B-cell prolymphocytic leukemia, also referred to as B-PLL, is a rare type of chronic lymphocytic leukemia that affects the prolymphocytes (B-cells) of the adaptive immune system. The disease represents less than 1% of all leukemia cases worldwide.
Prolymphocytes are immature white blood cells whose primary function it is to secrete antibodies against substances known as antigens, which include various toxins, bacteria, and viruses.
The interaction between these antibodies and the antigens causes B-cells to develop cellular memories, or immunities. It is because of B-cell memory that most humans only contract the chicken pox once, for instance. When malignant prolymphocytes result in the onset of B-cell prolymphocytic leukemia, this process of adaptive immunity is disrupted: a key feature of the disease.
Most cases of B-cell prolymphocytic leukemia affect individuals over the age of 60. B-PLL has a male predominance with a male to female ratio of 1.6 to 1. Unfortunately, Prognosis for this leukemia is typically poor.
B-cell Prolymphocytic Leukemia Symptoms and Features
This leukemia is characterized by the presence enlarged prolymphocytes, leading to the development of any of the following symptoms: a swollen spleen, weight loss, tiredness, and high concentrations of prolymphocytes in the bone marrow and/or blood. Like many leukemias, B-cell prolymphocytic leukemia is often initially asymptomatic and is frequently diagnosed during an unrelated procedure.
When a disease is asymptomatic, a physician will usually use the word “sign” to characterize the disease’s features, not “symptom”. The word “symptom” usually indicates the features of a disease that are observed and experienced by the patient; whereas “signs” of a disease are often only distinguishable through the eyes and skilled senses of a medical professional.
B-cell Prolymphocytic Leukemia Diagnosis
Diagnosis is dependent on a number of blood tests that will determine whether or not a patient’s blood cells are anomalous. Furthermore, these tests will reveal the quantities and ratios substances within the blood. In the case of B-PLL, diagnosis is dependent on the demonstration that there are high concentrations of anomalous prolymphpcytes in the blood. Once this abnormality has been confirmed, tissue samples are often recommended(biopsy).
Biopsies are very common operations that are usually performed while the patient is under local or general anaesthetic. Computed tomography (CT scan) and/or magnetic resonance imagining (MRI) are also usually employed to give the physician and patient a clearer understanding of the lymphoma’s location, size, rate of metastasis, and other features.
A physician may also recommend microscopically evaluating bone marrow samples and/or the employment of an x-ray in order to gather a more comprehensive understanding of the patient’s condition. The more in-depth the physician’s understanding of the lymphoma, the more appropriately he or she can treat the disease.
B-cell Prolymphocytic Leukemia Treatment
All types of chronic lymphocytic leukemia (CLL) are considered incurable and, thus, all treatments are palliative. Furthermore, B-cell prolymphocytic leukemia progresses considerably faster than most CLL’s, making effective treatment very difficult. The purpose of treating the disease is to reduce symptoms and control progression.
Occasionally, chemotherapy or radiation therapy is employed to reduce neoplastic cell proliferation, but neither treatment can be curative. Treatments such as bone marrow transplantation are usually considered to be too risky and only marginally effective.
The most common palliative treatments involve the administration of various drugs whose purpose it is to prolong functional and comfortable survival.
B-cell Prolymphocytic Leukemia Prognosis
B-cell prolymphocytic leukemia is so rare that it is difficult to assess what the approximate survival rates are for patients with the disease. Like all cancers, early detection improves a patient’s chances of successful treatment and longer survival. This type of leukemia is incurable and very aggressive; thus, resulting in the death of the patient