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November 28, 2016 00:10

Multiple Myeloma |Symptoms and treatment of multiple myeloma

Multiple myeloma (mielomatoz, plasma cell myeloma) is a plasma cell tumor that produces monoclonal immunoglobulin, which is being implemented and destroys surrounding bone.

most frequent manifestations of the disease are bone pain, renal failure, hypercalcemia, anemia, recurrent infections.For the diagnosis requires a M protein (often available in urine and serum is absent), destructive changes in the bones, determination of light chains in the urine, increased content of plasma cells in the bone marrow.It usually takes a bone marrow biopsy.Specific therapy includes standard chemotherapy (usually with alkylating agents, corticosteroids, anthracyclines, thalidomide), and high-melphalan with autologous peripheral blood stem cells.

incidence of multiple myeloma is from 2 to 4 per 100 000 population.Male to female ratio is 6: 1, most patients older than 40 years.The incidence of blacks in 2 times more than whites.The etiology is unknown, although some role chromosomal and genetic factors, radiation, ch

emical compounds.

Pathophysiology mnozhestvennnoy

myeloma plasma cell tumor (plasmacytoma) produce IgG in about 55% IgA and about 20% of patients with multiple myeloma.In 40% of these patients revealed proteinuria Bence-Jones, who is the availability of monoclonal or to X light chains in the urine.In 15-20% of patients with plasma cells secrete only Bence-Jones protein.These patients have a higher incidence osteodestruktsii, hypercalcemia, renal failure and amyloidosis, as compared to other patients who have myeloma.IgD myeloma occurs in about 1% of cases.

Diffuse osteoporosis or local bone destruction often develop in the pelvis, spine, ribs, and skull.Damage caused by the replacement bone spreading plasmacytoma or osteoclast activation by cytokines, which are secreted by malignant plasma cells.As a rule, osteolytic lesions are multiple, but sometimes found solitary intraosseous tumor.Vnekostnye plasmacytoma are rare, but can be detected in all tissues, especially the tissues of the upper respiratory tract.

Usually there hypercalcemia and anemia.Often there is renal insufficiency (myeloma kidney), which is caused by the filling of the renal tubules protein mass, atrophy of tubular epithelial cells and the development of interstitial fibrosis.

Increased sensitivity to bacterial infections due to a decrease of normal immunoglobulin production and other factors.Secondary amyloidosis occurs in 10% of patients with myeloma, the most common in patients with Bence Jones proteinuria.

Symptoms mnozhestvennnoy myeloma

resistant bone pain (especially in the spine and thorax), renal failure, recurrent bacterial infections are the most typical manifestations of multiple myeloma.Often there are pathological fractures.Destruction of the vertebral body can lead to spinal cord compression and paraplegia.The predominant symptom is often anemia, which can serve as the sole reason for the examination of the patient, some patients have symptoms of hyperviscosity syndrome (see. Below).Often there are peripheral neuropathy, carpal tunnel syndrome, abnormal bleeding, hypercalcemia symptoms (eg, polyuria, polydipsia).Lymphadenopathy and hepatosplenomegaly are not typical for patients with multiple myeloma.

Diagnostics mnozhestvennnoy myeloma

Multiple myeloma is suspected in patients older than 40 years with the presence of unexplained bone pain (especially at night or during a holiday), other typical symptoms, or the presence of laboratory abnormalities such as elevated protein levels in the blood and urine, hypercalcemia,renal failure or anemia.The survey consists of a standard definition of parameters of blood, protein electrophoresis, X-ray examination and bone marrow research.

options manifestations of multiple myeloma

Form

Feature

extramedullary plasmacytoma

plasmacytoma found outside the bone marrow

Solitary bone plasmacytoma

Singlebone lesions plasmacytoma, which usually produces the M-protein

Osteoskleroticheskaya myeloma (POEMS syndrome)

polyneuropathy (chronic inflammatory polyneuropathy), organomegaly (hepatomegaly, splenomegaly, lymphadenopathy), endocrinopathy (eg, gynecomastia, testicular atrophy), M protein, skin changes (such as hyperpigmentation, increased hair growth)

non-secretory myeloma

absence of M-protein in serum and urine, the presence of M-protein in the plasma cells

standard blood counts include complete blood count, erythrocyte sedimentation rate and blood chemistry.Anemia is present in 80% of patients, usually normocytic-normochromic to form a plurality of agglutinates comprising usually 3 to 12 erythrocytes.

number of white blood cells and platelets are usually normal.Often there is an increase urea, serum creatinine and uric acid, erythrocyte sedimentation rate may be greater than 100 mm / h.Anion gap is sometimes low.Hypercalcemia is present at diagnosis in 10% of patients.

