multiple myeloma



OVERVIEW

Definition.

Multiple myeloma is a malignant disease with abnormal proliferation of clonal plasma cells.

It is the second most common tumor of the hematologic system, accounting for 1% of all malignancies and 10% of hematologic tumors.

Disease Type

Clinical typing

Multiple myeloma is classified according to the presence or absence of characteristic clinical manifestations and the need for treatment:

Symptomatic (active) myeloma.

Asymptomatic (smoldering) myeloma.

M protein typing

According to immunoglobulin typing, it can be divided into IgG type, IgA type, IgD type, IgM type, IgE type, light chain type, biclonal type, and non-secretory type, and it can be further divided into κ type and λ type according to the light chain type of the M proteins, which are 14 types in total.

IgG type

IgA type

IgD type

IgM type: rare in China, prone to hyperviscosity or Raynaud’s phenomenon.

Light chain type

IgE type: very rare.

Non-secretory type

Incidence

Incidence: China still lacks precise epidemiological data on multiple myeloma, the estimated annual incidence rate is (1.0~2.5)/100,000, and the estimated number of patients diagnosed at the first diagnosis reaches 27,000 cases per year.

Prevalence: Multiple myeloma is mainly found in the elderly, half of the patients are over 65 years old at the time of diagnosis, 75% of the patients are over 55 years old at the time of diagnosis, and only about 2% of the patients are under 40 years old.

Causes

Causes

The exact cause of multiple myeloma is unknown and may be related to the following factors:

Radiation

Exposure to radiation has been documented to cause multiple myeloma.

After a latency period of 20 years, the incidence of multiple myeloma is significantly higher in survivors of the atomic bombings of World War II compared to the normal population.

Chemical Factors

Organic solvents such as benzene, herbicides and pesticides may be associated with the development of multiple myeloma.

Other Factors

Multiple myeloma may be associated with numerous factors such as viral infections, chronic antigenic stimuli, and genetic factors.

Pathogenesis

The exact pathogenesis of multiple myeloma is unknown, and current research has focused on theories of multiple myeloma stem cells, the bone marrow microenvironment, the two-strike model, and clonal evolution.

Pathogenesis of multiple myeloma bone disease

There is a large clonal proliferation of abnormal plasma cells (or myeloma cells) in the bone marrow.

Stimulation from malignant plasma cells and other cells in the bone marrow microenvironment causes osteoclasts to proliferate and become functionally active, resulting in extensive osteolytic bone destruction and osteoporosis.

At the site of primary osteolysis, new bone formation is reduced or absent.

Pathogenesis of multiple myeloma nephropathy

Monoclonal immunoglobulins (M proteins) and free light chains appear in the serum.

Free light chains can be filtered by the glomerulus and absorbed by the tubular epithelial cells, which have a direct toxic effect on the epithelial cells and promote apoptosis.

Excess free light chains form tubular patterns in the renal tubules, leading to tubular dilatation.

Immunoglobulin deposition in the glomerulus can lead to nephrotic syndrome.

Hypercalcemia due to osteolytic destruction, hyperuricemia due to chemotherapy, and renal infiltration of myeloma cells can lead to renal damage.

Pathogenesis of anemia in multiple myeloma

Myeloma cells invade the bone marrow and red lineage production is suppressed.

Absolute and relative deficiency of erythropoietin.

Combined autoimmune hemolytic anemia.

Hemodilution, etc.

Symptoms

Common Symptoms

Bone damage

Bone pain: bone pain is the most common symptom and often the first symptom, most common in the lumbosacral region, followed by the chest and lower limbs.

Pathological fracture: those with severe pain after activity or sprain have the possibility of pathological fracture.

Paraplegia: about 10% of patients develop paraplegia due to spinal cord compression.

Bone mass: myeloma cells infiltrate the bones and may be locally palpable as a mass.

