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. T-cell prolymphocytic leukemia was first described by Catovsky in 1973. 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. 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. 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.

Historical Perspective

• In 1973, T-cell prolymphocytic leukemia was first described by Catovsky.
• In 1994,

Classification

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

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:

• No remarkable findings

• On microscopic histopathological analysis, characteristic findings of T-cell prolymphocytic leukemia, include:

• 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:

• Genetic mutations (e.g. Trisomy 8, chromosomal abnormalities)

Differentiating T-cell Prolymphocytic Leukemia from Other Diseases

• T-cell prolymphocytic leukemia must be differentiated from other diseases that cause lymphadenopathy, hepatomegaly, and fever, such as:

• Sézary syndrome
• Cutaneous T cell lymphoma
• Angioimmunoblastic T cell lymphoma
• B-cell prolymphocytic leukemia

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.
• 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.

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:

• Graft-versus-host disease (allogeneic transplant)
• Infections
• Bleeding

• Prognosis is generally poor, and the median survival time of patients with T-cell prolymphocytic leukemia is approximately 7 months.

Diagnosis

History and Symptoms

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

• Fever
• Weight loss
• Night sweats

Physical Examination

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

• Hepatomegaly
• Splenomegaly
• Generalized lymphadenopathy
• Skin infiltration

Laboratory Findings

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

• High lymphocyte count (> 100 x 109/L)
• Anemia
• Thrombocytopenia
• Negative HTLV-1 serology 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

Other Diagnostic Studies

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

Treatment

Medical Therapy

• The mainstay of therapy for T-cell prolymphocytic leukemia, include:

• Alemtuzumab (anti-CD52)

• 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.