B cell prolymphocytic leukemia

B cell prolymphocytic leukemia (B-PLL) is a rare and aggressive type of leukemia characterized by the overproduction of B lymphocytes, a type of white blood cell that plays a crucial role in the immune system. It is a subtype of prolymphocytic leukemia, which also includes T cell prolymphocytic leukemia.

Epidemiology

B-PLL is extremely rare, accounting for less than 1% of all cases of chronic lymphocytic leukemia (CLL). It typically affects older adults, with a median age of diagnosis of around 70 years. There is no known gender predilection.

Pathophysiology

The pathophysiology of B-PLL involves the uncontrolled proliferation of mature B cells, which are typically larger than those seen in CLL. These cells infiltrate the bone marrow, spleen, and lymph nodes, leading to the clinical manifestations of the disease.

Clinical Features

Patients with B-PLL often present with symptoms related to anemia, such as fatigue and weakness, due to bone marrow infiltration. Splenomegaly (enlarged spleen) and lymphadenopathy (enlarged lymph nodes) are also common. Unlike CLL, B-PLL is typically aggressive and rapidly progressive.

Diagnosis

Diagnosis of B-PLL is based on clinical features, blood tests showing a high white blood cell count, and immunophenotyping to identify the abnormal B cells. Cytogenetic analysis may also be performed to identify any genetic abnormalities associated with the disease.

Treatment

Treatment options for B-PLL include chemotherapy, immunotherapy, and stem cell transplantation. However, the disease is often resistant to treatment and has a poor prognosis.

Prognosis

The prognosis of B-PLL is generally poor, with a median survival of less than 3 years. However, some patients may respond well to treatment and achieve long-term remission.

See Also

• Chronic lymphocytic leukemia
• Prolymphocytic leukemia
• Leukemia