UNIT 1.1 ANAEMIA 7.

Diaphoresis with exertion (excessive sweating due

ANEMIA: Condition of having a lower-than-normal to > metabolism)
numbers of haemoglobin where Hb value is < 12.5 8. Chronic fatigue
g/dL in adult (N: 14-18) DIAGNOSTIC TEST
COMPOSITION OF BLOOD 1. CBC - determine number of RBC & WBC
1. Plasma (55%) 2. Hemoglobin - measure level of Hb in blood
2. Blood cells (45%) 3. Hematocrit - % by volume of RBC in blood
PLASMA 4. Serum iron - measure num. of iron in blood
1. Plasma Protein 5. Serum ferritin - measure level of ferritin in blood
- Albumins (maintains osmotic pressure in blood) 6. Serum folate - check if have enough folate
- Globulins (provide immunity & antibody) MANAGEMENT
- Fibrinogen (clotting factor) 1. Blood transfusion (quickly > RBC in blood)
2. Minerals (cell formation, muscle contraction, 2. > intake of deficient nutrient (best sources of iron)
maintain blood pH) 3. Dietary supplement (iron supplement to > Hb
3. Nutrients (provide energy & heat, fix & replace level)
cells) 4. Changing cooking habit (high protein to > iron
4. Organic waste products (excreted by liver, kidney, absorption)
lungs) 5. < alcohol intake (RBC be destroyed prematurely)
5. Hormones (influences cellular activity) UNIT 1.2 IRON DEFICIENCY ANEMIA
6. Gases (O2 & CO2) DEFINITION – Chronic, hypochromic and microcytic
BLOOD CELLS anaemia due to the insufficient iron in red blood cell
1. Erythrocytes (RBC) that leads to low level of haemoglobin.
2. Leukocytes (WBC) CAUSES
3. Thrombocytes (Platelet) 1. Increased iron loss - GIT bleeding, Hemoptysis,
PRODUCTION OF RBC (erythropoiesis) Haematuria, Menorrhagia, Chronic hemorrhage
1. Stimulus < RBC count and availability of O2, > tissue 2. Decreased Iron Absorption - Malnutrition,
demand for O2 Imbalanced diet intake
2. < O2 level in blood 3. Decreased Iron Absorption - Celiac disease (gene
3. Kidney releases erythropoietin disorder characterized by autoimmune reaction to
4. Erythropoietin stimulate bone marrow ingestion of gluten & destroy small intestine),
5. More RBC produced inflammatory bowel disease, partial gastrectomy
6. > O2 carrying ability of blood 4. Increased Iron Requirements - Pregnancy,
CLASSIFICATION Lactation
1. Hypoproliferative Anemia RISK FACTORS
- underproduction of RBC production 1. Pregnancy - due to lactation process
2. Bleeding 2. Menorrhagia - loss of blood > 7d
- RBC loss 3. Strict vegetarian diets - low intake of iron
3. Hemolytic 4. Older population - reduced in iron absorption
- RBC destruction PATHOPHISIOLOGY
HYPOPROLIFERATIVE ANEMIA 1. Inadequate iron (low intake, absorption loss)
1. Iron deficiency anemia 2. Low formation of Hb
- microcytic hypochromic (large size, < colour) 3. Low Hb level leads to anaemia (signs & symptoms)
2. Vitamin B12 deficiency anemia CLINICAL MANIFESTATION
- macrocytic normochromic (large size, N colour) 1. Asymptomatic unless severe
3. Folic acid deficiency anemia 2. Fatigue
- macrocytic hypochromic (large size, < colour) 3. Headache
PATHOPHYSIOLOGY 4. Dyspnea
1. Lack of RBC reduces O2 being carried 5. Palpitations
2. Inadequate O2 to tissue 6. Pallor
3. Resulting in tissue hypoxia 7. Angular stomatitis (swollen/redness at end of lips)
4. Body attempts to compensate 8. Dizziness
5. > cardiac output by > stroke volume/heart rate 9. Glossitis (inflammation of tongue)
* CO: total blood pumps out by ventricle in a min 10. Cheilosis (inflammation of lips)
* SV: volume of blood pumps out by ventricle each 11. Brittle nails & spoon-shaped nails
