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

Hematology


Anemia

Anemia

  • deficiency of red blood cells and/or hemoglobin

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


Hematology

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

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

Diagnosis

  • Complete blood count (CBC)

  • Blood smear

  • Reticulocyte count

  • Mean corpuscular volume (MCV)

  • Mean corpuscular hemoglobin (MCH)

  • Mean corpuscular hemoglobin concentration (MCHC)


Hematology

  • Deficient red blood cell production

    • iron deficiency anemia

    • pernicious anemia

    • folate deficiency anemia


Hematology

Excessive red blood cell destruction

(hemolysis)

  • G-6-PD deficiency anemia

  • Thalassemia


Iron deficiency anemia

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


Diagnosis1

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

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

Prevention of complications

  • Detection and referral for diagnosis and treatment


Pernicious anemia

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

Folate deficiency anemia

  • Deficiency in daily intake or absorption


Diagnosis2

Diagnosis

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

  • High MCV, MCH

  • macrocytic anemia

  • low levels of serum vitamin B12


Treatment1

Treatment

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

  • Folic acid orally 1 mg daily


Potential problem

Potential problem

  • Infection

  • Bleeding

  • Delayed healing


Prevention of complications1

Prevention of complications

  • Detection and medical treatment

    (early detection and treatment can prevent permanent neurologic damage)


Glucose 6 phosphate dehydrogenase deficiency

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

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.


Hematology

Drugs :

  • antimalarial agents

  • sulfonamides (antibiotic)

  • aspirin

  • nonsteroidal anti-inflammatory drugs (NSAIDs)

  • nitrofurantoin

  • quinidine

  • quinine

  • others


Hematology

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).


Treatment2

Treatment

  • cause is an infection, it should be treated.

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


Potential problem1

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

Emergency care

  • Having hemolytic crisis : conservative control of pain and infection


Thalassemia

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


Hematology

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

Alpha () thalassemias

  • involve the genes HBA1 and HBA2

  • inherited in an autosomal dominant

  • connected to the deletion of the 16p chromosome


Hematology

  • 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


Hematology

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.


Hematology

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.


Hematology

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


Hematology

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

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


Hematology

  • 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


Hematology

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.


Hematology

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.


Hematology

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

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


Hematology

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


Diagnosis3

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 complications2

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

Leukemia

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


Symptoms

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


Hematology

  • 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


Diagnosis4

Diagnosis

  • blood tests

  • bone marrow biopsy


Related symptoms

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)


Hematology

  • Acute vs. chronic

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


Acute leukemia

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


Hematology

  • 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

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 leukemia1

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


Hematology

four main categories

  • Acute lymphocytic leukemia (ALL)

  • Acute myelogenous leukemia (AML)

  • Chronic lymphocytic leukemia (CLL)

  • Chronic myelogenous leukemia (CML)


Hematology

  • 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.


Hematology

  • 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.


Hematology

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


Causes

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


Hematology

  • 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


Hematology

  • 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 problem2

Potential problem

  • Prolonged bleeding

  • Infection

  • Delayed healing


Dental management

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


Hematology

  • 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 care1

Emergency care

  • As indicated, during remission

  • Conservative: antibiotics for infection; strong analgesics for pain

  • Drainage through pulp chamber rather than extraction


Hematology

Bleeding disorders


Normal hemostasis

Normal hemostasis

1. Blood vessel

2. Platelet

3. Coagulation and fibrinolysis


Hematology

Blood coagulation

cascade


Hematology

  • Thrombocytopenia

  • Platelet dysfunction


Hematology

  • Hereditary hemorrhagic telangiectasia

    autosomal dominant

  • Henoch Schoenlein purpura

    autoimmune disease

  • Drug : corticosteroid, iodine

  • Scurvy


Hematology

1.

2.Tourniquet test

3.


Potential problem3

Potential problem

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


Prevention of complications3

Prevention of complications

  • Referral and consultation

  • Local measures for control of bleeding

  • Splint

  • Prophylactic antibiotics in surgical cases to avoid postoperative infection


Thrombocytopenia

Thrombocytopenia

  • Megakaryocytes, 7 9 days

  • Normal : 150,000 400,000 /..

  • >50,000 100,000 /..

  • 20,000 50,000 /.. ,

    hematoma ,

  • <20,000 /.. Spontaneous bleeding


Thrombocytopenia1

Thrombocytopenia

1.

2.

3.

4.

5.splenomegaly


Hematology

  • (petechia, ecchymosis)

  • CBC, platelet count

  • bleeding time


Platelet dysfunction thrombocytopathia

Platelet dysfunction (Thrombocytopathia)

  • CBC, Platelet count

  • Bleeding time


Thrombocytopathia

Thrombocytopathia

1. ADP

2. Acute and chronic renal failure

3. Aspirin, NSAIDs

4. : Furosemide, antihistamine

etc.


