Thursday, 9 August 2018

MYELOMA

  • AKA Multiple myeloma, plasma cell neoplasms
  • This is a proliferation of neoplastic plasma cells derived from one B lymphocyte and producing a homogeneous immunoglobulin (M protein) without any apparent antigenic stimulation.
  • Plasma cells produce osteoclast-activating factor leading to extensive bone loss, severe pain, and pathologic fractures.
  • Abnormal immunoglobulin affects renal function, platelet function, resistance to infection, and may cause hyper viscosity of blood.

INCIDENCES

  • It accounts approximately 13% of all hematologic malignancies.
  • Affecting adults of any age but men are slightly more affected than women
  • The mean age at diagnosis is approximately 65–70 years of age.

ETIOLOGY

  • Exact cause is unknown.
  • Risk factors include:
    • Genetic and environmental factors
      • chronic exposure to low levels of ionizing radiation
      • Exposure to certain exotoxins, e.g. benzene, Agent Orange.
    • Monoclonal gammopathy of unknown significance (MGUS) - a condition in which an abnormal protein — known as monoclonal protein or M protein is in our blood. The protein is produced in plasma cells in our bone marrow.
    • Pernicious anaemia.
    • History of thyroid cancer.

CLINICAL MANIFESTATIONS

  • Fatigue and weakness
  • Unintentional weight loss.
  • Severe bone pain caused by bone lesions and pathologic fractures sites commonly affected include thoracic and lumbar vertebrae, ribs, skull, pelvis, and proximal long bones.
  • Vertebral collapse (may lead to spinal cord compression).
  • Hypercalcaemia.
  • hyperuricemia
  • Anaemia.
  • Infection.
  • Renal impairment.
  • Bruising.

DIAGNOSTIC INVESTIGATIONS

  • Bone marrow aspiration and biopsy—demonstrate increased number and abnormal form of plasma cells.
  • Bloods: peripheral blood smear (normocytic, normochromic anaemia), U&Es, creatinine, LFTs, ESR, CRP, calcium levels, alkaline phosphatase, beta-2 microglobulin.
  • Urine and serum analysis for presence and quantity of abnormal immunoglobulin.
  • Serum and urine electrophoresis: paraprotein (M protein), Bence Jones proteinuria.
  • Skeletal X-ray for bone deformities, e.g. pepper pot skull and generalised skeletal osteopaenia.
  • MRI scan may be useful.

COMPLICATIONS

  • Spinal cord compression.
  • Pathological fracture.
  • Hypercalcaemia.
  • Acute kidney injury.
  • Increased risk of infection.
  • Anaemia.

MEDICAL MANAGEMENT

  • Patients with "smoldering" multiple myeloma do not require treatment.
  • Management of multiple myeloma depends on the age of the patient and their state of health.
  • If they are <70/65 years and without significant co-morbidities like renal failure, few bone lesions, and good organ function then they are eligible for autologous bone marrow transplant, which is the most effective treatment. This involves an induction phase using the VAD regimen: vincristine, adriamycin, dexamethasone. After transplant the patient receives long-term therapy with melphalan.
  • Patients who are ineligible for autologous bone marrow transplant receive long-term treatment with melphalan and prednisolone.
  • Supportive care options:
    • Plasmapheresis to treat hyperviscosity or bleeding.
    • Radiotherapy may be required to treat bone pain and spinal cord compression.
    • Biphosphonates (eg, pamidronate), potent inhibitors of bone resorption, to treat hypercalcemia and alleviate bone pain.
      • administered as IV infusions, generally during 4 or more hours
      • rapid IV administration may cause renal failure
      • Long term use - jaw osteonecrosis
      • Other side effect are transient temperature elevations, hypophosphatemia, hypomagnesemia, hypocalcemia,
    • Allopurinol and fluids to treat hyperuricemia.
    • Hemodialysis to manage renal failure.
    • Surgical stabilization and fixation of fractures.
  • Surgical: Kyphoplasty surgery

Kyphoplasty surgery

  • A small incision is made in the back
  • Through which places a narrow tube using fluoroscopy guide
  • Inserts a special balloon through the tube and into the vertebrae,
  • Then gently and carefully inflates it.
  • As the balloon inflates, it elevates the fracture, returning the pieces to a more normal position.
  • It also compacts the soft inner bone to create a cavity inside the vertebrae.
  • The balloon is removed and specially designed instruments under low pressure to fill the cavity with a cement-like material called polymethylmethacrylate (PMMA).
  • After being injected, the pasty material hardens quickly, stabilizing the bone.

NURSING MANAGEMENT

Acute Pain related to destruction of bone and possible pathologic fractures.

(Controlling Pain)

  • Assess for presence, location, intensity, and characteristics of pain.
  • Administer pharmacologic agents, as ordered, to control pain.
  • Teach the use relxation therapies, such as music therapy, relaxation breathing, progressive muscle relaxation, distraction, and imagery, to help manage pain.
  • Assess effectiveness of analgesics and adjust dosage or drug used, as necessary, to control pain.

Impaired Physical Mobility related to pain and possible fracture.

(Promoting Mobility)

  • Encourage patient to wear back brace for lumbar lesion.
  • Advise the physical and occupational therapy consultation.
  • Discourage bed rest to prevent hypercalcemia but ensure safety of environment to prevent pathological fractures.
  • Assist patient with measures to prevent injury and decrease risk of fractures.
  • Advise avoidance of lifting and straining; use walker and other assistive devices.

Fear related to poor prognosis.

(Relieving Fear)

  • Develop trusting, supportive relationship with patient and family members.
  • Encourage patient to discuss medical condition and prognosis with health personals.
  • Assure patient that you are available for support, to provide comfort measures, and to answer questions.

Risk for Injury related to complications of disease process.

(Monitoring for Complications)

  • Report any sudden, severe pain, especially of back, which could indicate pathologic fracture.
  • Watch for nausea, drowsiness, confusion, polyuria, which could indicate hypercalcemia caused by bony destruction or immobilization.
  • Monitor serum calcium levels.
  • Monitor blood urea nitrogen (BUN), creatinine, and urine protein tests to detect renal insufficiency, caused by nephrotoxicity of abnormal proteins in multiple myeloma.
  • Encourage the patient to maintain high fluid intake (2 to 3 L/day) to avoid dehydration and prevent renal insufficiency and monitor intake and output, and weigh patient daily.

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