Anemia

The vast majority of patients develop varying degrees of anemia, which often manifests as dizziness, fatigue, tinnitus, and poor concentration.

Due to the slow onset of anemia, the symptoms of anemia are mostly inconspicuous, and the anemia is usually mild in the early stage and more severe in the late stage.

Hypercalcemia

The incidence of hypercalcemia in our patients is low, and hypercalcemia often indicates disease progression.

It manifests as symptoms such as lack of appetite, vomiting, fatigue, confusion, polyuria or constipation.

It is mainly caused by extensive osteolytic changes and renal insufficiency.

Renal impairment

Patients may have clinical manifestations such as a layer of fine foam floating on the surface of urine and reddish urine.

Manifestations of chronic renal failure, such as weakness, lumbago, increased nocturia and other mild discomforts, may occur.

Renal failure is one of the major causes of death in multiple myeloma.

Accompanying symptoms

Hyperviscosity syndrome

The increase of monoclonal immunoglobulin and the formation of polymorphism can make the blood viscosity too high, causing slow blood flow, microcirculation disorder, tissue bruising and hypoxia.

The main manifestations are dizziness, vertigo, blurred vision, tinnitus, numbness of fingers, visual impairment, congestive heart failure, impaired consciousness and even coma.

Raynaud’s phenomenon may occur in some patients

Abnormal coagulation function

Nosebleed, gum bleeding and skin purpura are common. Visceral and intracranial hemorrhage is seen in severe cases.

Thrombosis: Thrombosis in the venous system is the most common, which mostly occurs in small and medium veins, and deep vein thrombosis can also occur. Embolus dislodgement can cause pulmonary infarction.

Amyloidosis.

Complexes of immunoglobulin light chains and polysaccharides are deposited in tissues and organs, causing symptoms and functional impairment of the corresponding organs, with an incidence of about 10%.

It often manifests as enlargement of the tongue and parotid gland, deepening of the texture of the skin surface, and bark-like roughness when touched. Heart, liver, spleen, lungs and kidneys can be involved, and sudden death can occur when myocardial amyloidosis is severe.

Peripheral neuropathy may occur in some cases.

Nervous system damage

Peripheral neuropathy: progressive symmetrical distal sensory-motor deficits mainly manifested as muscle weakness, tingling sensation, numbness and dullness of pain sensation.

Spinal cord compression: it is a more serious manifestation of nerve damage, mainly manifested by limited motor disorders, severe pain distributed along the innervation area of nerve roots, etc.

Infiltration symptoms

Multiple myeloma patients can be combined with extramedullary plasmacytoma at the time of diagnosis, or it can appear with the progression of the disease during the treatment of multiple myeloma, and it is more common in the liver, spleen, lymph nodes and kidneys.

Liver and spleen: usually mildly enlarged.

Lymph nodes: enlargement is less common.

Oral cavity and respiratory tract: tumor cells can invade soft tissues such as the oral cavity and respiratory tract.

Other tissues: such as the thyroid, adrenal glands, ovaries, testes, lungs, skin, pleura, pericardium, gastrointestinal tract, and central nervous system can also be involved.

Consultation

Department of Medicine

Hematology

Prompt medical attention is recommended if symptoms such as dizziness, blurred vision, pallor, bone pain, recurrent infections, fatigue, lack of appetite, lumbago, and increased nocturia occur.

Orthopedics

Prompt medical consultation is recommended if bone pain in the lower back, chest or lower limbs occurs.

Preparation for medical treatment

Preparation for medical consultation: registration, preparation of documents, FAQs

Tips for seeking medical treatment

Do not abuse drugs without confirming the diagnosis.

Preparation Checklist

Pay particular attention to the time of onset of symptoms, special manifestations, etc.

Are there symptoms such as shortness of breath, fatigue, pale lips, pale eyelids? What were the aggravating or relieving factors?

When did the bone pain begin? Where is the specific site? Is there any relationship between the pain and position or labor?

