Multiple myeloma

MULTIPLE MYELOMA

Most common primary malignant tumor of bone.

Multiple myeloma is a malignant clonal proliferation of plasma cells in the bone marrow with lytic bone leisions.

TYPES

Multiple myeloma (50%)

Solitary myeloma (25%)

Myelomatosis (15%)

Plasma cell leukemia (1%)

Extra skeletal - nasopharyngeal / oral cavity

EPIDEMIOLOGY

Age group : 50 – 60 yrs

Sex : M:F- 3:1

Site : common in axial skeleton & flat bones than long bones.

- spine , skull, pelvis , ribs , sternum, proximal femur

CLINICAL FEATURES

Bone pain is the most common symptom , which is relieved by rest.

Persistent localised bone pain signifies pathological fracture.

No swelling / deformity unless there is pathologic al #

Compressive myelopathy due to vertebral collapse .

CLINICAL HALLMARKS OF MM

Weight loss , pallor, bleeding tendencies, fatigue

Susceptibility to bacterial infections

Hypercalcemia

Renal failure

Bone destructions lytic lesions)

Presence monoclonal antibody

INVESTIGATIONS

Haemogram shows anaemia , leucopenia , thrombocytopenia

ESR is very high

Peripheral smear – NCNA with increased rouleaux formation. Leucoerythroblastic picture.

Total protein increased with A:G reversal.

S.Ca is increased, ALP normal.

Electrophoresis on serum or urine shows ‘M’ band in the region of gamma globulin.

Urine may show Bence Jones protein.

Bone marrow – hypercellular with increased no. of plasma cells & myeloma cells.

Mature & Immature plasma cells with eccenteric nuclei and ‘cart-wheel appearance’ of nuclear chromatin

RADIOLOGICAL FINDINGS

Multiple punched out lesions in skull & other flat bones.

M M with pathological #

Pathological wedge collapse of vertebra, commonly thoracic spine. Pedicles usually spared.

Diffuse severe rarefaction of bones.

Erosion of borders of ribs.

CT & MRI

Bone scan in solitarybone lesions helps to detect other sites of involvement.

S. beta 2 microglogulin is the single most powerful predictor of survival.

DIAGNOSTIC CRITERIA (DURIE & SALMON)

Major criteria1.BM plasmacytosis ( > or = 30% BM plasma cells)

2.Monoclonal globulin spike Ig G or Ig A

3.Light chain excretion in urine ; > or = 1g/24 hrs.

4.Biopsy proven plasmacytoma

Minor criterias1.BM plasmacytosis ; 10-30 % BM plasma cells

2.Monoclonal globulin spike gut < for major criteria.

3.Lytic bone lesions

4.Decrease in normal Ig level.

Diagnosis - I major + 1 minor criteria

3 minor criteria that must include no. 1& no.2

TREATMENT “Watchful waiting", where the progress of the disease is monitored .

Chemotherapy • Melphalan, prednisone, thalidomide (MPT)• Bortezomib (Velcade), melphalan, prednisone (VMP)• Lenalidomide plus low-dose dexamethasone

• Every 3-4 weeks for 6-12 cycles.

Radiotherapy • Severe bone pain , pathological # , tumor lesions.

Bone marrow transplantationBisphosphonates (e.g. pamidronate or zoledronic acid) are routinely administered to prevent fractures and erythropoietin to treat anemia.

Pathological # prevented by internal splinting of affected part.

#s managed surgically by internal fixation .

Treatment of other complications.

In established cases survival is 2-3 yrs

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