Some important facts related to myeloma (plasma cell Myeloma)

Pathology

v  Type  - Cancer

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v  Cause(s)     - Unknown

v  Symptoms   - Bone pain, widespread infection, kidney failure, anaemia, neurological symptoms.

v  Mortality Rate        - Inevitably fatal if untreated. Even if treated, 50% mortality rate after five years.

v  Treatments  - Chemotherapy, thalidomide, steroids, stem cell transplant

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What is multiple myeloma?

Multiple myeloma, also known as plasma cell myeloma, is a cancer of plasma cells, a type of white blood cell normally responsible for producing antibodies. Often, no symptoms are noticed initially. When advanced, bone pain, bleeding, frequent infections, and anemia may occur. Complications may include amyloidosis.

You may not require treatment until symptoms develop, and most people respond well to treatments that include:

•        Chemotherapy

•        Radiation

•        Blood treatment called plasmapheresis.

In some cases, a bone marrow or stem cell transplant is an option.

Multiple myeloma is not considered “curable,” but symptoms wax and wane. There can be a long period of dormancy that could last several years. However, this cancer usually recurs.

There are several types of myeloma, but multiple myeloma accounts for 90 percent of cases, according to the Leukemia and Lymphoma Society. The Surveillance, Epidemiology, and End Results Program (SEER) list myeloma as the 14th most common type of cancer.

The cause is generally unknown. Risk factors include drinking alcohol and obesity. The underlying mechanism involves abnormal plasma cells producing abnormal antibodies which can cause kidney problems and overly thick blood. The plasma cells can also form a mass in the bone marrow or soft tissue. When only one mass is present, it is known as a plasmacytomawhile more than one is known as multiple myeloma. Multiple myeloma is diagnosed based on blood or urine tests finding abnormal antibodies, bone marrow biopsy finding cancerous plasma cells, and medical imaging finding bone lesions. Another common finding is high blood calcium levels.

Multiple myeloma is considered treatable, but generally incurable. Remissions may be brought about with steroids, chemotherapy, thalidomide or lenalidomide, and stem cell transplant. Bisphosphonates and radiation therapy are sometimes used to reduce pain from bone lesions.

Globally, multiple myeloma affected 488,000 people and resulted in 101,100 deaths in 2015. In the United States, it develops in 6.5 per 100,000 people per year and 0.7% of people are affected at some point in their lives. It usually occurs around the age of 61 and is more common in men than women. Without treatment, typical survival is seven months. With current treatments, survival is usually 4–5 years. This gives a five-year survival rate around 49%.The word myeloma is from the Greek myelo- meaning "marrow" and -oma meaning "tumour

Signs and symptoms.-

 Because many organs can be affected by myeloma, the symptoms and signs vary greatly. A mnemonic sometimes used to remember some of the common symptoms of multiple myeloma are CRAB:

C = calcium (elevated),

R = renal failure,

A = anaemia

B = bone lesions.

Myeloma has many other possible symptoms, including opportunistic infections (e.g., pneumonia) and weight loss. CRAB symptoms and proliferation of monoclonal plasma cells in the bone marrow are part of the diagnostic criteria of multiple myeloma.

Treatment

Treatment for multiple myeloma is focused on therapies that decrease the clonal plasma cell population and consequently decrease the signs and symptoms of disease. If the disease is completely asymptomatic (i.e. there is a paraprotein and an abnormal bone marrow population but no end-organ damage), as in asymptomatic ("smoldering") myeloma, treatment is typically deferred, or restricted to clinical trials.

In addition to direct treatment of the plasma cell proliferation, bisphosphonates (e.g., pamidronate or zoledronic acid) are routinely administered to prevent fractures; they have also been observed to have a direct anti-tumor effect even in patients without known skeletal disease. If needed, red blood cell transfusions or erythropoietin can be used for management of Anaemia.

Prognosis-

Durie-Salmon staging system

First published in 1975, the Durie-Salmon staging system is still in use. However, one of the limitations of the Durie-Salmon staging system is the subjectivity in determining the extent of bone disease.

•        Stage I: all of

•        Hb > 10g/dL

•        normal calcium

•        Skeletal survey: normal or single plasmacytoma or osteoporosis

•        Serum paraprotein level < 5 g/dL if IgG, < 3 g/dL if IgA

•        Urinary light chain excretion < 4 g/24h

•        Stage II: fulfilling the criteria of neither I nor III

•        Stage III: one or more of

•        Hb < 8.5g/dL

•        high calcium > 12 mg/dL

•        Skeletal survey: Three or more lytic bone lesions

•        Serum paraprotein > 7g/dL if IgG, > 5 g/dL if IgA

•        Urinary light chain excretion > 12g/24h

Stages I, II, and III of the Durie-Salmon staging system can be divided into A or B depending on serum creatinine:

•        A: serum creatinine < 2 mg/dL (< 177 μmol/L)

•        B: serum creatinine > 2 mg/dL (> 177 μmol/L)

Prevention

The risk of multiple myeloma can be reduced by maintaining a normal body weight

Diagnosis

Myeloma

There are two main systems used to stage multiple myeloma:

•        International Staging System (ISS)

•        Durie-Salmon system

Stage 1

In this stage you have a relatively small number of myeloma cells in your blood and urine. Your hemoglobin levels are only slightly below normal, and bone X-rays may look normal or show only one affected area.

Stage 2

In this stage a moderate number of myeloma cells are present. Hemoglobin levels are usually much lower than normal. Monoclonal immunoglobulin may be increased, and blood calcium levels may also be high. X-rays may show several areas of bone damage.

Stage 3

In the final stage of multiple myeloma, a high number of myeloma cells are found. Your hemoglobin level is also usually below 8.5 grams per deciliter, and calcium blood levels are high. And there are multiple areas of bone destruction caused by the cancer.

Survival rates

Survival rates are based on comparing people with myeloma to their peers who don’t have cancer. According to the American Cancer Society (ACS), these are the average survival rates by stage:

Stage 1: 62 months

Stage 2: 44 months

Stage 3: 29 months

It’s important to note that survival rates are calculated from the time treatment begins, and the average is the median survival rate. This means that half of the people with multiple myeloma lived longer than the average length for each stage.