Hematology
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Hematology. Anemia. deficiency of red blood cells and/or hemoglobin reduced ability of blood to transfer oxygen to the tissues, and this causes hypoxia. Three main classes excessive blood loss (acutely such as a hemorrhage or chronically through low-volume loss

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Hematology

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Hematology


Anemia

  • deficiency of red blood cells and/or hemoglobin

  • reduced ability of blood to transfer oxygen to the tissues, and this causes hypoxia


Three main classes

  • excessive blood loss (acutely such as a hemorrhage or chronically through low-volume loss

  • excessive red blood cell destruction (hemolysis)

  • deficient red blood cell production


Signs and symptoms

  • weakness or fatigue.

  • sometimes shortness of breath

  • severe anemia prompts the body to compensate by markedly increasing cardiac output, leading to palpitations and sweatiness; lead to heart failure in elderly

  • Pallor (pale skin and mucosal linings) : only notable in severe anemia, not a reliable sign


Diagnosis

  • Complete blood count (CBC)

  • Blood smear

  • Reticulocyte count

  • Mean corpuscular volume (MCV)

  • Mean corpuscular hemoglobin (MCH)

  • Mean corpuscular hemoglobin concentration (MCHC)


  • Deficient red blood cell production

    • iron deficiency anemia

    • pernicious anemia

    • folate deficiency anemia


Excessive red blood cell destruction

(hemolysis)

  • G-6-PD deficiency anemia

  • Thalassemia


Iron deficiency anemia

  • most common type of anemia

  • microcytic anemia

  • dietary intake or absorption of iron is insufficient

  • hemoglobin, which contains iron, cannot be formed


Diagnosis

  • complete blood count (CBC) : low hemoglobin or hematocrit

  • low MCV, MCH or MCHC

  • a peripheral blood smear : microcytic anemia

  • low serum ferritin, a low serum iron level

  • elevated serum transferrin and a high total iron binding capacity (TIBC).


Treatment

  • If the cause is dietary iron deficiency, iron supplements, usually with iron sulfate,

  • Oral iron supplements (ferrous sulfate) 300 mg three times a day

  • The hematocrit should return to normal after 2 months of iron therapy, but the iron should be continued for another 6 to 12 months to replenish the body's iron stores


Prevention of complications

  • Detection and referral for diagnosis and treatment


Pernicious anemia

  • a type of autoimmune anemia

  • Antibodies are directed against intrinsic factor or parietal cells which produce intrinsic factor

  • Intrinsic factor is required for vitamin B12 absorption, so impaired absorption of vitamin B12 can result

  • vitamin B12 malabsorption, and vitamin B12 deficiency


Folate deficiency anemia

  • Deficiency in daily intake or absorption


Diagnosis

  • complete blood count (CBC) : low hemoglobin or hematocrit

  • High MCV, MCH

  • macrocytic anemia

  • low levels of serum vitamin B12


Treatment

  • Vitamin B12 (hydroxycobalamin or cyanocobalamin) injected intramuscularly generally 1000 to 2000 mcg daily

  • Folic acid orally 1 mg daily


Potential problem

  • Infection

  • Bleeding

  • Delayed healing


Prevention of complications

  • Detection and medical treatment

    (early detection and treatment can prevent permanent neurologic damage)


Glucose-6-phosphate dehydrogenase deficiency

  • hereditary, sex-linked enzyme defect that results in the breakdown of red blood cells when the person is exposed to the stress of infection or drugs


Causes, incidence, and risk factors

  • primary effect is the reduction of the enzyme G-6-PD in red blood cells, causing destruction of the cells, called hemolysis.

  • hemolysis leads to anemia

  • disorder are not normally anemic and display no evidence of the disease until the red blood cells are exposed to an oxidant or stress.


Drugs :

  • antimalarial agents

  • sulfonamides (antibiotic)

  • aspirin

  • nonsteroidal anti-inflammatory drugs (NSAIDs)

  • nitrofurantoin

  • quinidine

  • quinine

  • others


Symptoms

  • Fatigue

  • Paleness

  • Shortness of breath, rapid heart rate

  • Yellow skin color (jaundice)

  • Dark urine

  • Enlarged spleen

    Note: Severe hemolysis may cause

    hemoglobinuria (hemoglobin in the urine).


Treatment

  • cause is an infection, it should be treated.