Has there been any recent fever?

Are there any symptoms such as increased nocturia or constipation?

Has there been any recent viral infection, such as a cold?

What is the occupational environment? Is there a history of exposure to ionizing radiation, chemical toxins, etc.?

Are there autoimmune diseases?

Are there any tumor patients in the family?

Test results in the past six months, which can be brought to the doctor’s office.

Blood test and blood biochemistry

X-rays, CT, Magnetic Resonance Imaging (MRI)

Medication for the last 3 months, if available, bring the box or package with you to the doctor’s office.

Bisphosphonates: clodronate, pamidronate disodium, zoledronic acid

Glucocorticoids: dexamethasone, prednisone, etc.

Others: iron, folic acid, vitamin B12

Diagnosis

Diagnosis is based on

Medical history

History of viral infection.

Family history of tumor.

Clinical manifestations

Common symptoms include bone pain, pallor, lack of appetite, malaise, lumbago, nocturia, and paraparesis.

Hepatosplenomegaly and lymph node enlargement are rare.

Extramedullary plasmacytoma can be found in about 5% of patients with initial diagnosis and 5% of patients with long-term follow-up, and the mass can be palpated on the surface of the body, which varies in size, and the diameter can be less than 1 cm or more than 10 cm.

Bone deformities, localized bone tenderness, and cutaneous purpura (dark purple patches or blotches on the skin) may be present.

Imaging

Imaging tests help to determine the presence of bone destruction, osteoporosis and fractures.

X-rays: can reveal changes such as osteolytic damage, osteoporosis and pathologic fractures. Bone damage is mostly seen in vertebrae, skull, thorax, pelvis and proximal limb bones.

CT, MRI examination: more sensitive and accurate than X-ray, and can find tiny lesions that cannot be shown by X-ray.

ECT examination: can show the whole body bone destruction.

Laboratory Tests

Blood picture

Blood M protein identification

Blood biochemical examination

Urine routine may show proteinuria, hematuria and tubular urine.

Detection of 24-hour urine light chain, urine immunofixation electrophoresis.

Protein of the week is present in the urine of about half of the patients. Protein of the week, i.e., light chains, either κ or X chains, excreted from the patient’s kidneys, has a small molecular weight and can be excreted in large quantities in the urine.

Bone marrow examination for multiple myeloma includes bone marrow smear and bone marrow biopsy, each with its own advantages and disadvantages. It is more comprehensive and reliable if performed at the same time.

Bone marrow smear

Bone marrow biopsy

Conventional chromosomal bands: Chromosomal abnormalities are detected at diagnosis in 30% to 50% of patients.

Fluorescence in situ hybridization (FISH) reveals cytogenetic abnormalities in more than 90% of MM patients.

It is now clear that some prognostically relevant chromosomal alterations such as t(4;14), 1q21 amplification, del(17p), t(14;16), t(14;20) suggest a poor prognosis, while hyperdiploidy and t(11;14) have a better prognosis.

Diagnostic criteria

The Chinese multiple myeloma diagnosis and treatment guidelines (2020 edition) comprehensively refer to the guidelines of the National Comprehensive Cancer Network (NCCN) and the International Myeloma Working Group (IMWG), and the diagnostic criteria for symptomatic (active) myeloma and asymptomatic (smoldering) myeloma are as follows.

Diagnostic Criteria for Symptomatic (Active) Multiple Myeloma

Fulfillment of 1 and 2, plus any 1 of 3.

Bone marrow monoclonal plasma cell percentage ≥10% and/or tissue biopsy evidence of plasmacytoma.

Presence of monoclonal M protein in serum and/or urine (Note 1).

Manifestation of target organ damage (CRAB) (Note 2)

No manifestations of target organ damage but abnormalities in 1 or more of the following (SLiM)

Diagnostic criteria for asymptomatic (smoldering) multiple myeloma

Need to fulfill Article 3 + Article 1 / Article 2.

