ABNORMAL

BLEEDING
IN
CHILDREN
Dr Barnabas Atwiine
Paediatric Haematologist and
Oncologist
MUST
Hemostasis - Balance
Hemostasis - Imbalance
 Hemostasis:
4 major events
1. Vascular constriction: to limit the flow of
blood to the area of injury.
2. Platelet aggregation: secretion, clumping
and activation
3. Clot formation: to stabilize the platelet plug.
A fibrin mesh (the clot) forms and entraps
the plug.
4. Fibrinolysis: the clot is dissolved to resume
normal blood flow following tissue repair
through plasmin.
 Abnormal bleeding occurs when
there’s a defect in any of the above
processes
Bleeding is abnormal when it is:
• Spontaneous
Or/And
• Excessive
 Causes of abnormal bleeding
• Platelet disorders (primary or secondary,
quantitative or qualitative)
• Clotting factor disorders (congenital and
acquired)
• Vascular disorders
• DIC
 Quantitative Platelet disorders

Primary Secondary
• ITP • Malignancy
• Neonatal immune • Aplastic anemia
• TAR syndrome • DIC
• Wiscott-Aldrich syndrome • Sepsis
• Drug-induced
• HUS
• Hypersplenism
• Autoimmune (SLE)
• Viruses
Qualitative platelet d/os
 Clotting factor disorders

Primary Secondary
• Von Willebrand disease • DIC
• Hemophilia (A and B) • Anticoagulants eg
warfarin
• Vit K deficiency (incl HDN)
• Hepatic failure
 Vascular disorders
• Vasculitis e.g HSP
• Telangiectasia
• Angiodysplasia
 Key to diagnosis
• History
• History
• History
 History
– age at onset and Sex
– soft tissues/joints (think clotting factor deficiency,
coagulation disorder)
– epistaxis, menorrhagia, gum bleeding, mucous
membranes (suggestive of platelet disorder)
– GI bleeding, hematuria
– previous surgeries, immunizations, tooth extractions,
circumcision, etc
– excessive bleeding with fractures, minor cuts
– Significant or not? Generalized or localized?.
 History

Detailed family history of bleeding disorders?

• sex linked recessive (hemophilia A,B)
• autosomal recessives disease : clotting factors
deficiency 2,5,7,10,11,13.
• autosomal dominant : (VWD, qualitative platelet
disorders)
• autoimmune disorders
 More history..
-Recent illness, medications?
-Presence of chronic disease e.g. liver disease?
• Screen for other cytopenias
– systemic symptoms (wt loss, night sweats)
– recurrent infections
– anemia (fatigue, pallor)

• Remember non-accidental injury! Consider child abuse

with unusual bruising
 Nature of bleeding

Vascular and platelet dysfunction:

• Usually spontaneous subcutaneous or mucus
membrane bleeding eg; purpura, petechiae, epistaxis
Coagulation factor deficiency:
• Bleeding usually occurs deep into joints, muscles,
retroperitoneal space; post traumatic bleeds are often
prolonged (sometimes hours).
• May recur & bleeding may not get controlled by direct
pressure.
 Physical Examination

• Vital signs: Pulse, BP, RR, Temp

• General appearance, pallor
• Mucosal bleeding, joint/muscle hematomas
• Petechiae/purpura/ecchymosis,
• Splenomegaly +/- hepatomegaly
• Lymphadenopathy
• Signs of sepsis
Ecchymoses, Purpura, Petechiae
 Physical examination:
• Sites of hemorrhage – abdominal, pelvis, long
bones, etc
• Evidence of chronic disease, malignancy.
• Musculoskeletal examination for bleeding and
dysmorphic features
Feature Thrombocytopen Vascular Coagulation
ia or Disorders Disorders
Qualitative (HSP,
Platelet meningococca,
Disorder vasculitides)
Typical Superficial Palpable Ecchymoses:
appearanc ecchymoses petechiae or large and
e (small and purpura solitary
multiple), Petechiae
PETECHIAE RARE

Sites of Mucous Extremities, Subcutaneous

bleeding membranes buttocks in HSP hematoma,
Occassionally Intramuscular
hematuria Intra-articular
Rarely
hematomas
 Laboratory tests
• Initial laboratory screening .
-Bleeding time
-Clotting time
- Complete blood count including Platelet count
- Blood film
- Prothrombin Time (PT)
- activated Partial Thromboplastin Time (aPTT)
Test Mechanism Reference value Disorder
tested

PT Extrinsic & <12s beyond Vit K deficiency, HDN,

common neonate, 12-18s in malabsorption, Liver disease, oral
pathways neonate anticoagulants

aPTT Intrinsic & 20-40s beyond Hemophilia, vWD, DIC, lupus

common neonate, 70s in anticoagulant, Factor XI & XII
pathways neonate deficiency

Thrombin Fibrinogen to 10-15s beyond Fibrin split products, DIC, heparin,

Time fibrin neonate, 12-17s in uremia, hypofibrinogenemia
neonate

Bleeding Hemostasis, 3-7min Platelet dysfunction,

Time capillary & thrombocytopenia, aspirin, von
platelet Willebrand disease
function

Platelet Platelet 150,000-450,000/ Causes of Thrombocytopenia

count number mm3
 Diagnosis of coagulation defects
• Prolonged APTT Defect in Intrinsic
No change in PT