contraction (N: 70) DIAGNOSTIC TEST
CLINICAL MANIFESTATION 1. Blood Test
1. Pallor (pale skin colour) - FBC
2. Tachycardia (> PR) - Hematocrit
3. Tachypnea (> RR) - Hemoglobin
4. Dyspnea(difficulty in breathing) - Serum iron
5. Palpitations (noticeably rapid HR) - Serum ferritin
6. Dizziness TIBC (total iron binding capacity) >
- FBC (full blood picture) CLINICAL MANIFESTATION
2. Radiographic Study 1. Weight loss
- OGDS (esophagogastroduodenoscopy) 2. Poor appetite
- gastroscopy 3. Nausea
- sigmoidoscopy 4. Vomiting
3. Stool for occult blood (FOBT) 5. Abdominal distension
- detect the hidden blood in feces 6. Diarrhea
MANAGEMENT 7. Constipation
1. Diagnosis & correction of underlying cause 8. Neurologic disorder (paresthesia, memory loss
2. Diet modification (high iron food) depression, cognitive prob)
3. Supplemental iron DIAGNOSTIC TEST
4. Monitoring on the compliant on treatment 1. Blood Test
5. Change of lifestyle (avoid alcohol & correct cooking - FBC
method) - FBP
IRON ABSOPRTION - Reticulocyte count (measure RBC to evaluate bone
INHIBITING marrow in producing RBC/low reticulocyte)
1. Coffee and tea (tannins inhibits absorption) - Hb & HCT level
2. Milk (casein binds to iron & slow down absorption) - Cobalamin Test (measure how much VB12 in blood)
3. Dietary supplement contained Ca, Zn, Mg or Cu - Schilling Test (oral radioactive VB12>check
(compete for absorption) urine>absence means PA) *not practice now
4. Antacid, H2 blockers & proton pump inhibitors MEDICAL MANAGEMENT
(neutralizes stomach acid) 1. Cobalamin Therapy
5. Tetracycline antibiotics (ferrous sulphate < - Lifelong therapy
absorption) - Parental administration of VB12 (injection)
FACILITING - cyanocobalamin/hydroxocobalamin
1. Vitamin C (acidic foods) UNIT 1.4 - FOLIC ACID DEFICIENCY ANAMEIA
2. Non enteric coated iron tablets (to protect iron in DEFINITION - Insufficient of Folic acid (VitB) for the
gastric environment) formation of heme, pigmented, iron-containing
3. Fasting ingestion of iron supplement (absorb portion of haemoglobin in RBC
better when fasting for about 8h) ETIOLOGY
HEALTH TEACHING ON TAKING IRON SUPPLEMET 1. Diet
1. Take iron tablet on empty stomach (absorption <  Lack intake of green leafy vegetables, fruits
with foods) & nuts
2. > intake of Vit C (enhance iron absorption) 2. Chronic alcoholism
3. Encourage intake of high fibres (minimize  Impairs folate absorption in small intestine
constipation) 3. Eating disorder
4. Remind pt that stool will be dark colour (due to the  Anorexia causes not eating foods
active agent in iron pills) containing folic acid
COMPLICATIONS 4. Hemodialysis patient
1. Heart failure - low Hb to carry O2  Remove folate by diffusion & lead to a
2. Problems during pregnancy - premature births & deficiency after several weeks of therapy
low weight of baby 5. Medications such as long-term use of anti-
3. Growth problems - delay growth & development convulsant medications
4. Susceptibility to infections - prone to get one  Medications can antagonize folate
UNIT 1.3 - PERNICIOUS ANAEMIA utilization & inhibit its absorption
DEFINITION - Autoimmune disorder characterized by CLINICAL MANIFESTATION
the absence of intrinsic factor in gastric secretions, 1. Weight loss
leading to malabsorption of cobalamin (VitB12) 2. Poor appetite
ETIOLOGY & RISK FACTORS 3. Nausea
1. Gastric Atrophy 4. Vomiting
- no parietal cell so intrinsic factor can’t be produced 5. Abdominal distension