  • cause is a drug, the offending agent should be stopped


Potential problem

  • Accelerated hemolysis of RBC

  • Prevention of complications

    • control infection

    • avoid drugs : antibiotics, aspirin

    • often increased sensitivity to sulfa drugs, aspirin , chloramphenicol


Emergency care

  • Having hemolytic crisis : conservative control of pain and infection


Thalassemia

  • inherited disease of the red blood cells, classified as a hemoglobinopathy

  • genetic defect results in synthesis of an abnormal hemoglobin molecule

  • blood cells are vulnerable to mechanical injury and die easily

  • Prevalence : 3-14 % in Thailand


Classification

  • according to which chain of the globin molecule is affected:

  • thalassemia, the production of globin is deficient

  • thalassemia the production of globin is defective


Alpha () thalassemias

  • involve the genes HBA1 and HBA2

  • inherited in an autosomal dominant

  • connected to the deletion of the 16p chromosome


  • thalassemias result in excess chain production in adults and excess chains in newborns

  • excess chains form unstable tetramers that have abnormal oxygen dissociation curves.

    four genetic loci for globin

  • The more of these loci that are deleted or affected by mutation, the more severe will be the manifestations of the disease


All four loci are affected

  • infants are dead at birth with hydrops fetalis, born alive die shortly after birth

  • edematous and have little circulating hemoglobin

  • tetrameric chains (hemoglobin Barts).

  • homozygous inheritance of an alpha thalassemia trait, type 1.


three loci are affected, Hemoglobin H

disease results

  • in blood, both hemoglobin Barts (tetrameric chains) and hemoglobin H (tetrameric chains)

  • noticed in childhood or in early adult life, when the anemia and splenomegaly

  • usually due to compound heterozygous inheritance of alpha thalassemia type 1 and type 2 traits.


two of the four loci are affected, alpha

thalassemia trait, type 1 results

  • Two loci permit nearly normal erythropoiesis

  • mild microcytic hypochromic anemia

  • deletion of one of the two loci on chromosomes


one of the four loci is affected, alpha

Minor or alpha+ thalassemia trait or alpha

thalassemia trait, type 2 results

  • minimal effect.

  • Three -globin loci are enough to permit normal hemoglobin production

  • no anemia or hypochromia

  • called thalassemia carriers.


Beta () thalassemias

  • mutations in the gene on chromosome 11

  • inherited in an autosomal dominant

  • excess chains are produced, but these do not form tetramers

  • bind to the red blood cell membranes producing membrane damage

  • at high concentrations have the tendency to form toxic aggregates


  • severity of the damage depends on the nature of the mutation

  • individual has two globin alleles

  • mutations (o) prevent any formation of chains

  • (+) allow some chain formation to occur


thalassemia major or Cooley's anemia :

both have mutations, a severe microcytic,

hypochromic anemia

  • Untreated, this results in death before age twenty

  • Treatment :

    • periodic blood transfusion

    • splenectomy (splenomegaly)

    • transfusion-caused iron overload

    • Cure is possible by bone marrow transplantation.


Thalassemia minor (sometimes referred to as

thalassemia trait) : only one globin allele

mutation

  • results mild anemia with microcytosis

  • Symptoms include weakness and fatigue

  • most cases thalassemia minor may be asymptomatic and may be unaware

  • Detection usually involves counting the mean corpuscular volume (size of red blood cells) and noticing a slightly decreased mean volume than normal.


Thalassemia intermedia

  • condition intermediate between the major and minor forms

  • often normal life but may need occasional transfusions e.g. at times of illness or pregnancy

  • depends on the severity of their anemia.


Treatment and complications

Thalassemia Major and Intermedia

  • receive frequent blood transfusions that lead to iron overload. Iron chelation treatment is necessary

  • Untreated thalassemia Major eventually leads to death usually by heart failure, therefore birth screening is very important

  • bone marrow transplant


Thalassemia Intermedia

  • patients vary in treatment needs depending on the severity of their anemia

    Thalassemia Minor

  • not life threatening, can affect quality of life due to the effects of a mild to moderate anemia


Diagnosis

  • complete blood count (CBC) : low hemoglobin or hematocrit

  • low MCV or MCHC

  • a peripheral blood smear : hypochromic microcytic anemia

  • increase serum iron level


Prevention of complications

  • Detection and referral for diagnosis and treatment

  • Severe anemia : hemoglobin, hematocrit before dental treatment

  • Severe anemia :

  • Hematocrit < 15 % - blood transfusion

  • Splenectomy : infection

  • Antibiotic prophylaxis before dental surgery


Leukemia

  • cancer of the blood or bone marrow characterized by an abnormal proliferation of blood cells, usually white blood cells (leukocytes)


Symptoms

  • Damage bone marrow, by displacing the normal marrow cells with increasing numbers of malignant cells,

  • results in a lack of platelets, which are important in the blood clotting process.