Article 1: Serum monoclonal M protein ≥30 g/L and 24-hour urinary light chain ≥0.5 g.

Article 2: Bone marrow monoclonal plasma cell percentage 10% to 59%.

Article 3: No damage to related organs and tissues, i.e., no manifestation of end-organ damage such as SLiM-CRAB.

Note: For details of SLiM-CRAB, please refer to the section “Diagnostic Criteria for Symptomatic (Active) Multiple Myeloma”.

Extended Reading

Note 1: There is no restriction on the amount of blood or urine M-protein, but if no M-protein is detected (diagnosis of non-secretory MM), then myeloma monoclonal plasma cells ≥30% or plasmacytoma on biopsy are required.

Note 2: Other types of end-organ damage also occur occasionally, and the diagnosis and classification may be further supported if damage to these organs is confirmed to be associated with myeloma.

Note 3: Corrected serum calcium can be calculated in either of the following ways.

Note 4

Note 5: A value of at least ≥100 mg/L for the involved light chain is required.

Tumor staging

Staging is according to the traditional Durie-Salmon (DS) staging system and and the Revised International Staging System (R-ISS).

DS staging system for multiple myeloma

All of the following conditions are met:

Hemoglobin > 100 g/L.

Serum calcium ≤ 2.65 mmol/L (11.5 mg/dl).

Skeletal radiographs: normal bone structure or isolated plasmacytoma of bone.

Low serum or urine myeloma protein production rate

All patients who do not meet Stages I and III.

Meet 1 or more of the following conditions:

Hemoglobin <85 g/L.

Serum calcium > 2.65 mmo/L (11.5 mg/dl).

Greater than 3 osteolytic lesions on skeletal examination.

High serum or urinary myeloma protein production rate

Subtype A: Normal renal function, i.e., creatinine clearance >40 ml/min or serum creatinine level <177 μmol/L (2.0 mg/dl).

Subtype B: renal insufficiency, i.e. creatinine clearance ≤40 ml/min or serum creatinine level ≥177 μmol/L (2.0 mg/dl).

Revised International Staging System (R-ISS)

Those who fulfill both of the following conditions:

β2 microglobulin <3.5 mg/L and albumin ≥35 g/L.

Non-cytogenetic high-risk patients with concomitant normal levels of lactate dehydrogenase (LDH).

All patients not eligible for R-ISS stage I and III.

Those who fulfill both of the following conditions:

β2 microglobulin ≥ 5.5 mg/L.

Patients at high cytogenetic risk or with higher than normal levels of LDH.

Cytogenetic high risk is defined as interphase fluorescence in situ hybridization detection of del(17p), (t4;14), t(14;16).

Differential Diagnosis.

The differential diagnosis of multiple myeloma is complex and requires a comprehensive analysis by a specialized physician based on laboratory tests and other clinical data.

It is often differentiated from reactive plasmacytosis, monoclonal gammaglobulinopathy of undetermined significance (MGUS), Wahl’s macroglobulinemia (WM), and AL amyloidosis.

Treatment

Principles of treatment

Symptomatic patients should be treated systematically, and asymptomatic patients can be left untreated, but asymptomatic or non-progressing patients should be observed periodically.

Treatment of newly diagnosed multiple myeloma

Induction therapy

The patient’s age (in principle, ≤65 years), fitness, and coexisting disease status determine his or her suitability for autologous hematopoietic stem cell transplantation (HSCT).

Induction therapy should not be longer than 4 to 6 sessions in transplant candidate patients to avoid damaging HSCT cells and affecting their mobilization for collection.

Bortezomib/dexamethasone (BD).

Lenalidomide/dexamethasone (RD).

Lenalidomide/bortezomib/dexamethasone (RVD).

Bortezomib/doxorubicin/dexamethasone (PAD).

Bortezomib/cyclophosphamide/dexamethasone (BCD).

