Disclaimers: This is a real case reports encountered by the authors. However, the discussion in this entry was taken either edited or un edited from a textbook The Internal Medicine Casebook: Real Patients, Real Answers, 3rd edition by Robert W. Schrier, 2007 published by Lippincott Williams @ Wilkins
This 40 years old lady presented to casualty with complaint of right thigh pain, swollen and inability to move following a trivial fall at home due to slippery. Examination reveals right thigh tenderness, swollen, warmness and deformity. Right femur X ray shows subtrochanteric fracture with reduce bone density arounds the trochanter region. Apart from that she also complained of easily lethargy and occasionally back pain. Otherwise, other systems are unremarkable with no hepatosplenomegally, pink conjunctiva and spine examination was normal.
Laboratory findings was uneventful except hypercalcaemia and normal renal function test. Skeletal survey was nil of significant except for skull x ray which shows multiple rounded lytic lesion
Discussion
1) Monoclonal gammopathy
A monoclonal gammopathy is defined as the overproduction of a particular immunoglobulin protein by a single clone of overactive or malignant B cells. This clone can produce a whole immunoglobulin, composed of both heavy and light chains, or it can produce just heavy chains, just light chains, or a combination of whole immunoglobulin plus excess light chains. The monoclonal light chains are called Bence Jones protein. [Robert W. Schrier et al,]
2) Typical presentation
- Back pain, anemia, hypercalcemia and renal disease.
- Waldenstrom's macroglobulinemia resembles lymphoma symptoms which is fever, lymphadenopathy and hepatosplenomegally
- Hyperviscosity
- Amyloidosis due to light chain disease
3) Further investigations
- Liver function test (Elevated total protein but decrease albumin level which suggestive of increase in globulin fraction)
- Urine or serum electrophoresis for Bence Jones Protein
- Skeletal survey (Skull, Complete Spine, Pelvis and Chest)
- CT Abdomen in case of solitary extramedullary plasmacytoma
- Serum calcium level
- Bone marrow aspiration to demonstrate clumping and sheets of plasma cells
4) Immunologic capability in this patient
- High chance of compromised with susceptible to high grade bacterial pathogens.
- Do not provide adequate antibodies after prophylactic immunizations
5) Management
a) For multiple myeloma
- Melphalan and prednisone (chemo agents)
- Bone marrow transplant
- IV gamma globulin for prophylaxis against infection
- Thalidomide
b) For fracture
- Open reduction and Internal Fixation should be done in center with specialty of orthopaedic oncology unit and reconstructive surgery and may require specialized plating to prevent implant failure.
c) Hypercalcaemia
- Fluid rehydration with normal saline together with IV furosemide enhance excretion of calcium.
- IV hydrocortisone 200mg QID or prednisolone 30-60 mg daily works well in patient with myeloma.
- Calcitonin
- Biphosphonate
- Dialysis in severe hypercalcaemia.
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