ANEAMIA

NOTES : -There are no slides for this lecture. - The book is your reference.

- The difference between anemia and bleeding disorders has to be very distinct. Bleeding disorders :inclination of the patient to have bleeding from any small cut . anemia :can be defined as either of the following: Decrease in number of circulating RBCs. Hemoglobin level below normal (the most important indication). * for adult females below 11.5 g/dl. * For adult males below 13.5 g/dl * For the under age children(<11 years old) below 11 g/dl Haematocrit below the normal range. *hematocrit: how many cells in plasma. We have something called MCV (mean corpuscular volume) which gives us an idea about the size of the blood cell that indicates the different types of anemia. MCV Patient hematocrit / RBCs count. Normal MCV: 79-98 fl. Therefore, indication of types anemia will be: Macrocytic anemia : > 98 fl. Microcytic anemia: < 79 fl (the most common anemia due to iron deficiency). Normocytic anemia: 79-98 fl

SO hemoglobin level and MCV are important to classify patients as anemic.

General Clinical features of anemia (symptoms): - Chronic fatigue - headache . - fainting.(not specific) - Breathlessness (very important indication) - Angina pectoris.(chronic anemia can cause Ischemic heart disease) - Palpitations. - Ankle edema. - Intermittent claudication (pain in the muscles of the leg upon walking) - Dyspnea. Note:
*Symptoms: what the patient complain from *Signs: what the clinician can see

Nonspecific Signs of anemia: 1. Pallor but not jaundice. * Jaundice is a yellowish pigmentation of the skin

*Pallor is reduced amount of oxy hemoglobin in skin or mucous membrane, a pale color which can be caused by illness, emotional shock or stress, stimulant use, or anemia. 2. Tachycardia- more than 100 beat /min ( NORM 60-100 beat /min). 3. palpitation 4. Full pulse. * Tachycardia is something you can hear * Pulse is something you count 5. systolic flow murmur. 6. congestive cardiac failure due to lower oxygen-carrying capacity of blood that leads to ankle edema. (Heart is normal but blood is abnormal). 7.rarely they develop papilledema and retinal hemorrhage in an acute bleed .

Specific signs for severe anemia: 1. Koilonychias: spoon-shaped nails, usually we see it in iron deficiency anemia.

2. Jaundice may be in some types of anemia mainly hemolytic anemia(destructed RBCs will increase the bilirubin level which leads to jaundice) 3. Bone deformities; mainly in thalassemia major and sickle cell anemia. 4.leg ulcers: mainly in sickle cell anemia

Note: Normochromic-normal color of RBCs(red) Normocytic-normal average RBC size Hyperchromic-very reddish color of RBCs Macrocytic-large average RBC size>99 fl Hypochromic-RBCs are paler than normal (low redness) Microcytic- small average RBC size

Classification of anemia:
Types of anemia Hypochromic Microcytic (MCV is low <79 fl) -RBCs on the blood film are small . -low Iron content -high iron binding capacity to compensate the deficiency. Examples Iron deficiency. (most common anemia)

Thalassemia.( pseudo plastic anemia)

-inherited autosomal recessive blood disorders caused by the weakening and destruction of red blood cells -Small RBCs -Low MCV -Normal iron content-the patient source of consumption and absorption of iron is normal but the problem in the RBCs itself.

Hyperchromic Macrocytic

Vitamin B12 deficiency Malabsorption (Post-gastrectomy) Foliate deficiency , hemolysis Aplastic anemia, liver diseases, hypothyroidism. Chronic diseases. Renal failure. Hemolysis. Hypothyroidism.

Normochromic Normocytic normal MCV

Microcytic anemia

Type 1--Iron deficiency anemia-It is the most common one of all anemia. -Small RBCs found in blood films. - MCV <79 fl. - Low iron content. This type of anemia requires good diet it gives us around (15-20 mg)of iron .The amount of iron absorbed from various foods ranges from around 1-10% from plant foods and 10-20% from animal foods. Iron absorption occurs in duodenum and jejunum so any patient has diseases in duodenum and jejunum will be anemic. *Ferrous form of iron is better in absorption than ferric form. *Gastric acid is needed for the adequate conversion of iron salts from ferric to ferrous forms for their absorption from the proximal small intestine. So people who take anti-acids to neutralize their low acidity has less iron absorption. Causes of iron deficiency: Poor iron intake (the most common cause in males). Pregnancy which requires an increased demand of iron (pregnant ladies must take supplements) 3. Malabsorption. 4. chronic blood loss : a. GI tract ex:Peptic ulcer(most common cause),malignancy in the intestine , patients with esophageal varices due to portal hypertension have a strong tendency to develop bleeding. b.Menstruation:
Menorrhagia or Hematomunia is an abnormally heavy and prolonged menstrual period at regular intervals.

