1. Myeloma & Amyloidosis

2. Multiple myeloma
A cancer of plasma cells
(responsible for antibody production)

3. Clinical features
 Bone involvement-
 Pain & fractures, due to lytic lesions, seen in X-rays of skull, ribs, spine, pelvis
 Can cause hypercalcemia
 Infection-
 LRTI & UTI, due to hypogammaglobulinemia
 Renal failure-
 Due to hypercalcemia, BJ proteins, amyloidosis, hyperuricemia, UTI
 Anemia- N/N, due to BM infiltration
 Neurological symptoms-
 Weakness, fatigue, confusion due to hypercalcemia
 Headache, visual changes due to hyperviscosity
 Radicular pain, paraplegia due to spinal cord compression
 Carpal tunnel syndrome due to amyloid deposition
Calcium.Renal failure.Anemia.Bone lesions- CRAB

4. Diagnosis
 Presence of bone pains/pathological fracture,
anemia, raised ESR & elevated creatinine
raises possibility of MM
 Dx- any two of three-
 1- Monoclonal M-band in serum electrophoresis
 2- BM with >10% plasma cells- CD56,38,138 +ve
 3- C.R.A.B
 Asymptomatic myeloma-
1 >3 gm% &/or 2, not 3
 MGUS- 1 <3 gm%, not 2 or 3

5. Staging
 Durie-Salmon staging system-
 I- Hb>10, Ca normal, normal/osteoporosis, serum paraprotein-
IgG<5 gm% & IgA<3gm%, urinary light chain <4 gm/24 hr
 II- between I & III
 III- Hb<8.5, Ca>12, >3 lytic lesions, serum paraprotein-
IgG>7 gm% & IgA>5 gm%, urinary light chain >12 gm/24 hr
 All- A- Cr<2 or B- Cr>2
 International staging system- for Px-
 I- β2 microglobulin <3.5 mg/L- survival 62 months
 II- β2 microglobulin 3.5-5.5 mg/L- 45 months
 III- β2 microglobulin >5.5 mg/L- 29 months

6. Treatment
 Initial Rx-
 Melphalan + Prednisolone- preferred in elderly
 Thalidomide + Dexamethasone
 Bortezomib based regimen
 Curative Rx- offered to patients <65-
 Autologous SCT
 Supportive Rx-
 Epo-anemia, bisphosphonates-prevent fracture, dialysis-ESRD
 Px- ISS- β2 microglobulin

7. Amyloidosis
 Variety of conditions characterized by
deposition of amyloid
 Amyloid- an altered protein,
with β-pleated sheet structure
 Amyloid deposition leads to organ dysfunction
 Identified histologically by Congo red staining,
seen as apple-green birefringence under
polarized light

8. Classification
 AL- immunoglobulin light chains in MM
 AA- amyloid associated protien in chronic
inflammatory diseases- TB, RA, SSA
 Transthyretin- senile systemic amyloidosis
 β2 microglobulin- long-term hemodialysis
 Aβ- brain lesions in Alzheimer disease
 Amylin- pancreas of T2DM patients
 Calcitonin- medullary carcinoma of thyroid

9. Manifestation
 AL- nephrotic syndrome, CHF,
peripheral neuropathy, hepatomegaly,
carpal tunnel syndrome, macroglossia
 AA- nephrotic syndrome
 Transthyretin- peripheral neuropathy,
cardiac & renal involvement, purpura
 β2 microglobulin- joint involvement

10. Diagnosis
Biopsy of involved organ or
rectal submucosa/abdominal fat
Diagnostic ECHO, if heart involved

11. Treatment
Mainly supportive
Rx of underlying disease
Liver/kidney transplantation