ANAEMIA AND BLEEDING

Raja Noor Azimah Mohd Norhalimi Siti Nazihah Yim Wei Sen Poo Suk Ting

CONTENTS
Anaemia Bleeding - classification of - classification anaemia - causes of bleeding - causes of anaemia disorder - History & Examination - History & examination - Iron deficiency - Haemophilia anaemia (IDA) - Immune - Thalassemia Thrombocytopenic Purpura (ITP)

ANAEMIA

DEFINITION
WHO definition: condition in which the Hb level below a defined range, their O2 carrying capacity is insufficient to meet physiological needs, which vary by age, sex, altitude, smoking, and pregnancy status.
Age 2 week 3 month 6 month- 6 year 7-12 year 12-18 year female 12-18 year male Hb (g/dl) 13.7-20.1 9.5 -14.5 10.5 - 14.0 11.0 -16.0 12.0 - 16.0 13.0 -18.0 <11 <12 <13 <14

STRUCTURE

 Hemoglobin (Hb) - The amount of hemoglobin in the blood, expressed in grams per decilitre (g/dL) Mean corpuscular volume (MCV) - the average volume of the red cells, measured in femtolitres (fL). Mean corpuscular hemoglobin (MCH) - the average amount of hemoglobin per red blood cell, in picograms (pg). Mean corpuscular hemoglobin concentration (MCHC) - the average concentration of hemoglobin in the cells Hematocrit or packed cell volume (PCV) - This is the fraction of whole blood volume that consists of red blood cells. Red blood cell distribution width (RDW) - a measure of the variation of the RBC population

Classification of anemia based on MCV CLASSIFICATION

(MCV)

Microcytic
(abnormal small red blood cell MCV <75)

Normocytic
(normal red blood cell)

Macrocytic
(abnormal large red blood cell MCV >95)

anisocytosis, microcytes and marked hypochromia

large, dense, oversized of red blood cells

Causes: IDA Thalassemia

Causes: Blood loss Hemolytic anaemia Chronic illness

Causes: Vit B12 deficiency Folate deficiency

Microcytic Normocytic -Iron deficiency* -Chronic inflammatory disease -Thalassemias* -Chronic inflammatory disease -Sideroblastic anemia -Copper deficiency

Macrocytic With megaloblastic bone marrow -Vitamin B12 deficiency* -Recent blood loss -Folic acid deficiency* Without megaloblastic -Malignancy/marrow bone marrow infiltration -Liver disease -Chronic renal failure -Hypothyroidism -Down Syndrome -Transient Bone marrow failure erythroblastopenia states -Myelodysplasia -G6PD deficiency -Acquired aplastic anemia -Marrow aplasia/hypoplasia -Fanconi anemia

CLINICAL FEATURES
Symptoms
Lethargy SOB Poor effort tolerance Headache Excessive sleeping (especially in infants) Poor feeding Syncope

Signs
Pallor, jaundice Tachycardia, tachypnoea Collapsing pulse, bounding pulse Flow murmur, cardiomegaly Brittle spoon nail (koilonychia), angular stomatitis, glossitis Bleeding site, ecchymosis Hepatosplenomegaly Lymphadenophaty Sn of cardiac failure

HOW TO APPROACH??
History - Gender - Symptoms such as pallor, fatigue, exercise intolerance, irritability, increase in sleeping, jaundice, dark urine, weight loss - Onset of symptoms: rapid/ gradual - History of blood loss: Blood in diapers, malaena, menstrual history

History of trauma Growth parameters acute/chronic Any worms in faeces Neonatal jaundice or episodes of jaundice in the past Other signs of hypothyroidism?

