What is mutliple myeloma and how it affects your whole body

It is a malignant plasma cell disease that typically affects older individuals more than 70 years old. In particular, it is a malignancy of the bone marrow, a soft substance found in some bones. It could affect the spine, brain, pelvis, and ribs, among other body parts. Because the patients first exhibit no clear indications or symptoms, it is extremely difficult to identify.

 

Pasma cells in Mutiple Myeloma

 

 

Clinical manifestations of multiple myeloma

 

We will try to list the most common sign and symptoms of this disease.

Generalized bone disease that mainly affects vertebra, ribs, skull and sternum etc.

·      Persistent and continuous back pain that does not go away with the use of routine painkillers.

 

Myeloma cells affecting spine

 

·      Punched out lytic bone lesions

·      Pathological fractures

·      Hypercalcemia of malignancy due to release of calcium by weakened bones in the blood that in turn causes other symptoms like lethargy, dehydration, confusion and weakness.

 

 

·      Normocytic normochromic anaemia due to reduced production of erythropoietin as the result of kidney damage.

·      Primary amyloidosis, which damages the endothelium of the blood vessels.

·      Peripheral neuropathy, which can cause numbness, pins and needles, or tingling owing to the build-up of amyloid in the skin's soft tissues or can cause carpal tunnel syndrome by affecting the median nerve in your hands.

·      Radiculopathy that includes impingement of origins of nerve roots from the spinal cord owing to the vertebral fractures.

·      Renal failure, which includes proteinuria, where Para proteins, such as Bence-Jones proteins, exceed the body's capacity to reabsorb them.

·      Kidney stones due to excessive light chain proteins like lambda and kappa or due to increased calcium contents in your blood.

·      Recurrent infections due to immunosuppression of other immunoglobulin.

·      Shortness of breath, headache and general fatigue due to hyper viscosity syndrome as M proteins are everywhere in the body.

 

Hperviscosity syndrome

 

·      Local tumours that may appear as local masses.

 

Pathophysiology of multiple myeloma

 

Osteoblasts, osteoclasts, and stromal cells that support blood synthesis can be found in your bone. Multiple myeloma, as its name implies, is a malignancy of the myeloid tissue that also goes by the name monocolonal plasmacytoma since it comprises more than 10% of plasma cells that come from the same colony.

These plasma cells or cancer cells emit cytokines that promote cell development and migration to the site of the damaged area. This leads to a poor chain of events, such as a reduction in osteoblast formation and an increase in osteoclast activity, as well as the proliferation of malignant cells.

 

Multiple myeloma cells

 

Additionally, it could result in more para proteins being produced, which are short, light-chain proteins that can easily flow through the kidney tubules, causing injury to the kidneys and proteinuria. But none of these proteins is albumin; rather, they are all globulin in origin. This malignancy affects your humoral immunity because it is a B-lymphocyte cancer.

·       

Investigations for multiple myeloma

 

·      Blood test to check for weak blood, higher levels of calcium

·      Renal function tests like increase in urea and creatinine and BUN

·       Serum alkaline phosphatase will be within normal range.

 

 

·      Urine dipstick is usually normal so it does not hep to diagnose this condition.

·      Urine immuno electrophoresis detects Bence-Jones proteins. 

 

 

·      Bone examination includes aspiration of bone marrow that shows abundant plasma cells.

·      X-ray of bone shows thinning of bones or osteoporosis.

·      CT scan of bone shows lesion of bones and other soft tissues.

 

Early onset of multiple myeloma seen in skull

 

 

Diagnosis of multiple myeloma

 

It really depends upon presence of these four factors like

·      Hypercalcemia

·      Renal failure

·      Anaemia

·      Bone lesion 

 

 

 

Complications of multiple myeloma

 

·      Complications of multiple myeloma includes renal tubular acidosis and dehydration due to high calcium in the body.

·      Mostly people may develop leukaemia later (ALL).