5. achlorhydria : production of gastric acid in the stomach is absent or low. 6.GI disease 7. Dietary factors Low iron low vitamin C high coffee and tea intake ( neutralize gastric acidity ) . 8. Demographic factor elderly teenager females immigrant aborigine widower . 9. Socioeconomic factor depression. poverty. Abuse (specially alcohol abuse) Poor attention

Clinical features of iron deficiency anemia: 1. Brittle nail (koilonychias)- thin and bent 2. Angular stomatitis:is fungal infx sometimes it can be bacterial infx leads to crusting over the angle of the mouth ,as we will see later on it is more common in some anemias than others. 3. Brittle hair.

4. Atrophy of tongue papilla (red and smooth tongue burning tongue). 5. Glossitis (reddish and depapillated tongue accompanied with pain)

PlummerVinson syndrome (PVS), also called PatersonBrownKelly syndrome , presents as a triad of dysphagia, glossitis, and iron deficiency anemia. It most usually occurs in postmenopausal women. This patients are very prone to SCC(squamous cell carcinoma). Some claim that if iron deficiency anemia in this syndrome declined you might save this patient from the SCC.

6. parotid gland enlargement 7. Splenomegaly 8. Failure to grow

Investigation for iron deficiency anemia:

1. If Hemoglobin level is <10 then need to test MCV, as we mentioned should be <79 fl. 2. Iron binding capacity is very high in iron deficiency anemia, due to low iron content. 3. Send Blood film to pathologist , you will see a. Poikilocytosis: variation in shape of blood cells. b. Anisocytosis: variation in size of blood cells. 4. Endoscopy for GI bleeding.

Type II Thalassaemia

MCV <79 FL Iron level is normal. It is an Inherited autosomal recessive blood disorders. The disease is caused by the excessive destruction or degradation of red blood cells due to formation of abnormal hemoglobin molecules, because of a defect through a genetic mutation. There are two major forms of the disease : a. b. Thalassemia : defect in globin chains Thalassemia : defect in globin chains (more common in our region)

The body cannot produce one of the globin chain because of a Defect through a genetic mutation. This will lead to over production of the second globin chain (for ex: impaired production of alpha globin chains, leading to a relative excess of beta globin chains), so an abnormal hemoglobin molecule.So excessive destruction or degradation of red blood cells at the end of the day.

 Thalassemia divided to :

1. Major thalassemia ( Cooley's anemia )

2. Minor thalassemia (very common); carrier patient without symptoms. If mother and father have traits ( carriersminor thalassemia ) then the kids : 25% major thalassemia 25% normal kids 50% minor thalassemia

Symptoms of major thalassemia : 1. failure to Thrive 2. Intermittent infection: can easily attract infection. 3. Severe anemia ( hemoglobin is 6 or 7) which lead to extra medullary haemopoiesis - overproduction of defected blood from unusual sites, like: Bone marrow , Spleen which lead to splenomegaly , Mandible, Maxilla. The thick Maxilla leads to chipmunk faces, the increased production of bone trys to get more blood supply as possible. 4.Hair on end skull: space between 2 dipole of skull with thin cancellous bone (hair appearance on x-rays).

THE END OF 1st PART : DONE BY: ABEER ABU SOBEH So, the body will start producing blood from places which don't originally produce blood... we call this process "extra-medullary haemopayosis"....for example the liver, the spleen when they start to produce blood they will enlarge as they become functioning, so we'll find spleenomegaly, or hepatomegaly, also bone expansion, the skull for example, the medulla inside the skull bone shouldn't be functioning in blood production, blood is normally produced in the bone of the extremities, but here we'll have thalasemic facias; chipmunk faces, the bone of the skull enlarges because of blood production, it actually gets thinner, but it empty from the inside where it produces blood... When we look at the x-ray .. we can see an empty space inside that looks like a collection of hair connecting the outer plate and the inner plate of the skull... this area is the functioning area, contains living cells that produce blood, and as it enlarges it pushes the bone to the outside! this area is trying to amend the anaemia, the body is trying to produce blood, but here the blood is not functioning and it gets destroyed easily!

The aspects:
Suspect that those patients have blood-borne viruses... why? Because of frequent blood transfusion (every few months they attend the hospital for blood transfusion; 2, 3 or even 4 times a year) so we should suspect infections, blood borne viruses. For local anaesthesia, such patients are safe in general. For sedation, it's ok, but you should know that the functioning blood is low, And for his body to be able to stand the stress of any procedure, we should make sure to supply the patient with full oxygenation; (there is 21% of O2 in the air, but we should give the patient 100% O2) to make sure that the functioning blood is saturated with oxygen. For general anaesthesia, it's sort of hard with SEVERE thalasemia, for the enlarged maxilla because of the extramedullary haemopayosis, the maxilla is very big that the anaesthetist will have a problem to introduce the tube inside the patients airway. These patients will have rarefaction of the maxilla; rare = , now when u take an x-ray for the maxilla you'll see the maxilla bone as if it's "empty" and also this is because of the extramedullary haemopayosis.

