Anaemia & Bleeding

8/3/2019 Anaemia & Bleeding

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ANAEMIA ANDBLEEDING

Raja Noor Azimah

Mohd Norhalimi

Siti Nazihah

Yim Wei Sen

Poo Suk Ting


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CONTENTS

Anaemia

- classification ofanaemia

- causes of anaemia

- History & Examination

- Iron deficiency

anaemia (IDA)- Thalassemia

Bleeding

- classification

- causes of bleedingdisorder

- History & examination

- Haemophilia

- ImmuneThrombocytopenicPurpura (ITP)


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ANAEMIA


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DEFINITION

WH

Odefinition:

condition in which the Hb level below a defined

range, their O2carrying capacity is insufficient to

meet physiological needs, which vary by age, sex,

altitude, smoking, and pregnancy status.

Age Hb (g/dl)

2 week 13.7-20.1


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STRUCTURE


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Hemoglobin (Hb) - The amount of hemoglobin in the blood,expressed in grams per decilitre (g/dL)

Mean corpuscular volume (MCV) - the average volume of thered cells, measured in femtolitres (fL).

Mean corpuscular hemoglobin (MCH) - the average amount ofhemoglobin per red blood cell, in picograms (pg).

Mean corpuscular hemoglobin concentration (MCHC) - theaverage concentration of hemoglobin in the cells

Hematocrit or packed cell volume (P

CV

) - This is the fractionof whole blood volume that consists of red blood cells.

Red blood cell distribution width (RDW) - a measure of thevariation of the RBC population


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Classification of anemia based on MCV

anisocytosis, microcytesand marked hypochromia

large, dense, oversizedof red blood cells

Causes:

IDA

Thalassemia

Causes:

Vit B12 deficiency

Folate deficiency

Causes:

Blood loss

Hemolytic anaemia

Chronic illness

CLASSIFICATION

(MCV)

Microcytic(abnormal small red

blood cell MCV 95)

Normocytic(normal red blood cell)


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Microcytic Normocytic Macrocytic

-Iron deficiency*

-Thalassemias*

-Chronic

inflammatory

disease

-Sideroblastic

anemia

-Copper

deficiency

-Chronic inflammatory

disease

-Recent blood loss

-Malignancy/marrow

infiltration

-Chronic renal failure

-Transient

erythroblastopenia

-G6PD deficiency

-Marrow aplasia/hypoplasia

With megaloblastic bone

marrow

-Vitamin B12 deficiency*

-Folic acid deficiency*

Without megaloblastic

bone marrow

-Liver disease-Hypothyroidism

-Down Syndrome

Bone marrow failure

states-Myelodysplasia

-Acquired aplastic anemia

-Fanconi anemia


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CLINICAL FEATURES

Lethargy

SOB

Poor effort tolerance

Headache

Excessive sleeping

(especially in infants)

Poor feeding

Syncope

Pallor, jaundice

Tachycardia, tachypnoea

Collapsing pulse, boundingpulse

Flow murmur, cardiomegaly

Brittle spoon nail (koilonychia),angular stomatitis, glossitis

Bleeding site, ecchymosis Hepatosplenomegaly

Lymphadenophaty

Sn ofcardiac failure

Symptoms Signs


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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


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History of trauma

Growth parameters acute/chronic

Any worms in faeces Neonatal jaundice or episodes of jaundice in

the past

Other signs of hypothyroidism?


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Systemic enquiry

- infection

- liver disease

- renal disease

Past Medical History

- any iron supplement before?

- previous blood transfusion?

Birth History


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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) orchew on ice?

- Picky eater? lead poisoning

- Vegetarian?- Mothers diet if breast feeding.

- Detail of all the meals daily


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Development History

- mental retardation?

