common complications of adult T-cell leukemia include cardiac arrhythmias, opportunistic infections, and bone fractures. The prognosis varies between the sub-types of adult T-cell leukemia. Acute and lymphomatous sub-types have a poor prognosis; whereas, chronic and smoldering sub-types have a good prognosis.

Natural History, Complications, and Prognosis

Natural History

• The natural history of adult T-cell leukemia varies between the different sub-types of the disease.
• Patients with acute adult T-cell leukemia usually have an aggressive clinical course with a median survival period of less than 12 months. If left untreated, most of the patients with acute adult T-cell leukemia will develop constitutional symptoms, lymphadenopathy, and organomegaly within a few weeks of diagnosis.
• Patients with chronic adult T-cell leukemia will usually have an stable clinical course. If left untreated, most of the patients with chronic adult T-cell leukemia will develop lymphocytosis for months, or even years, before presenting with the typical cutaneous manifestations.
• Most patients with smoldering adult T cell leukemia are initially asymptomatic. If left untreated, most of the patients with smoldering adult T cell leukemia will develop steroid-responsive skin rash and multiple lung infiltrates.

Complications

• Common complications of adult T-cell leukemia include:

• Cardiac arrhythmias (due to hypercalcemia)
• Opportunistic infections (Strongyloides stercoralis infection is a frequent cause of death among adult T-cell leukemia patients)
• Bone fractures (due to lytic bone lesions)
• Anemia
• Recurrent bleeding

Prognosis

• The prognosis varies between the sub-types of adult T-cell leukemia. Acute and lymphomatous sub-types have a poor prognosis; whereas, chronic and smoldering sub-types have a good prognosis.

• The 4-year overall survival rate of patients with adult T-cell leukemia is approximately 16%.
• The 4-year overall survival rate of patients with acute adult T-cell leukemia is approximately 11%.
• The 4-year overall survival rate of patients with chronic adult T-cell leukemia is approximately 36%.
• The 4-year overall survival rate of patients with smouldering adult T-cell leukemia is approximately 52%.

• The table below lists prognostic factors for adult T-cell leukemia patients:

Prognostic Factor	Description
Clinical subtype	Acute and lymphomatous subtypes have a poor prognosis, where as chronic and smouldering subtypes have a good prognosis.
Gender	Males are associated with a worse prognosis when compared to females.
Performance status	Patient's poor performance status is associated with a worse prognosis.
Calcium level	Hypercalcemia is associated with a worse prognosis.
Leukocyte count	An elevated leukocyte count is associated with a worse prognosis.
Lactate dehydrogenase (LDH) level	Elevated level of LDH is associated with a worse prognosis.
β2-microglobulin level	An elevated β2-microglobulin level is associated with a worse prognosis.
Lymphocyte surface markers	Over expression of CD25 is associated with a worse prognosis.
Neuron‐specific enolase	An elevated neuron‐specific enolase level is associated with a worse prognosis.