B-cell prolymphocytic leukemia, referred to as B-PLL, is a rare blood cancer. It is a more aggressive, but still treatable, form of leukemia. Specifically, B-PLL is a prolymphocytic leukemia (PLL) that affects prolymphocytes – immature forms of B-lymphocytes and T-lymphocytes – in the peripheral blood, bone marrow, and spleen. It is an aggressive cancer that presents poor response to treatment.Mature lymphocytes are infection-fighting immune system cells. B-lymphocytes have two responsibilities:
Production of antibodies – In response to antigens, B-lymphocytes produce and release antibodies specific to foreign substances in order to aid in their identification and elimination phagocytes
Generation of memory cells – Interactions between antibodies and antigens allow B-lymphocytes to establish cellular memories, otherwise known as immunities that allow the body to respond more rapidly and efficiently to previously encountered species

Classification
It is categorized as a lymphoproliferative disorder due to the excessive production of lymphocytes, in B-PLL there is excess production of B-prolymphocytes by the bone marrow. These immature lymphocytes are not normally found in the blood; part of their maturation process is being programmed to produce antibodies against foreign material prior to their departure from the bone marrow. In B-PLL, malignant B-prolymphocytes disrupt the adaptive capabilities of the immune system due to the lack of mature B-lymphocytes.It has been suggested that some cases may represent a variant of mantle cell lymphoma. Signs and symptoms
This type of leukemia is characterized by:
More than 55% of circulating cells in peripheral blood (red blood cells, white blood cells and platelets collectively) are prolymphocytes.
This mutant shows a higher sensitivity to acetylcholine and unchanged desensitization compared to wild-type. Diagnosis
Diagnosis is typically made upon patient history, although EEG recordings can be confirmatory if they occur during attacks. Management
Anti-epileptic drugs are normally used to combat ADNFLE. These drugs are discussed in the main epilepsy article. Footnotes
Further reading
GeneReviews/NCBI/NIH/UW entry on Autosomal Dominant Nocturnal Frontal Lobe Epilepsy


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