  • people with leukemia may become bruised, bleed excessively, or petechiae


  • White blood cells, which are involved in fighting pathogens, may be suppressed or dysfunctional, putting the patient at the risk of developing infections

  • red blood cell deficiency leads to anaemia, which may cause dyspnea


Diagnosis

  • blood tests

  • bone marrow biopsy


Related symptoms

  • Fever, chills, and other flu-like symptoms

  • Weakness and fatigue

  • Loss of appetite and/or weight

  • Swollen or bleeding gums

  • Neurological symptoms (headache)


  • Acute vs. chronic

  • Leukemia is clinically and pathologically split in to its acute and chronic forms


Acute leukemia

  • characterized by the rapid growth of immature blood cells

  • bone marrow unable to produce healthy blood cells

  • Acute forms of leukemia can occur in children and young adults


  • Immediate treatment in acute leukemias

  • rapid progression and accumulation of the malignant cells, which then spill over into the bloodstream and spread to other organs of the body.

  • If left untreated, the patient will die within months or even weeks


Chronic leukemia

  • excessive buildup of relatively mature, but still abnormal white blood cells

  • progress months to years

  • the cells are produced at a higher rate than normal cells, resulting in many abnormal white blood cells in the blood.

  • mostly occurs in older people, but can occur in any age group


Chronic leukemia

Lymphoid vs. myeloid

  • classified according to the type of abnormal cell found most in the blood.

  • Lymphocytic leukemia : when leukemia affects lymphoid cells

  • When myeloid cells are affected, the disease is called myeloid or myelogenous leukemia


four main categories

  • Acute lymphocytic leukemia (ALL)

  • Acute myelogenous leukemia (AML)

  • Chronic lymphocytic leukemia (CLL)

  • Chronic myelogenous leukemia (CML)


  • Acute lymphocytic leukemia (ALL) :

    most common type of leukemia in young children. This disease also affects adults, especially those age 65 and older.

  • Acute myelogenous leukemia (AML) : occurs more commonly in adults than in children. This type of leukemia was previously called acute nonlymphocytic leukemia.


  • Chronic lymphocytic leukemia (CLL) : most often affects adults over the age of 55, sometimes occurs in younger adults, but it almost never affects children.

  • Chronic myelogenous leukemia (CML) : occurs mainly in adults. A very small number of children also develop this disease.


  • The most common forms in adults are AML and CLL, whereas in children ALL is more prevalent


Causes

  • The exact cause of leukemia is unknown

  • influenced by both genetic and environmental factors

  • from somatic mutations in the DNA which activate oncogenes or inactivate tumor suppressor genes, and disrupt the regulation of cell death, differentiation or division


  • mutations :

    • may occur spontaneously or

    • as a result of exposure to radiation or carcinogenic substances

  • exposure to petrochemicals, such as benzene, and hair dyes to the development of leukemia


  • Viruses have also been linked to some forms of leukemia

  • ALL are associated with viral infections by either the human immunodeficiency virus (HIV, responsible for AIDS)

  • human T-lymphotropic virus (HTLV-1 and -2, causing adult T-cell leukemia/lymphoma).

  • Fanconi anemia is also a risk factor for developing acute myelogenous leukemia


Potential problem

  • Prolonged bleeding

  • Infection

  • Delayed healing


Dental management

  • Detection and referral for diagnosis and treatment

  • Determine platelet status (> 80,000/mm3) on day of surgical procedure, bleeding time (within normal range)

  • Avoidance of postoperative infection and osteoradionecrosis : prophylactic antibiotics


  • During acute stages : avoidance of dental care

  • In state of remission : treat all active dental disease and hygiene maintenance program

  • Avoidance of long, drawn-out dental procedures

  • Poor prognosis : no complex restorative procedures


Emergency care

  • As indicated, during remission

  • Conservative: antibiotics for infection; strong analgesics for pain

  • Drainage through pulp chamber rather than extraction


Bleeding disorders


Normal hemostasis

1. Blood vessel

2. Platelet

3. Coagulation and fibrinolysis


Blood coagulation

cascade


  • Thrombocytopenia

  • Platelet dysfunction


  • Hereditary hemorrhagic telangiectasia

    autosomal dominant

  • Henoch Schoenlein purpura

    autoimmune disease

  • Drug : corticosteroid, iodine

  • Scurvy


1.

