THE PARAPROTEINAEMIAS

Multiple myeloma

Multiple myeloma is a malignant disease of the plasma cells of bone marrow, accounting for 1% of all malignant disease. There is clonal proliferation of bone marrow plasma cells usually capable of producing monoclonal immunoglobulins (paraproteins), which in most cases are IgG or IgA. The paraproteinaemia may be associated with excretion of light chains in the urine (Bence Jones protein) which are either kappa or lambda; sometimes there are light chains without a paraproteinaemia.

Clinical features

The peak age of presentation is 60 years. There is:

■ Bone destruction - increased osteoclastic activity causes bone pain (back ache is the most common presenting symptom), osteolytic lesions, patho-logical fractures, spinal cord compression and hypercalcaemia.

■ Bone marrow infiltration with plasma cells resulting in anaemia, infec-tions and bleeding.

■ Acute kidney injury has multiple causes: deposition of light chains in the tubules, hypercalcaemia, hyperuricaemia and amyloid deposition in the kidneys.

Paraproteins may form aggregates in the blood, which greatly increase the viscosity, leading to blurred vision, gangrene and bleeding. Infections are also due to a reduction in the normal polyclonal immunoglobulin levels (immune paresis).

Investigations

Two out of three diagnostic features should be present:

■ Paraproteinaemia on serum protein immunofixation or Bence Jones protein in the urine.

■ Radiological evidence (CT, MRI) of lytic bone lesions.

■ An increase in bone marrow plasma cells on bone marrow aspirate or trephine biopsy.

Other essential investigations are as follows:

■ Blood count, which may show anaemia, thrombocytopenia and leuco-penia. The ESR is almost always high.

■ Serum biochemistry may show evidence of renal failure and hyper-calcaemia. The alkaline phosphatase is usually normal.

■ Serum β2-microglobulin and albumin are used in prognosis.

Management

With good supportive care and chemotherapy with autologous stem cell transplantation, median survival is now 5 years with some patients surviving to 10 years. Young patients receiving more intensive therapy may live longer.

Supportive therapy includes correction of anaemia with blood transfusion or erythropoietin, prompt treatment of infections, and treatment of bone pain with radiotherapy or high-dose dexamethasone. Acute kidney injury (p. 383) and hypercalcaemia (p. 651) may be corrected by adequate hydration alone. Progression of bone disease is reduced by bisphosphonates e.g. zoledronate, which inhibit osteoclastic activity. Hyperviscosity is treated by plasma-pheresis together with systemic therapy.

Specific treatment involves combination chemotherapy with melphalan (an alkylating agent), prednisolone and thalidomide. Younger patients (<65-70 years) are treated with high-dose melphalan with peripheral blood stem cell rescue. Lenalidomide, a thalidomide analogue, and bortezomib, a proteosome inhibitor, are currently used for relapsed myeloma.

Monoclonal gammopathy of undetermined sigmíicance

This is usually seen in older patients, where a raised level of paraprotein (usually IgA) is found in the blood, but without other features of myeloma. Patients are often asymptomatic and no treatment is required. Follow-up is necessary as 20-30% go on to develop multiple myeloma over a 25-year period.

Ebook Essentials of Kumar and Clark's Clinical Medicine, 5e

1. Ethics and communication

Ethics and communication

2. Infectious diseases

Infectious diseases

3. Gastroenterology and nutrition

Gastroenterology and nutrition

4. Liver, biliary tract and pancreatic disease

Liver, biliary tract and pancreatic disease
LIVER BIOCHEMISTRY AND LIVER FUNCTION TESTS
SYMPTOMS AND SIGNS OF LIVER DISEASE
JAUNDICE
HEPATITIS
NON - ALCOHOLIC FATTY LIVER DISEASE (NAFLD)
CIRRHOSIS
COMPLICATIONS AND EFFECTS OF CIRRHOSIS
LIVER TRANSPLANTATION
TYPES OF CHRONIC LIVER DISEASE AND CIRRHOSIS
PRIMARY SCLEROSING CHOLANGITIS
BUDD - CHIARI SYNDROME
LIVER ABSCESS
LIVER DISEASE IN PREGNANCY
LIVER TUMOURS
GALLSTONES
THE PANCREAS
CARCINOMA OF THE PANCREAS
NEUROENDOCRINE TUMOURS OF THE PANCREAS

5. Haematological disease

Haematological disease
ANAEMIA
Assessment and treatment of suspected neutropenic sepsis
HAEMOLYTIC ANAEMIA
INHERITED HAEMOLYTIC ANAEMIAS
ACQUIRED HAEMOLYTIC ANAEMIA
MYELOPROLIFERATIVE DISORDERS
THE SPLEEN
BLOOD TRANSFUSION
THE WHITE CELL
HAEMOSTASIS AND THROMBOSIS
THROMBOSIS
THERAPEUTICS

6. Malignant disease

Malignant disease
MYELOABLATIVE THERAPY AND HAEMOPOIETIC STEM CELL TRANSPLANTATION
THE LYMPHOMAS
THE PARAPROTEINAEMIAS
PALLIATIVE MEDICINE AND SYMPTOM CONTROL