·      Acute renal failure

·      Presence of immature red blood cells and immature white blood cells in the blood

 

Treatment of multiple myeloma

 

Chemotherapy, steroids, thalidomide, or bortezomib are all used in its treatment. But be mindful of their side effects, which may include hair loss, neuropathy, or in the case of thalidomide, blood clot development. Your doctor will give you additional medications to treat these issues because they work well at killing myeloma cells.

 

treatment of multiple meyeloma

 

 

            Autologous stem cell transplant in which you will be your own donor.

Painkillers

Radiotherapy is used further for bone pains

Use of certain special medicines like bisphosphonates

Blood transfusion

Surgery in case of oncological emergency or pathological fractures

Dialysis along with plasma exchange in the case of renal failure

 

 

 

 

 

Why abdominal aortic aneurysms are life-threatening?

Abdominal aortic aneurysm occurs when a portion of the aorta bulges out more than 3cm in diameter. It can affect any artery in your body, although the aorta is the most usually affected. The blood vessel wall becomes weak and fragile, and it has an 80 per cent chance of rupturing. This is usually classified as a vascular emergency that necessitates rapid medical attention in order to save the person's life.

abdominal aortic aneurysm

 

 

Signs and symptoms of AAA

 

We'll try to list a few of these symptoms for your information.

 

Lower middle back, loin or pelvic pain

Dizziness

Light-headedness

Pulsating sensation in the abdomen

Faster heartbeat

Sweaty, pale and clammy skin

Fainting or passing out

Weight loss

 

Who is at the risk of developing abdominal aortic aneurysm?

 

The person who has history of

Hypertension

Diabetes mellitus

COPD

Raised cholesterol level

Positive family history of abdominal aortic aneurysm

Because smoking hardens the walls of blood arteries, making them more prone to dilatation, there is a history of smoking.

 

Causes of abdominal aortic aneurysm

Ageing

 The loss of elastic lamellae, deterioration of their endothelium by leukocyte infiltration into the vessels, and loss of smooth muscle cells coupled with their proteolysis all enhance your chances of developing an aortic aneurysm as you become older.

As a result, in most nations, males over the age of 65 and women over the age of 70 undergo screening.

 

 

Atheroma development

 

 The build-up of fatty substances or fat-like tissue alongside the lining of your blood vessels is known as atherosclerosis. Most of the time, atheroma will line your aortic aneurysm. The formation of these aggregates within your blood vessel walls can be caused by a variety of factors, including:

smoking

 

Male gender

Hypertension

Increased Blood cholesterol

Obesity

Chronic obstructive pulmonary disease

Lack of regular exercise

Unhealthy eating habits

All of these factors have the potential to cause major health problems such as heart attacks and strokes.

Your genetic makeup plays a key part in putting you at risk for aneurysms.

Other persons who may develop these include those who have had a blood vessel injury, those who have been infected with tuberculosis or HIV, and those who have inherited diseases such Marfan's syndrome.

 

Examination

You doctor may examine your abdomen to detect any aneurysm by performing bimanual palpation of your abdomen above the umbilicus.

 

Sometimes, he can find flank bruising due to rupture of abdominal aortic aneurysm.

 

Investigations

 

The blood tests include full blood count, renal and clotting profile. ESR and CRP if any inflammatory disease is suspected.

Abdominal X-ray

Abdominal ultrasound to check the size and shape of aneurysm.

Abdominal CT scan for more details to know about aneurysm affecting the origin of other arteries like renal arteries coming from the aorta.

 

ultrasound scan of aortic aneurysm

 

 

Treatment

 Unfortunately, some non-modifiable risk variables, such as male gender or rising age, as well as some modifiable risk factors, such as diabetes and high blood pressure, increase the risk of rupture.

The rupture will cause a rush and pressure of blood inside the aorta, which it will not be able to hold, causing the aneurysm to burst. This can result in significant internal bleeding, which can be fatal. The type of treatment is determined by the size of the aneurysm.

