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Multiple myeloma

Updated: Wikipedia source

Multiple myeloma

Multiple myeloma (MM), also known as plasma cell myeloma and simply myeloma, is a cancer of plasma cells, a type of white blood cell that normally produces antibodies. Often, no symptoms are noticed initially. As it progresses, bone pain, anemia, renal insufficiency, and infections may occur. Complications may include hypercalcemia and amyloidosis. The cause of multiple myeloma is unknown. Risk factors include obesity, radiation exposure, family history, age and certain chemicals. There is an increased risk of multiple myeloma in certain occupations. This is due to the occupational exposure to aromatic hydrocarbon solvents having a role in causation of multiple myeloma. Multiple myeloma is the result of a multi-step malignant transformation, and almost universally originates from the pre-malignant stage monoclonal gammopathy of undetermined significance (MGUS). As MGUS evolves into MM, another pre-stage of the disease is reached, known as smoldering myeloma (SMM). In MM, the abnormal plasma cells produce 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 one tumor is present, it is called a plasmacytoma; more than one is called multiple myeloma. Multiple myeloma is diagnosed based on blood or urine tests finding abnormal antibody proteins (often using electrophoretic techniques revealing the presence of a monoclonal spike in the results, termed an m-spike), 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, targeted therapy, and stem cell transplant. Bisphosphonates and radiation therapy are sometimes used to reduce pain from bone lesions. New approaches utilizing CAR-T cell therapy have been included in the treatment regimens. Globally, about 175,000 people were diagnosed with the disease in 2020, while about 117,000 people died from the disease that year. In the U.S., forecasts suggest about 35,000 people will be diagnosed with the disease in 2023, and about 12,000 people will die from the disease that year. In 2020, an estimated 170,405 people were living with myeloma in the U.S. It is difficult to judge mortality statistics because treatments for the disease are advancing rapidly. Based on data concerning people diagnosed with the disease between 2013 and 2019, about 60% lived five years or more post-diagnosis, with about 34% living ten years or more. People newly diagnosed with the disease now have a better outlook, due to improved treatments. The disease usually occurs around the age of 60 and is more common in men than women. It is uncommon before the age of 40. The word myeloma is from Greek myelo- 'marrow' and -oma 'tumor'.

Infobox

Other names
Plasma cell myeloma, myelomatosis, Kahler's disease, myeloma
Specialty
Hematology and oncology
Symptoms
Bone pain, fatigue: 653
Complications
Amyloidosis, kidney problems, bone fractures, hyperviscosity syndrome, infections, anemia: 653
Usual onset
Around 60
Duration
Long term
Causes
Unknown
Risk factors
Obesity
Diagnostic method
Blood or urine tests, bone marrow biopsy, medical imaging
Treatment
Steroids, chemotherapy, thalidomide, stem cell transplant, bisphosphonates, radiation therapy
Prognosis
Five-year survival rate 54% / life expectancy 6 years (USA)
Frequency
488,200 (affected during 2015)
Deaths
101,100 (2015)

Tables

· Prognosis › Genetic testing
Deletion/isolated monosomy 13
Deletion/isolated monosomy 13
Genetic abnormality
Deletion/isolated monosomy 13
Gene(s)
RB1, DIS3
Incidence among myelomas
45–50%
Prognostic impact
Effect on prognosis is unclear
Trisomies
Trisomies
Genetic abnormality
Trisomies
Incidence among myelomas
40–50%
Prognostic impact
Median overall survival: 7–10 years
1q21 gain, as an addition to another abnormality
1q21 gain, as an addition to another abnormality
Genetic abnormality
1q21 gain, as an addition to another abnormality
Gene(s)
CKS1B, ANP32E
Incidence among myelomas
35–40%
Prognostic impact
Median overall survival: 5 years
t(11;14)(q13;q32)
t(11;14)(q13;q32)
Genetic abnormality
t(11;14)(q13;q32)
Gene(s)
IgH and CCND1
Incidence among myelomas
15–20%
Prognostic impact
Median overall survival: 7–10 years
Trisomies plus any one IgH translocation
Trisomies plus any one IgH translocation
Genetic abnormality
Trisomies plus any one IgH translocation
Incidence among myelomas
15%
Prognostic impact
May neutralize high risk IgH and del 17p translocations
Hypodiploidy
Hypodiploidy
Genetic abnormality
Hypodiploidy
Incidence among myelomas
13–20%
Prognostic impact
Unfavorable prognosis, high risk of progression
t(4:14)(p16;q32)
t(4:14)(p16;q32)
Genetic abnormality
t(4:14)(p16;q32)
Gene(s)
IgH and FGFR3/MMSET
Incidence among myelomas
10–15%
Prognostic impact
Median overall survival: 5 years
17p deletion, as an addition to another abnormality
17p deletion, as an addition to another abnormality
Genetic abnormality
17p deletion, as an addition to another abnormality
Gene(s)
TP53
Incidence among myelomas
10%
Prognostic impact
Median overall survival: 5 years
t(14;16)
t(14;16)
Genetic abnormality
t(14;16)
Gene(s)
IgH and C-MAF
Incidence among myelomas
2–5%
Prognostic impact
Median overall survival: 5 years
t(6;14)(p21;q32)
t(6;14)(p21;q32)
Genetic abnormality
t(6;14)(p21;q32)
Gene(s)
IgH and CCND3
Incidence among myelomas
2%
Prognostic impact
Median overall survival: 7–10 years
t(14;20)(q32;q12)
t(14;20)(q32;q12)
Genetic abnormality
t(14;20)(q32;q12)
Gene(s)
MAFB
Incidence among myelomas
1%
Prognostic impact
Median overall survival: 5 years
Genetic abnormality
Gene(s)
Incidence among myelomas
Prognostic impact
Deletion/isolated monosomy 13
RB1, DIS3
45–50%
Effect on prognosis is unclear
Trisomies
40–50%
Median overall survival: 7–10 years
1q21 gain, as an addition to another abnormality
CKS1B, ANP32E
35–40%
Median overall survival: 5 years
t(11;14)(q13;q32)
IgH and CCND1
15–20%
Median overall survival: 7–10 years
Trisomies plus any one IgH translocation
15%
May neutralize high risk IgH and del 17p translocations
Hypodiploidy
13–20%
Unfavorable prognosis, high risk of progression
t(4:14)(p16;q32)
IgH and FGFR3/MMSET
10–15%
Median overall survival: 5 years
17p deletion, as an addition to another abnormality
TP53
10%
Median overall survival: 5 years
t(14;16)
IgH and C-MAF
2–5%
Median overall survival: 5 years
t(6;14)(p21;q32)
IgH and CCND3
2%
Median overall survival: 7–10 years
t(14;20)(q32;q12)
MAFB
1%
Median overall survival: 5 years

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