Bortezomib/thalidomide/dexamethasone (BTD).

Thalidomide/doxorubicin/dexamethasone (TAD).

Thalidomide/dexamethasone (TD).

Thalidomide/cyclophosphamide/dexamethasone (TCD).

In addition to the above regimens, the following regimens may be used:

Marfan/prednisone/bortezomib (VMP).

Marfan/prednisone/thalidomide (MPT).

Marfan/prednisone/lenalidomide (MPR).

Lenalidomide/low dose dexamethasone (Rd).

Marfan/prednisone (MP).

Hematopoietic stem cell transplantation (HSCT)

Hematopoietic stem cell transplantation is a general term used to refer to the intravenous transplantation of normal hematopoietic stem cells from various sources into the recipient’s body to replace the original pathological hematopoietic stem cells after the patient has received an excessive dose of chemotherapy (radiotherapy), thus allowing the patient’s normal hematopoietic and immune functions to be re-established.

According to donor genetics, HSCT can be classified into autologous hematopoietic stem cell transplantation (AHSCT), allogeneic HSCT and allo-HSCT.

The main approaches in the treatment of multiple myeloma are autologous HSCT and allogeneic HSCT.

Autologous hematopoietic stem cell transplantation (ASCT)

Allogeneic HSCT: Allogeneic HSCT may be considered in young patients with high-risk, relapsed refractory disease.

Autologous stem cell transplantation increases remission rates, improves overall patient survival and event-free survival, and is the standard of care for transplant-suitable patients. Transplantation after chemotherapy-induced remission is more effective.

Efficacy is independent of age and gender and is related to conventional chemotherapy sensitivity, tumor load size, and serum β2-microglobulin levels.

Allogeneic HSCT may be considered in young, high-risk patients, and in relapsed refractory patients, but transplantation-related mortality is high.

Consolidation therapy

The need for consolidation therapy after transplantation is controversial. It is recommended that re-stratification be performed after autologous HSCT, and consolidation therapy may be used in high-risk patients. Consolidation therapy is usually a previously effective regimen of 2 to 4 courses, followed by entry into maintenance therapy.

For patients who are not suitable for autologous HSCT, if the induction regimen is effective, it is recommended that the effective regimen be continued to maximum efficacy, followed by a maintenance phase of treatment.

Maintenance therapy

Maintenance therapy may include lenalidomide, bortezomib, isazomib, and thalidomide.

Maintenance therapy with a proteasome inhibitor-containing regimen for 2 years or more is advocated for patients with high-risk factors.

Combination of the two agents is recommended for high-risk patients; thalidomide should not be used alone.

Treatment of relapsed multiple myeloma

First relapse

The goal of treatment is to achieve maximum remission and prolong the progression-free survival (PFS) period.

A 3- to 4-agent combination chemotherapy containing proteasome inhibitors, immunomodulators, or daratumumab is used as tolerated by the patient.

Sequential autologous hematopoietic stem cell transplantation is indicated when available.

The treatment regimen should take into account the patient’s time to relapse; if relapse occurs within 6 months, the patient should try to switch to a regimen consisting of drugs with a different mechanism of action than the one before relapse.

Multi-line relapse

The main therapeutic goal is to improve the patient’s quality of life and to obtain the maximum possible remission on this basis.

Generally, 2 to 4-agent combination chemotherapy containing proteasome inhibitors, immunomodulators, and dalteplumab as well as nuclear output protein inhibitors and cytotoxic agents can be considered.

Invasive/symptomatic relapse and biochemical relapse

Chemotherapy should be initiated in patients with invasive relapse and symptomatic relapse.

Treatment does not need to be started immediately in patients with biochemical relapse only; these patients should only be started if they develop an accelerated rate of increase in monoclonal gammaglobulin (e.g., a 1-fold increase in 3 months).