2. Loss of intrinsic factor or loss of parietal cells 6. Diarrhea
- gastrectomy 7. Constipation
- ileal resection UNIT 1.5 - BLOOD TRANSFUSSION
- congenital DEFINITION - Procedure of administering whole
PATHOPHYSIOLOGY blood or blood components via intravenous route
1. < or no cobalamin PURPOSE
2. Impaired DNA synthesis & cell replication 1. To replace circulatory blood volume
3. RBC precursor (erythroblast /reticulocyte) don’t 2. To replace plasma loss by replacing plasma protein
divide normally & RBC poorly function 3. To correct any blood components deficiency
4. Production of myelin on nerve is affected =  Packed cells
neurologic deterioration  Platelets concentration
  Cryoprecipitate (clotting factors)
TYPE OF BLOOD PRODCUCT
1. Whole Blood
 Large amount contains plasma & osmotic
components (albumin) to help draw fluid
back into vessels from surrounding tissue
2. Packed RBC (PRBC)
 Made up of removal of virtually all plasma
to > O2 level
3. Fresh Frozen Plasma (FFP)
 To > clotting factor level, albumin, globulin
& fibrinogen
4. Cryoprecipitate
 Consist all clotting factors (fibrinogen,
Factor VIII, Factor IX)
5. Platelet concentrate (50ml)
 To > platelet count in dengue patient
CALCULATION OF DROP PER MIN
mls ordered x 20 (drop factor fixed)
min
TIME FRAME FOR TRANSFUSSION
1. Whole blood: within 4 hours
2. Packed cells: within 4 hours
3. FFP: Within 30 mins
4. Cryoprecipitate: within 30 mins
5. Platelets concentrate: within 30 mins
BLOOD TRANSFUSSION
1. Obtained from blood bank after branulla is
inserted
2. Confirm pt’s identification
 Wrist band
 Pt state name)
3. Confirm prescription by checking the doc order
 Verify right blood with doc
 Verify right blood transfusion type
 Verify screening
 Verify expiry date
 Verify consent
4. Perform baseline monitoring of vital sign
 BP (1st h: every 15mins, 2nd h: every
30mins, 3rd h: hourly)
5. Prime line with 0.9% N/S. Infuse first 50ml of N/S
6. Transfuse blood pack & titrate flow rate
accordingly
 Start transfusion with 10dpm
 Monitor pt within first 15mins of
transfusion (restlessness, hives, SOB,
nausea/vomit, flushing, fever/chills,
hematuria)
 Check vital sign hourly till completed
7. Monitor time limit of transfusion
 Inform pt time of completion
8. Identify reactions
 Febrile non-hemolytic transfusion reaction
- fever > 38’C
 Acute hemolytic transfusion reaction (triad
components)
- fever, flank pain, red/brown urine
 Anaphylactic reaction
- acute BP drop & airways narrow
 Transfusion-associated graft-vs-host
disease (GVHD)
- systemic disorder occurs when immune
cell recognize host as foreign & attack them
 Infection
- sepsis
9. Check for accuracy & completeness of
documentation
 I/O chart
 Observation chart
 Nursing notes (time start, time completion,
reactions)
NURSING RESPONSIBILITY BEFORE
1. Blood must be transfused within 30min after it had
been collected to prevent bacterial growth &
destruction of RBC
2. Practice aseptic technique when administer blood
transfusion
3. Blood transfusion must be counterchecked with
another SN regarding:
 Name
 Age
 Sex
 Blood group
 Date of expiry
4. Check for signed consent for transfusion for
medical legal purpose
5. Inform pt & explain procedure to allay anxiety &
gain cooperation
6. Ask pt to pass urine to ensure pt is comfortable
during procedure
7. Insert large bore of branulla (20G)
8. Check IV canulla is present
9. Check pt’s bital sign for baseline data to detect any
transfusion reaction
10. Administer IV diuretics as ordered prior to
