Multiple myeloma overview: Difference between revisions
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'''Multiple myeloma''' is a type of [[cancer]] of [[plasma cell]]s which are [[immune system]] cells in bone marrow that produce [[antibody|antibodies]]. Its [[prognosis]], despite therapy, is generally poor, and treatment may involve [[chemotherapy]] and [[stem cell transplant]]. It is part of the broad group of diseases called [[Hematological malignancy|hematological malignancies]]. | '''Multiple myeloma''' is a type of [[cancer]] of [[plasma cell]]s which are [[immune system]] cells in bone marrow that produce [[antibody|antibodies]]. Its [[prognosis]], despite therapy, is generally poor, and treatment may involve [[chemotherapy]] and [[stem cell transplant]]. It is part of the broad group of diseases called [[Hematological malignancy|hematological malignancies]]. | ||
==Pathophysiology== | |||
It is a disorder affecting post [[germinal center]] [[B lymphocytes]]. A chromosomal [[translocation]] between the [[Heavy-chain immunoglobulin|immunoglobulin heavy chain]] gene and one of many [[oncogenes]] leads to dysregulated multiplication of plasma cells. This leads to both localised damage, resulting in punched out bony lesions as well as various organ damage and other symptoms. | It is a disorder affecting post [[germinal center]] [[B lymphocytes]]. A chromosomal [[translocation]] between the [[Heavy-chain immunoglobulin|immunoglobulin heavy chain]] gene and one of many [[oncogenes]] leads to dysregulated multiplication of plasma cells. This leads to both localised damage, resulting in punched out bony lesions as well as various organ damage and other symptoms. | ||
== | ==Causes== | ||
The exact | The exact cause for multiple myeloma is not known, but several risk factors are implicated in the same. | ||
== | ==Differentiating Multiple Myeloma from other Diseases== | ||
It needs to be differentiated from other [[plasma cell]] disorders such as [[Waldenström's macroglobulinemia|Waldenstrom's hypergammaglobulinema]] and [[MGUS|monoclonal gammopathies of uncertain origin]] as well as lytic bony lesions. | It needs to be differentiated from other [[plasma cell]] disorders such as [[Waldenström's macroglobulinemia|Waldenstrom's hypergammaglobulinema]] and [[MGUS|monoclonal gammopathies of uncertain origin]] as well as lytic bony lesions. | ||
==Epidemiology and Demographics== | |||
It is the second most common blood cancer after [[non-Hodgkin's lymphoma]]. It is typically a disease of old age,however recent trends indicate earlier involvement as well and is slightly more prevalent in men than women. The average survival is about 3 years. About 43.5% survive after 5 years as per a census conducted from 2003 to 2008. There are an estimated 77,617 people currently living with myeloma in the United States.<ref>{{Cite web | last = | first = | title = Myeloma - SEER Stat Fact Sheets | url = http://seer.cancer.gov/statfacts/html/mulmy.html | publisher = | date = | accessdate = 17 February 2014 }}</ref> | It is the second most common blood cancer after [[non-Hodgkin's lymphoma]]. It is typically a disease of old age,however recent trends indicate earlier involvement as well and is slightly more prevalent in men than women. The average survival is about 3 years. About 43.5% survive after 5 years as per a census conducted from 2003 to 2008. There are an estimated 77,617 people currently living with myeloma in the United States.<ref>{{Cite web | last = | first = | title = Myeloma - SEER Stat Fact Sheets | url = http://seer.cancer.gov/statfacts/html/mulmy.html | publisher = | date = | accessdate = 17 February 2014 }}</ref> | ||
===Natural History | ==Risk Factors== | ||
The exact etiology behind multiple myeloma is not known but a number of risk factors are implicated, most commonly [[carcinogenic]] chemicals. | |||
==Natural History, Complications and Prognosis== | |||