Vitamin B12 Deficiency

Vitamin B12 and folic acid deficiencies are part of the Macrocytic Anaemia (high MCV; above 98). Vitamin b12 deficiency is the most common type of macrocytic anaemia, it is either Autoimmune disease (we call it Pernicious Anaemia) or due to nutritional problems. In general people may have normal B12 deficiency. The pernicious anaemia: is the presence of auto antibodies against the intrinsic factors of the parietal cells, or against the parietal cells itself that produce the intrinsic factors! In both ways we won't have intrinsic factor

(the intrinsic factor normally binds to vitamin B12 and carries it to somewhere in the intestine, so it ease the absorption of the vitamin in the intestine; in the ileum). So if the patient has problem with the intrinsic factor the vitamin will not be easily absorbed from that area. Pernicious anaemia patients will have other autoimmune diseases like the Thyroid disease, Addison's disease... what I want to know is that the autoimmune diseases come as a combination of diseases as the body has the inclination to destroy itself! Also those patients have higher incidence of gastric carcinoma.

Causes of vitamin B12 deficiency:

Insufficient dietary intake (vegan/vegetarian) as the main source of the vitamin is red meat. So any person who doesn't eat red meats may have vitamin b 12 deficiency unless he takes supplements; vitamin b12 or multivitamins. Impaired absorption because there will be problems in the stomach so there won't be production of the intrinsic factors. Or there will be small bowel or celiac and chron's disease ( it is an inflammatory bowel disease), tropical sprauce??? , bacterial overgrowth in the ileum; where the vitamin is mainly absorbed, or ileac disease , or the patient simply has had a surgery. Chronic pancreas diseases for example zollinger-ellison syndrome. Nitrous Oxide this is theoretical, coz a test was made in vitro not in vivo, and they found that the nitrous oxide inactivates vitamin B12, so they ask the patients who have vitamin B12 deficiency not to take nitrous oxide (which is usually used in pediatric dentistry).

Neurological syndromes:
Peripheral neuropathy: patients with vitamin B12 deficiency sometimes complain from symmetrical parasthesia in their fingers and toes. Regressive weakness and ataxia: patients cannot move well, but this happens in sever vitamin b12 deficiency cases.

Syndrome paraplegia: "" . Mental changes: the patient can develop psychosis, dementia. Digestive syndromes: glossitis (red sore tongue), angular stomatitis. These are very uncommon here, they are more common in iron deficiency. Hepato-spleenomegaly . Gastric atrophy and achlorohydria: the parietal cells produce many things including intrinsic factor and HCl. The b12 deficiency patient doesn't have HCl in their stomach so it loses its acidity.

Dental aspects:
Nitrous oxide sedation -- we'll talk about this theoretically as the experiments were made in vitro", it worsens the problem. So we try to avoid using it in this case. Aphtus ulcers.

Folic Acid Deficiency:

Here the body doesn't have stores for folic acid, this means "the body gets his needs of the acid from the food u eat in the day; to produce blood cells, but the day in which u don't eat food with folic acid u won't have folic acid for that day. folic acid is absorbed in the proximal small intestine "in the duodenum just like the iron".

Clinical features:
Pregnancy : pregnant ladies take supplements, but the thing that they really have to take is folic acid, otherwise they will have higher rates of neural tube defects that leads to cleft lip and palate .

Dental aspects:

Just like b12 deficiency; but the angular stomatitis : it's more common here than in b12 deficiency.

Normocetic Anaemias
Normal cells shape, normal cells number, less Hb content.

Acute Blood Loss

Aplastic Anaemia
Aplasia of bone marrow with peripheral blood pan-cytopenia. Now due to autoimmune diseases, due to medications or due to cancer or whatever the bone marrow; that produces all the types of blood cells, becomes destroyed, so the patient has aplasia ( no cells in the blood, only the white plasma. And this disease can be reversed if the bone marrow goes back to its normal state and starts to produce blood cells.

Causes:
Congenital ( fanconi anaemia) it was found commonly between the people that marry their relatives, and it's still seen now. Acquired (ionizing radiation; radiotherapy (for example lymphoma), chemicals, drugs, insecticide; that's why farmers develop aplastic anaemia (low blood pressure and bleeding tendency due to the absence of blood cells)). Infections, measles : might lead to aplastic amaemia but not often. Salacious infections : tuberculosis for example.

