Pathology of multiple myeloma

2021-01-26 12:00 AM

Due to its origin and function, the cytoplasm is present in most organs and organs in the body, so when malignant proliferation will manifest symptoms in many places.

Define

Multiple myeloma is a malignant proliferation of cytoplasm (plasmocyte) in the bone marrow and several other organs.

Cell proliferation leads to:

Increased immunoglobulins in the blood.

The formation of many bone fissures leads to pathological fractures.

Dysfunction of many organs: kidney failure, anaemia, hypercalcemia, neurological symptoms ...

Epidemiology

The rate of disease is about 3-4 / 100,000 people; 1 - 2% of malignancies.

Common age:> 40; male> female.

Blacks are twice as many as whites and their onset is earlier.

The cause and pathogenesis remain unclear. However, epidemiological studies show that low doses of radiation increase morbidity.

Symptoms

Due to its origin and function, the cytoplasm is present in most organs and organs in the body, so when malignant proliferation will manifest symptoms in many places. The disease usually starts slowly with increasing dissipation symptoms, so it is easy to confuse it with other diseases: systemic depression, manifestations in bones, kidneys, blood, nerves ...

Expression in bones

Clinical:

Bone pain (90%): Common locations are in the spine, pelvis, chest, pain increases with movement and decreases with rest, after that, pain is continuous, sometimes pain in the form of pressing nerve roots.

Natural fractures (50%): fractures of the ribs, collarbone, sternum, subsidence and displacement of the spine, causing scoliosis. Usually detected by x-ray.

Bone tumours: 10% of patients have this symptom; Painless molluscum contagiosum on bone base, between 0.5 cm and 2 cm in diameter; often found in positions such as the skull, collarbone, sternum, scapula, spine ... rarely seen in limbs.

X-ray:

Cavity-shaped bone resorption: on the skull, ribs, spinal pelvis ... the holes are round or oval, with clear boundaries, ranging in size from a few millimetres to several centimetres, looking like a hollow bone.

Partial or partial bone resorption in long bones.

When the cavities are small and dense, diffuse osteoporosis is formed.

The body of the vertebrae is deformed (concave, flat, tongue-shaped), can be displaced, causing hunchback.

Currently, it is possible to use stroboscopic radiation, density tomography, and magnetic resonance imaging to detect lesions early.

Extrinsic manifestations

Body: Thinness, fatigue, loss of appetite, pale skin, persistent fever that lasts.

Kidney: Kidney damage is seen in 70% of cases.

Proteinuria is usually a thermostatic (light chain: Bence Jones) protein, coagulated at 56 ° C and dissolved when boiling.

Chronic renal failure.

Anuria: Suddenly, due to the rapid increase in globulin in the blood, the renal tubules are blocked.

Blood: Anaemia, bleeding due to thrombocytopenia.

Nervous: Direct compression of tumours or damage from immunoglobulins.

Squeeze the marrow and nerve roots.

Damage to the cranial nerves.

Polyneuritis.

Increased intracranial pressure.

Damage to the bottom of the eye.

Hypercalcemia:

Lethargy, fatigue, depression, confusion.

Vomiting, water and electrolyte disturbances, perception disorders, coma.

Other manifestations:

Easy to infect with superinfection.

Liver, spleen, lymphadenopathy.

Digestive disorders.

Faint infiltrates in the lungs (plasmocyte).

Amyloid infection in some organs.

Haematological and biochemical changes

Plasmocytic

Puncture: Bone tumours on the skin or areas with bone resorption (on the X-ray) cytology will see a lot of cytoplasms.

Bone marrow: cytoplasm increased> 15% (normal <5%). In the peripheral blood, the cytoplasm is rarely seen.

Morphologically, this cytoplasm can be normal, maybe large and irregular, the nucleus has many seeds.

Monoclonal gamma globulin increase

Due to the pathological cytoplasm secreted, mainly IgG, IgA, IgD and IgE are rarely seen, Increased gamma globulin is shown by:

High blood sedimentation rate (increased erythrocyte adhesion).

Health Protide increased much.

Serum protein electrophoresis revealed a large increase in gamma globulin.