 Systemic enquiry - infection - liver disease - renal disease Past Medical History - any iron supplement before? - previous blood transfusion? Birth History

 Diet History - fava bean G6PD - When did he start whole milk? - How much milk does he drink now? - Does he eat anything unusual (paper, dirt) or chew on ice? - Picky eater? lead poisoning - Vegetarian? - Mother s diet if breast feeding. - Detail of all the meals daily

 Development History
- mental retardation? - developmental delay

Family History - anemia - iron supplements - regular transfusion, gallstone early in life

PHYSICAL EXAMINATION General

Alertness consciousness activity pallor jaundice growth characteristic facies: tower skull, frontal bossing, maxillary hypertrophy with prominent cheek

 Peripheral Examination
koilonychia increase pulse rate (tachycardia)

Interdigitation of Gum (gum hypertrophy) Evidence of CHF

cardiomegaly ejection systolic murmur hepatomegaly

Hepatosplenomegaly

COMPLICATIONS
Heart failure Pulmonary oedema Cardiovascular collapse Sudden cardiac death

PHYSIOLOGICAL ANAEMIA IN EARLY INFANCY

 Hb concentration in newborn infants is 1523g/dL. Min level is at 2-3mo. Lower limit is 9.5g/dL bcoz - erythroid hypoplasia of BM ( dec in blood volume) - change to HbF to HbA (from fetal life to 6mo)

CONTENTS
Anaemia Bleeding - classification of - classification anaemia - causes of bleeding - causes of anaemia disorder - History & Examination - History & examination - Iron deficiency - Haemophilia anaemia (IDA) - Immune - Thalassemia Thrombocytopenic Purpura (ITP)

IRON DEFICIENCY ANEMIA

 The most common cause of anemia in childhood Prevalence higher in poorer socioeconomic groups Incidence of iron deficiency in most reported series appears to be related to socioeconomic factors but not to age, sex or race.

Pathophysiology
Iron absorption Iron transport and usage Iron storage Iron deficiency
Occur in the duodenum and upper jejunum aided by ascorbic acid, fructose and amino acid
Iron from intestinal mucosal cells is transferred to transferrin transport to the bone marrow for haemoglobin synthesis ( 70%) Small amount( 4%) are used for synthesis myoglobin, and haem enzymes(cytochromes)

Additional iron (25%) is stores in the liver, spleen and bone marrow as ferritin and haemosiderin
iron requirement exceeds intake, iron stores are used up, and the patient becomes iron deficient. Poor iron stores results in impaired haemoglobin synthesis and a hypochromic, microcytic anaemia.

Classification of iron deficiency

Stages Iron stores Serum iron ug/dl 90-150 Transferrin saturation% 30 Serum ferritin ug/dl 35 anaemia

Normal

present

None

Iron depletion Iron deficiency Iron deficiency anemia

low

< 60

20

10-30

None

absent

< 60

< 15

< 10

None

absent

< 30

< 10

< 10

Microcytic hypochromic

AETIOLOGY
Chronic blood loss - Meckel s diverticulum, peptic ulcer, ITP, parasitic infection(hookworn), hemoglobinuria Increase demand - Prematurity/ low birth weight infant - rapid rate of growth Poor dietary intake - poor socioeconomic - excessive cow s milk diet - delayed weaning Impaired absorption - Chronic diarrhea, GI abnormalities, hookworm infection, malabsorption

History and Physical Examination

History
Irritability, prolonged fatigue History of bleeding episode( from intestinal tract, menstruation) Pallor Pica : persistent craving and inappropriate eating of non-food substances ( soil, chalk) Diet (age of weaning)

Physical examination
Pallor( conjunctiva, palmar) Atrophic glossitis, angular stomatitis Koilonychias( spoon shaped nail) Tachycardia, cardiac enlargement and cardiac failure

Investigations
Full blood count HB RDW MCV MCH MCHC Normal WCC and Plt Serum Ferritin -- male : 22 322 g/L female : 10 291 g/L Stool for occult blood loss, ova and cyst for parasititc infection