- developmental delay

Family History

- anemia

- iron supplements

- regular transfusion, gallstone early in life


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PHYSICAL EXAMINATION

General

Alertness

consciousness

activity pallor

jaundice

growth

characteristic facies: tower skull, frontal bossing,maxillary hypertrophy with prominent cheek


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Peripheral Examination

koilonychia increase pulse rate (tachycardia)

Interdigitation of Gum (gum hypertrophy)

Evidence ofCHF cardiomegaly

ejection systolic murmur

hepatomegaly

Hepatosplenomegaly


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COMPLICATIONS

Heart failure

Pulmonary oedema Cardiovascular collapse

Sudden cardiac death


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PHYSIOLOGICAL ANAEMIA IN EARLY

INFANCY


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Hb concentration in

newborn infants is 15-

23g/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)


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CONTENTS

Anaemia

- classification ofanaemia

- causes of anaemia

- History & Examination

- Iron deficiency

anaemia (IDA)- Thalassemia

Bleeding

- classification

- causes of bleeding

disorder

- History & examination

- Haemophilia

- ImmuneThrombocytopenicPurpura (ITP)


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IRO

N DEFIC

IENC

Y ANEMIA


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The most common cause of anemia in

childhood

Prevalence higher in poorer socioeconomicgroups

Incidence of iron deficiency in most reported

series appears to be related to socioeconomic

factors but not to age, sex or race.


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Pathophysiology

Occur in the duodenum and upperjejunum aided by ascorbic acid, fructoseand amino acid

Iron absorption

Iron transport and

usage

Iron from intestinal mucosal cells is transferredto transferrin transport to the bone marrowfor haemoglobin synthesis ( 70%)

Small amount( 4%) are used for synthesismyoglobin, and haem enzymes(cytochromes)

Iron storage Additional iron (25%) is stores in the liver,

spleen and bone marrow as ferritin andhaemosiderin

Iron deficiency iron requirement exceeds intake, iron stores are

used up, and the patient becomes irondeficient. Poor iron stores results in impairedhaemoglobin synthesis and a hypochromic,microcytic anaemia.


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Classification of iron deficiency

Stages Iron stores Serum

iron

ug/dl

Transferrin

saturation%

Serum

ferritin

ug/dl

anaemia

Normal present 90-150 30 35 None

Iron

depletion

low < 60 20 10-30 None

Iron

deficiency

absent < 60 < 15 < 10 None

Iron

deficiency

anemia

absent < 30 < 10 < 10 Microcytic

hypochromic


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AETIOLOGY Chronic blood loss

- Meckels 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 cows milk diet

- delayed weaning

Impaired absorption

- Chronic diarrhea, GI abnormalities, hookworm

infection, malabsorption


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History and Physical Examination

History Physical examination

Irritability, prolongedfatigue

History of bleedingepisode( from intestinaltract, menstruation)

Pallor

Pica : persistent craving

and inappropriate eatingof non-food substances( soil, chalk)

Diet (age of weaning)

Pallor( conjunctiva,palmar)

Atrophic glossitis,angular stomatitis

Koilonychias( spoonshaped nail)

Tachycardia, cardiac

enlargement and cardiacfailure


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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 parasititcinfection


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FULL BLOOD PICTURE

Anisocytosis

Poikilocytosis

Hypochromia

microcytosis


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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 wholegrains, legumes, seeds, and nuts

Iron supplementation

Maintain breastfeeding


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2) Oral iron

y A daily total dose of 6 mg/kg/ day ofelemental 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 normaly Side effects- Nausea, vomiting, epigastric

pain, constipation


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Failure of response to oral iron caused by:

- Non compliance

- Inadequate iron dosage- Unrecognized blood loss

- Incorrect diagnosis

- Impaired GI absorption


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3) Parenteral iron

Eg: Oral dextran, Sodium ferric gluconate and ironsucrose

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


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4) Blood transfusion

indicated only in severe anemia and cardiacfailure

slow administration of 5 10 ml/kg body weightof packed red cells over 4-6 hours is adequate toraise the hemoglobin to a safe level ( rapid bloodtransfusion may cause hyperkalemia and cardiacdecompensation).

If severe anemia( hb< 4g/dl), add IV frusemide1mg/kg ( to prevent fluid overload Pulmonaryedema)


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Prognosis

Good.

In most cases, the blood counts will return to

normal in2

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.


