Monoclonal Gammopathy with Pathological fracture By: Muhamad Na’im B. Ab Razak (MD USM)Disclaimers: This is a real case reports encountered by the authors. However, the discussionin this entry was taken either edited or un edited from a textbook The Internal MedicineCasebook: Real Patients, Real Answers, 3rd edition by Robert W. Schrier, 2007 published byLippincott Williams @ WilkinsThis 40 years old lady presented to casualty with complaint of right thigh pain, swollen andinability to move following a trivial fall at home due to slippery. Examination reveals rightthigh tenderness, swollen, warmness and deformity. Right femur X ray shows subtrochantericfracture with reduce bone density arounds the trochanter region. Apart from that she alsocomplained of easily lethargy and occasionally back pain. Otherwise, other systems areunremarkable with no hepatosplenomegally, pink conjunctiva and spine examination wasnormal.
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 roundedlytic lesionDiscussion1) Monoclonal gammopathyA monoclonal gammopathy is defined as the overproduction of a particular immunoglobulinprotein by a single clone of overactive or malignant B cells. This clone can produce a wholeimmunoglobulin, composed of both heavy and light chains, or it can produce just heavychains, 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.- Waldenstroms 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 cells4) Immunologic capability in this patient- High chance of compromised with susceptible to high grade bacterial pathogens.- Do not provide adequate antibodies after prophylactic immunizations5) Managementa) For multiple myeloma- Melphalan and prednisone (chemo agents)- Bone marrow transplant- IV gamma globulin for prophylaxis against infection- Thalidomideb) For fracture- Open reduction and Internal Fixation should be done in center with specialty of orthopaediconcology unit and reconstructive surgery and may require specialized plating to preventimplant failure.c) Hypercalcaemia- Fluid rehydration with normal saline together with IV furosemide enhance excretion ofcalcium.- IV hydrocortisone 200mg QID or prednisolone 30-60 mg daily works well in patient withmyeloma.- Calcitonin- Biphosphonate- Dialysis in severe hypercalcaemia.