FULL BLOOD PICTURE



Anisocytosis Poikilocytosis Hypochromia microcytosis

TREATMENT
1) Nutritional counselling(Dietary advise)  Well balance diet  Do not give cow's milk  Food with high iron  If formula, give formula fortified with iron  High vitamin C food to increase absorption  No food containing tannin (tea)  Less high fibre food containing phytates ( raw whole grains, legumes, seeds, and nuts  Iron supplementation  Maintain breastfeeding

2) Oral iron y A daily total dose of 6 mg/kg/ day of elemental iron in 3 divided doses y Syrup FAC(ferrous ammonium citrate) 1mg/ml or T.ferrous fumarate 200mg y Should be continued for 6-8 weeks after Hb is normal y Side effects- Nausea, vomiting, epigastric pain, constipation

 Failure of response to oral iron caused by: - Non compliance - Inadequate iron dosage - Unrecognized blood loss - Incorrect diagnosis - Impaired GI absorption

3) Parenteral iron
Eg: Oral dextran, Sodium ferric gluconate and iron sucrose Indication: - Poor absorption, eg. Gut resection - compensation of frequent blood loss - Intolerance iron by mouth - When patient cannot relied upon to continue medication at home Rarely given because a lots of side effect such as fever, chills, backache, myalgia, dizziness, syncope, rash and anaphylactic shock

4) Blood transfusion indicated only in severe anemia and cardiac failure slow administration of 5 10 ml/kg body weight of packed red cells over 4-6 hours is adequate to raise the hemoglobin to a safe level ( rapid blood transfusion may cause hyperkalemia and cardiac decompensation). If severe anemia( hb< 4g/dl), add IV frusemide 1mg/kg ( to prevent fluid overload Pulmonary edema)

Prognosis
Good. In most cases, the blood counts will return to normal in 2 months. Must continue taking iron supplements for another 6 to 12 months. Iron supplementation improves learning, memory, and cognitive test performance in adolescents who have low levels of iron.

THALASSEMIA

Definition
Is a blood disorder passed down through families (inherited) in which the body makes an abnormal form of haemoglobin Resulting in 1)Excessive destruction of red blood cells 2)Ineffective erythropoisis 3)Premature removal of red blood cells by spleen which leads to hypochromic microcytic anemia

Thalassemia Syndromes
Syndromes

Thalassemia Minor (trait or carrier)

Thalassemia Intermedia

Thalassemia Major

Clinical Features
Syndrome Clinical Age of Presentation Need for Blood Transfusion None None ; occ some Regular

Trait Thalintermedia Thal-major

Asymptomatic Any age Moderately Severe Beta: Severe Alpha: Death After age 2 or later 1-2

Clinical Forms of Alpha & Beta thalassemia

Alpha
(1) (2) (3)

Beta Trait Beta-thalassemia intermedia

Trait Hb H disease

Hb Bart s hydrops fetalis Homozygous beta ( 0)thalassemia

Clinical Severity
Syndrome Trait Thal-Intermedia Thal-Major Degree of Globin Hb Level Chain Imbalance + >10gm/dL ++ +++ 7-10gm/dL <7 gm/ dL

Iron Status In Thalassemia Syndrome

Thalassemia trait Thalassemia intermedia Thalassemia major Normal Normal or iron overload Iron overload in the absence of iron chelation therapy

Thalassemia Diagnosis
Laboratory Tests Screening Confirmatory or definitive

Basic Screen Tests

Full Blood Picture Hb analysis Iron Status
RBC indices Reticulocyte count Blood smear

Screening for abnormal haemoglobin Quantification of Hb A, A2, Hb F

Confirmatory Tests
DNA studies Globin chain synthesis Structural analysis

Beta-thalassemia

 Decrease or absence in the synthesis of beta globin chains B+ : some globin chain synthesis B0 : no B globin chain synthesis

Clinical Features of Beta-Thalassemia Major

Globin chain imbalance (excess of alpha globin chain)