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THALASSEMIA


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Definition

Is a blood disorder passed down through families (inherited)

in which the body makes an abnormal form of haemoglobin

Resulting in1)Excessive destruction of red blood cells

2)Ineffective erythropoisis

3)Premature removal of red blood cells by spleen

which leads to hypochromic microcytic anemia


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Syndromes

Thalassemia Minor(trait or carrier)

ThalassemiaIntermedia

ThalassemiaMajor

Thalassemia Syndromes


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Syndrome Clinical Age of

Presentation

Need for

Blood

Transfusion

Trait Asymptomatic Any age None

Thal-

intermedia

Moderately

Severe

After age 2 or

later

None ; occ

some

Thal-major Beta: Severe

Alpha: Death

1-2 Regular

Clinical Features


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Alpha Beta

(1) Trait Trait

(2) Hb H disease Beta-thalassemia intermedia

(3) Hb Barts hydrops fetalis Homozygous beta ( 0)-

thalassemia

Clinical Forms of Alpha & Beta

thalassemia


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Syndrome Degree of Globin

Chain Imbalance

Hb Level

Trait + >10gm/dL

Thal-Intermedia ++ 7-10gm/dL

Thal-Major +++


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Thalassemia trait Normal

Thalassemia intermedia Normal or iron overload

Thalassemia major Iron overload in the absence

of iron chelation therapy

Iron Status In Thalassemia Syndrome


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Thalassemia Diagnosis

Laboratory Tests

Screening

Confirmatory or definitive


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Basic Screen Tests

RBC indices

Reticulocyte count

Blood smear

Full BloodPicture

Screening for abnormal haemoglobin

Quantification ofHb A, A2, Hb FHb analysis

Iron Status


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Confirmatory Tests

DNA studies

Globin chain synthesis

Structural analysis


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Beta-thalassemia


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Decrease or absence in the synthesis of beta

globin chains

B+ : some globin chain synthesis

B

0

: no B globin chain synthesis


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Clinical Features of Beta-Thalassemia

Major

Globin chain imbalance (excess of alpha globin chain)

RBC damage

Ineffective erythropoises

Peripheral hemolysis

Anaemia


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Anaemia

Hypoxia

Erythropoieten increase


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General Effects of Anaemia

Pallor

Generalised malaise, reduce activity

Retardation of growth

Cardiac failure


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Thalassemia Facies


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Laboratory Diagnosis

Beta-thalassemia Trait

Hb > 10gm/dL ~ N

MCV, MCH low

MCHC N

RDW N

Serum ferritin N

Hb A2 raised


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Beta-thalassemia Carrier Identification

Hallmark ofclassical beta-thalassemia carrier

Presence of an elevated Hb A2 level > 4%


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Beta-thalassemia Intermedia

Hb 7-10 gm/ dL

MCV, MCH, MCHC Low

RDW Increase

Serum ferritin Increase

Hb F raised


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Beta-thalassemia Major

Hb < 7gm/dL

MCV, MCH, MCHC Low

RDW Increase

Serum ferritin Increase

Hb F markedly raised


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Thalassemia picture :Intermedia & thal major

Anisopoikilocytosis

Hypochromasia Target cells

Basophilic stippling

Nucleated RBCs


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ALPHA THALASSEMIA


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Reduce synthesis of the alpha globin chains

+

: 1 globin deleted

0 : 2 globins deleted


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Hb Barts Hydrops Fetalis

Hallmark of the condition

-Presence of useless Hb : Hb Barts

Incompatible with life

Death in utero (23-38weeks) or soon after birth (within6 hours)

High oxygen affinity

Functionally useless Hb for oxygen transfer


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Functionally useless Hb for oxygen transfer

Severe hypoxia

Hydropic fetus


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Laboratory Diagnosis

Hb Barts Hydrops Fetalis

Hb low

RBC low

HCT low

MCV Increase

MCH, MCHC low

RDW increase


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Full Blood Picture

Anisopoikilocytosis

Macrocytosis

Hypochromia

Numerous nucleated RBCs


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Management

Thalassemia trait/carrier

- no need treatment

Thalassemia intermedia

-may require blood transfusion duringfulminant infection

Thalassemia Major

-Transfusion dependent for life-Curative with stem cell transplantation

(bone marrow, peripheral blood)


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Initiation ofBT

- 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


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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


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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


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Alternative options

Oral iron chelator

- Deferasirox.- > 2 y/o

- 20-30 mg/kg/day, O.D.