transfusion to prevent pulmonary edema
11. Assess pt:
 Itching
 Hives
 Fever & chills
 SOB
 Swelling
 Backache
NURSING RESPONSIBILITY DURING
1. Blood to be transfused within 30min after removal
from refrigerator
2. Observe flow rate of blood transfusion
3. Observe infusion site for extravasation or branulla
dislodgement
4. Ensure tubing is not kinked & no air bubble &
blood clot
5. Observe pt closely for any reaction & monitor vital
sign
6. Inform pt about sign & symptoms of reaction &
advice to report to nurse or doc
7. Maintain I/O chart balance
8. Duration of transfusion don’t exceed 4h due to >
risk of bacterial proliferation
9. Never inject any med into branulla with blood
because bacterial contamination of blood product
10. Change blood tubing each time for a new pack
NURSING RESPONSIBILITY AFTER
1. Dispose used material into clinical waste
2. Empty blood packs to be returned to blood bank 4. Bacterial & viral infections such as TB & hepatitis
according to hosp policy  Supress 3 cells line caused pancytopenia
3. N/S solution should be infused after transfusion is PATHOPHYSIOLOGY
completed to flush the line BM become > fatty cells, < in hemopoietic cell
ADVERSE REACTIONS II
1. Febril Non-hemolytic reaction Insufficient RBC
 Due to presence of donor leukocytes II
 Treat with antipyretics Resulting in pancytopenia
2. Acute Hemolytic reaction CLINICAL MENIFESTATIONS
 Due to incompatible of donor & recipient’s 1. Result in < Hb, < O2 transport, lead to weakness
blood  Fatigue
 Most dangerous  Pallor
3. Allergic reaction  SOB
 Due to sensitivity reaction to plasma  Tachycardia & heart failure
protein  Headache
 Treat with antihistamine  BM failure
4. Anaphylactic reaction  Bleeding
 Treat with adrenaline, corticosteroid (anti-  Infection
inflammatory)  Bruises
5. Circulatory overload DIAGNOSTIC TEST
 Due to fast blood flow 1. Blood test
 Treat with diuretics  FBC - Hb <7gm, RBC normocytic
 Dyspnea, tachycardia, jugular vein  < total iron binding capacity
distension  > Iron serum level
6. Infection (sepsis) 2. Bone marrow Biopsy
 Due to delay of transfusion, > 4h  Dry tap: Condition when no marrow in
NURSING MANAGEMENT FOR REACTION aspiration
1. Stop transfusion temporarily & inform doc  Blood tap: Obtain blood but no marrow
2. St up N/S infusion (another site) at low rate particle from aspiration
3. Send blood pack & tubing to blood bank for repeat MANAGEMENT
typing & culture 1. Early identification & correction of underlying prob
4. Obtain blood specimen for blood investigation in 2. Most measure aims at prevention of infection &
laboratory bleeding
5. Obtain urine specimen for investigation in lab to 3. BM transplant
detect hemoglobin in urine 4. Medication
6. Monitor pt’s vital sign  Erythropoietin stimulates production of
7. Monitor urine output RBC
8. Fill in “Adverse Transfusion Reaction Report Form”  Filgrastim stimulates production of WBC
9. Document reactions & actions in nursing notes  IV anti-thymosin immunoglobulin
UNIT 2.1 APLASTIC ANEMIA - Action: kill T-lymphocyte in immune
DEFINITION system to prevent it from attacking BM
 Disorder of unknown etiology characterized stem cells
by low num. of WBC (leukopenia) & low - Given by IV for 8-12 a day for 4 days
platelet count (thrombocytopenia) PREVENT INFECTION
resulting from aplasia of bone marrow 1. Prevent high risk places
(bone marrow w/o its tissue)  Crowded
 Condition where bone marrow doesn’t  Hospital
produce sufficient new cells to replenish 2. Personal hygiene