Immune electrophoresis: 1 increase in immunoglobulins: IgG, IgA, IgD, IgE ...

Urine: 50% contain Bence Jones protein.

Other tests

Increased blood calcium (> 105mg / l) is a consequence of extensive, widespread bone resorption.

Red blood cells and platelets decrease in the blood.

Decreased kidney function.

Diagnose

Implementing the quadrants

Clinical: A au bone, bone tumours.

X-ray: Osteoporosis shaped cavity, diffuse osteoporosis.

Plasmocyte increases Tumour puncture, myelogram.

Test for blood protein, electrophoresis, and urine.

Differential diagnosis

Bone tumours due to metastatic cancer.

Diseases that cause bone resorption and osteoporosis: hyperparathyroidism, postmenopausal osteoporosis, primary osteoporosis.

Blood diseases are present in the bones.

Disease forms

Diffuse forms of calcium loss like osteoporosis (Lièvre and Weissenbach).

Non-institutional and no secretion of gamma globulin, only X-ray images, no changes in the fluid.

A lone tumour.

Can coordinate with Leucémie.

The globulin type: light chain form, IgM form, combination 2 clone.

Diagnose the stage of the disease

Stage I: Including the following criteria: (≥ 1).

Haemoglobin > 100g/l.

Blood calcium <3mmol / l (<12 mg%).

Normal bone x-ray or a single lesion.

Immune electrophoresis: IgG <50g / l

IgA <30g / l.

Light chain urine <4g / 24 hours.

Phase II: G iua phase I and III.

Stage III: ≥ 1 of the following standards:

Haemoglobin < 85g/l.

Blood calcium> 3mmol / l.

XQ: N understand lesions.

Immune electrophoresis: IgG> 70g / l.

IgA> 50g / l.

Light chain urine> 12g / 24 hours.

Based on kidney function:

A: Creatinine blood < 177 μmol/l (<2mg%) B: Creatinine blood > 177 μmol/l (>2mg%)

The standard combination of stage and kidney function evaluated the prognosis of the disease (Dan.Longo):

IA live average: 61 months.

IIA, B live average: 55 months.

IIIA lives an average of 30 months.

Average life of IIIB: 15 months.

Treatment

Multiple myeloma is a disease that causes damage to the bone marrow and many external organs due to the malignant proliferation of the cytoplasm. Consequently, treatment includes the following major problems: Bone pain and bone destruction, hypercalcemia, myelosuppression with persistent anaemia, thrombocytopenia and leukopenia, renal failure and superinfection.

Most patients require chemotherapy to control tumours and symptomatic treatment to prevent complications.

Root treatment

Intravenous infusion in batches, in the following types: Phenylalanine Mustard (L-PAM, Melphalan): 8mg / m2 / day.

Cyclophosphamide 200 mg/m2/day.

Coordinate with Prednisolone 25-60 mg / m2 / day. Each session 4-6 days, 4 -6 weeks apart.

It can then be administered orally with a dose.

Melphalan: 1-3 mg / day Cyclophosphamide: 1 - 2mg / kg / day Prednisolone: ​​1mg / kg / day.

Use 1 - 4 weeks in instalments.

Treatment time lasts 1 - 2 years. Follow closely clinically and laboratory to adjust drugs.

High doses of steroids that are administered by intravenous alone may be used:

200mg Prednisolone every other day.

Or Methylprednisolone 1g / m2 / day x 5 days.

Alternate combination chemotherapy can be used:

Vincristine 0.4 mg / day x 4 days.

Next: Doxorubicin 9mg / m2 / day x 4 days.

Next Dexamethasone 40mg / day x 4 days.

Other treatment

Irradiation with localized, slow-progressing, ectopic tumour.

Surgery: single tumour, releasing compression.

Combined symptomatic treatment:

Blood transfusion.

Antibiotics.

Dialysis.

Calcitonin.

Diuretic.

Progression and prognosis

15% die in 3 months, 15% die in 1 year, the rest are chronic from 2 to 5 years.

Cause of death:   

Kidney failure.

Sepsis.

Spread of the tumour.

Heart attack, chronic lung disease.

Diabetes, stroke

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