Potential problem4

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 complications4

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


Hematology

- bleeding time Platelet count

- platelet

50,000/..


Coagulopathy

Coagulopathy

Hereditary

  • Hemophilia

  • Von Willebrands disease

    Acquired

  • Drug : Heparin, Coumadin

  • Liver disease

  • DIC

  • Massive blood transfusion


Hemophilia

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

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.


Hematology

  • 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

Hemophilia A

  • factor


Hematology

VIII

(%)

50 100

25 50

5 25

1 5

0 1


Factor viii

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

Hemophilia B

  • Factor IX

  • Hemophilia A

  • Hemophilia A Factor IX


Factor xi deficiency

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 willebrand s disease

Von Willebrands disease

  • bleeding time

  • von Willebrandfactor factor Factor VIII

  • Autosomal dominant trait


Von willebrand s disease1

Von Willebrands disease

  • Hemophilia ,,

  • PT

  • PTT

  • Bleeding time


Hematology

  • PTT

  • clotting time (severe)

  • PT bleeding time

  • Factor assay


Hematology

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.


Hemophilia1

Hemophilia

Potential problem

  • Excessive bleeding


Prevention of complications5

Prevention of complications

1. Identification

2. Consult and referral

3. Replacement options

  • Cryoprecipitate

  • Fresh frozen plasma

  • Factor VIII concentrates


Hematology

4. Mild and moderate factor VIII deficiency

  • DDAVP

  • antifibrinolytic agent

  • Fibrin glue

  • Factor VIII replacement for some cases


Hematology

5. Severe factor VIII deficiency

  • DDAVP

  • antifibrinolytic agent

  • Fibrin glue

  • Higher dose factor VIII


Hematology

6. Stable level of inhibitors

  • DDAVP

  • antifibrinolytic agent

  • Fibrin glue

  • Very high dose factor VIII


Hematology

7. Inducible inhibitors

  • No elective surgery

  • DDAVP,antifibrinolytic agent, Fibrin glue

  • High doses of factor VIII concentrate

  • Nonactivated prothrombin-complex concentrate

  • plasmapheresis


Hematology

8. Local measures for control of bleeding

9. Prophylactic antibiotic : prevent postoperative infection

10. Avoid aspirin, NSAIDs


Hematology

  • Factor VIII

    50% 12 . 3-5

    - factor

    - IM

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

    nerve block factor VIII > 50-70%


Factor viii1

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

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

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


Hematology

1. Aspirin

2. Anticoagulants : heparin, coumadin

3. Broad-spectrum antibiotics

4. Alcohol

5. Anticancer or cancer therapeutic drugs


Heparin

Heparin

  • platelet

  • thromboplastin

  • prothrombin thrombin

  • fibrinogen fibrin

  • half life 1-3 . 4-6 .

  • protamine sulfate50 mg, IV


Hematology

  • heparin

  • PTTclotting time

  • heparin protamine sulfate6 .

  • Antibiotics

  • Heparin , 6-12 .


Coumadin

Coumadin

  • Coumadin, warfarin, dicumarol

  • Vit. K antagonist

  • Vit. K

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

  • ~ 8 10 ., ~ 36 .

  • ~ 72 .


Hematology

  • PT , PT ~ 1.5-2 , INR 3.0

  • prothrombin activity > 30 40%

  • Vit. K 10 mg IV, 6-8 .

  • Antibiotics


Hematology

  • ~ 3

  • Antibiotics sulfonamides, erythromycin, metronidazole, tetracycline


Aspirin

Aspirin

  • Antiplatelet aggregation

  • factor II VII


Hematology

  • bleeding time, PTT coagulation phase

  • 5


Vitamin k deficiency

Vitamin K deficiency

  • Fat soluble vitamin

    Major causes

    - inadequate dietary intake

    - intestinal malabsorption

    - Hepatocellular disease (storage site)

  • factor II, VII, IX X


Vitamin k deficiency1

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

Fibrinolytic defect

plasminogen proactivator

XIIa

activator

plasminogen plasmin

fibrin fibrin split product


Fibrinolytic defect1

Fibrinolytic defect

  • plasmin inhibitor deficiency

    excessive fibrinolysis and fibrin deposit

    after trauma and surgery

  • Cirrhosis impair clearance of tissue

    plasminogen activator


Fibrinolytic defect2

Fibrinolytic defect

Diagnosis

  • low fibrinogen

  • normal PT, PTT, platelet count

  • should not receive heparin

    Therapy

  • Plasma therapy

  • fibrinolytic inhibitor (Epsilon-

    aminocaproic acid)


Prevention of complications6

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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