T-cell prolymphocytic leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and Keywords: T-cell chronic lymphocytic leukemia; "Knobby" type of T-cell leukemia; T-prolymphocytic leukemia/T-cell lymphocytic leukemia- Kiel; T-PLL

Overview

T-cell-prolymphocytic leukemia (also known as T-PLL) is a rare, mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin.[1] T-cell prolymphocytic leukemia was first described by Catovsky in 1973.[2] There is no classification system for T-cell prolymphocytic leukemia. The inversion of chromosome 14 (14q11) has been associated with the development of T-cell prolymphocytic leukemia. T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults. T-cell prolymphocytic leukemia is more commonly observed among young adult patients aged between 30 to 40 years old. Males are slightly more affected with are more commonly affected with T-cell prolymphocytic leukemia than females. Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include: high lymphocyte count (> 100 x 109/L), anemia, thrombocytopenia, and negative HTLV-1 serology.[3] There are no specific imaging findings associated with T-cell prolymphocytic leukemia. Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately 7 months.[3] The mainstay of therapy for T-cell prolymphocytic leukemia is alemtuzumab (anti-CD52). However, T-cell prolymphocytic leukemia is often resistant to therapy. Autologous and allogeneic stem cell transplants is the mainstay of therapy for patients who achieve remission.[3]

Historical Perspective

  • T-cell prolymphocytic leukemia was first described by Catovsky in 1973.[2]

Classification

  • There is no classification system for T-cell prolymphocytic leukemia.[3]

Pathophysiology

  • T-cell prolymphocytic leukemia arises from mature (post-thymic) T-cell, which is normally involved in in cell-mediated immunity.
  • The inversion of chromosome 14 (14q11) has been associated with the development of T-cell prolymphocytic leukemia.
  • Patients with T-cell prolymphocytic leukemia have TCR gene rearrangements for the γ and δ chains.
  • Mutations of chromosome 8 are seen in approximately 75% of patients.
  • On gross pathology, characteristic findings of T-cell prolymphocytic leukemia, include:[3]
  • No remarkable findings
  • On microscopic histopathological analysis, characteristic findings of T-cell prolymphocytic leukemia, include:[3]
  • The immunophenotype CD4+/CD8- (present in 60% of cases)
  • The immunophenotype CD4+/CD8+ (present in 25%)
  • The immunophenotype CD4-/CD8+ (15% of cases)

Pan-T antigens

  • CD2 negative
  • CD3 negative
  • CD7 negative
  • TdT positive
  • CD1a positive

Causes

  • Common causes of T-cell prolymphocytic leukemia, include:[3]
  • Genetic mutations (e.g. Trisomy 8, chromosomal abnormalities)

Differentiating T-cell Prolymphocytic Leukemia from Other Diseases

Epidemiology and Demographics

  • T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults.

Age

  • T-cell prolymphocytic leukemia is more commonly observed among patients aged between 30 to 40 years old.[3]
  • T-cell prolymphocytic leukemia is more commonly observed among young adults.

Gender

  • Males are slightly more affected with are more commonly affected with T-cell prolymphocytic leukemia than females.

Race

  • There is no racial predilection for T-cell prolymphocytic leukemia.

Risk Factors

  • There are no risk factors associated with the development of T-cell prolymphocytic leukemia.[3]

Natural History, Complications and Prognosis

  • The majority of patients with T-cell prolymphocytic leukemia are symptomatic at the time of diagnosis.
  • Early clinical features, include fever, fatigue, and lymphadenopathy.
  • If left untreated, patients with T-cell prolymphocytic leukemia may progress to develop multiple organ failure.
  • Common complications of T-cell prolymphocytic leukemia, include:[3]
  • Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately 7 months.[3]

Diagnosis

Symptoms

  • Symptoms of T-cell prolymphocytic leukemia may include the following:[3]

Physical Examination

  • Patients with T-cell prolymphocytic leukemia usually appear pale and malnourished.
  • Physical examination may be remarkable for:[3]

Peripheral Blood Smear

  • Medium-sized lymphocytes
  • Single nucleoli and basophilic cytoplasm
  • The nuclei are usually round to oval in shape,
  • Irregular nuclear outline that is similar to the cerebriform nuclear shape seen in Sézary syndrome.
  • A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include:[3]

Imaging Findings

  • There are no specific imaging findings associated with T-cell prolymphocytic leukemia.[3]

Treatment

Medical Therapy

  • The mainstay of therapy for T-cell prolymphocytic leukemia, include:[3]
  • T-cell prolymphocytic leukemia is often resistant to therapy.

Surgery

  • Autologous and allogeneic stem cell transplants is the mainstay of therapy for patients who achieve remission.

Prevention

  • There are no primary preventive measures available for T-cell prolymphocytic leukemia.

References

  1. Jaffe E.S., Harris N.L., Stein H., Vardiman J.W. (eds): World Health Organization Classification of Tumors. Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues. IARC Press: Lyon 2001
  2. 2.0 2.1 Catovsky D, Galetto J, Okos A, Galton DA, Wiltshaw E, Stathopoulos G. Prolymphocytic leukaemia of B and T cell type. Lancet
  3. 3.00 3.01 3.02 3.03 3.04 3.05 3.06 3.07 3.08 3.09 3.10 3.11 3.12 3.13 3.14 3.15 3.16 Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759Freely accessible. PMID 23382603. 



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