Multiple myeloma (kahler's disease)

Introduction

This is a malignant proliferation of plasma cells. Normal plasma cells are derived from B cells and produce immunoglobulins which contain heavy and light chains. Normal immunoglobulins are POLYCLONAL, but in myeloma plasma cells produce immunoglobulin of a single heavy and light chain, a MONOCLONAL protein called as paraprotein.

In some cases only light chain is produced and this appears in the urine as BENCE JONES PROTEINURIA.

The malignant plasma cells produce cytokines, which stimulates osteoclasts and result in net bone reabsorption which causes bone pain, fractures and hypercalcaemia.

Clinical features and investigations

Bone pain/ fractures

Lytic lesions

Nephrotic syndrome, carpal tunnel syndrome

Panda eyes

Renal failure due to paraprotein deposition, hypercalcaemia, infection, NSAIDs, amyloid.

Spinal cord compression

Retinal bleeds

Bruising

Heart failure

Cerebral ischaemia

Diagnosis of myeloma requires two of the following criteria:

1. Increase malignant plasma cells in bone marrow

2. Serum and/ or urinary M-protein

3. Skeletal lytic lesions.

Investigations

X-ray for skeletal survey

Alkaline phosphatase

MRI spine

Blood and urine protein electrophoresis done for the presence of M-protein

Blood and urine IMMUNOELECTROPHORESIS for the TYPE of M-protein

Bone marrow aspiration and trephine

Full blood count

Urea, creatinine, urate and electrolytes to access renal function

Blood calcium and albumin to know presence of hypercalcaemia.

Management

Immediate support

• High fluid intake to treat renal impairment and hypercalcaemia.

• Analgesia for bone pain.

• Bisphosphonates for hypercalcaemia.

• Allopurinol to prevent urate nephropathy.

• Plasmapheresis for hyperviscosity.

Chemotherapy with or without HSCT

First line treatment is with THALIDOMIDE and more recently with the BORTEZOMIB.

Thalidomide along with the alkylating agent MEPHALAN and PREDNISOLONE has increased the median overall survival to more than 4 years.

Treatment is administered untill PARAPROTEIN levels have stopped falling. This is termed as PLATEAU PHASE.

In UK at present, bortezomib is recommended followed by LENALIDOMIDE if there is subsequent progression.

Radiotherapy

This is effective for localised bone pain not responding to simple analgesia.

Bisphosphonates

Long term therapy reduces bone pain and skeletal events.

I/V ZOLEDRONATE in combination with anti-myeloma therapy confers a survival advantage over oral bisphosphonates.

Comments

Mechanism of Normal Labor

Mechanism of normal labor Definition: The series of movements that occur on the head in the process of adaptation during its journey through the pelvis is called mechanism of labor. Mechanism: In normal labor, the head enters the brim more commonly through the available transverse diameter (70%) and to the lesser extent through one of the oblique diameters. The position is either occipitolateral or oblique occipitoanterior. Left occipitoanterior is more common than right occipitoanterior as the left oblique diameter is encroached by the rectum. The engaging anteroposterior diameter of the head is either suboccipitobregmatic 9.5 cm or in slight deflexion-the suboccipitofrontal 10 cm. The engaging transverse diameter is biparietal 9.5 cm. As the occipitolateral position is most common, the mechanism of the labor in such position will be described. The principal movements are: Engagement Descent Flexion Internal rotation Crowning Extension Restitution External rotation Expulsion of

Alopecia (Baldness)

Alopecia: causes, diagnose, and treatment Alopecia : Alopecia means loss of hair. There are two types of alopecia. Non-cicatricial alopecia Cicatricial alopecia Difference between cicatricial and noncicatricial alopecia Alopecia Areata (AA) Etiology The pathogenesis of AA is uncertain and following factors are incriminated: Immunological factors: AA is considered an immunological disease because of: Association of AA with other auto-immune diseases (auto-immune thyroid disease, pernicious anemia, vitiligo, and atopy) Cytokines produced by dermal papillae in lesions not only attract lymphocytes to perifollicular region but also stimulate them to multiply. As opposed to normal hairs, strong major histocompatibility complex (class 1 and class 2) immunoreactivity found in affected follicles. 2. Genetic factors: AA may be present in some families, so it may have a genetic basis. 3. Emotional factors: In some patients, AA is precipitated by emotional stress. Epidemiology Prevalence: A

Hereditory spherocytosis

• This is the abnormality of red blood cells. • This is usually inherited as an AUTOSOMAL DOMINANT condition. • The most common abnormalities are deficiencies of BETA SPECTRIN or ANKYRIN. • Most cases are associated with an asymptomatic compensated chronic haemolytic state with spherocutes present on the blood film, a reticulocytosis and mild hyperbilirubinaemia. • Pigment GALLSTONES are present in up to 50% of patients and may cause chlolecystitis. The clinical course may be complicated by crisis : • A HAEMOLYTIC CRISIS occurs when the severity of haemolysis increases. • A MEGALOBLASTIC CRISIS follows the development of folate deficiency. • An APLASTIC CRISIS occurs in association with PARVOVIRUS B19 infection. Investigations • The blood film will show spherocytes but the direct Coombs test is negative, excluding immune haemolysis. • An OSMOTIC FRAGILITY TEST may show increased sensitivity to lysis in hypotonic saline solutions but is limited by lack of sensitivity and specificity. •

MBBS question bank

Search This Blog