Australian Doctor Australian Doctor 15th September 2017 | Page 28

Case study
Conclusion

How to Treat – Multiple myeloma

Case study

JEAN, 60, presents to her GP complaining of worsening right hip pain. It is thought to be arthritis, secondary to longstanding obesity. Hip and pelvis X-rays reveal multiple lytic lesions and the radiologist recommends a myeloma screen.
Jean’ s blood tests reveal she is mildly anaemic, with a haemoglobin of 110g / L and an IgG paraprotein of 21g / L. A skeletal survey reveals multiple lytic lesions. She is referred to the haematology clinic, where a bone marrow biopsy confirms multiple myeloma, with 30 % plasma cells. CyBorD chemotherapy( cyclophosphamide, bortezomib, dexamethasone) is started, and she completes six cycles with minimal side effects. She is also given monthly zoledronic acid infusions to prevent further skeletal events.
Re-staging shows a partial response— the paraprotein falling from 21g / L to 11.9g / L, and her bone marrow biopsy demonstrates 10 % plasma cells. She undergoes a stem cell harvest and autologous stem cell transplant. Her transplant is complicated by febrile neutropenia and neutropenic colitis. However she recovers fully and is discharged from hospital after three weeks.
She is subsequently followed up
MULTIPLE MYELOMA IS A RARE, BUT IMPORTANT, HAEMATOLOGICAL MALIGNANCY THAT CAN PRESENT IN A VARIETY OF WAYS.
in the haematology clinic every two weeks. Further staging following the autologous transplant reveals an undetectable paraprotein and bone marrow biopsy shows only 1-2 % plasma cells. She remains in partial remission for three years following the transplant, when her paraprotein starts to rise again, reaching13g / L.
Following repeat bone marrow biopsies and skeletal surveys, thalidomide-dexamethasone is started. Unfortunately she develops peripheral neuropathy secondary to the thalidomide, and despite again achieving partial response, it has to be stopped. She has remained stable since, with only slowly increasing paraprotein, and no change in her haemoglobin or renal function.

Conclusion

MULTIPLE myeloma is a rare, but important, haematological malignancy that can present in a variety of ways. An understanding of the screening and diagnostic modalities, and complications of both the disease and treatment is more important now more than ever. This is because improving therapy options and life expectancy is transforming myeloma into a chronic disease.
Key points
• Myeloma is a malignancy of plasma cells, which results in the secretion of a monoclonal immunoglobulin.
• There are a wide range of clinical presentations; think of, and screen for, myeloma in any patient with unexplained anaemia, progressive renal impairment, hypercalcaemia or bone pain.
• Diagnostic workup includes testing for serum and urine paraproteins and serum free light chains, as well as a skeletal survey and bone marrow biopsy.
• Diverse treatment options are now available.
• Complications of myeloma and its treatment are best managed with a multidisciplinary approach.

How to Treat Quiz GO ONLINE TO COMPLETE THE QUIZ

Multiple myeloma
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1. Which TWO statements regarding the epidemiology of multiple myeloma are correct? a) Multiple myeloma accounts for about 10 % of haematological malignancies. b) The mean age at diagnosis is 50. c) Monoclonal gammopathy of uncertain significance, the premalignant disease, occurs in around 4 % of people over 50. d) Despite the introduction of new therapeutic agents, the life expectancy of multiple myeloma has remained static over the past two decades.
2. Which THREE statements regarding the pathophysiology of multiple myeloma are correct? a) Most multiple myeloma involves secretion of whole immunoglobulin in which the heavy chain is clonally IgG or IgA. b) Excess light chains, known as Bence Jones proteins, are excreted in the urine. c) Paraproteins can only be detected in the blood. d) Clonal expansion of plasma cells results from genetic mutations.
3. Which TWO statements regarding the pathophysiology of multiple myeloma are correct? a) Dysregulation of the balance between
osteoclast and osteoblast activity is responsible for the osteyolytic bone lesions seen in multiple myeloma. b) The anaemia of multiple myeloma results from expansion of plasma cells within the marrow, as well as an inflammatory state. c) The most common causes of renal impairment in myeloma are light chain amyloidosis and monoclonal immunoglobulin deposition disease. d) Infection in myeloma is rarely related to treatment, but is always as a result of neutropenia.
4. Which is the MOST common presenting symptom in multiple myeloma? a) Bone disease. b) Anaemia. c) Renal impairment. d) Spinal cord compression.
5. Under which TWO circumstances would one consider screening for multiple myeloma? a) Complaints of bone pain with pathological fractures in the presence of osteoporosis. b) Acute kidney injury in an over-50-year-old with no alternative explanation. c) Anaemia, after exclusion of common causes. d) Hypoproteinaemia.
6. Which THREE are myeloma defining events? a) Renal failure. b) Anaemia. c) More than one focal lesion on MRI. d) Hyperkalaemia.
7. Which TWO statements regarding the diagnosis of multiple myeloma are correct? a) The preferred method for detection of cytogenetic abnormalities is conventional cytogenetic testing. b) Cytogenetic investigations provide prognostic information for patients with both smouldering multiple myeloma and active multiple myeloma. c) Hyperdiploidy is associated with low risk of smouldering multiple myeloma progression and a poor prognosis in patients with symptomatic multiple myeloma. d) Certain cytogenetic abnormalities portend a poor prognosis in multiple myeloma
8. Which THREE statements regarding the treatment of multiple myeloma are correct? a) Newer treatment regimens and improved risk stratification have produced a significant improvement in the cure rates of multiple myeloma. b) In confirmed multiple myeloma, treatment should ideally be initiated to prevent further
organ injury, unless the situation is clearly palliative. c) Most patients respond to initial treatment, but because of the lack of curative treatment, relapse is inevitable. d) Most new agents are given in combinations with traditional chemotherapy agents and corticosteroids.
9. Which TWO statements regarding the treatment of multiple myeloma are correct? a) Autologous stem cell transplant is superior to a non-transplant approach in terms of both progression-free and overall survival. b) Allogeneic stem cell transplantation is recommended in the frail and elderly, as it has fewer complications than autologous stem cell transplant. c) Radiation therapy may be used both at diagnosis and when there is disease progression. d) Venous thromboembolism is the main cause of death in patients with myeloma
10. Which TWO drugs used to treat multiple myeloma increase the risk of venous thromboembolism? a) Lenalidomide. b) Bortezomib. c) Cyclophosphamide. d) Thalidomide.
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