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]

Overview

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

Natural History, Complications, and Prognosis

Natural History

  • The natural history of adult T-cell leukemia varies between the different subtypes of the disease.[1]
  • 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:[1][2]

Prognosis

  • The prognosis varies between the subtypes of adult T-cell leukemia; acute and lymphomatous subtypes have a poor prognosis, whereas chronic and smoldering subtypes have a good prognosis.[3]
  • 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%.
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
Calcium level
Leukocyte count
Lactate dehydrogenase (LDH) level
β2-microglobulin level
Lymphocyte surface markers
Neuron‐specific enolase

References

  1. 1.0 1.1 1.2 Matutes E (2007). "Adult T-cell leukaemia/lymphoma". J Clin Pathol. 60 (12): 1373–7. doi:10.1136/jcp.2007.052456. PMC 2095573. PMID 18042693.
  2. Adult T-cell leukemia/lymphoma. Wikipedia (2015) https://en.wikipedia.org/wiki/Adult_T-cell_leukemia/lymphoma Accessed on November, 3 2015
  3. 3.0 3.1 Katsuya H, Ishitsuka K, Utsunomiya A, Hanada S, Eto T, Moriuchi Y; et al. (2015). "Treatment and survival among 1594 patients with ATL". Blood. 126 (24): 2570–7. doi:10.1182/blood-2015-03-632489. PMID 26361794.
  4. Mahieux R, Gessain A (2007). "Adult T-cell leukemia/lymphoma and HTLV-1". Curr Hematol Malig Rep. 2 (4): 257–64. doi:10.1007/s11899-007-0035-x. PMID 20425378.