blood cells  Mask
*Pancytopenia: lower counts of RBC, WBC &
 Hand washing
platelets
3. Antibiotic
ETIOLOGY  Whenever have infection
1. Congenital  Non-prophylactic is important of normal
 Trait abnormality since birth such as flora
immune system attacks stem cells in BM PREVENT HEMORRHAGE
2. Exposure to toxic substances such as industrial
1. Transfuse 6-8 units of platelet when there’s
chemicals
haemorrhage
 Benzenes
TREAT ANEMIA
3. Chemotherapy medications
1. Keep Hb 12-14mg%
 Temporary side effects due to
2. Donor blood < 24h
chemotherapy drugs damage healthy stem
3. Repeat transfusion after 6-8 weeks when Hb<7-9
cells
BONE MARROW
1. Done to pt <40y 2. Anterior superior iliac chest
2. Procedure  pt who can’t lie on their abdomen
 Immunosuppressive drug 3. Sternum
- 2-3 weeks pt don’t have immunity  only in adults & kids > 12y, only aspiration
- isolate pt to avoid acquire disease  dangerous
 Transplant intravenously 4. Anterior medial surface of tibia
 After 3 weeks, pt regains immune function  infants < 1y
& hemopoietic cells 5. Spinous process of L3-L4 vertebrae
 To prevent host versus graft reaction  rarely used
 Complication is 50% liver failure resulting INSTRUMENTS REQUIRED
from host versus graft reaction 1. 16-18 gauge, 1-1 ¾ inch BM biopsy needle
MEDICATION 2. 10-20ml sterile syringe
1. IV anti-thymocyte immunoglobulin for 1 week 3. Clean slide
2. Anabolic agent for mild aplastic anemia 4. Clear petri or watch glass
 (100mg) 6-12month 5. EDTA & saline
3. High dose of corticosteroid 6. Surgery prep. Equipment
 Osteoporotic fracture, susceptibility to 7. Sedative/anesthesia
infection glucose intolerance NURSING RESPONSIBILITY AFTER
4. Epoetin Injection 1. Make sure pt comfortable
 Stimulate RBC production in BM 2. Maintain supine position for 6h if taken from iliac
PROGNOSIS OF APLASTIC ANEMIA crest, lie pt on affected site
1. Difficult to predict 3. Observe client
2. Very serious  vital sign
 Die within 6 month – 1 year after  hematoma
presentation of symptoms  pain/discomfort
UNIT 2.2 BONE MARROW puncture  after 48h for warmth & redness
 Perform when peripheral blood smears 4. Ensure specimen is labelled correctly with form
failed to diagnose the abnormalities & 5. Clean up BM aspiration set & send to CSSD (centre
diseases sterile supply department) for autoclaving
TYPE OF BONE MARROW EXAMINATION COMPLICATION
1. BM aspiration 1. Excessive bleeding
 Use a thin needle to remove small amount  concern with bleeding
of liquid BM in the back of hipbone (pelvis) disorder/thrombocytopenic
2. BM biopsy  Iatrogenic marrow infection
 Remove small piece of bone tissue & UNIT 2.3 SICKLE CELL ANEMIA
enclosed marrow DEFINITION - Inheritance Hb disorder when RBC are
INDICATIONS FOR BM ASPIRATION in abnormal crescent shape
1. Diagnosis of cause & severity of haematological PATHOPHYSIOLOGY
conditions: When exposed to < in O2
 Anemia II
 Leukemia (acute & chronic) Defective Hb forms a gel like substance contains Hb
 Myelodysplastic disorder (cancer/ crystals with affected RBS
immature cells in BM), Myeloproliferative II
neoplasm (overproduction) Crystal clump together assumes a crescent sickle
 Fever of unknown origin shape
 Thrombocytopenia II
 Pancytopenia Neutropenia (< neutrophils) Sickled cells become rigid, sticky & fragile (hemolysis)
 Immunodeficiency Cells impede circulation, causing microinfarcts tissue
2. Staging hepoxia
 Used to assess severity, progress of CLINICAL MANIFESTATION