RBC damage

Ineffective erythropoises Anaemia Peripheral hemolysis

Anaemia

Hypoxia

Erythropoieten increase

General Effects of Anaemia

Pallor Generalised malaise, reduce activity Retardation of growth Cardiac failure

Thalassemia Facies

Laboratory Diagnosis
Beta-thalassemia Trait Hb > 10gm/dL ~ N MCV, MCH low MCHC N RDW N Serum ferritin N Hb A2 raised

Beta-thalassemia Carrier Identification

Hallmark of classical beta-thalassemia carrier Presence of an elevated Hb A2 level > 4%

Beta-thalassemia Intermedia Hb 7-10 gm/ dL MCV, MCH, MCHC Low RDW Increase Serum ferritin Increase Hb F raised

Beta-thalassemia Major Hb < 7gm/dL MCV, MCH, MCHC Low RDW Increase Serum ferritin Increase Hb F markedly raised

Thalassemia picture : Intermedia & thal major Anisopoikilocytosis Hypochromasia Target cells Basophilic stippling Nucleated RBCs

ALPHA THALASSEMIA

 Reduce synthesis of the alpha globin chains

+

:1 :2

globin deleted globins deleted

Hb Bart s Hydrops Fetalis

Hallmark of the condition -Presence of useless Hb : Hb Barts Incompatible with life Death in utero (23-38weeks) or soon after birth (within 6 hours) High oxygen affinity Functionally useless Hb for oxygen transfer

Functionally useless Hb for oxygen transfer

Severe hypoxia

Hydropic fetus

Laboratory Diagnosis
Hb Bart s Hydrops Fetalis Hb low RBC low HCT low MCV Increase MCH, MCHC low RDW increase

Full Blood Picture

Anisopoikilocytosis Macrocytosis Hypochromia Numerous nucleated RBCs

Management
Thalassemia trait/carrier - no need treatment Thalassemia intermedia -may require blood transfusion during fulminant infection Thalassemia Major -Transfusion dependent for life -Curative with stem cell transplantation (bone marrow, peripheral blood)

Initiation of BT - Hb < 7 on 2 occasions 2 weeks apart - Hb > 7 but presence of Sx & Sn Target - Pre-transfusion 9-10; post-transfusion 13.5-15.5 - 15-20ml/kg (max) packed red cell over 4 hours

Iron chelation therapy -Desferrioxamine (Desferal) Initiation - Child > 2 y/o and serum ferritin > 1000ng/ml (10-20 BTs) How? - 20-60mg/kg/day S.C. 8-10hrs/day, 5-7 nights/week. - Vit. C augments iron excretion Aim - Serum ferritin < 1000ng/ml

Complications of Desferal (MUST CHECK during ROUTINE F/UP) Skin reactions Yersinia infection Toxicity (>50mg/kg/day with low serum ferritin) - Ocular > Reduced vision, visual fields, night blindness; reversible - auditory > high tone deafness; not really reversible - growth retardation - skeletal > pseudo rickets, metaphyseal changes and vertebral growth retardation Iron overload > iron precipitates in organs - Endocrine (pituitary, thyroid, pancreas) --endocrine dysfunction - cardiac -- arrhythmias, pericarditis - liver -- hepatitis

Alternative options Oral iron chelator - Deferasirox. - > 2 y/o - 20-30 mg/kg/day, O.D. Bone marrow transplantation

Supplementation
Vitamin C Folate Zinc

Bleeding Disorder

 is the loss of blood or blood escape from the circulatory system

Bleeding

Internal -blood leaks from blood vessels inside the body

External

-through a natural opening -eg: vagina, mouth, nose, ear or anus

through a break in the skin

Severity of bleeding (WHO)

Grade 0 Grade 1 Grade 2 Grade 3 Grade 4 No bleeding Petechial bleeding Mild blood loss (clinical significant) Gross blood loss, requires transfusion (severe) debilitating blood loss, retinal or cerebral associated with fatality