A number of organ systems are commonly involved, the most common symptoms being [[bone pain]] usually involving the [[spine]] and the [[ribs]]. These frequently lead to pathological fractures and resultant [[hypercalcemia]]. Also common are infections such as [[pneumonia|pneumonias]] and [[pyelonephritis]]. [[Renal failure]] from [[hypercalcemia]] and [[Bence Jones protein]] filtration, [[anemia]] from suppressed bone marrow production and neurological symptoms from brain [[metastasis]] are also prominent. | A number of organ systems are commonly involved, the most common symptoms being [[bone pain]] usually involving the [[spine]] and the [[ribs]]. These frequently lead to pathological fractures and resultant [[hypercalcemia]]. Also common are infections such as [[pneumonia|pneumonias]] and [[pyelonephritis]]. [[Renal failure]] from [[hypercalcemia]] and [[Bence Jones protein]] filtration, [[anemia]] from suppressed bone marrow production and neurological symptoms from brain [[metastasis]] are also prominent. | ||
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Complications associated with [[multiple myeloma]] include renal failure, hematologic complications, infections, bony and neurological complications. | Complications associated with [[multiple myeloma]] include renal failure, hematologic complications, infections, bony and neurological complications. | ||
===Staging | ==Diagnosis== | ||
==Staging== | |||
International Staging System for myeloma, based on [[beta-2-microglobulin|β2-microglobulin]] (β2M) levels and [[serum albumin]] levels, was published by International myeloma working group in 2003 and is the most widely used staging system. It is used for both guiding treatment as well as predicting prognosis.<ref name="ISS">Greipp PR, San Miguel J, Fonseca R, Avet-Loiseau H, Jacobson JL, Rasmussen E, Crowley J, Durie BMG. Development of an international prognostic index (IPI) for myeloma: report of the international myeloma working group. ''Hematology Journal'' 2003;4:S42. NLM ID 100965523.</ref> | International Staging System for myeloma, based on [[beta-2-microglobulin|β2-microglobulin]] (β2M) levels and [[serum albumin]] levels, was published by International myeloma working group in 2003 and is the most widely used staging system. It is used for both guiding treatment as well as predicting prognosis.<ref name="ISS">Greipp PR, San Miguel J, Fonseca R, Avet-Loiseau H, Jacobson JL, Rasmussen E, Crowley J, Durie BMG. Development of an international prognostic index (IPI) for myeloma: report of the international myeloma working group. ''Hematology Journal'' 2003;4:S42. NLM ID 100965523.</ref> | ||
===Symptoms | ===Laboratory Findings=== | ||
The most frequently advised tests are [[complete blood count]] (CBC), [[erythrocyte sedimentation rate]] (ESR), [[Basic metabolic panel]], [[electrophoresis]], [[immunohistochemistry]] and imaging procedures like X ray, CT scans and MRI's. | |||
===History and Symptoms=== | |||
The sign and symptomatology associated with multiple myeloma is most commonly associated with following 4 systems: Calcium (elevated), kidneys (renal failure), blood (anemias) and cells and bones (fractures and pains). | The sign and symptomatology associated with multiple myeloma is most commonly associated with following 4 systems: Calcium (elevated), kidneys (renal failure), blood (anemias) and cells and bones (fractures and pains). | ||
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Most common signs include [[pallor]], pain without tenderness, altered sensations on neurological examination etc. | Most common signs include [[pallor]], pain without tenderness, altered sensations on neurological examination etc. | ||
=== | ===Physical Examination=== | ||
Multiple myeloma affects not only the bone marrow, but also spreads to other organ systems. This therefore, requires a detailed assessment of various organ systems. A number of times, any of these physical findings may warrant a further evaluation, and thus leads to an incidental diagnosis of multiple myeloma. [[Pallor]], [[purpura]], signs of [[cord compression]], [[hepatosplenomegaly]], bony pain without tenderness and [[carpal tunnel syndrome]] are some of the most common physical findings, in a patient presenting with multiple myeloma. | |||
===X Ray=== | |||
Simple radiography is the current gold standard for the initial diagnosis and evaluation of relapses of multiple myeloma. Well defined punched out bony lesions and hair on end appearance are two characteristic findings. The [[long bone]]s and the spine must always be evaluated. The evaluation of other bones merit consideration based on the symptoms of the patient. | |||
===CT=== | |||
Computed Tomography scan is not the best modality for the diagnosis of multiple myeloma. Contrast CT scans lead to renal failure in multiple myeloma more frequently than otherwise. Hence, other modalities are preferred over CT scan. However, occasionally a [[CT scan]] is performed to measure the size of soft tissue [[plasmacytoma]]s among patients with multiple myeloma. | |||