Bone marrow transplantation


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Supplementation

Vitamin C

Folate

Zinc


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Bleeding Disorder


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is the loss of blood or blood escape from the

circulatory systemBleeding

Internal External

-blood leaks from blood

vessels inside the body

-through a naturalopening

-eg: vagina,

mouth, nose, ear

or anus

through a breakin the skin


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Severity of bleeding (WHO)

Grade 0 No bleeding

Grade 1 Petechial bleeding

Grade 2 Mild blood loss (clinical significant)

Grade 3 Gross blood loss, requires transfusion (severe)

Grade 4 debilitating blood loss, retinal or cerebral associated

with fatality


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Causes

Traumatic Injury Medical Condition

- Abrasion

- Excoriation

- Hematoma

- Laceration

Intravascular changes;

Intramural changes

-aneurysms

-dissections-Vasculitides

-AVM

Extravascular changes

- H.pylori infection

- brain abscess- brain tumor

Acquired disorders Inherited disorders

- Vitamin K deficiency

- Liver disease

- ITP- DIC

- Haemophilia

- Von Willebrands disease


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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

bd l


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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


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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


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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


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Examination;

Petechiae, bruising, mucosal bleeding, and oozing

from venipuncture sites

Joint swelling

Anaemia, lymphadenopathy, hepatosplenomegaly


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Investigation

FBC

BUSE

LFT

ESR

Coagulation Tests

Special test; measurement of vWF


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ITPITP


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Immunomediated Thrombocytopenia

Purpura (ITP) Idiopathic Thrombocytopenic Purpura /Idiopathic Thrombocytopenic Purpura /

Immune Thrombocytopenia.Immune Thrombocytopenia.

Clinical syndrome in which there is isolated thrombocytopeniaClinical syndrome in which there is isolated thrombocytopeniawith otherwise normal blood count with no clinically apparentwith otherwise normal blood count with no clinically apparentassociated condition that can cause thrombocytopenia.associated condition that can cause thrombocytopenia.

Commonest cause of thrombocytopenia in childhood


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Affects children between 2 and 10 years old, with onset often1-2 weeks after a viral infection

In 90% ofchildren acute & self-limiting

Prevalence in children =

Results from an immune-mediated destruction ofcirculatingplatelets within the reticuloendothelial system, mainly thespleen compensatory in megakaryocytes within the bonemarrow

2 types acute & chronic


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Pathophysiology

Platelet sensitisation with autoantibodies (usually IgG) results in theirpremature removal from the circulation by macrophages ofreticuloendothelial system, especially the spleen

Immune mediated destruction ofcirculating platelet due toImmune mediated destruction ofcirculating platelet due to

autoantibodies to platelet membranes antigen.autoantibodies to platelet membranes antigen.

The normal lifespan of a platelet is about 7 days but in ITP this isreduced to a few hours

Reduce platelet countReduce platelet count compensatory increase in megakaryocytes in bone marrowcompensatory increase in megakaryocytes in bone marrow

Total megakaryocytes mass and platelet turnover are increased inparallel to about 5 times normal

ITPITP Classification :Classification :


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ACUTEACUTE CHRONICCHRONIC

11 2 wks2 wks

80% children80% children

male:female = 1:1male:female = 1:1

postpost -- viral infection (1viral infection (1--2 wks)2 wks)

rapid onset purpurarapid onset purpura

Sudden in onsetSudden in onset

> 6 m> 6 m

20% children (> in adult)20% children (> in adult)

Male:female = 1:3Male:female = 1:3

No history of viralNo history of viral

infectioninfection

wide spectrum ofwide spectrum of

manifestationmanifestation

insidiousinsidious


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ACUTEACUTE CHRONICCHRONIC

Platelet count >20 xPlatelet count >20 x101099/L/L

self limiting (95%)self limiting (95%)

(spontaneous remitting: 6(spontaneous remitting: 6

8 wks)8 wks)

5% only5% only chronicchronic

VariableVariable

most:most:

remittingremitting within 3 yrswithin 3 yrs stabilise with moderate,stabilise with moderate,

asymptomaticasymptomatic

thrombocytopenia.thrombocytopenia.