 Pregnancy

Clinical features:
Blood anaemia Bleeding tendency Infections ; because there are no WBCs so they can develop any sort of infection including fungal infections which we call "the disease of the diseased" . so when we see someone with fungal infection , we don't just say it is a fungal infection, we should say that this person has a very bad disease that even the fungi could take over!

Anaemia of chronic diseases

Hemolytic Anaemia "sickle cell anaemia"

(thalasemia is called hemolytic anaemia, but thalasemia is microcytic anaemia, here we're talking about normocytic anaemia). ** Red cells usually survive for 120 days, but in hemolysis the RBC lives just for a short period of time then it gets lysed. This leads to anaemia and for one of the symptoms that we talked about before "Jaundice" .

Causes :
We ask the patients with glucose 6 phosphate not to take certain medications and they can live a normal life! these medications are: NSAID (like aspirin, prufin), and some antibiotic. We usually give aspirin as an antiplatelet.

 We as dentists always prescribe prufin as it's a very good dental analgesic. But we shoudn't give it to a glucose 6 phosphate deficiency. Inherited : like RBC membrane defects; spherocytosis, hereditary elepto or hemoglobin abnormality (not abnormality in the cell shape, but in the hemoglobin) and like thalasemia, iron deficiency anaemia and sickle cell anaemia. Metabolic defects such as glucose six phosphate deficiency " ; " glucose 6 phosphate dehydrogenase enzyme will be defect. The amino acids that form proteins "glubin chains", the patient will have substitution in these chains " and chains", but any substitution or change in any amino acid will lead to sickle cell anaemia. Sickle cell anaemia has 2 types: Sickle cell major: homozygous, very severe. Sickle cell trait: heterozygous, patients have normal life, but at some point they might develop sickle cell disease.

Painful crisis: the clinical features: In sickle cell patient, It is always there. In the sickle cell trait, they develop painful crisis too. the sickle RBC gets destroyed bcoz it's not functioning, and the destroyed cells will accumulate in the area where they were destroyed, if they were destroyed in the bone the patient will feel pain in that area because the vessels are closed, the patient will have infarction (very painful). And if they were destroyed in the heart it will lead to MI (fatal). or in the jaws. Or in the liver, spleen or lung, and so on. The main problem about the infarction is the pain!

Hematological crisis:

In heterozygous "the trait", the para-virus for example will lead to hematological disorders; SCA. Chronic anaemia. Hyperbilirubinemia; increase bilirubin production; when the blood cell are destroyed, it will produce bilirubin, part of this bilirubin will go the GIT, and the body reabsorb small part of it, but when the produced bilirubin amount is very high, it will go to the blood causing jaundice, and also causing higher rate of stones formation in the gallbladder in the liver where the destruction process occur and bilirubin is produced! Infection: the MAIN cause of death!!! They can easily get infected. Sequestration syndrome; blood accumulation also.

Dental aspects:
in heterozygous; First you need to do very good investigations for such patients. By electrophoresis, then they take the cells and examine them very well. And this gives us accurate results and diagnosis (positive diagnosis) . Full Oxygenation: the patient has normal life, but he might develop SCA under stressful situations that need high oxygenation; infection, starvation, or any low oxygenation cases (like traveling by planes), or dental treatment these are situations where the patient can develop crisis or SCA. Local anesthesia: is fine but they say don't use the one with prilocaine, coz it causes methemoglobinemia. Avoid NSAIDs Prophylactic antibiotics: because they're prone to have infections, that might cause crisis!

Orofacial manifestations: The patient may come to your clinic complaining from pain in his teeth but you examine him and see nothing, and no pain around the teeth. So simply the cause is infarction! That might lead to osteomyelitis "infection in the bone" if more infracted sells accumulate. Hypersementosis Skeletal, not dental development is delayed

Steroids
( = cortisone ) under stress, patients will have adrenal insufficiency. So they need to take supplements if they are going to do something stressful. We have these examples: A patient who had taken steroids a yrea ago or less; we ask the patient to come in the early morning and ask him to take steroids "same amount of steroids", we sometimes give them IV hydrocortisone (25 -50 mg). As a cover! And others said we should give the patient a "post-operation" hydrocortisone (25 50 mg) (for 1 3 hours). Or a patient who had taken the steroids more than 1 year (we treat them normally) A patient who is taking steroids now, here we don't only care about the steroids intake we care more about the cause or the disease that caused the patient to take steroids! Liver and lung transplant for example look how big is the problem! Or Behet's disease "but it's not very related to dentistry" , but we need to know that any patient with Behet's disease is taking steroids THE END 2nd PART DONE BY: RAZAN TANNUS

CHECKED BY: HEBAH RAID RAMADNEH