disease, distribution of disease & enable 1. Hb: < 7-10
administration of potentially curative 2. Pallor
radiation therapy (Hodgkin Lymphoma) 3. Fatigue (lack of O2 due to low Hb)
3. Therapeutic monitoring to note progress of disease 4. < tolerance for exercise
& effectiveness of therapy (Hodgkin & Non- 5. Jaundice (> bilirubin)
Lymphoma) 6. Tachycardia
STAFF REQUIRED 7. Cardiac murmurs (abnormal heart sound)
1. Doc to perform 8. Cardiomegaly (heart enlargement)
2. Nurse to assist SICKLE CELL CRISIS
3. Lab technician 1. Sudden onset
COMMON SITE OF COLLECTION 2. Serious symptoms require immediate medical
1. Posterior superior iliac crest attention
 3. Acute chest syndrome 2. Transportation
4. Heart failure & dysrhythmias  Bilirubin leaves site of production &
5. Microvascular-occlusive transported in plasma bound to albumin
 Tissue pain & necrosis caused by plugs of  Binding capacity of serum albumin to bind
sickled cells in microcirculation bilirubin
BLOOD TEST 3. Hepatic uptake
1. FBC  Selective & highly efficient system for
 < HCT removing unconjugated bilirubin from
2. Full Blood Picture plasma
 Sickled cells smear  Transferred to endoplasmic reticulum (site
TREATMENT of bilirubin conjugation)
1. Pain management 4. Conjugation
 NSAID (Ibuprofen)  Binding unconjugated bilirubin to
 Morphine glucuronic acid
2. Exchange transfusion (acute chest syndrome) 5. Excretion
3. Blood transfusion  Conjugated bilirubin is excreted into bile
4. Pharmacology  Transported to small intestine & excreted
 Hydroxyurea: chemotherapy agent > HbF (< as stool
formation of sickled cells) 6. Enterohepatic (GIT) circulation
5. Oxygen therapy  Deconjugation of bilirubin in intestine
6. Peripheral bloods stem cell transplant resulting formation of urobilinogen where
COMPLICATIONTS it’s absorbed & transported by portal
1. Pulmonary embolism circulation
2. Pulmonary infarction  Urobilinogen excreted in urine & converted
3. Pulmonary hypertension to stercobilinogen b4 excreted in feces as
4. Death stercobilin
NURSING PRIORITIES TYPE JAUNDICE
1. Promote adequate cellular O2 1. Prehepatic (haemolytic)
2. Alleviate pain  Due to > production bilirubin with
3. Prevent complications erythrocyte destruction
4. Provide information about disease process,  Extrinsic causes like reabsorption of large
prognosis & treatment needs haematoma
UNIT 2. 4 JAUDICE (ICTERUS)  Intrinsic defects in RBC leading to hemolytic
DEFINITION - Yellowish discolouration of skin, anemia
mucous membrane & sclera off eyes due to elevated 2. Hepatic (hepatocellular)
levels of serum bilirubin > 2.5mg/dL  Due to dysfunction of hepatocytes < ability
(hyperbilirubinemia) to remove bilirubin from blood & convert it
BILIRUBIN METABOLISM into bile
- Formed by breakdown of heme in RBC &  Caused by virus infection, alcohol, rare
liver excretes it out genetic (Gilbert’s syndrome), medication
TYPE OF BILIRUBIN (acetaminophen toxicity)
1. Unconjugated bilirubin (indirect) 3. Post-hepatic (obstructive)
 Heme released from Hb, it’s converted to  Due to obstruction of flow of bile &
unconjugated resulting in clogged-up bile through
 Unconjugated is not H2O soluble & it travels hepatocytes to blood
in bloodstream to liver  Caused by cholestasis due to gallstone &
2. Conjugated bilirubin (direct) cancer at head pancreas
 Bilirubin converted from unconjugated > CLINICAL MANIFESTAION
conjugated in liver 1. Severity symptoms depends on underlying causes
 H2O soluble & progress of diseases
