Approach To Child with

Bleeding Tendency

Halima Al.Dhali98181

Case Scenario

Parents brought a 3 year old girl to emergency room with history of bruising on her lower limbs and chest for the last 4 days. The bruises were not painful but were increasing in number every day. No active bleeding seen. Child otherwise very active and playful.

What further history you would like to obtain from parents?What else you will be searching in your examination?What investigations you will order?

History•Patient ID ,•PRESENTING

COMPLAINT – NATURE OF BLEED Onset – Acute

bleeds >> Acquired disorder– Bleeding since birth >> Congenital disorder Site – Superficial: skin, mucosa (nasal,gingival) >> Platelet – Deep: joint, muscle >> Coagulopathy

Other types of bleed – Hematemesis,Hematuria,

Hematochezia, Melena, epistaxis

Preceding/Precipitating event - Trauma, surgery, dental procedure

Duration and frequency – bleeding that stops and recur quickly → Coagulopathy

• Associated symptoms• nausea, vomiting, headache,

weakness• postural giddiness, pallor,

fatigue, chest pain,, palpitations, joint swelling

• Review of systemsPAST MEDICAL HISTORY• Previously Diagnosed

Bleeding disorder (eg Hemophilia, ITP)

• Liver disease• Renal disease• Malignancies(leukemia,

neuroblastoma)• Infections• Connective tissue disorders

AutoimmunePrevious admissions, surgeries and blood

transfusion

HistoryPrenatal, perinatal and postnatal Hx Postcircumcision bleeding Birth cephalohematoma Umbilical stump bleeding or delayed stump separationNutritional Hx Protein malnutrition Vitamin C deficiency, or scurvy Vitamin K deficiency >>bacterial overgrowth, celiac disease,

chronic pancreatitis, inflammatory bowel disease.Developmental Hx gross motor (to corroborate the described mechanism of injury)

History FAMILY HISTORY

• consanguinity >> autosomal recessive inherited bleeding disorder 

• Known bleeding disorder or other heritable medical disorder that may predispose to bruising (eg, Ehlers-Danlos syndrome, osteogenesis imperfecta). 

• Male relatives affected (Hemophilia, X-linked Recessive trait)

• Female relatives - Menstrual and Obstetric Hx• males and females affected >>an autosomal disorder

such as von Willebrand factor deficiency• autosomal dominant traits such as hereditary

hemorrhagic telangiectasia Social Hx

• child’s behavior and the family’s methods of discipline

Examination General appearance: dysmorphisms , built. Vital signs Growth parameters: malnutrition General examination: hydration, pallor, jaundice,

lymphadenopathy  Skin :Telangiectasia, Petechiae, Purpurae, Ecchymoses,

Hematoma (size, location, pattern) Mucosae : Nasal (Epistaxis), Gingival bleeding The musculoskeletal system : Intramuscular Bleed,

HemearthrosisJoint contractures ( chronic hemearthroses) , joint hypermobility, skin laxity and bony deformity

GI system: Hepatosplenomegaly

petechiae Pinpoint flat round red spots under the

skin surface caused by intradermal hemorrhage (bleeding into the skin)

Purpura

Purpura is purple-colored spots and patches that occur on the skin, and in mucus me

Group of adjoining petechiae

Ecchymoses

Isolated lesion larger than petechiae ( larger than 1cm)

Bleeding Disorder

CoagulationFactor

•Deficiency in coagulation Factor

Blood VesselsIntegrity

•Weak vessele•Inflammation

Platelet

disorder

•Decrease number•Abnormal function

Platelet Its called thrombocyte from

megakaryocte Life span 8- 10 days One third of them sequestered in spleen production control by thromboprotein Normal Range150-400 X 109/L Less than 150X 109/L ,

thrombocytopenia.

Causes of Thrombocytopenia

Decrease platelet

production

Increase platelet consump

tion

Splenic Sequestrat

ion

Dilution

-Bone marrow failure-Aplastic syndrome-cyanotic congenital heart D _Infection(HIV,CMV, Episten Barr viruse-Drug(heparin-Anti-biotic –quinidine)

-Idiopathic thrombocytopenic purpura (ITP)

-Neonatal alloimmune thrombocytopenia (NAIT)-SLE-Disseminated intravascular coagulation (DIC)-Sepsis

-Increase pooling in

spleen

Hypersplenism

InfectionInflammationCongestionRed cell disordersStorage diseases

-Massive Blood transfusion

manifestations of thrombocytopenia Petechiae Bruises or purpura Bleeding of mucous membranes:

epistaxis, gingival bleeding Acute gastrointestinal bleeding Hematuria Acute CNS hemorrhage: the rarest but

MOST FEARED consequence of low platelets

Degree of trauma and bleeding

Platelet Count Risk of bleeding Example

Less than 80×10^9 Primary hemostasis impaired

After Major trauma, Surgery

Less than 50×10^9 Spontaneous bleeding mostly in

Skin

Petechiae ,purpura

Less than 20×10^9 Noticeable hemorrhage

Seen in skin and mucosa

EpistaxisGingival bleeding

Immune Thrombocytopenic (ITP)

isolated thrombocytopenia with normal bone marrow and in the absence of other causes of thrombocytopenia.

