Some facts about this disease:
* Malignant disorder of plasma cells
* Peak incidence between 60-70 years
* Rare under the age of 30
* Males are affected more than females.
Pathogenesis:
-The tissues are infiltrated by proliferating cells.
-One type of abnormal immunoglobulins (paraprotein) usually IgG or IgA.
-This paraprotein may cause auto-immune manifestations.
-The paraprotein may coat the platelets and coagulation factors.
-Production of incomplete immunoglobulins (light chain only) by plas. cells which are excreted in urine (Bence-Jones protein).
-Diminished production of normal immunoglobulins increases infections.
Clinical picture:
i- Skeletal:
1. Bone aches and pathological fractures
2. Multiple osteolytic lesions in the skull and osteoclast stimulation
ii- Neurological:
1. Compression of spinal cord by vertebral lesion
2. Infiltration of roots, nerves and muscles by plasma cells.
iii- Renal Failure: due to:
1. Bence-Jones proteins: precipitated in kidneys
2. Nephrocalcinosis: caused by hypercalcaemia
3. Hyperuricaemia: due to destruction of plasma cells
4. Amyloidosis of kidneys
5. Pyelonephritis: due to susceptibility to infections
iv- Anemia: due to:
1. BM (bone marrow) replacement by plasma cells
2. BM inhibition by chemotherapy and radiotherapy
3. Haemolysis caused by paraprotein
4. Renal impairment
v- Bleeding Tendency due to:
1. Coating of coagulation factors by paraprotein
2. Coating of platelets by paraprotein
vi- Cardio-Vascular
1. Raynoud’s phenomenon due to cold antibodies
2. Hyperviscosity syndrome
vii- Others:
1. Repeated infections
2. Amyloidosis
Investigations:
1- Blood picture:
* Anemia
* Increased ESR
2- BM Examination:
Full of plasma cells
3- Biochemical:
* Increased calcium in blood
* Increased uric acid in blood
* Alkaline phosphatase: normal
4- Immuno electrophoresis:
Shows marked increase in one type of immunoglobulins
5- Renal:
* Bence-Jones proteins
* Urine electrophoresis: for paraprotein and light chains
* Kidney function tests: may be impaired
6- X-ray:
Multiple osteolytic lesions in bones especially in vertebrae, skull and pelvis.
Treatment:
1- Chemotherapy:
Melphalan or cyclophosphamide.
2- Radiotherapy:
To control tumour masses.
3- Symptomatic:
e.g. infection, renal failure and pain.
Brought to you by Sayed EL Assal who is a physician graduated from faculty of medicine 7 years ago and since then he’s working in health field.
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