===MRI=== | |||
The workup of suspected multiple myeloma includes a [[skeletal survey]].[[Magnetic resonance imaging]] (MRI) is more sensitive than simple X-ray in the detection of lytic lesions, and may supersede skeletal survey, especially when vertebral disease is suspected. | |||
===Other Imaging Findings=== | |||
[[Positron emission tomography]] (PET) scan: When doing this test, a small amount of a radioactive medium is injected into your body and absorbed by the organs or tissues. This [[radioactive]] substance gives off energy which in turn is used to produce the images. PET can provide more helpful information than either CT or MRI scans. It is useful to see if the cancer has spread to the lymph nodes and it is also useful for your doctor to locate where the cancer has spread. | |||
==Treatment== | |||
=== | ===Medical Therapy== | ||
The treatment of multiple myeloma is based on differentiating suitability for transplantation or not. Those who are candidates for transplantation are treated with a combination of [[dexamethasone]] and one of the following [[lenalidomide]]/[[bortezomib]]. [[Alkylating antineoplastic agents|Alkylating agents]] are usually avoided in such candidates. Several other agents are also used these include [[thalidomide]], [[prednisone]], [[melphalan]] etc. | The treatment of multiple myeloma is based on differentiating suitability for transplantation or not. Those who are candidates for transplantation are treated with a combination of [[dexamethasone]] and one of the following [[lenalidomide]]/[[bortezomib]]. [[Alkylating antineoplastic agents|Alkylating agents]] are usually avoided in such candidates. Several other agents are also used these include [[thalidomide]], [[prednisone]], [[melphalan]] etc. | ||
===Surgery=== | |||
Surgery is not a main treatment for multiple myeloma. Emergency surgery may be needed to help support weight-bearing bones in the spine or legs to prevent bones from breaking. | |||
==References== | ==References== | ||
Revision as of 01:19, 18 February 2014
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Multiple myeloma is a type of cancer of plasma cells which are immune system cells in bone marrow that produce antibodies. Its prognosis, despite therapy, is generally poor, and treatment may involve chemotherapy and stem cell transplant. It is part of the broad group of diseases called hematological malignancies.
Pathophysiology
It is a disorder affecting post germinal center B lymphocytes. A chromosomal translocation between the immunoglobulin heavy chain gene and one of many oncogenes leads to dysregulated multiplication of plasma cells. This leads to both localised damage, resulting in punched out bony lesions as well as various organ damage and other symptoms.
Causes
The exact cause for multiple myeloma is not known, but several risk factors are implicated in the same.
Differentiating Multiple Myeloma from other Diseases
It needs to be differentiated from other plasma cell disorders such as Waldenstrom's hypergammaglobulinema and monoclonal gammopathies of uncertain origin as well as lytic bony lesions.
Epidemiology and Demographics
It is the second most common blood cancer after non-Hodgkin's lymphoma. It is typically a disease of old age,however recent trends indicate earlier involvement as well and is slightly more prevalent in men than women. The average survival is about 3 years. About 43.5% survive after 5 years as per a census conducted from 2003 to 2008. There are an estimated 77,617 people currently living with myeloma in the United States.[1]
Risk Factors
The exact etiology behind multiple myeloma is not known but a number of risk factors are implicated, most commonly carcinogenic chemicals.
Natural History, Complications and Prognosis
A number of organ systems are commonly involved, the most common symptoms being bone pain usually involving the spine and the ribs. These frequently lead to pathological fractures and resultant hypercalcemia. Also common are infections such as pneumonias and pyelonephritis. Renal failure from hypercalcemia and Bence Jones protein filtration, anemia from suppressed bone marrow production and neurological symptoms from brain metastasis are also prominent.
