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Plasma Cell Leukemia: Causes, Symptoms, and Treatment

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A rare and severe form of leukemia and blood disorder affecting plasma cells is known as plasma cell leukemia (PCL).

Written by

Dr. Kinjal Shah

Medically reviewed by

Dr. Abdul Aziz Khan

Published At November 3, 2023
Reviewed AtNovember 3, 2023

Introduction

The Wiener Klinische Wochenschrift published a description of the first instance of PCL in 1906 by Professor A. Gluzinski and Dr. M. Reichenstein. The writers were employed at the University Hospital of Lemberg (Lvov), which is today a major city in western Ukraine but was formerly a part of the Austrian Empire. An uncommon and severe form of blood cancer known as plasma cell leukemia (PCL) is characterized by the uncontrolled development and proliferation of plasma cells, a kind of white blood cell that produces antibodies. Another cancer that affects plasma cells, multiple myeloma, is thought to be a variation of PCL.

What Is Plasma Cell Leukaemia?

Plasma cells in the bone marrow give rise to plasma cell leukemia (PCL), a rare and severe kind of blood cancer. It is a subtype of multiple myeloma, a plasma cell malignancy that usually affects the bone marrow and results in the generation of aberrant antibodies (M-proteins). In contrast to multiple myeloma, which often has a proportion of plasma cells in the blood that is less than five percent, PCL is defined by the presence of a significant number of malignant plasma cells in the peripheral blood (at least 20 percent of the total white blood cells). Less than five percent of all plasma cell illnesses are PCL, and compared to other types of multiple myeloma, it has a worse prognosis.

What Are the Plasma Cell Leukaemia Causes and Risk Factors?

Although the precise causes of PCL are not yet fully known, a number of risk factors have been identified, including:

  1. Age: Those over 60 are more likely to have PCL.

  2. Gender: Men are somewhat more likely than women to have PCL.

  3. Genetic Mutations: Many genetic changes, including changes in the TP53 and BRAF genes, have been linked to PCL.

  4. Multiple Myeloma Prior Diagnosis: PCL is more likely to develop in those who have previously been diagnosed with multiple myeloma.

  5. Exposure to Radiation or Specific Chemicals: PCL risk may be increased by exposure to high radiation levels or specific chemicals, such as benzene.

  6. Immune System Dysfunction: PCL risk may be higher in those with compromised immune systems, such as those with HIV/AIDS or organ transplant recipients.

Remember, these risk factors may make PCL more likely to occur; not everyone who possesses them will experience the condition. Moreover, many PCL instances include persons who have no identified risk factors.

What Are the Signs and Symptoms of Plasma Cell Leukemia?

The majority of known PCL's symptoms come from numerous case studies because the disease is so uncommon. Due to the significant presence of aberrant plasma cells in the blood, many of PCL's symptoms are linked to organ damage. The following are examples of possible PCL symptoms:

  1. Anemia.

  2. Several infections.

  3. Simple bleeding or bruising of the bones.

  4. Elevated blood calcium levels (hypercalcemia).

  5. An enlarged lymph node.

  6. Increased spleen (splenomegaly).

  7. Overgrown liver (hepatomegaly).

  8. The lungs' fluid (pleural effusion).

  9. Renal failure.

  10. Abnormal blood test results, such as high calcium, creatinine, or M-protein levels.

How Is Diagnosis of Plasma Cell Leukemia Made?

The presence of peripheral blood plasma cells in an amount of at least 20 percent, together with additional clinical and laboratory findings that point to the existence of a plasma cell disease, are used to make the diagnosis of PCL.

  1. Blood Test: Complete blood counts (CBCs), which measure the quantity of all blood cells, including platelets, red blood cells, and white blood cells, can be used to identify abnormalities. Certain proteins, such as beta-2 microglobulin and immunoglobulins, which are frequently high in PCL patients can also be checked with blood examinations.

  2. Bone Marrow Biopsy: To test for aberrant plasma cells, a tiny sample of bone marrow is removed from the hip bone or another major bone and examined under a microscope.

  3. Imaging Studies: The degree of the disease and if it has progressed to other body areas can be ascertained with the aid of imaging examinations such as X-rays, CT scans, MRI scans, and PET scans.

  4. Flow Cytometry: With the use of a laser, cells' size, shape, and surface proteins are examined by flow cytometry in order to ascertain whether they are aberrant plasma cells.

  5. Cytogenetic Analysis: This examination of a cell's chromosomes looks for any anomalies or mutations that could be connected to PCL.

If PCL is suspected, in order to confirm the diagnosis and choose the most effective course of action, more tests could be required. It is important to work with an experienced healthcare worker of blood cancers, such as a hematologist-oncologist.

How Is Plasma Cell Leukemia Treated?

The amount of the disease, the patient's age and general health, and the existence of any underlying medical disorders are some of the variables that affect how plasma cell leukemia (PCL) is treated. Some of the typical PCL treatments include the following:

  1. Chemotherapy: The most typical form of treatment for PCL is chemotherapy. It includes administering medications intravenously or orally in order to destroy cancer cells.

  2. Transplantation of Stem Cells: In some circumstances, a stem cell transplant may be advised. In this operation, healthy donor stem cells are used to replenish the patient's bone marrow.

  3. Targeted Therapy: Drugs are used in targeted therapy to selectively target cancer cells while causing the least amount of harm to healthy cells.

  4. Radiation Treatment: To destroy cancer cells, high-energy radiation is used in radiation therapy. Localized illness is often treated with it.

  5. Supporting Care: An essential component of PCL therapy is supportive care. This may involve taking drugs to control pain, stop infections from happening, and support the patient's general health and well-being.

How Is the Prognosis of Plasma Cell Leukemia?

PCL has a poor prognosis in general, with a median survival duration of less than a year. Nonetheless, some individuals may benefit from their therapy and live longer. The patient's age and general health, the severity of the disease, the existence of other medical disorders, and the patient's response to therapy are all variables that might affect the prognosis. In order to track the disease's development and alter the course of therapy as necessary, follow-up care is crucial.

Conclusion:

Plasma cell leukemia is an uncommon and severe type of blood cancer that has to be identified and treated very quickly. While PCL is a rare and aggressive form of cancer, advances in diagnosis and treatment have led to improved outcomes for many patients. Despite the typically dismal prognosis, some patients' fates may be enhanced by recent developments in supportive care and therapeutic alternatives.

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Dr. Abdul Aziz Khan
Dr. Abdul Aziz Khan

Medical oncology

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