2.Tourniquet test

3.


Potential problem

  • Prolong bleeding following surgical procedures or any insult to integrity of oral mucosa


Prevention of complications

  • Referral and consultation

  • Local measures for control of bleeding

  • Splint

  • Prophylactic antibiotics in surgical cases to avoid postoperative infection


Thrombocytopenia

  • Megakaryocytes, 7 9 days

  • Normal : 150,000 400,000 /..

  • >50,000 100,000 /..

  • 20,000 50,000 /.. ,

    hematoma ,

  • <20,000 /.. Spontaneous bleeding


Thrombocytopenia

1.

2.

3.

4.

5.splenomegaly


  • (petechia, ecchymosis)

  • CBC, platelet count

  • bleeding time


Platelet dysfunction (Thrombocytopathia)

  • CBC, Platelet count

  • Bleeding time


Thrombocytopathia

1. ADP

2. Acute and chronic renal failure

3. Aspirin, NSAIDs

4. : Furosemide, antihistamine

etc.


Potential problem

  • Prolong bleeding

  • Infection in patients with bone marrow replacement

  • In patients being treated with steroids, stress may lead to serious medical emergency


Prevention of complications

  • Referral and consultation

  • Correction of underlying problem or replacement therapy before surgery

  • Local measures for control of blood loss

  • Prophylactic antibiotics in surgical cases to prevent postoperative infection

  • Additional steroids for patients being treated with steroid

  • Avoid aspirin, NSAIDs


- bleeding time Platelet count

- platelet

50,000/..


Coagulopathy

Hereditary

  • Hemophilia

  • Von Willebrands disease

    Acquired

  • Drug : Heparin, Coumadin

  • Liver disease

  • DIC

  • Massive blood transfusion


Hemophilia

Forms

  • Haemophilia A - factor VIII deficiency, "classic haemophilia" (X-linked)

  • Haemophilia B - factor IX deficiency, "Christmas disease" (X-linked)

  • Haemophilia C - factor XI deficiency (autosomal recessive)


Willebrand disease (vWD)

  • milder than haemophilias

  • caused by mutations in the coagulation protein von Willebrand factor.

  • the most common coagulation disorder present in 1% of the population.


  • Haemophilia A and B are inherited in an X-linked recessive pattern

  • caused by mutations affecting the genes encoding one of the clotting factors.

  • genes for Haemophilia A and Haemophilia B are located on the X chromosome


Hemophilia A

  • factor


VIII

(%)

50 100

25 50

5 25

1 5

0 1


Factor VIII

  • Factor VIII 1.0 u/ml plasma Factor VIII 100 %

  • Factor VIII 75 % Factor VIII

    0.75 u/ml

    ( Factor VIII 55 - 145 %)


Hemophilia B

  • Factor IX

  • Hemophilia A

  • Hemophilia A Factor IX


Factor XI deficiency

  • autosomal recessive trait

  • No correlation between factor level and

    propensity to bleed

  • less spontaneous bleeding

  • hemarthrosis rare

  • Post traumatic bleeding + perioperative

    bleeding

  • Daily infusion of FFP, T1/2 24 hr


Von Willebrands disease

  • bleeding time

  • von Willebrandfactor factor Factor VIII

  • Autosomal dominant trait


Von Willebrands disease

  • Hemophilia ,,

  • PT

  • PTT

  • Bleeding time


  • PTT

  • clotting time (severe)

  • PT bleeding time

  • Factor assay


Treatment

  • no cure for haemophilia

  • controlled with regular injections of the deficient clotting factor,

  • i.e. factor VIII in haemophilia A or factor IX in haemophilia B.

  • Some haemophiliacs develop antibodies (inhibitors) against the replacement factors - amount of the factor has to be increased or non-human replacement products must be given, such as porcine factor VIII.


