Paraproteinemia

What is Paraproteinemia? It is a medical condition characterized by a high level of paraprotein in the blood.

What is a monoclonal gammopathy? It is another term for Paraproteinemia. The paraprotein in the blood contains monoclonal gammaglobulin. This condition is common in people with immunoproliferative disorder. ⁽¹⁾

What is a paraprotein band?

Paraproteins are immunoglobulins produced at the bone marrow. Excess paraprotein in urine can severely damage the kidneys. Paraproteins present itself as a light chain present not only in the urine but in the blood. It is the clonal population of mature B cells that produce paraprotein. A perfect example of mature B cell is plasma cell. ⁽²⁾

Photo 1: A closer look at the plasma cells in a patient with multiple myeloma.
Picture Source: image.slidesharecdn.com

Picture 2: An image of an elderly’s hands with bruising as a result of paraproteinemia.
Photo Source: piel-l.org

What are the types of Paraproteinemia?

1. Light chain Paraproteinemia (Bence Jones Protein) – This is linked with immunoproliferative disorder. Examples are multiple myeloma and amyloidosis. It can escape from the blood vessels and will then be excreted by the kidneys. The paraprotein found in urine is known as Bence Jones proteins.
2. Heavy chain Paraproteinemia – It is also known as heavy chain disease.
3. Whole immunoglobulin Paraproteinemia – It is characterized by the presence of M-proteins in the blood. Both the heavy chain and whole immunoglobulin Paraproteinemia are restricted to the blood vessels. Some patients experienced macroglobulinemia, a condition wherein whole immunoglobulins bind with one another. It severely affects the health of the person. ^{(2, 3, 4)}

Paraproteinemia Symptoms

Paraproteinemia symptoms are often confused with other diseases. The typical clinical manifestations include fever, body weakness, and bone pain. The patient complains of persistent back pain and other related bone disease. Other clinical manifestations include:

• Impaired renal function
• Anemia
• Hypercalcemia (high calcium level in the blood)
• Hyperviscosity (increased viscosity of the blood)
• Recurrent bacterial infections
• High ESR (erythrocyte sedimentation rate)
• Peripheral neuropathy (damage to the peripheral nerves)
• Carpal tunnel syndrome (numbness, tingling, and weak sensation of the hands)
• Nephrotic syndrome
• Cardiac failure ^{(4, 5)}

What are the causes of paraproteinemia?

Leukemia and Lymphoma

• Myeloma – It is the cancer of the plasma cells. The abnormal cell collect at the bone marrow level and affects the production of healthy blood cells.
• B-cell non-Hodgkin lymphoma – It is a cancer originating from the lymphocytes (white blood cells). ⁽⁶⁾
• Lymphoplasmacytic lymphoma – It is a certain type of cancer affecting the B white cells. It spreads in a gradual manner.
• Plasmacytoma – it is a malignant type of tumor affecting the plasma cells. It affects the soft tissues as well as the axial skeleton. ⁽⁷⁾

Other probable causes of paraproteinemia include the following:

• Benign monoclonal gammopathy
• Idiopathic or no identified cause at all
• Light chain paratoproteinemia/primary amyloidosis
• Rheumatoid arthritis
• Scleroderma
• Hashimoto disease
• Thyroiditis
• Liver disease such as hepatitis and liver cirrhosis
• Mycobacterium tuberculosis
• Endocarditis ^{(6, 7, 8)}

What methods are used to diagnose paraproteinemia?

A series of tests and diagnostic procedures should be done to accurately diagnose paraproteinemia. They are as follows:

• Blood testing to check for serum protein electrophoresis narrow band/spike, serum immunofixation electrophoresis, and haematocrit.
• Blood viscosity analysis to check for the presence of immunoglobulins in the blood.
• Serum free light chain which will be correlated with urine analysis. A 24-hour collection of urine is needed.
• Computed tomography (CT Scan)
• A biopsy of the bone marrow, especially if the doctor is suspecting leukemia or lymphoma.
• Skeletal investigation
• Identifying of light and heavy chains
• Checking of both normal and abnormal proteins ^{(8, 9)}

When is the best time to rule out paraproteinemia?

One of the commonly associated health conditions with paraproteinemia is multiple myeloma. It presents a wide range of symptoms. Hence, it is important that the patient should be screened for M-protein. The blood and urine sample of the patient should be checked along with serum protein electrophoresis.

By doing so, paraproteinemia will be ruled out at an early stage, which will increase the possibility of healing. The key is to detect the condition the earliest time possible. ^{(4, 7, 10)}

Paraproteinemia Treatment

The treatment for paraproteinemia depends on the clinical manifestations and associated disorders. The treatment focuses on the acute management and supportive care. ⁽²⁾

Improve the condition of the bones

To improve the condition of the bones of patients with paraproteinemia, the doctor prescribes calcium, vitamin D, and bisphosphonates. ⁽³⁾

To prevent neuropathy

The patient should take analgesics and narcotics to alleviate the pain and relieve discomfort. ⁽⁵⁾

Cancer management

The patient should receive a combination of radiation, chemotherapy, biologic therapy, and autologous transplant when deemed necessary.

Drugs of choice for patients with paraproteinemia

• Antibiotics
• Antivirals
• Pain medications
• Corticosteroids
• Immune-modulatory drugs
• Proteasome inhibitors
• Anti-inflammatory drugs ^{(4, 6, 8)}

Incidence of paraproteinemia

Paraproteinemia is high in people aged 50 years old and above. Men are more susceptible than women. The incident is higher in African Americans than the white population. ⁽⁷⁾

Prognosis

The prognosis primarily depends on the general health condition of the patient. Usually, the prognosis for paraproteinemia is poor. In patients suffering from benign monoclonal gammopathy, the patients do not have any symptoms at all. As a matter of fact, there is no evidence of impairment of antibody response of malfunctioning of the bone marrow.

Paraproteinemia is a medical condition wherein there is a high level of paraprotein in the blood. It is commonly associated with immunoproliferative disease specifically cancer of the bone marrow. Various blood works and diagnostic procedures are done to correctly and accurately diagnose the condition.

One of the reliable procedures is serum electrophoresis. As for the treatment and management approach, the focus of care is on the underlying condition. The survival rate is low, but with urgent care and religious treatment, the survival rate can be significantly improved. If cancer is the root cause of the problem, it should be treated so as to improve the overall condition of the patient.

Most of the time, the underlying conditions can be extremely difficult to treat. So, the best and only option is supportive care. ^{(3, 5, 10)}

References:

1. https://en.wikipedia.org
2. mddk.com
3. www.ncbi.nlm.nih.gov
4. https://patient.info
5. www.slideshare.net
6. www.wikilectures.eu
7. https://bmcneurol.biomedcentral.com
8. www.themmrf.org
9. www.bloodjournal.org
10. www.wisegeek.org