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Acute

Spontaneous remissions are usual but in 5-10% ofcases thedisease 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), Hodgkins disease or autoimmune haemolyticanaemia

ITPITP Clinical features :Clinical features :


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OnsetOnset acuteacute

Cutaneous bleedingCutaneous bleeding (esp over legs) :(esp over legs) :

-- purpurapurpura

-- petecheipetechei

-- bruisingbruising

Mucosal bleeding :Mucosal bleeding :

-- palatal petecheipalatal petechei

-- gum bleedgum bleed

-- epistaxisepistaxis

-- haematuriahaematuria

-- maenorrhagiamaenorrhagia-- GIT bleedingGIT bleeding

-- Intracranial bleed (rare, but serious)Intracranial bleed (rare, but serious)

Profuse bleedingProfuse bleeding uncommon (plt < 10 x 10uncommon (plt < 10 x 1099 /L)/L)


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No hepatosplenomegalyNo hepatosplenomegaly

No LympadenopathyNo Lympadenopathy

FBC, FBPFBC, FBP thrombocytopenia , larger pltthrombocytopenia , larger plt..

Prolonged BTProlonged BT (N= 11min or less)(N= 11min or less)

Bone marrow aspirationBone marrow aspiration

-- increase in megakaryocytesincrease in megakaryocytes

ITPITP Diagnose :Diagnose :


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By exclusionBy exclusion

-- other causes of purpura/easy bleed.other causes of purpura/easy bleed.

HistoryHistory

PEPE

FBC and peripheral blood smearFBC and peripheral blood smear


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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 oflarge 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

Investigations

Acute ITPAcute ITP Treatment:Treatment:


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self limiting (95%)self limiting (95%)

not required any therapy or admissionnot required any therapy or admission

-- spontaneous remitting: 6spontaneous remitting: 6 8 wks8 wks

Treatment:Treatment:

-- lifelife--threatening bleeding (ICH), regardless plt.threatening bleeding (ICH), regardless plt.

-- plt: < 20,000/mmplt: < 20,000/mm33 + mucosal bleed+ mucosal bleed

-- plt: < 10,000/mmplt: < 10,000/mm33 + any bleed+ any bleed

Hospitalisation:Hospitalisation:-- severe lifesevere life--threatening (ICH), regardless plt.threatening (ICH), regardless plt.

-- plt: < 20,000/mmplt: < 20,000/mm33 + evidence of bleed+ evidence of bleed

-- plt: < 20,000/mmplt: < 20,000/mm33 (x bleed, but inaccessible to health care(x bleed, but inaccessible to health care

Treatment option:Treatment option:


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Treatment option:Treatment option:

-- oral prednisoloneoral prednisolone

-- IV

MethylprednisoloneIV

Methylprednisolone-- IVIGIVIG

-- IV AntiIV Anti--Rh(D)Rh(D)

Platelet transfusionPlatelet transfusion reserve only for lifereserve only for life--threatening haemorrhagethreatening haemorrhage(rise plt only for a few hours)(rise plt only for a few hours)

Chronic ITPChronic ITP Management :Management :


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MajorityMajority remit spontaneously if given enough time.remit spontaneously if given enough time.

Revisit Dx to exclude other causesRevisit Dx to exclude other causes

AsymptomaticchildAsymptomaticchild need no treatment, kept under observation.need no treatment, kept under observation.

Symptomaticchild :Symptomaticchild : -- intermittent pulses of IVIGintermittent pulses of IVIG

-- intermittent pulses of steroidintermittent pulses of steroid

-- intermittentintermittent AntiAnti--Rh(D) IG (Rh +ve patient)Rh(D) IG (Rh +ve patient)

SplenectomySplenectomy indicated:indicated:

-- persist >12 mpersist >12 m

-- bleeding sxbleeding sx

-- plt:plt: --


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ICHICH

Most fear cx, mortality: 50%Most fear cx, mortality: 50%

Risk in newly Dx ITP child within 1Risk in newly Dx ITP child within 1stst yr < 1%yr < 1%

Highest risk :Highest risk :

--


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Prognosis

More than 80% ofchildren 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


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Causes

Traumatic Injury Medical Condition

- Abrasion

- Excoriation

- Hematoma

- Laceration

Intravascular changes;