Produced electrophoresis of serum proteins in the absence of a specific protein electrophoresis results carried out 24-hour urine concentrate.In 80-90% of cases by electrophoresis of serum proteins is determined by M-protein.The remaining 10-20% of the patients are usually defined by monoclonal free light chains (Bence Jones protein) or IgD.In these patients, almost always determined by M-protein in urine protein electrophoresis.Immunofixation electrophoresis to identify protein-immunoglobulin M class and often determines the light chain protein if immunoelectrophoresis of serum proteins was false negative.Electrophoresis with immunofixation recommended in cases where the serum protein electrophoresis was negative when there are substantial grounds for suspicion of myeloma.

radiographic examination includes scenic images of bones.In 80% of cases there is diffuse osteoporosis or lytic bone changes rounded shape.Radionuclide bone scan is not usually informative.MRI can provide more detailed picture and recommended in the presence of pain and neurological symptoms and the absence of data on plain radiography.

also perform aspiration and bone marrow biopsy, in which revealed diffuse distribution or accumulation of plasma cells, indicating the presence of bone marrow cancer.Bone marrow involvement is usually uneven and often determined by an increased number of plasma cells with varying degrees of maturation.Sometimes the number of plasma cells is normal.Morphology plasma cells does not depend on the class of immunoglobulin synthesized.

in patients with M protein in the serum, it is reasonable to assume the presence of myeloma at the level of proteinuria Bence-Jones more than 300 mg / 24 h, osteolytic lesions (without evidence of metastatic cancer or granulomatous disease), and the presence of high content of plasma cells in the bonebrain.

Treatment mnozhestvennnoy

myeloma patients in need of serious maintenance treatment.Outpatient maintenance therapy helps preserve bone density.Analgesics and palliative doses of radiotherapy (18-24 Gy) can relieve pain in the bones.However, radiation therapy may impede the course of chemotherapy.All patients must also receive bisphosphonates, which reduce the risk of developing skeletal complications, alleviate pain in the bones and have antitumor activity.

Adequate hydration is the prevention of kidney damage.Even patients with prolonged, massive Bence Jones proteinuria (10-30 g / day) can retain kidney function if they maintain urine output of more than 2000 ml / day.Patients with proteinuria Bence Jones dehydration that accompanies the introduction of vysokoosmolyarnogo intravenous contrast, can cause acute renal failure.

used to treat hypercalcemia abundant hydration and bisphosphonates, sometimes in conjunction with prednisone 60-80 mg orally per day.Although most patients do not require admission allopurinol, receiving 300 mg per day is shown in the presence of renal failure or the symptoms of hyperuricemia.

shown conducting preventive vaccination against pneumococcal disease and influenza.Antibiotics produced with documented bacterial infection and is not recommended routine prophylactic antibiotics.The prophylactic administration of intravenous immunoglobulin may reduce the risk of infectious complications, it is usually prescribed to patients with frequent recurrent infections.

recombinant erythropoietin (40,000 units subcutaneously three times per week) used in patients with anemia, chemotherapy is not correctable.If anemia leads to violations on the part of the cardiovascular system, use the transfusion of red cells.With the development of hyperviscosity syndrome plasmapheresis is performed.Chemotherapy shown to reduce serum or urine M-protein.Posttsitostaticheskaya neutropenia may contribute to the development of infectious complications.

standard chemotherapy usually consists of courses of oral melphalan [0,15mg / (kg x d) inwardly] and prednisolone (20 mg three times a day) every 6 weeks response estimate 3-6 months.Chemotherapy may be effected using different modes with intravenous drugs.These modes do not improve the long term survival as compared with the combination of melphalan and prednisone, but may provide more rapid response in patients with renal dysfunction.Conducting autologous hematopoietic stem cell transplantation is indicated in patients younger than 70 years with adequate function of the heart, liver, lung and kidney with stable disease or a good response after several courses of standard chemotherapy.These patients performed the initial chemotherapy with vincristine, doxorubicin and dexamethasone or dexamethasone with thalidomide.The appointment of myeloid growth factor drugs that suppress bone marrow function, alkylating agents, nitrosourea drugs are not assigned.Implementation of non-myeloablative allogeneic transplantation conditioning modes (eg, low dose tsiklofosfomida and fludarabine or radiation therapy), some patients can improve disease-free survival to 5-10 years due to the reduction of toxicity and the availability of allogeneic immune anti-myeloma effect.This method is indicated for patients younger than 55 years with good physiological reserve.In relapsed or refractory myeloma apply new drugs (thalidomide, immunomodulatory drugs, proteasome inhibitors), the effectiveness of these drugs as a therapy 1st line study.

Supportive therapy is nehimioterapevticheskimi drugs, including interferon, which provide long-term effect, but have some side effects.The use of corticosteroids as maintenance therapy is being studied.

prognosis of multiple myeloma

Multiple myeloma is continuously progressing, the median survival with standard chemotherapy is about 3-4 years, with high-dose chemotherapy with stem cell transplantation - about 4-5 years.The treatment improves the quality and duration of life in 60% of patients.Poor prognostic signs at diagnosis are high levels of M-protein in the blood serum or urine, increased serum beta2-microglobulin (& gt; 6 ug / ml), Bone diffuse damage, hypercalcemia, renal insufficiency and anemia.