Hemophilia

Potential problem

  • Excessive bleeding


Prevention of complications

1. Identification

2. Consult and referral

3. Replacement options

  • Cryoprecipitate

  • Fresh frozen plasma

  • Factor VIII concentrates


4. Mild and moderate factor VIII deficiency

  • DDAVP

  • antifibrinolytic agent

  • Fibrin glue

  • Factor VIII replacement for some cases


5. Severe factor VIII deficiency

  • DDAVP

  • antifibrinolytic agent

  • Fibrin glue

  • Higher dose factor VIII


6. Stable level of inhibitors

  • DDAVP

  • antifibrinolytic agent

  • Fibrin glue

  • Very high dose factor VIII


7. Inducible inhibitors

  • No elective surgery

  • DDAVP,antifibrinolytic agent, Fibrin glue

  • High doses of factor VIII concentrate

  • Nonactivated prothrombin-complex concentrate

  • plasmapheresis


8. Local measures for control of bleeding

9. Prophylactic antibiotic : prevent postoperative infection

10. Avoid aspirin, NSAIDs


  • Factor VIII

    50% 12 . 3-5

    - factor

    - IM

    - LA : nerve block, pericemental, intraligamental, intrapulpal, infiltration

    nerve block factor VIII > 50-70%


Factor VIII

  • FFP

    1 Factor VIII 200 U

    (Factor VIII1 U/ml plasma)

  • Cryoprecipitate

    Factor VIII 80 -100 U/ 1 (20-25ml)

    (Factor VIII4-5 U/ml)

  • Lyophilized concentrate factor VIII

    Factor VIII 250 1,500 U/ 1 (25ml)

    (Factor VIII10-60 U/ml)


Factor IX

1. Fresh frozen plasma (FFP) Factor IX 1 U/ml

2. Prothrombin complex concentrates Factor II, VII, IX, X 500-1,000 U/25 ml

Half life ~ 24 hr

FFP 24-36 . 30 %

16-24 .


Synthetic vasopressin analogue

  • Desmopressin ( 1-deamino-8-D-argenine vasopressin; DDAVP)

  • 0.3-0.4 g/kg + NSS 50 ml, IV

  • Desmopressin endothelium

    Factor VIII

  • inhibitor Factor VIII


1. Aspirin

2. Anticoagulants : heparin, coumadin

3. Broad-spectrum antibiotics

4. Alcohol

5. Anticancer or cancer therapeutic drugs


Heparin

  • platelet

  • thromboplastin

  • prothrombin thrombin

  • fibrinogen fibrin

  • half life 1-3 . 4-6 .

  • protamine sulfate50 mg, IV


  • heparin

  • PTTclotting time

  • heparin protamine sulfate6 .

  • Antibiotics

  • Heparin , 6-12 .


Coumadin

  • Coumadin, warfarin, dicumarol

  • Vit. K antagonist

  • Vit. K

  • factor -carboxylation Vit. K(factor II, VII, IX, X)

  • ~ 8 10 ., ~ 36 .

  • ~ 72 .


  • PT , PT ~ 1.5-2 , INR 3.0

  • prothrombin activity > 30 40%

  • Vit. K 10 mg IV, 6-8 .

  • Antibiotics


  • ~ 3

  • Antibiotics sulfonamides, erythromycin, metronidazole, tetracycline


Aspirin

  • Antiplatelet aggregation

  • factor II VII


  • bleeding time, PTT coagulation phase

  • 5


Vitamin K deficiency

  • Fat soluble vitamin

    Major causes

    - inadequate dietary intake

    - intestinal malabsorption

    - Hepatocellular disease (storage site)

  • factor II, VII, IX X


Vitamin K deficiency

  • Prolonged PT, normal PTT

    Treatment

    Vit K 10 mg, IV

    restore vit K in liver

    normal production of prothrombin

    complex in 8-10 h

    Severe hemorrhage : FFP


Fibrinolytic defect

plasminogen proactivator

XIIa

activator

plasminogen plasmin

fibrin fibrin split product


Fibrinolytic defect

  • plasmin inhibitor deficiency

    excessive fibrinolysis and fibrin deposit

    after trauma and surgery

  • Cirrhosis impair clearance of tissue

    plasminogen activator


Fibrinolytic defect

Diagnosis

  • low fibrinogen

  • normal PT, PTT, platelet count

  • should not receive heparin

    Therapy

  • Plasma therapy

  • fibrinolytic inhibitor (Epsilon-

    aminocaproic acid)


Prevention of complications

  • Referral and consultation

  • Screening laboratory test

  • Platelet count, bleeding time normal

    • PT, PTT prolonged

    • Thrombin time prolonged

  • Epsilon-aminocaproic acid therapy will inhibit plasmin and plasmin activators


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