Intramural changes

-aneurysms

-dissections-Vasculitides

-AVM

Extravascular changes

- H.pylori infection

- brain abscess- brain tumor

Acquired disorders Inherited disorders

- Vitamin K deficiency

- Liver disease

- ITP- DIC

- Haemophilia

- Von Willebrands disease


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Coagulation Defect

Hereditary:

Haemophilia A

Haemophilia B

von Willebrands disease

Acquired:

ITP

Deficiency of Vitamin K-dependant factors

Liver disease

DIVC


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Haemophilia

Inherited bleeding disorders caused by

defective production ofcoagulation factor VIII

(haemophilia A) or IX (haemophilia B)

Dorlands Medical Dictionary

C t i h it d l ti


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Commonest severe inherited coagolationdisorder

X linked recessive disorder

Prevalence :

1 in 5000 male birth (haemophilia A)

1 in 30000 male birth (haemophilia B)

Significant rates of spontaneous mutation andacquired immunologic processes can result in thisdisorder as well.


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h h i l


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Pathophysiology

Disruption of the normal intrinsiccoagulation

cascade, resulting in spontaneous

haemorrhage or excessive haemorrhage in

response to trauma

Cli i l f t


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Clinical featureDepend on severity

Age of onset:

Neonate: intracranial haemorrhage

Toddler: Starting to crawl/walk


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Pattern of bledding:

Bruises

Hematuria, epistaxis, gum bleeding

Into muscle/joints: haemarthrosis ischaracteristic of haemophilia


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Bleeding history:

Dental extraction

Post-circumscision

Prolonged oozing in venepuncture sitesPost-trauma / spontaneously

Haemarthrosis


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large joints (elbow, ankle

and knee)

Swollen, painful

bruises

Haemophilic arthropathy

S i f h hili


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Severity of haemophilia

Classification Factor level

Mild 5 30%

Moderate 1 5%

Severe


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Hallmark of severe hemophilia

-Recurrent spontaneous bleeding into joints and muscles

-Can lead to crippling arthritis and progressive arthropathy

with permanent damage

I ti ti


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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


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Further investigation

Hepatitis B surface antigen HIV serology

Diagnosis ofcarrier status for geneticcounseling.

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.

T t t


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Treatment

Replace the missing factor

FVIII/FIX concentrates

Plasma derived

Recombinant The type of treatment depends on:

Type of bleeding

Severity

T t t


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Treatment

Dose depends on type

Type of bleeding Percentage of factor

aimed

Factor VIII dose

Haemarthrosis 30 % 20 U/kg

Soft tissue/muscle 30 50% 30-40 U/kg

ICH/surgery 100% 50 U/kg


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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


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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


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Treatment

Severity

Mild haemophilia.

Give desmopressin (DDAVP) to raise the body's levels of

factorV

III. 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.


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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


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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.


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Cont TreatmentCont Treatment

Prophylactic F

V

III/IX to reduce risk ofchronic joint damage Desmopressin (DDAVP) to stimulates endogenous release of

FVIII

Analgesia

Dental

Care

Home treatment education

Immunisation

Activity at school

Haemophilia Society

Complication


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Complication Deep internal bleedingDeep internal bleeding e.g. deep-muscle bleeding, leading to

swelling, numbness or pain of a limb.

Joint destructionJoint destruction oeteoarthritis & derformity

TransfusionTransfusion

--Transmitted viral infectionTransmitted viral infection--Development of inhibitorsDevelopment of inhibitors bodys immune system rejects factorbodys immune system rejects factor

concentrateconcentrate

--Central venous accessCentral venous access infected/ thrombosesinfected/ thromboses

IntracranialIntracranial haemorrhagehaemorrhage is a serious medical emergency caused

by the buildup of pressure inside the skull. It can causedisorientation, nausea, loss ofconsciousness, brain damage,and death

Life expectancy


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Life expectancy

varies with severity and adequate treatment.

average life expectancy was only 11 years

effective treatment became available at 1960

By the 1980s- 5060 years with appropriate

treatment

Today- near normal quality of life with an

average lifespan approximately 10 years

shorter than an unaffected male


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Thank You

Anaemia & Bleeding

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