Table 1 shows the criteria for diagnosis of MM using the old method. In the newer method, initiation of therapy is an evidence of organ or tissue damage end-organ damage [ 9 ]. Table 2 shows the current criteria of diagnosis of MM. Evidence of end-organ damage that can be attributed to the underlying plasma cell proliferative disorder, specifically:.
BMPC percentage should preferably be estimated from a core biopsy specimen; in case of a disparity between the aspirate and core biopsy, the highest value should be used. The staging of MM is another important step after diagnosis. The essence of staging is for decision-making on therapeutic interventions and for prognostication of the disease.
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There are two clinical staging systems for MM. Here, the staging of MM is based on five parameters viz. This is shown in Table 3. The modified Salmon-Durie assesses myeloma tumor mass using the old system to stage MM into high tumor mass stage III , low tumor mass I , and intermediate tumor mass myelomas II , which is shown in Table 4. The ISS is based on two widely available parameters, serum beta-2 microglobulin and albumin.
This staging system recognizes three stages and can be useful for prognostication of survival intervals of MM patients Table 5 [ 13 ].
Intermediate tumor mass stage II 0. All patients who do not qualify for high or low tumor mass categories are considered to have intermediate tumor mass. Data adapted from Durie and Salmon [ 12 ]. A remodified D-S staging system. The standard assessment of MM requires a panel of investigations, which are carried out periodically postdiagnosis for prognostication and monitoring of the disease response to treatment. These investigations include complete blood count, blood chemistry, serum and urine monoclonal protein assay, C-reactive protein, beta-2 microglobulin test, marrow study, skeletal survey, echocardiogram, immunophenotyping, cytogenetic tests, etc.
Table 6. The prevalence of MM is on the increase in developing countries such as those found in Sub-Saharan Africa [ 3 , 14 ]. The oil-rich regions are worse hit probably due to a wide range of environmental pollution, flaring of gases, water pollution, oil spillage, and lack of effective environmental policies [ 6 ]. This is understandable based on the hypothesis that occupation studies of chemical, petroleum, and radiation industry workers have provided inconsistent evidence of causal association with MM [ 5 ]. Another potential etiologic factor that could be a key player in the increasing prevalence is the median age of diagnosis.
The implication of this early age of diagnosis is that more people may likely be diagnosed with MM by the time they attend the age of 65, hence increasing the burden of the disease. The male to female ratio of about recorded by most of the studies shows a gender disparity of the disease. However, the later may not have much role to play on the increased prevalence of MM in developing countries.
There is a dearth of data on the diagnosis or prevalence of premalignant plasma cell disease in low- and some middle-income countries. However, due to lack of resources for making diagnosis at this early stage, these premalignant diagnoses are missed. This ultimately leaves the attending physicians with MM patients who present at advanced stages of the disease. The mean duration from onset of symptoms to diagnosis in a study was The lack of modern equipments for diagnosis and staging of the disease are the key players in the late diagnosis of MM in most developing countries including Nigeria [ 14 ].
Most health institutions in developing countries especially the low-income do not have the infrastructural and medical capacities to handle comprehensive assessment investigations for MM patients. Out of this number, 43 and The commonest assessment tests done by the patients are hematocrit, erythrocyte sedimentation rate, skeletal x-ray, bone marrow aspiration, and trephine biopsy in centers where there are hematologists [ 14 , 15 , 16 , 17 ].
The serum albumin is one of the analytes essential for international prognostic staging of MM. The implication of this is that most MM diagnosed in these regions are cytogenetically unknown and are not internationally staged. Hence, MM patients do not benefit from accurate risk stratification and prognostic assessments as offered to their counterparts in developed countries [ 19 ]. These challenges in diagnoses and disease staging contribute to the poor survival outcome of people living with MM in these regions.
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In a year retrospective study of 26 MM patients in Niger-delta region of Nigeria, only one 3. In the study, only four subjects could afford immunofixation test, which showed IgA:IgG-type myeloma ratio of and this was in keeping with previous study by Salawu and Durosimi [ 16 ]. MM poses a diagnostic dilemma for the orthopedic surgeons because of the frequent skeletal manifestations. At advanced stage, it causes multiple lytic bone lesions with severe osteoporosis and pathological fracture. A recent observational study in Nigeria [ 14 ] found that about Pathological fracture constitutes about It is surprising to note that These cytokines, especially VEGF and PDGF, have angiogenic effect on the bone marrow microenvironment and this effect favors the growth of myeloma cells in the bone.
IL-6, an important osteoclast-activating cytokine, plays an important role in the pathogenesis of osteoporosis in MM [ 21 ]. Annibali et al. Other complications such as anemia, hemiplegia, nephropathy, and constipation accounted for Anemia in MM results from bone marrow invasion by abnormal plasma cells that secret erythropoiesis-suppressive cytokines, and this anemia is usually anemia of chronic disorder [ 23 ].
The last step in the management of multiple myeloma is the therapeutic intervention. The current standard treatment for MM is palliative care. This is a holistic treatment that offers supportive, definitive, and psychosocial care for people living with MM [ 24 ]. There is a gross inadequacy in the palliative care of MM in developing countries, hence the call to scale-up the care of people living with MM. This is because of the life-threatening nature and the suffering associated with the disease.
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A recent study has shown that inadequate palliative care accounts significantly for the low survival interval of MM patients [ 3 ]. In one of the studies, it was found that only about 7. This was far below the estimated 5-year period survival of 32 and The two major challenges in the treatment of MM in developing countries are anchored on the supportive and definitive treatment of MM. The standard supportive care for MM patients at advanced stage of the disease, which include the use of analgesics, bisphosphonates BPs , component blood therapy, antibiotics therapy, renal dialysis viz-a-viz renal transplant, radiotherapy, orthopedic care, is grossly inadequate.