The slow rate of increase in the affected globulin requires observation only, and follow-up is recommended once every 3 months.

Supportive Treatment

Treatment of bone disease

Oral or intravenous bisphosphonates, including clodronate, pamidronate disodium, and zoledronic acid, are indicated for all symptomatic patients with multiple myeloma.

Surgery is indicated for impending or existing pathologic fractures of the long bones, spinal fractures compressing the spinal cord, or spinal instability.

Low-dose radiotherapy may be used as palliative care for pain that cannot be controlled by medications and to prevent impending pathologic fractures or spinal cord compression.

Hypercalcemia

Aggressively treat the primary disease.

Bisphosphonates inhibit bone resorption and have a lowering effect on blood calcium.

Other measures: hydration, alkalinization, diuresis, etc.

Renal insufficiency

Hydration, alkalinization, diuresis to reduce uric acid formation and promote uric acid excretion to avoid renal insufficiency.

Those with renal failure should be actively dialyzed.

Avoid drugs that affect renal function such as nonsteroidal anti-inflammatory drugs and intravenous contrast agents.

Long-term use of bisphosphonates requires monitoring of renal function.

Anemia

Treatment with erythropoietin (EPO) may be considered, and supplementation of hematopoietic raw materials such as iron, folic acid, and vitamin B12 may be appropriate.

Infection

Intravenous immunoglobulin may be considered for recurrent infections or the development of life-threatening infections.

High-dose dexamethasone regimens should be used to prevent Pneumocystis carinii and fungal infections.

Patients using proteasome inhibitors, daltuzumab, may use acyclovir or valacyclovir for herpes zoster virus prophylaxis.

In patients who are serologically positive for hepatitis B virus, prophylactic use of drugs that inhibit viral replication and careful monitoring of viral load are indicated.

Coagulation/Thrombosis

In patients receiving immunomodulator-based regimens such as thalidomide or lenalidomide, venous thromboembolism risk should be assessed and prophylactic anticoagulation or antithrombotic therapy should be given based on the risk of thrombosis.

Hyperviscosity

Plasma exchange is feasible in symptomatic individuals.

Frontline therapy

Clinical trials that have the potential to give patients better outcomes than the current standard of care. Given that there are still a number of limitations to the efficacy of current standard treatments, patients may voluntarily participate in clinical trials that are compatible with their condition with a view to achieving better survival.

CAR-T therapy

Chimeric antigen receptor T (CAR-T) cells, as a new cellular immunotherapy approach, have been successfully used in the clinic for the treatment of a variety of hematologic tumors, such as acute lymphoblastic leukemia, chronic lymphocytic leukemia, and non-Hodgkin’s lymphoma.

CAR-T cells have also achieved remarkable efficacy in the treatment of relapsed/refractory MM, and the reports of clinical studies related to CAR-T cells targeting B-cell maturation antigen (BCMA) are particularly encouraging, with efficacy rates of more than 80% and progression-free survival (PFS) of 10 to 14 months.

Monoclonal antibody therapy

Monoclonal antibodies induce antibody-dependent cell-mediated cytotoxicity (ADCC), antibody-dependent cellular phagocytosis (ADCP), and complement-dependent cytotoxicity (CDC) by binding to the corresponding target antigens on the surface of MM cells, and ultimately achieve the goal of killing tumor cells.

Isatuximab (SAR650984, SAR): another anti-CD38 monoclonal antibody that has shown more satisfactory efficacy when used as a monotherapy in patients with refractory/relapsed MM.

Erlotuzumab: a humanized antibody specific for SLAMF7, which induces myeloma cell death via two pathways: direct activation of NK cells and triggering induction of antibody-dependent cell-mediated cytotoxicity.

The relevant safety and efficacy data are not yet sufficient, and further studies and research are needed.

Treatment-Related Care

Knowledge of treatment-related care by patients and their families will help them understand and cooperate with the medical staff, and will facilitate the recovery of the patient’s condition.