Causes
Traumatic Injury - Abrasion - Excoriation - Hematoma - Laceration Intravascular changes; Medical Condition Extravascular changes - H.pylori infection - brain abscess - brain tumor

Acquired disorders Vitamin K deficiency Liver disease ITP DIC

Inherited disorders - Haemophilia - Von Willebrand s disease Intramural changes -aneurysms -dissections -Vasculitides -AVM

Symptoms
Blood coming from an open wound Bruising Shock : Confusion or decreasing alertness Dizziness or light-headedness after an injury Low blood pressure Paleness (pallor) Rapid pulse, increased heart rate Shortness of breath Weakness

Abdominal pain Abdominal swelling Chest pain External bleeding through a natural opening
Blood in the stool Blood in the urine Blood in the vomit Vaginal bleeding

How to approach a patient with bleeding

History
Age of onset Is there a lifelong bleeding history Cause of bleeding (after procedure or trauma) Pattern of bleeding: mucosal bleeding, bleeding into muscles or into joints Any bruises

 Systemic enquiry; bone marrow failure, infection, liver disease, renal disease Past medical Hx;
previous known bleeding disorder surgery done before dental extraction

Family history Medication history

 Examination;
Petechiae, bruising, mucosal bleeding, and oozing from venipuncture sites Joint swelling Anaemia, lymphadenopathy, hepatosplenomegaly

Investigation
FBC BUSE LFT ESR Coagulation Tests Special test; measurement of vWF

ITP

Immunomediated Thrombocytopenia Purpura (ITP)

Idiopathic Thrombocytopenic Purpura / Immune Thrombocytopenia. Clinical syndrome in which there is isolated thrombocytopenia with otherwise normal blood count with no clinically apparent associated condition that can cause thrombocytopenia. Commonest cause of thrombocytopenia in childhood

 Affects children between 2 and 10 years old, with onset often 1-2 weeks after a viral infection In 90% of children acute & self-limiting =

Prevalence in children

Results from an immune-mediated destruction of circulating platelets within the reticuloendothelial system, mainly the spleen compensatory in megakaryocytes within the bone marrow 2 types acute & chronic

Pathophysiology
Platelet sensitisation with autoantibodies (usually IgG) results in their premature removal from the circulation by macrophages of reticuloendothelial system, especially the spleen Immune mediated destruction of circulating platelet due to autoantibodies to platelet membranes antigen. The normal lifespan of a platelet is about 7 days but in ITP this is reduced to a few hours Reduce platelet count compensatory increase in megakaryocytes in bone marrow Total megakaryocytes mass and platelet turnover are increased in parallel to about 5 times normal

ITP Classification :
ACUTE CHRONIC

 1 2 wks  80% children  male:female = 1:1

>6m  20% children (> in adult)  Male:female = 1:3

 post - viral infection (1-2 wks) No history of viral (1infection rapid onset purpura Sudden in onset  wide spectrum of manifestation insidious

ACUTE

CHRONIC

Platelet count >20 x109/L x10  self limiting (95%)

(spontaneous remitting: 6 8 wks)

Variable  most: remitting within 3 yrs stabilise with moderate, asymptomatic thrombocytopenia.

 5% only chronic

Acute Spontaneous remissions are usual but in 5-10% of cases the disease becomes chronic (>6months) Low morbidity and mortality Chronic Most common cause of thrombocytopenia without anaemia/ neutropenia Usually associated with SLE, HIV, chronic lymphocytic leukemia (CLL), Hodgkin s disease or autoimmune haemolytic anaemia

ITP Clinical features :

Onset acute Cutaneous bleeding (esp over legs) : - purpura - petechei - bruising Mucosal bleeding : - palatal petechei - gum bleed - epistaxis - haematuria - maenorrhagia - GIT bleeding - Intracranial bleed (rare, but serious)  Profuse bleeding uncommon (plt < 10 x 109 /L)