 Conjugated turns into bile & enters small 2. Yellow colouration of skin & sclera
intestine & eventually eliminated 3. If infection:
BILE  Fever & chills
1. Some bile will be absorbed in kidney  Abdominal pain
2. Emulsify lipid in small intestine  Flu-like symptoms
3. Store in gall bladder  Dark urine & clay stool
BILIRUBIN METABOLISM 4. If not infection:
1. Production  Weight loss
 Bilirubin is end product of catabolism of  Itchy skin
heme, major source is circulating Hb  Nausea & vomit
 75% unconjugated bilirubin produced from  Malaise
normal destruction of RBS  Anorexia
  Fatigue 4. If progressed to hemeloytic anemia, > aggressive
 Dark urine treatment maybe required
 Clay stool MANAGEMENT
5. If biliary tract cancer: 1. Severe hemolytic
 Weight loss  Hospitalized
 Itchy skin  Blood transfusion
 Nausea & vomit  O2 therapy
 Malaise PATIENT EDUCATION
 Anorexia fatigue 1. Genetic counselling & screening fam history of
 Dark urine G6PD
 Clay stool 2. Explain pathophysiology & process of disease
DIAGNOSTIC TEST 3. Medical alert bracelet for disease identification
1. Blood test 4. Avoid triggers
 AST & ALT <  Fava beans
 GGT <  Naphthalene mothball
 Total bilirubin <  Oxidant drugs
 Prothrombin Time (PT) prolonged - Antibiotic: sulfamethoxazole
2. Urine test - Antimalarials: primaquine
 Urobilinogen > - Antipyretics: aspirin
3. Ultrasound COMPLICATIONS
4. ERCP 1. Prolonged neonatal jaundice, possible kernicterus
 Endoscopic retrograde (brain damage)
 Cholangiopancreatography 2. Ophthalmological damage due to intraocular IV
TREATMENT hemolysis (eye bleeding)
1. Treatment depends on underlying cause 3. Susceptibility to infection
2. Injection IV Vit K 4. Hemolytic crises in response:
3. Antihistamine  Illness
4. Antiemetics  Certain drugs
COMPLICATIONS  Certain foods (beans)
1. Neonatal  Certain chemicals
 Acute bilirubin encephalopathy 5. Severe crises caused acute kidney failure
 Kernicterus (> bilirubin lead to brain 2.6 THALASSEMIA
damage) DEFINITION - Disorder of the Hb due to lack of globin
2. Adult chain that leads to hemolysis
 Anemia GENETIC TYPE OF THALASSEMIA
 Bleeding 1. Beta thalassemia: < production of normal beta
 Infection/sepsis globin chain
 Chronic hepatitis 2. Alpha thalassemia: < normal alpha globin chain
 Cancer PATHOPHYSIOLOGY
 Liver failure 1. Results when there’s deletion in production of
 Kidney failure globin chain from any/all globin genes
2.5 GLUCOSE 6-PHOSPHATE DEHYDROGENASE 2. Genes responsible for regulating synthesis &
DEFICIENCY (G6PD) structure of globins
DEFINITION - Is a genetic abnormality that lack of 3. Hb formed is unstable & precipitates to form Heinz
G6PD leads to hemolysis of RBC due to the bodies which may damage RBC
obstruction of the membrane TYPE OF THALASSEMIA
*X-linked recessive, commonly in men Thalassemia trait/minor
CLINICAL MANIFESTATION  Carry genetic trait but don’t usually
1. Pallor experience any health probs except mild
2. Tachycardia anemia
3. SOB  May have either alpha or beta trait,
4. Jaundice depends on which form of protein lack
5. Hemoglobulinuria TYPE OFBETA THALASSEMIA
6. Tae colour urine 1. Thalassemia Major (Cooley/s Anemia)
DIAGNOSTIC TEST  Severe B-thalassemia
1. Neonatal screening  Presence of 2 mutated genes caused either
 Check blood right after birth severe < or no any beta globin
TREATMENT 2. Thalassemia Minor
1. Removing triggers that’s caused symptoms  Presence of 1 normal gene & 1 mutation
2. If infection, infection is treated accordingly  Caused mild-moderate mild anemia