Nursing care for impaired mobility

Patients with obvious bone destruction should absolutely rest in bed, apply hard board beds and avoid using elastic mattresses to prevent fractures.

Help patients to move within the range of activities, pay attention to safety, prevent fall and fracture, and pay attention to rest after activities.

Encourage the patient to cough and take deep breaths. Assist patients in washing, eating, urinating and defecating and personal hygiene, etc. Scrub the whole body with warm water every day to keep the skin clean and dry.

Paralyzed patients should be assisted to change position every 1~2 hours, keep the bed dry, flat and clean to prevent bedsores. Massage the limbs regularly to prevent atrophy of the lower limbs.

Bleeding site care

Some patients may have bleeding tendency and need to pay close attention to nursing.

Nosebleed: lean forward and pinch the nose to stop bleeding. If the bleeding does not stop, inform the doctor in time for treatment.

Bleeding gums: keep oral hygiene, gargle or oral care after meals. Local bleeding can be stopped by cotton ball pressure.

Gastrointestinal bleeding: record the amount of vomiting blood, blood in stool or black stool, pay attention to the patient’s dizziness, palpitation, rapid pulse, cold sweat, blood pressure drop and other signs of blood loss, inform the doctor in time to rescue, give hemostasis and supplement blood volume.

If the patient develops periorbital petechiae, change of respiratory rate, convulsions, drowsiness, coma, etc., it is mostly suggestive of fundus hemorrhage and intracranial hemorrhage, and the patient should be strictly lying down, avoiding activities, and informing the doctor for treatment in time.

Dietary care

Patients with renal insufficiency should apply low-protein diet, and patients with polyuria should not restrict sodium intake, such as those with low urine and edema with low-salt diet (salt intake is controlled at 2~3g/day).

Encourage patients to drink more water to avoid dehydration and renal function damage caused by hyperuricemia and hypercalcemia.

Chemotherapy care

Eat small meals, light, easy-to-digest diet is the main focus, avoid eating stimulating, greasy food, to reduce the occurrence of nausea, regurgitation.

Some chemotherapeutic drugs can cause stomatitis, avoid eating stimulating food, and be sure to rinse your mouth after meals.

Exercise in moderation, relax your mood, communicate with your family more often, and keep a good mood.

Drink plenty of water in moderation and in small amounts to promote the excretion of chemotherapy drugs.

Follow the doctor’s requirement to have chemotherapy on schedule, avoid increasing or decreasing on your own.

If there is any discomfort, consult the doctor in time.

Prognosis

Survival rate

Median survival

Without treatment, the median survival of patients with progressive multiple myeloma is only 6 months.

Conventional chemotherapy has a therapeutic efficiency of 40% to 60% and a median survival of no more than 3 years.

10-year survival

Currently reported efficacy rates for combination chemotherapy with different new agents are 80% to 95%. For patients under 50 years of age, the 10-year survival rate of new drugs combined with autologous hematopoietic stem cell transplantation can exceed 40%.

With the current proliferation of new drugs, the search for the optimal new drug regimen or combination will further improve the natural course of multiple myeloma.

[Special Note

Median survival is the survival time of patients with the same disease who survive in the middle of the total number (e.g., the 50th of 99 patients), in order of time.

Statistical data such as median survival and 10-year survival rate are only used for scientific research and do not represent the patient’s own survival. Individual survival is determined by a variety of factors, so it is recommended to consult with the physician for details.

Prognostic factors

Factors affecting prognosis are categorized into three major groups: patient’s own factors, tumor characteristics, treatment modality and response to treatment, and a single factor is often insufficient to determine prognosis.

Patient factors: Age, physical status, and Geriatric Assessment of Health (GA) scores can be used to assess prognosis.

Tumor factors: DurieSalmon stage of multiple myeloma mainly reflects the tumor load and clinical progression; RISS is mainly used for prognosis.