 No hepatosplenomegaly No Lympadenopathy

FBC, FBP thrombocytopenia , larger plt. plt. Prolonged BT (N= 11min or less) Bone marrow aspiration - increase in megakaryocytes

ITP Diagnose :
By exclusion - other causes of purpura/easy bleed. History PE FBC and peripheral blood smear

Investigations
Full blood cell count
 Low platelet count (10-50x109/L)

Peripheral blood smear

 Morphology of red blood cells and leukocytes normal  Morphology of platelets typically normal, with varying numbers of large platelets

Bone marrow aspiration

 Normal or number of megakaryocytes  Do not show platelet budding (formation of platelets)

Antiplatelet antibody may or may not be detected

Acute ITP Treatment:

 self limiting (95%) not required any therapy or admission - spontaneous remitting: 6 8 wks  Treatment: - life-threatening bleeding (ICH), regardless plt. life- plt: < 20,000/mm3 + mucosal bleed - plt: < 10,000/mm3 + any bleed  Hospitalisation: - severe life-threatening (ICH), regardless plt. life- plt: < 20,000/mm3 + evidence of bleed - plt: < 20,000/mm3 (x bleed, but inaccessible to health care

 Treatment option:
- oral prednisolone - IV Methylprednisolone - IVIG - IV Anti-Rh(D) Anti Platelet transfusion reserve only for life-threatening haemorrhage life(rise plt only for a few hours)

Chronic ITP Management :

Majority remit spontaneously if given enough time. Revisit Dx to exclude other causes Asymptomatic child need no treatment, kept under observation. Symptomatic child : - intermittent pulses of IVIG - intermittent pulses of steroid - intermittent Anti-Rh(D) IG (Rh +ve patient) Anti Splenectomy indicated: - persist >12 m - bleeding sx - plt: - <10,000/mm3 (3 -12 y/o) - < 10,000/mm3 - 30,000/mm3 (8 -12 y/o) - no/transient response to intermittent IVIG, anti D, or steroid - no contra-indication to surgery contra-

ITP Complication :
   ICH Most fear cx, mortality: 50% Risk in newly Dx ITP child within 1st yr < 1% Highest risk : - < 20,000/mm3 - hx of head trauma - aspirin - cerebral AV malformation

 50% occur : after 1 m of presentation 30% occur : after 6 m  Tx : IVIG, anti-D, methylprednisolone, plt transfuse, or/and antineurosurgical.

Prognosis
More than 80% of children with untreated ITP have a spontaneous recovery with completely normal platelet counts in 2-8 weeks Fatal bleeding occurs in 0.9% upon initial presentation

Causes
Traumatic Injury - Abrasion - Excoriation - Hematoma - Laceration Intravascular changes; Medical Condition Extravascular changes - H.pylori infection - brain abscess - brain tumor

Acquired disorders Vitamin K deficiency Liver disease ITP DIC

Inherited disorders - Haemophilia - Von Willebrand s disease Intramural changes -aneurysms -dissections -Vasculitides -AVM

Coagulation Defect
Hereditary:
Haemophilia A Haemophilia B von Willebrands disease

Acquired:
ITP Deficiency of Vitamin K-dependant factors Liver disease DIVC

Haemophilia
Inherited bleeding disorders caused by defective production of coagulation factor VIII (haemophilia A) or IX (haemophilia B)

Dorland s Medical Dictionary

 Commonest severe inherited coagolation disorder X linked recessive disorder Prevalence :

1 in 5000 male birth (haemophilia A) 1 in 30 000 male birth (haemophilia B)

Significant rates of spontaneous mutation and acquired immunologic processes can result in this disorder as well.