7. Rheumatology

Rheumatology
COMMON INVESTIGATIONS IN MUSCULOSKELETAL DISEASE
COMMON REGIONAL MUSCULOSKELETAL PROBLEMS
BACK PAIN
OSTEOARTHRITIS
INFLAMMATORY ARTHRITIS
THE SERONEGATIVE SPONDYLOARTHROPATHIES
Clinical features, Investigations
INFECTION OF JOINTS AND BONES
AUTOIMMUNE RHEUMATIC DISEASES
SYSTEMIC INFLAMMATORY VASCULITIS
DISEASES OF BONE
THERAPEUTICS

8. Water, electrolytes and acid–base balance

WATER AND ELECTROLYTE REQUIREMENTS
BODY FLUID COMPARTMENTS
REGULATION OF BODY FLUID HOMEOSTASIS
PLASMA OSMOLALITY AND DISORDERS OF SODIUM REGULATION
DISORDERS OF POTASSIUM REGULATION
DISORDERS OF MAGNESIUM REGULATION
DISORDERS OF ACID - BASE BALANCE
THERAPEUTICS

9. Renal disease

Renal disease
INVESTIGATION OF RENAL DISEASE
GLOMERULAR DISEASES
NEPHROTIC SYNDROME
URINARY TRACT INFECTION
TUBULOINTERSTITIAL NEPHRITIS
HYPERTENSION AND THE KIDNEY
RENAL CALCULI AND NEPHROCALCINOSIS
URINARY TRACT OBSTRUCTION
ACUTE RENAL FAILURE/ACUTE KIDNEY INJURY
CHRONIC KIDNEY DISEASE
RENAL REPLACEMENT THERAPY
CYSTIC RENAL DISEASE
TUMOURS OF THE KIDNEY AND GENITOURINARY TRACT
DISEASES OF THE PROSTATE GLAND
TESTICULAR TUMOUR
URINARY INCONTINENCE

10. Cardiovascular disease

COMMON PRESENTING SYMPTOMS OF HEART DISEASE
INVESTIGATIONS IN CARDIAC DISEASE
CARDIAC ARRHYTHMIAS
HEART FAILURE
ISCHAEMIC HEART DISEASE
RHEUMATIC FEVER
VALVULAR HEART DISEASE
PULMONARY HEART DISEASE
MYOCARDIAL DISEASE
CARDIOMYOPATHY
PERICARDIAL DISEASE
SYSTEMIC HYPERTENSION
ARTERIAL AND VENOUS DISEASE
ELECTRICAL CARDIOVERSION
DRUGS FOR ARRHYTHMIAS
DRUGS FOR HEART FAILURE
DRUGS AFFECTING THE RENIN - ANGIOTENSIN SYSTEM
NITRATES, CALCIUM - CHANNEL BLOCKERS AND POTASSIUM - CHANNEL ACTIVATORS

11. Respiratory disease


Respiratory disease
TUBERCULOSISnd
DIFFUSE DISEASES OF THE LUNG PARENCHYMA
OCCUPATIONAL LUNG DISEASE
CARCINOMA OF THE LUNG
DISEASES OF THE CHEST WALL AND PLEURA
DISORDERS OF THE DIAPHRAGM

12. Intensive care medicine

Intensive care medicine

13. Drug therapy, poisoning, and alcohol misuse

Drug therapy, poisoning, and alcohol misuse

14. Endocrine disease

Endocrine disease
PITUITARY HYPERSECRETION SYNDROMES
THE THYROID AXIS
MALE REPRODUCTION AND SEX
FEMALE REPRODUCTION AND SEX
THE GLUCOCORTICOID AXIS
THE THIRST AXIS
DISORDERS OF CALCIUM METABOLISM
DISORDERS OF PHOSPHATE CONCENTRATION
ENDOCRINOLOGY OF BLOOD PRESSURE CONTROL
DISORDERS OF TEMPERATURE REGULATION
THERAPEUTICS

15. Diabetes mellitus and other disorders of metabolism

DIABETES MELLITUS
DIABETIC METABOLIC EMERGENCIES
COMPLICATIONS OF DIABETES
SPECIAL SITUATIONS
HYPOGLYCAEMIA IN THE NON - DIABETIC
DISORDERS OF LIPID METABOLISM
THE PORPHYRIAS

16. The special senses

THE EAR
THE NOSE AND NASAL CAVITY
THE THROAT
THE EYE

17. Neurology

COMMON NEUROLOGICAL SYMPTOMS
COORDINATION OF MOVEMENT
THE CRANIAL NERVES
COMMON INVESTIGATIONS IN NEUROLOGICAL DISEASE
UNCONSCIOUSNESS AND COMA
STROKE AND CEREBROVASCULAR DISEASE
EPILEPSY AND LOSS OF CONSCIOUSNESS
NERVOUS SYSTEM INFECTION AND INFLAMMATION
HYDROCEPHALUS
HEADACHE, MIGRAINE AND FACIAL PAIN
SPINAL CORD DISEASE
DEGENERATIVE NEURONAL DISEASES
DISEASES OF THE PERIPHERAL NERVES
MUSCLE DISEASES
MYOTONIAS
DELIRIUM
THERAPEUTICS

18. Dermatology

Dermatology

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