Multiple myeloma (kahler's disease)

Introduction

This is a malignant proliferation of plasma cells. Normal plasma cells are derived from B cells and produce immunoglobulins which contain heavy and light chains. Normal immunoglobulins are POLYCLONAL, but in myeloma plasma cells produce immunoglobulin of a single heavy and light chain, a MONOCLONAL protein called as paraprotein.

In some cases only light chain is produced and this appears in the urine as BENCE JONES PROTEINURIA.

The malignant plasma cells produce cytokines, which stimulates osteoclasts and result in net bone reabsorption which causes bone pain, fractures and hypercalcaemia.

Clinical features and investigations

Bone pain/ fractures

Lytic lesions

Nephrotic syndrome, carpal tunnel syndrome

Panda eyes

Renal failure due to paraprotein deposition, hypercalcaemia, infection, NSAIDs, amyloid.

Spinal cord compression

Retinal bleeds

Bruising

Heart failure

Cerebral ischaemia

Diagnosis of myeloma requires two of the following criteria:

1. Increase malignant plasma cells in bone marrow

2. Serum and/ or urinary M-protein

3. Skeletal lytic lesions.

Investigations

X-ray for skeletal survey

Alkaline phosphatase

MRI spine

Blood and urine protein electrophoresis done for the presence of M-protein

Blood and urine IMMUNOELECTROPHORESIS for the TYPE of M-protein

Bone marrow aspiration and trephine

Full blood count

Urea, creatinine, urate and electrolytes to access renal function

Blood calcium and albumin to know presence of hypercalcaemia.

Management

Immediate support

• High fluid intake to treat renal impairment and hypercalcaemia.

• Analgesia for bone pain.

• Bisphosphonates for hypercalcaemia.

• Allopurinol to prevent urate nephropathy.

• Plasmapheresis for hyperviscosity.

Chemotherapy with or without HSCT

First line treatment is with THALIDOMIDE and more recently with the BORTEZOMIB.

Thalidomide along with the alkylating agent MEPHALAN and PREDNISOLONE has increased the median overall survival to more than 4 years.

Treatment is administered untill PARAPROTEIN levels have stopped falling. This is termed as PLATEAU PHASE.

In UK at present, bortezomib is recommended followed by LENALIDOMIDE if there is subsequent progression.

Radiotherapy

This is effective for localised bone pain not responding to simple analgesia.

Bisphosphonates

Long term therapy reduces bone pain and skeletal events.

I/V ZOLEDRONATE in combination with anti-myeloma therapy confers a survival advantage over oral bisphosphonates.

Comments

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