Prolymphocytic leukemia

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Carlos A Lopez, M.D. [2], Maria Fernanda Villarreal, M.D. [3]

For more information regarding B-cell prolymphocytic leukemia, please click here.
For more information regarding T-cell prolymphocytic leukemia, please click here.

Overview

Prolymphocytic leukemia is a rare lymphoid leukemia, which account for only about 2% of all mature lymphoid leukemias. Prolymphocytic leukemias present similar to leukemia. Like lymphomas, they originate in the lymphocytes, but do not form solid tumors. Prolymphocytic leukemias are also considered lymphoproliferative disorders, which mean that lymphocytes are produced in large amounts.

Prolymphocytic leukemia may be classified according to the type of cell involved: B-cell prolymphocytic leukemia and T-cell prolymphocytic leukemia. Originally, it was thought to be a rare variation of chronic lymphocytic leukemia, but is now considered a distinct disease. It is usually classified as a kind of chronic lymphocytic leukemia. Although these 2 types of prolymphocytic leukemias share some of the same characteristics, the World Health Organization (WHO) classifies them as different types of lymphoid leukemias.[1]

Classification

For more details about each specific type of prolymphocytic leukemia, please select:

 
 
 
 
 
 
Prolymphocytic leukemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
B-cell prolymphocytic leukemia
 
 
 
 
 
T-cell prolymphocytic leukemia
 
 

Differentiating B-Prolymphocytic Leukemia from T-Prolymphocytic Leukemia

In order to distinguish B-prolymphocytic leukemia from T-prolymphocytic leukemia, see the table below.

Characteristics B-PLL T-PLL
Epidemiology
  • Rare
  • Very rare
Age
  • 60-70 years
  • 60-70 years
Onset
  • Elevated white blood cell count >100,000/microL
  • Hepatosplenomegaly
  • Elevated white blood cell count >100,000/microL
  • Splenomegaly
  • Generalized lymphadenopathy
  • Occasional skin involvement
Clinical Features

B symptoms

Diagnosis Peripheral blood smear
  • Prolymphocytes (90%)

Biomarkers

  • CD20+
Peripheral blood smear
  • Medium-sized lymphoid cells
  • Moderately condensed chromatin and a visible nucleolus

Biomarkers

  • CD52+

References


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