According to a report published by National Cancer Institute there is a 43.25 chance of 5 year survival.[2]
Complications associated with multiple myeloma include renal failure, hematologic complications, infections, bony and neurological complications.
Diagnosis
Staging
International Staging System for myeloma, based on β2-microglobulin (β2M) levels and serum albumin levels, was published by International myeloma working group in 2003 and is the most widely used staging system. It is used for both guiding treatment as well as predicting prognosis.[3]
Laboratory Findings
The most frequently advised tests are complete blood count (CBC), erythrocyte sedimentation rate (ESR), Basic metabolic panel, electrophoresis, immunohistochemistry and imaging procedures like X ray, CT scans and MRI's.
History and Symptoms
The sign and symptomatology associated with multiple myeloma is most commonly associated with following 4 systems: Calcium (elevated), kidneys (renal failure), blood (anemias) and cells and bones (fractures and pains).
Some of the most common symptoms include weakness, bone pains, back pain, numbness and paresthesia, bleeding and recurrent infections.
Most common signs include pallor, pain without tenderness, altered sensations on neurological examination etc.
Physical Examination
Multiple myeloma affects not only the bone marrow, but also spreads to other organ systems. This therefore, requires a detailed assessment of various organ systems. A number of times, any of these physical findings may warrant a further evaluation, and thus leads to an incidental diagnosis of multiple myeloma. Pallor, purpura, signs of cord compression, hepatosplenomegaly, bony pain without tenderness and carpal tunnel syndrome are some of the most common physical findings, in a patient presenting with multiple myeloma.
X Ray
Simple radiography is the current gold standard for the initial diagnosis and evaluation of relapses of multiple myeloma. Well defined punched out bony lesions and hair on end appearance are two characteristic findings. The long bones and the spine must always be evaluated. The evaluation of other bones merit consideration based on the symptoms of the patient.
CT
Computed Tomography scan is not the best modality for the diagnosis of multiple myeloma. Contrast CT scans lead to renal failure in multiple myeloma more frequently than otherwise. Hence, other modalities are preferred over CT scan. However, occasionally a CT scan is performed to measure the size of soft tissue plasmacytomas among patients with multiple myeloma.
MRI
The workup of suspected multiple myeloma includes a skeletal survey.Magnetic resonance imaging (MRI) is more sensitive than simple X-ray in the detection of lytic lesions, and may supersede skeletal survey, especially when vertebral disease is suspected.
Other Imaging Findings
Positron emission tomography (PET) scan: When doing this test, a small amount of a radioactive medium is injected into your body and absorbed by the organs or tissues. This radioactive substance gives off energy which in turn is used to produce the images. PET can provide more helpful information than either CT or MRI scans. It is useful to see if the cancer has spread to the lymph nodes and it is also useful for your doctor to locate where the cancer has spread.
Treatment
=Medical Therapy
The treatment of multiple myeloma is based on differentiating suitability for transplantation or not. Those who are candidates for transplantation are treated with a combination of dexamethasone and one of the following lenalidomide/bortezomib. Alkylating agents are usually avoided in such candidates. Several other agents are also used these include thalidomide, prednisone, melphalan etc.
Surgery
Surgery is not a main treatment for multiple myeloma. Emergency surgery may be needed to help support weight-bearing bones in the spine or legs to prevent bones from breaking.
References
- ↑ "Myeloma - SEER Stat Fact Sheets". Retrieved 17 February 2014.
- ↑ "Myeloma - SEER Stat Fact Sheets". Retrieved 17 February 2014.
- ↑ Greipp PR, San Miguel J, Fonseca R, Avet-Loiseau H, Jacobson JL, Rasmussen E, Crowley J, Durie BMG. Development of an international prognostic index (IPI) for myeloma: report of the international myeloma working group. Hematology Journal 2003;4:S42. NLM ID 100965523.