Treatment modality and treatment response: the depth of treatment response and the level of microscopic residual disease have a significant impact on the prognosis of multiple myeloma.

Recurrence.

Multiple myeloma is prone to relapse after treatment and is still considered an incurable disease.

Daily

Life Management

Mindfulness and Emotional Adjustment

A good mood and mindset cannot be replaced by medication.

After diagnosis, patients may develop a sense of fear and may be afraid of pain, abandonment and death. Family members should pay attention to listen to the patient’s heart, improve the patient’s psychological tolerance and relieve anxiety symptoms.

During the period between treatments and after treatment, family members are advised to encourage the patient to do work and household chores that are within his/her ability and reintegrate into social roles.

Living

Keep the living environment clean, with frequent ventilation, sufficient sunlight, and suitable temperature and humidity.

Disinfect the room regularly to avoid infection.

Maintain good hygiene and cleanliness to prevent accidental bodily injury.

Rinse your mouth with saline solution and use a soft-bristled toothbrush after meals and before bedtime.

Maintain a positive and optimistic state of mind, reduce tension and anxiety, and avoid excessive activity and trauma for those who are prone to bleeding.

If the lesion is in the lower limbs, try not to get out of bed to avoid fracture.

Dietary regulation

Balanced dietary structure, diversified food types and rich nutrition.

Pickled, fried and deep-fried food should be avoided.

Eat more vitamin-rich vegetables and fruits, such as broccoli, tomatoes, celery, lettuce, kiwi, apples and bananas.

Eat more protein-rich foods, such as eggs, milk, lean meat and fish.

It is recommended not to eat foods that stimulate the secretion of stomach acid, such as foods that are too sweet and spicy.

Rest and Exercise

Pay attention to rest, avoid staying up late or straining, and ensure sufficient sleep and rest to reduce physical exertion and promote recovery.

When the condition improves, you can start with low intensity exercise such as walking and gradually resume normal activities.

Review and Follow-up

Asymptomatic myeloma

It is generally recommended to review the relevant indicators every 3 months. However, each patient’s condition is different, and the frequency of review and related examination items should strictly follow the doctor’s instructions.

Blood: blood creatinine, albumin, lactate dehydrogenase, serum calcium, β2 microglobulin, serum immunoglobulin quantification, serum protein electrophoresis and blood immunofixation electrophoresis, serum free light chain (FLC), etc.

Urine: 24-hour total urine protein, urine protein electrophoresis and urine immunofixation electrophoresis.

Skeletal examination: performed once a year or when clinically indicated.

Isolated plasmacytoma

Isolated plasmacytomas are categorized as bony and extraosseous, and multiple myeloma needs to be excluded.

Follow-up and monitoring is performed every 4 weeks at the beginning.

If M protein disappears completely after treatment of plasmacytoma, it is performed every 3 to 6 months or when there are clinical symptoms.

If M protein persists, continue monitoring 1 every 4 weeks. Imaging is performed every 6 to 12 months.

Symptomatic myeloma

Efficacy assessment every 2 to 3 sessions during induction therapy.

Efficacy assessment every 3 months during consolidation and maintenance therapy.

Bone marrow examination is required for efficacy assessment of non-secretory myeloma.

Serum FLC is useful for efficacy assessment, especially for non-secretory myeloma.

Bone marrow examination is performed every 6 months or as clinically indicated.

Prevention

There are no standard precautions for this disease; the following recommendations may reduce the incidence of multiple myeloma.

Avoid excessive exposure to x-rays and other harmful radiation.

Personal protection is required for those who work with radiation.

Avoid exposure to various carcinogens such as arsenic, pesticides, petrochemicals and other poisons.

Protect against cold and keep warm to avoid catching cold.

Exercise appropriately to improve physical fitness and resistance.

Regarding daily diet, pay attention to balanced nutrition and ensure the intake of protein, vitamins and other nutrients.