3. If current medication then discontinue CLINICAL MANIFESTATION
1. Minor
  Characterized by mild anemia  Give drug to < iron level
2. Beta 3. Folate supplement
 Symptoms appear at first 2 years  > RBC formation
- fatigue & weakness 4. BM transplant
- pallor or jaundice  Change mother cell due to gene
- protruding abdomen with enlarged spleen 5. Surgical treatment
& liver  Enlargement of spleen
- dark urine 6. Supportive measure
- abnormal facial bone & poor growth  Need extra observation
- poor appetite 7. Genetic counselling
- adolescent with severe form of beta may  Sharing opinion about having child or not
experience delayed puberty BENEFITS BLOOD TRANSFUSION
DIAGNOSTIC TEST 1. Improve tissue oxygenation & prevent chronic
1. Physical examination may reveal hypoxia
hepatosplenomegaly - extramedullary hematopoiesis 2. Improve normal growth & development
2. FBP 3. Prevent erythropoiesis thus avoiding expansion of
 Microcytic hypochromic anemia BM & extra medullary hemolysisi
 Immature RBC 4. Reduce hemolysis
3. FBC reveals anemia 5. Reduce hepatosplenectomy
4. Hb electrophoresis 6. Reduce GIT absorption of iron
 Measure different type Hb COMPLICATION TRANSFUSION
5. Mutational analysis to detect thalassemia that 1. Non hemolytic febrile transfusion reaction
can’t be detected with Hb electrophoresis 2. Allergic reaction
COMPLICATIONS THALASSEMIA MAJOR 3. Acute & delayed hemolytic reactions
1. Severe anemia 4. Transfusion associated circulatory overload
 Ineffective erythropoiesis 5. Alloimmunization (immune response to foreign
 Extramedullary hematopoiesis antigens after exposure to genetically different cells)
2. Iron overload resulting from: 6. Iron overload
 Transfusion 7. Transfusion transmitted infections
 Iron absorption CHELATING AGENTS
3. Pallor & jaundice 1. Deferoxamine (DFO)
 Excessive bilirubin 2. Deferipone (Kelfer)
4. Skull & bones maybe deformed 3. Deferasirox (Exjade)
 Due to erythroid hyperplasia with TREATMENT OF THALASSEMIA
intramedullary expansion cortical bone 1. Chelation therapy
thinning (compact bone thinner)  Remove excess iron
5. Cardiac failure related to severe anemia or iron 2. Folate supplements
overload  Folic acid
 Severe anemia 3. Transplant
 Iron overload  BM
6. Repeated blood transfusion  Stem cells
 Hepatomegaly or chronic hepatitis due to SURGICAL TREATMENT
iron overload 1. Splenectomy
7. Gallbladder contains bilirubin stones due to  To < transfusion requirements
lifelong hemolytic state 2. Surgical or orthodontic correction
8. Hepatosplenomegaly  To correct skeletal deformities of skull &
9. Iron overload caused endocrine dysfunction maxilla caused by erythroid hyperplasia
10. Transfusion-associated viral hepatitis resulting in 3. Cholecystectomy due to presence of bilirubin
portal hypotensionn stones
COMPLICATION MANAGEMENT THALASSEMIA MAJOR
1. Iron overload 1. Further treatment
2. Infection  Gene replacement
3. Bone deformities 2. Supportive care
4. Enlarged spleen  Management of endocrine & cardiac
 Due to destruction of large num. RBC complications
5. Slowed growth rate PREVENTION OF THALASSEMIA
 Slow child’s growth & delays puberty 1. Carrier screening
6. Congestive heart failure  Minor or carrier can be detected by Hb
MANAGEMENT electrophoresis
1. Periodic transfusion 2. Population education, mass screening, genetic
 Packed cell blood transfusion counselling & antenatal diagnosis & therapeutic
2. Iron chelation absorption of affected pregnancy
UNIT 3 POLYCYTHEMIA