B-cell prolymphocytic leukemia overview

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B-cell prolymphocytic leukemia

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating B-cell prolymphocytic leukemia from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Biopsy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Carlos A Lopez, M.D. [2]

Synonyms and Keywords: B-PLL, Prolymphocytic leukemia, B-cell type

Overview

B-cell prolymphocytic leukemia is a form of leukemia or cancer of the white blood cells. Prolymphocytic leukemia is classified in B-cell prolymphocytic leukemia and T-cell prolymphocytic leukemia, B-cell prolymphocytic leukemia is part of a subclassification of prolymphocytic leukemias. Arises from mature B-cells, which are hematologic white cells that are normally involved in the in the humoral immunity component of the adaptive immune system by secreting antibodies. B-cell prolymphocytic leukemia must be differentiated from other diseases such as T-cell prolymphocytic leukemia, chronic lymphocytic leukemia and hairy cell leukemia. The incidence of B-cell prolymphocytic leukemia is 1% of lymphocytic leukemias. B-cell prolymphocytic leukemia affects men and women equally. The prognosis is generally poor. Symptoms of B-cell prolymphocytic leukemia include generalised weakness and, anemia,massive splenomegaly, weight loss, loss of appetite, thrombocytopenia and peripheral lymphadenopathy. Common physical examination findings include tachycardia, pallor, splenomegaly and lymphadenopathy. Laboratory findings consistent with the diagnosis of B-cell prolymphocytic leukemia include anemia, lymphocytosis, abnormal blood chemistry studies and abnormal blood peripheral smear. B-cell prolymphocytic leukemia bone marrow biopsy is considered a definitive diagnosis. Other diagnostic studies include immunophenotyping.

Historical Perspective

B-cell prolymphocytic leukemia was first described in 1974 by Galton et al.

Classification

Prolymphocytic leukemia is classified in B-cell prolymphocytic leukemia and T-cell prolymphocytic leukemia. B-cell prolymphocytic leukemia is part of a subclassification of prolymphocytic leukemias.

Pathophysiology

B-cell prolymphocytic leukemia arises from mature B-cells, which are hematologic white cells that are normally involved in the in the humoral immunity component of the adaptive immune system by secreting antibodies.

Differentiating B-cell prolymphocytic leukemia from other Diseases

B-cell prolymphocytic leukemia must be differentiated from other diseases such as T-cell prolymphocytic leukemia, chronic lymphocytic leukemia and hairy cell leukemia.

Epidemiology and Demographics

The incidence of B-cell prolymphocytic leukemia is 1% of lymphocytic leukemias. B-cell prolymphocytic leukemia affects men and women equally.

Risk Factors

Common risk factors in the development of B-cell prolymphocytic leukemia are age, gender, race and ethnicity.

Screening

Screening for B-cell prolymphocytic leukemia is not recommended.

Natural History, Complications and Prognosis

The prognosis for B-cell prolymphocytic leukemia is generally poor.

Diagnosis

History and Symptoms

Symptoms of B-cell prolymphocytic leukemia include generalised weakness, anemia, massive splenomegaly, weight loss, loss of appetite, thrombocytopenia and peripheral lymphadenopathy.

Physical Examination

Common physical examination findings of B-cell prolymphocytic leukemia include tachycardia, pallor, splenomegaly and lymphadenopathy.

Laboratory Findings

Laboratory findings consistent with the diagnosis of B-cell prolymphocytic leukemia include anemia, lymphocytosis, abnormal blood chemistry studies and abnormal blood peripheral smear.

Biopsy

B-cell prolymphocytic leukemia bone marrow biopsy is considered a definitive diagnosis.

Other Diagnostic Studies

Other diagnostic studies for B-cell prolymphocytic leukemia include immunophenotyping.

Medical Therapy

Chemotherapy, biological therapy, and splenectomy or radiation therapy to the spleen are indicated in the treatment of B-cell prolymphocytic leukemia.

References


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