T-cell prolymphocytic leukemia

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

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Treatment

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Qurrat-ul-ain Abid, M.D.[2], Maria Fernanda Villarreal, M.D. [3]

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

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating T-cell prolymphocytic leukemia historical perspective from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X-Ray Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Interventions | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1


Causes

  • Common causes of T-cell prolymphocytic leukemia, include genetic factors and chromosomal abnormalities:[1]
  • Trisomy 8, chromosomal abnormalities
  • Ataxia telangiectasia (ATM) gene mutation
  • TP53 gene mutation

Epidemiology and Demographics

  • T-cell prolymphocytic leukemia is very rare, and it represents 2% of all small lymphocytic leukemias in adults.
  • The incidence of T-cell prolymphocytic leukemia increases with age; the median age at diagnosis is 65 years.[1]
  • Patients with ataxia telangiectasia and T-cell prolymphocytic leukemia are young adults; the median age at diagnosis is 30 years.
  • Males are slightly more affected with T-cell prolymphocytic leukemia than females.
  • 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.[1]

Screening

There is insufficient evidence to recommend routine screening for T-cell prolymphocytic leukemia.

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:[1]
  • Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately one to two years.[1]
  • Patients with CD45RO+/CD45RA- immunophenotype tend to have a more indolent course.
  • It seems following factors are associated with worse prognosis:
    • Increased expression of TCL1
    • Increased activity of the serine-threonine kinase AKT

Diagnosis

Diagnostic Study of Choice

  • There are no established criteria for the diagnosis of T-cell prolymphocytic leukemia. Patients with T-cell prolymphocytic leukemia are diagnosed by clinical presentation, pathology evaluation of the peripheral blood and bone marrow. Flow cytometry and immunostains should be performed to diagnose a T cell immunophenotype.

History and Symptoms

  • Symptoms of T-cell prolymphocytic leukemia may include the following:[1][2]

Physical Examination

  • Patients with T-cell prolymphocytic leukemia usually appear pale and malnourished.
  • Physical examination may be remarkable for:[1]

Laboratory Findings

  • Laboratory findings consistent with the diagnosis of T-cell prolymphocytic leukemia, include:[1]
  • High lymphocyte count (> 100 x 109/L)
  • Anemia
  • Thrombocytopenia
  • Negative human T lymphotropic virus (HTLV) serology
  • Peripheral Blood Smear demonstrated predominance of lymphocytes:
    • Typical variant:
      • Medium-sized lymphocytes
      • Condensed chromatin and a visible nucleolus
      • Round nucleus
      • Slightly basophilic cytoplasm
      • Cytoplasmic protrusion
    • Small cell variant
      • Small tumor cells with condensed chromatin
      • Small nucleolus visible by electron microscopy
    • Cerebriform (Sézary cell-like) variant
      • Irregular nuclear outline
      • Similar to cerebriform nucleus of Sézary cells seen in mycosis fungoides

Electrocardiogram

There are no ECG findings associated with T-cell prolymphocytic leukemia.

X-ray

There are no x-ray findings associated with T-cell prolymphocytic leukemia. However, an x-ray may be helpful in the diagnosis of complications of T-cell prolymphocytic leukemia, which include pleural effusion and lung involvement.

Echocardiography or Ultrasound

There are no echocardiography findings associated with T-cell prolymphocytic leukemia.

Ultrasound may be helpful in the diagnosis of T-cell prolymphocytic leukemia. Findings on an ultrasound suggestive of/diagnostic of T-cell prolymphocytic leukemia include hepatomegaly and splenomegaly.

CT scan

CT scan may be helpful in the diagnosis of T-cell prolymphocytic leukemia. Findings on an CT scan suggestive of/diagnostic of T-cell prolymphocytic leukemia include hepatomegaly and splenomegaly.

MRI

There are no MRI findings associated with T-cell prolymphocytic leukemia.

Other Imaging Findings

There are no specific imaging findings associated with T-cell prolymphocytic leukemia.[1]

Other Diagnostic Studies

Flow cytometry and immunohistopathology must be done to diagnose T-cell prolymphocytic leukemia.

Treatment

Medical Therapy

  • The mainstay of therapy for T-cell prolymphocytic leukemia, include:[1][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.

Primary Prevention

  • There are no established measures for the primary prevention of T-cell prolymphocytic leukemia.

Secondary Prevention

  • There are no established measures for the secondary prevention of T-cell prolymphocytic leukemia.

References

  1. 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 1.9 Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759. PMID 23382603.
  2. Sud A, Dearden C (April 2017). "T-cell Prolymphocytic Leukemia". Hematol. Oncol. Clin. North Am. 31 (2): 273–283. doi:10.1016/j.hoc.2016.11.010. PMID 28340878.
  3. Robak T, Robak P (April 2007). "Current treatment options in prolymphocytic leukemia". Med. Sci. Monit. 13 (4): RA69–80. PMID 17392661.