Sandbox: 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 mature T-cell leukemia with aggressive behavior and predilection for blood, bone marrow, lymph nodes, liver, spleen, and skin involvement.[1]


Historical Perspective

  • T-cell prolymphocytic leukemia was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].

Classification

  • T-cell prolymphocytic leukemia may be classified according to World Health Organization (WHO) classification system into subtypes:
  • [group1]
  • [group2]
  • [group3]


Pathophysiology

  • The pathogenesis of t-cell prolymphocytic leukemia is characterized by:
  • The has been associated with the development of t-cell prolymphocytic leukemia.
  • On gross pathology, characteristic findings of t-cell prolymphocytic leukemia, include:
  • On microscopic histopathological analysis, characteristic findings of t-cell prolymphocytic leukemia, include:

Causes

  • Common causes of T-cell prolymphocytic leukemia, include:

Differentiating T-cell Prolymphocytic Leukemia from Other Diseases

  • T-cell prolymphocytic leukemia must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
  • [Differential dx1]
  • [Differential dx2]
  • [Differential dx3]

Epidemiology and Demographics

  • The prevalence of t-cell prolymphocytic leukemia is approximately [number or range] per 100,000 individuals worldwide.
  • 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

  • T-cell prolymphocytic leukemia affects men and women equally.

Race

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

Risk Factors

  • Common risk factors in the development of t-cell prolymphocytic leukemia are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

Natural History, Complications and Prognosis

  • The majority of patients with t-cell prolymphocytic leukemia remain asymptomatic for [duration/years].
  • Early clinical features, include
  • If left untreated, patients with t-cell prolymphocytic leukemia may progress to develop
  • Common complications of t-cell prolymphocytic leukemia, include:
  • Prognosis is generally poor, and the 5 year survival rate of patients with t-cell prolymphocytic leukemia is approximately

Diagnosis

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:
  • Hepatosplenomegaly
  • Generalized lymphadenopathy
  • Skin infiltration

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:
  • High lymphocyte count (> 100 x 109/L)
  • Anemia
  • Thrombocytopenia

Imaging Findings

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

Treatment

Medical Therapy

  • There is no treatment for t-cell prolymphocytic leukemia; the mainstay of therapy is supportive care.
  • The mainstay of therapy for t-cell prolymphocytic leukemia is [medical therapy 1] and [medical therapy 2].
  • [Medical therapy 1] acts by [mechanism of action1].
  • Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

  • Surgery is the mainstay of therapy for t-cell prolymphocytic leukemia.
  • [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of t-cell prolymphocytic leukemia.
  • [Surgical procedure] can only be performed for patients with [disease stage] t-cell prolymphocytic leukemia.

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