If it is little or less than 5.5 cm in diameter, surgery is not recommended because the danger of death from surgery outweighs the chance of rupture. Serial ultrasound scans will be performed to see if the size of aneurysm is increasing.

 

Your vascular surgeon will operate if the aneurysm is more than 5.5 cm in diameter, however due to the patient's general poor health, this decision can be tough. However, if an aneurysm ruptures, a team of experts, including surgeons and anaesthetists, must prepare for an emergency treatment, which in 80 per cent of cases fails owing to internal bleeding.

 

When it comes to aneurysm surgery, there are two options to think about.

 

 

There are two types of procedures to consider for operating the aneurysm

1-Open abdominal surgery by performing laparotomy

2-Endovascular surgeries, in which the draft is used to support the weak part of blood vessel through the femoral artery.

Open surgey for aortic aneurysm

 

 

Complications

 

Rupture of aorta

Aortic dissection

Blood clots Low blood pressure, slow breathing, palpitations, and abrupt back discomfort are all signs of a shock-like condition that necessitates immediate medical attention.

 

Lifestyle advice when the aneurysm is small less than 4.4cm

 

Atherosclerosis can cause atheroma plaques to form in other parts of the body, such as arteries leading to the brain and coronary arteries leading to the heart, resulting in major consequences.

As a result, patients with minor aneurysms should follow some general guidelines.

 

Quit smoking.

150 minutes of physical activity each week is recommended.

Limit your salt intake.

At least five pieces of vegetables and fruits should be consumed each day.

Alcohol should be consumed in moderation.

This is how we can diagnose and manage Giant cell arteritis

Giant cell arteritis(temporal arteritis) is an autoimmune illness that affects the body’s large arteries, particularly the temporal artery.  The temporal artery is placed on the side of the head, and it becomes irritated for no apparent reason, resulting in blockage, inflammation, and prominence of the temporal artery beneath the skin.Females over the age of 60 are the most typically affected by this disease, for no apparent cause. 

 

 

Symptoms of Giant cell arteritis

The clinical manifestations are as follows:

·      Patients typically report a sudden or slow-growing headache for days or weeks. The intensity of a headache is severe, and it occurs frequently.

·      When chewing food or talking, jaw pain can occur.(jaw claudication)

·      Inflamed vessels beneath the epidermis make the scalp tender and painful to touch.

·      Visual problems, this may include acute loss of vision. There are also other unusual symptoms patients may present with;

·      Flu like illness or persistent fever

·      Continuous cough

·      Depression

·      Exhaustion

·      Loss of appetite

·      Lack of energy

·      Sleep deprivation

·      Weight reduction that is unexpected.

 

 

Diagnosis of giant cell arteritis

 

Initially, Some blood tests to detect the intensity of inflammations are usually requested for this type of patient such as CRP and ESR .In this case, an increased ESR value is a critical and sensitive. Regardless of the results of the temporal artery biopsy, if it is more than 40mm/hr, your doctor should start steroids right once to avoid the most catastrophic consequence like loss of vision.

 If its suspicion has been established then temporal artery biopsy is performed to conform the diagnosis, which involves the removal of a part of this artery under the effect of local anaesthetic injection and then this sample is examined under the microscope to detect the pathological granulomatous inflammatory changes in the temporal artery if any. In some patients the biopsy report comes normal but this does not rule out the condition.

  As it is a day care procedure, there is no need to stay in hospital for long. Your doctor will prescribe you with some simple painkillers and topical antibiotic cream to apply on the wound to avoid any further complication. Following the removal of the dressing, you can resume typical activities such as driving and working. To avoid wound infection, you should avoid going in the dirt or in an open area.

 

Temporal artey biopsy

 

Liver function tests usually indicate a higher level of serum alkaline phosphatase, which is of no use in confirming the diagnosis.

Cranial ultrasound that is a new way to investigate this disease but further research needed for this.