• Prolonged PT Defect in Extrinsic

• No change in APTT

• Prolonged APTT Defect in common

Prolonged PT
 PT PTT What does it mean?
Elevated Elevated •DIC, Vitamin K deficiency, Liver disease
•Heparin, anticoagulant therapy
• Deficiency of Factor X, Prothrombin (F II) or
Fibrinogen (FI)
Normal Elevated • Deficiency of F VIII, IX, XI, or XII, HMW
kininogens or prekallikrien

Elevated Normal • Deficiency of Factor VII

• Warfarin therapy
•Vitamin K deficiency
Normal Normal •No Bleeding: Normal!
• Bleeding: von Willebrand disease, F XIII def
 Immune Thrombocytopenic Purpura
(ITP)
• One of the most common acquired bleeding
disorders in children
• Incidence of symptomatic children:
– 3-8 in 100,000 per year
Acute ITP Persistent ITP Chronic ITP
(10%)
< 3months 3-12 months > 12 months
 Pathophysiology

• Platelet destruction from auto-antibodies

– Auto-antibodies generated during immune response
to viral or bacterial infection
• Auto-antibodies cross-react with platelet antigens
• Spleen and other organs of RES then destroy antibody-
coated platelets
– Similar mechanism: autoimmune hemolytic anemia
 Clinical Features - ITP
• Acute onset of petechiae and ecchymoses in otherwise
healthy child, usually after viral infection
• Mucosal bleeding (epistaxis, gingival)
• Other bleeding sites rarely affected
– GIT, vaginal mucosa, urinary tract, retina, conjunctiva
• Absence of other pathological findings:
– Hepatomegaly
– Splenomegaly
– Lymphadenopathy
– Fever, night sweats, weight loss
– Jaundice
– Pallor (depends on degree of hemorrhage)
 Investigations - ITP
• Platelets are the ONLY cell line affected
– Typically platelets <30,000/mcL
– Platelets appear large on peripheral smear
• RBCs and WBCs (including differential) are NORMAL
– No blasts, schistocytes, etc
– Mild normocytic anemia in <15% as a result of
hemorrhage
• Coagulation studies NOT required if FBC consistent with
ITP
• Bone marrow aspirate/biopsy NOT indicated if classic
FBC and clinical features
 Investigations - ITP
• Peripheral blood smear is necessary
– Rule out ‘pseudothrombocytopenia’
(clumping of platelets on slide or in EDTA tube)
– RBC morphology: rule out concomitant hemolytic
process (HUS, DIC, Evans syndrome)
– WBC morphology: rule out primary or secondary bone
marrow malignancy
– Platelet morphology: increased size in ITP, decreased size
suggests other pathology
Giant platelets of ITP
 Management – Acute ITP
• 80-90% of cases will self-resolve within 6 months

• Goal: To prevent serious bleeding complications from

thrombocytopenia

• Limit activity and potential trauma in children (when

possible)

• Restrict medications that alter platelet quantity or function

 Management – Acute ITP
• Watch & wait

• Steroids

• IVIG

• Splenectomy
 Hemophilia
• Hemophilia A and B: Factor 8 and 9 deficiency
respectively
• X-linked recessive disease
• Disease Classification (Factor Levels)
– Mild disease 6-40%
– Moderate disease 1-5%
– Severe disease <1%
 Hemophilia A
• Clinically
– Mild Disease
• Bleed with trauma & surgery
– Moderate Disease
• Bleed spontaneously occasionally
– Severe Disease
• Frequent spontaneous bleeds
• Type of bleeding:
– soft-tissue hematomas and hemarthroses leading to
hemarthropathy
Bruising in Hemophilia
Hemarthrosis
 Disseminated Intravascular
Coagulation
• A syndrome characterized by widespread
deposition of fibrin in circulation as a result of
• systemic intravascular activation of
coagulation
• impaired fibrinolysis and,
• suppression of physiological anticoagulant
mechanisms
 Disseminated Intravascular Coagulation

systemic activation of coagulation

widespread fibrin Consumption of

deposition platelets & clotting
factors

Microvascular
Hemorrhage
thrombosis
 Disseminated Intravascular Coagulation
• Triggers of DIC:
– Cancer cells esp Leukemic blasts
– Placenta in obstetric complications
– Bacterial endo and exotoxins
– Antigen-antibody complexes =S.L.E.
– Temperature extremes= Heat stroke or burns
– Microorganisms=Rickettsae, meningococci
• Sequelae= tissue hypoxia due to microinfarcts (Thrombotic)
or bleeding problems
 Disseminated Intravascular
Coagulation
• Clinically= Bleeding tendency in presence of
widespread coagulation
– Acute D.I.C.= dominated by bleeding
• seen in obstetrical complications and trauma
– Chronic D.I.C.= presents with thrombotic
complications
• seen in cancers
• Manifestations = variable
– Minimal to profound shock, renal failure,
dyspnea, cyanosis, convulsions, and coma
– Hypotension is characteristic.
 Disseminated Intravascular Coagulation
• Lab = PT and aPTT are typically prolonged.
– Thrombocytopenia
– low Fibrinogen
– Elevated plasma Fibrin split products (D-Dimers or
FDP)
• Prognosis = Highly variable
– Depends upon:
• Underlying disorder
• Degree of intravascular clotting
• Treatment of the underlying disorder is most
important!!
 DIC Treatment
Supportive therapy
- fresh blood
- fresh frozen plasma
- fibrinogen concentrates
- platelet concentrates
 Summary
• When you face a child with a
bleeding problem what should
you do?
- Careful history including past
and family history.
- Detailed clinical examination.
- Few screening test then
appropriate specialized tests
- Appropriate referral when
necessary