Chronic bone pain appears to be one of the commonest clinical features of MM, and analgesic drug is the first supportive therapy offered to patients with the disease. However, in the assessment and treatment of pain in MM patients in some low-income countries such as Nigeria, the WHO analgesic ladder for cancer pain control is not usually adhered to, as only few centers can access oral morphine and other opiate analgesics [ 27 ].
This leads to analgesic abuse self-medication , most of which are nephrotoxic, hence, worsening the prognosis of the disease. BPs are useful in preventing, reducing, and delaying MM SREs such as bone pain, osteoporosis, and other lytic bone lesions. They can also help to control the growth of extramedullary tumors, hence the need to scale-up their usage in MM [ 22 , 28 ].
There is a gross inadequate access to radiation therapy in LICs including Nigeria. Studies have shown that only about 3. The major reason is that the megavoltage radiotherapy machine per population size is grossly inadequate 1-MV machine per 24 million population as against the International Atomic Energy Agency IAEA requirement of 1-MV machine per , population or per — new cancer patients in centers with excellent cancer registry [ 29 ]. The implication is that they will rely on blood transfusion therapy in order to improve the quality of their life.
Unfortunately, many of the LICs do not practice safe blood transfusion. They depend majorly on commercial paid blood donation as against voluntary non-remunerated blood donation VNRBD , thereby predisposing the patients to transfusion transmissible infectious diseases TTIs including HIV [ 30 ]. The facilities for component blood therapy i. For instance, there was no documented beneficiary from component blood therapy in previous studies in Nigeria.
About However, the later is usually expensive and only very few patients can afford it, hence worsening the survival outcome of the disease [ 3 ]. A striking finding about the nephropathies in MM patients in LICs is their severity at presentation, which qualifies most of them for renal dialysis or renal transplant. However, this is an expensive palliative intervention as only very few patients can comply with the courses of dialysis, which may not be available in some centers.
In African continent, the major complications that bring MM patients to the hospital for the first time are operable surgical complications. A recent study revealed that Surprisingly, these complications have set in long before diagnoses were made. The presence of extramedullary plasmacytoma indicates poor prognosis, and this is worsened further in the absence of involved field radiotherapy IFR [ 33 ].
The standard definitive interventions for people living with MM are antimyeloma chemotherapy regimens and stem cell transplantation autologous stem-cell transplantation ASCT. The antimyeloma chemotherapeutic regimens have undergone series of transformation and evolution over the years. The current antimyeloma therapeutic agents have changed the paradigm in the management of the disease. These agents have the best effect in improving the quality of life and overall survival intervals of MM patients. They have positively changed the course of the disease especially in high-income countries where they are relatively more available.
This has been due in large part to a better understanding of the biology of the disease and the development of several highly effective therapies. They include proteasome inhibitors [PI] bortezomib, carfilzomib, ixazomib, marizomib, and oprozomib , immunomodulatory [IMiD] agents thalidomide, lenalidomide, and pomalidomide , monoclonal antibody therapies elotuzumab, daratumumab, and siltuximab , Bcl inhibitor navitoclax , FGFR3 inhibitor dovitinib , and histone deacetylase HDAC inhibitors panobinostat, romidepsin, vorinostat, and rocilinostat.
These agents include those that target the myeloma itself, some that target the bone marrow microenvironment, and those that target both [ 34 ]. Unfortunately, these agents are not readily available in low- and some middle-income countries LMICs including Nigeria. The huge disparity in income, health-care infrastructure, and access to novel drugs in LMICs hinders the delivery of optimum care to every patient with MM in the region [ 35 ] due to limitation in purchasing power.
The treatment approaches that are often referred to as standard are usually those with strong evidence of clinical efficacy. Although a recent clinical trial has shown that a combination of PI and IMiD will make for a standard regimen when added with dexamethasone [ 36 ], the current opinion is in favor of individualized treatment options, which is based not only on cytogenetic risk classification, but also on host factors, disease stage, and a variety of other prognostic factors.
This consensus standard of treatment of MM is yet to be achieved in many developing countries. In Nigeria, the major antimyeloma chemotherapy drug is the old conventional alkylating agent known as melphalan M , which is usually combined with a steroid i. MP is still the most accessible commonly used regimen for treating MM patients because of the cost and availability, long after it has been phased out for treating MM patients in developed countries.
This is contrary to the standard RVD triplet regimen accepted worldwide as the current treatment of choice for MM. However, a recent study in Nigeria has shown that up to Stem cell transplantation i. The only patient 3. There is paucity of data regarding stem cell transplantation in most LICs especially those from Sub-Saharan African region. Although few successful attempts on allogeneic stem-cell transplantation have been made in a center in Southern Nigeria on sickle cell disease , but it has not been sustainable due to technological inequalities, brain drain of health workers, lack of funding, and political-will from the government.
The public health system does not guarantee health insurance coverage for oncology treatment and stem-cell transplantation. Transplant-eligible patients who require stem-cell transplantation usually pay out from their pockets, and this could add to another burden to the patients [ 41 , 42 , 43 ].
A multiple myeloma diagnosis can be overwhelming for a loved one. They'll need encouragement and positive energy. Here are a few tips to help a loved…. A doctor answers questions about targeted therapy for multiple myeloma. Getting a diagnosis of multiple myeloma can feel overwhelming and isolating. Joining a cancer support group may help you feel more at ease as you…. Multiple myeloma is a type of cancer in which abnormal cells reproduce in bone marrow.
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Targeted therapy is just one of several drugs your doctor might give you to treat multiple myeloma. Before you start on a targeted therapy medication…. Treatment for multiple myeloma includes taking multiple medications and keeping up with ongoing testing. While it's understandable to feel overwhelmed,.
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