Magnetic resonance angiography

CT angiography

PET scanning

 

 

Treatment of Giant cell arteritis

 

High doses of steroids, such as prednisolone, are used to treat this condition due to their anti-inflammatory properties.

The treatment is provided in two stages to the patients.

The first step of treatment comprises a very high dose of steroids orally for weeks or months, after which it is gradually decreased to the lowest feasible maintenance dose of steroid under the supervision of a medical doctor in the second phase of treatment. This treatment lasts for almost three years.

However, in the case of sight loss, the treatment could be different, such as injectable steroids to alleviate the problem as much as possible.

Even if this arteritis isn't diagnosed yet, your doctor will start you on steroids to prevent disease complications. You'll also be told to take low-dose aspirin on a daily basis to prevent inflammation and complete blockage of other arteries in your head and neck, which could lead to a catastrophic condition like stroke.

 

When you use steroids, you're more likely to get stomach ulcers. Your doctor prescribes proton pump inhibitors to protect your stomach. Try not to use other anti-inflammatory drugs without doctor’s advice because their use may lead to stomach ulcers more rapidly.

 

Other drugs known as bisphosphonates are frequently recommended to prevent bone weakening, which is a side effect of steroid treatment.

 

You've probably figured out that this is an inflammatory condition in which your immune system becomes overly reactive to your own body tissues, producing substances like interleukin and causing immune cells to migrate to the site of action. Your doctor may prescribe an immunosuppressant such as methotrexate to prevent the disease from recurring and to keep you free of symptoms for long time.

 

Side effects of taking steroids 

 

There is a chance that you will acquire certain steroid-related side effects if you use steroids.

We'll try to sketch down some of them in case you come up with any of them, as well as some solutions to go along with them.

 

High blood sugar

These medications can cause blood sugar levels to rise, which can lead to diabetes. For that, try to keep an eye on your carbohydrate and sugar intake.

 

High blood pressure

As a result of the increased volume burden on your body, you may develop hypertension. Regular exercise can be beneficial to combat this issue.

 

Osteoporosis and avascular necrosis of bones

 Patients who take a larger dose of steroids are more likely to develop osteoporosis, which is characterised by bone weakening. It could indicate that your bone mineral density is low, making you more susceptible to fractures.

Special types of drugs, such as bisphosphonates, are normally recommended to be used on a regular basis to avoid this. Your doctor may also encourage you to consume more dairy products, such as milk and cheese.

To maintain bone health, you will also be encouraged to take supplements such as vitamin D and calcium-containing pills.

Avascular necrosis of the bones is an uncommon complication that can arise as a result of steroid treatment. The blood supply to the affected bone or joint will be entirely cut off. Same dietary changes are recommended.

 

Diet recommendation for osteoprosis

 

Weight gain

Another issue you may encounter is weight gain. To address these problems, a low-calorie diet, moderate exercise, and adequate water consumption are required.

Low mood and disturbed sleep

You should expect mood swings, such as feeling depressed, irritated, or energised at times. It's also possible that this will disrupt your sleep.

If you're having any of these issues, talk to your doctor.

 

Eye problems

If you use more than 25mg of high-dose steroids per day, you are at risk of developing cataracts and glaucoma.  If any of these are bothering you, make an appointment with your ophthalmologist. They may conduct some ocular tests to ensure that you are in good health.

Because every adverse effect does not occur in every individual, the benefits of taking steroids and their negative effects should be carefully discussed and weighed with your healthcare professional.

In addition, side effects are affected by other factors too such as the length of time you've been taking drugs and the dose you're taking.

 

Side effects of steroids

 

 

Complications of giant cell arteritis

 Polymyalgia rheumatica and aortic aneurysm can affect up to two-thirds of patients with giant cell arteritis. Aortic aneurysm is an abnormal dilation of the body's main blood vessel, the aorta, which can rupture as a result of the inflammatory insult and cause mortality.

This disease can affect the heart and other essential organs, such as the brain, resulting in a myocardial infarction or stroke.

 

Polymyalgia rheumatica