Usually follow an acute viral infection IgG,IgM that bind platelet membrane,

result in Fc receptor mediated splenic destruction

signs, symptoms, and precipitating factors

Abrupt onset, Purpura Epistaxis Bruising tendency Gingival bleeding Recent live virus immunization Recent viral illness mainly after1-4 week Normal RBC,WBC count Sever type of thrombocytopenia Adenopathy or hepatosplenomegaly is unusual

Diagnosis Usually based on clinical presentation Platelet Count low Atypical finding-Do Bone marrow

Examination( Increase megakaryocyte, Normal Erythroid,myloid element).

In ITP shows increased megakaryocytes and normal erythroid and amyloid elements.

(WBC,RBC,Hb) Normal Normal pt aptt time

Our patient:Hb: 11g/dLWBC 5x109/L with normal differentialPlatelets 4 x109/LPT and APTT = normal

Treatment Optimal treatment is Controversial 80% of children will have spontaneous

resolution(few weeks). child with mild

bleeding(bruising,petechia) no treatment required.

Severe and moderate bleeding with platelet count less 10,000mm

Treatment option

Prednisone for 2 weeks IVIG for(1-2 Days)expensive,

Platelet transfusion: Actively bleeding patients with thrombocytopenia \central nervous system bleeding

Splenectomy (life threating condition/not responding to other medication)

Decrease clearance of sensitized platelet

Coagulation Disorder Hemophilia

Von Willebrand Disease

Hemophilia an inherited (genetic) disorder

blood doesn't clot normally because it lacks sufficient blood-clotting proteins (clotting factors).

you may bleed for a longer time after an injury than you would if your blood clotted normally.

Types of hemophilia Types Hemophilia A Hemophilia

Binheritance X-linked X-linked

Factor Deficiency factor8 factor9

Bleeding site Muscle, joints ,surgical

Muscle, joints ,surgical

Prothrombin time Normal Normal

Activated partial thromboplastin time

prolong prolong

Hemophilia A

Hemophilia B

Clinical Manifestation Bleeding In soft Tissue ,GI ,Hip ,Joint,

elbow , and ankle Joint. Spontaneous joint bleeding occur when

child begin to walk Intracranial hemorrhage

uncommon(important cause of death) Petechiae usually do not occur in

patients with hemophilia

Musculoskeletal (joints) - Tingling, cracking, warmth, pain, stiffness, refusal to use the joint (young children)

Central nervous system (CNS) - Headache, stiff neck, vomiting, lethargy, irritability, spinal cord syndromes

Gastrointestinal (GI) - Hematemesis, melena, frank red blood per rectum, abdominal pain

Genitourinary - Hematuria, renal colic, post-circumcision bleeding

Other - Epistaxis, oral mucosal hemorrhage, hemoptysis, dyspnea (hematoma leading to airway obstruction)

contusions, excessive bleeding with routine dental procedures

Severity, Factor Activity, and Hemorrhage Type

Classification Factor Activity% Cause of hemorrhage

Mild 5-40% Major trauma, surgery

Moderate 1-5% Mild to moderate trauma

Severe Spontaneous

Diagnosis

CBC(normal platelet count)

Coagulation Study:(aPTT) is prolonged,the bleeding time and prothrombin time are normal Factor 8,9 assay:levels are compared

with a normal pooled-plasma standard

Mixing study

Mixing Study

Management

Prevention of trauma is important Should avoid Aspirin and other NSAID Iv infusion whenever there is any bleeding

recombinant FVIII concentrate for haemophilia A recombinant FIX concentrate for haemophilia B

Highly purified, virally inactivated plasma-derived products should be used if recombinant products are unavailable

Von Willebrand Disease VWF found in( Endothelia cell,

Megakaryocyte). Two Rules :Carrier Molecule for factor 8 Promote platelet Adhesion An Inherited bleeding disorder Women are worse affected than men 3types ( 1,2,3) Either Qualitative or Quantitative

Disorder

Type1 Type2 Type3Quantitative partial Deficiency

70-80%-Mild clinical

symptoms-Autosomal

Dominant

Functional abnormality

15-20% -Mild-Moderate

-Autosomal Recessive/Dominan

t -4 subtypes

(2A ,2B,2M,2N)

Complete Deficiency

Most Sever Type -Autosomal

Recessive

-Similar to hemophilia A

Manifestations Wide variation in Clinical Manifestation (even in the

same family).

Many children are asymptomatic .

Increased or easy bruising

Recurrent Epistaxis.

Post-operative bleeding( Tonsillectomy –dental Extraction )

Laboratory finding Factor 8 levels are low. The APTT may be prolonged . VWF levels are low . Platelet count is normal (except in type

2B)

Management DDAVP (Desmopressin Acetate) -synthetic form of vasopressinStimulate release of VWF from cells after 30min (for type1) . Purified plasma-derived

concentrates of vWF/FVIII are used for treatment of bleeds and for surgical prophylaxis when DDAVP is ineffective or contraindicated

Prophylactic (antifibronolytics ), before dental Extraction .

A five-year-old child who is not clinically ill but presents with moderate mucocutaneous purpura in the wake of a viral infection

A male infant who is starting to walk and presents with a painful swollen joint after a fall

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