Pathophysiology
Disruption of the normal intrinsic coagulation cascade, resulting in spontaneous haemorrhage or excessive haemorrhage in response to trauma

Clinical feature
 Depend on severity

Age of onset: Neonate: intracranial haemorrhage Toddler: Starting to crawl/walk

 Pattern of bledding: Bruises Hematuria, epistaxis, gum bleeding Into muscle/joints: haemarthrosis is characteristic of haemophilia

 Bleeding history: Dental extraction Post-circumscision Prolonged oozing in venepuncture sites Post-trauma / spontaneously

 Haemarthrosis
large joints (elbow, ankle and knee) Swollen, painful

bruises

Haemophilic arthropathy

Severity of haemophilia
Classification Mild Moderate Severe Factor level 5 30% 1 5% <1%
Spontaneous joint/muscle bleed, risk of ICH

Bleed after trauma/surgery

Bleed after minor trauma/surgery

Hallmark of severe hemophilia

-Recurrent spontaneous bleeding into joints and muscles -Can lead to crippling arthritis and progressive arthropathy with permanent damage

Investigation
Full blood count Coagulation screen: APTT Specific factor assay: FVIII level (low in H.A) Specific factor assay: FIX level (low in H.B)

Further investigation
Hepatitis B surface antigen HIV serology Diagnosis of carrier status for genetic counseling.
Mother of a newly diagnosed son with haemophilia Female siblings of boy with haemophilia Daughter of a man with haemophilia
Viral status at diagnosis and yearly, treatment carries risk of acquiring viruses. Immunized against Hepatitis B.

Treatment
Replace the missing factor FVIII/FIX concentrates
 Plasma derived  Recombinant

The type of treatment depends on:

 Type of bleeding  Severity

Treatment
Dose depends on type
Type of bleeding Haemarthrosis Soft tissue/muscle ICH/surgery Percentage of factor aimed 30 % 30 50% 100% Factor VIII dose 20 U/kg 30-40 U/kg 50 U/kg

Alternative formula:
Unit of F VIII = (% rise require) x (weight in kg) x 0.5 Unit of F IX = (% rise require) x (weight in kg) x 1.4

 FVIII-Given every 8 to 12 hourly FIX-Given every 12 to 24 hourly

Duration depend on type of bleeding -haemarthroses 2-3 days -soft tissue bleeds 4-5 days -intracranial/surgery 7-10 days

Treatment
 Severity Mild haemophilia.
Give desmopressin (DDAVP) to raise the body's levels of factor VIII. Since the effect wears off with chronic use, it is applied only in certain situations e.g. prior to dental work or participation in sports Desmopressin does not help in haemophilia B.

 Moderate haemophilia
treatment only when bleeding occurs Educate signs and symptoms of bleeding to get treatment as quickly as possible. may also have treatment to prevent bleeding that could occur when participating in some activity

 Severe haemophilia.
usually need long-term or shorter term preventive therapy to prevent bleeding that could cause permanent damage. Some people with severe haemophilia receive treatment only when bleeding occurs, however. It is important to get treatment as soon as possible. Delayed treatment can lead to complications.

Cont Treatment Prophylactic FVIII/IX to reduce risk of chronic joint damage Desmopressin (DDAVP) to stimulates endogenous release of FVIII Analgesia Dental Care Home treatment education Immunisation Activity at school Haemophilia Society

Complication
Deep internal bleeding e.g. deep-muscle bleeding, leading to swelling, numbness or pain of a limb. Joint destruction oeteoarthritis & derformity Transfusion
-Transmitted viral infection -Development of inhibitors body s immune system rejects factor concentrate -Central venous access infected/ thromboses

Intracranial haemorrhage is a serious medical emergency caused by the buildup of pressure inside the skull. It can cause disorientation, nausea, loss of consciousness, brain damage, and death

Life expectancy
varies with severity and adequate treatment. average life expectancy was only 11 years effective treatment became available at 1960 By the 1980s- 50 60 years with appropriate treatment Today- near normal quality of life with an average lifespan approximately 10 years shorter than an unaffected male

Thank You