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Approach Bleeding
Approach Bleeding
Coagulation defects
FXIII deficiency poor wound healing, severe scar formation
Thrombocytopenia
Wiskott-Aldrich syndrome eczema, immunodeficiency
Blanchette VS, Breakey VR, Revel-Vilk S (eds): SickKids Handbook of Pediatric Thrombosis and Hemostasis.
Basel, Karger, 2013, pp 14–22 (DOI: 10.1159/000346914)
vasculature. There may be prolonged bleeding hematoma, hemarthrosis, central nervous system
following surgery and/or dental extractions. In bleeding and ‘other’, pediatric-specific bleeding
contrast, spontaneous or excessive bleeding into symptoms (postcircumcision bleeding, umbili-
soft tissues, muscles and joints, or delayed sur- cal stump bleeding, cephalohematoma, macro-
gical bleeding suggests disorders of coagula- scopic hematuria, postvenipuncture bleeding,
tion factors. It should be noted that coagulation conjunctival hemorrhage). The mean bleeding
factor disorders may also cause mucocutaneous score in healthy children was 0.5, and a bleeding
bleeding, epistaxis, or gastrointestinal bleeding. score of ≥2 was defined as abnormal. The PBQ
ICH, postcircumcision bleeding or severe muco- was validated prospectively as a screening tool for
sal bleeding in early infancy requires immediate the diagnosis of VWD and studies validating it
investigation for a coagulation factor deficiency. for the diagnosis of other bleeding disorders are
Bleeding from the umbilical cord stump within ongoing.
the first days of life is strongly suggestive of FXIII
Blanchette VS, Breakey VR, Revel-Vilk S (eds): SickKids Handbook of Pediatric Thrombosis and Hemostasis.
Basel, Karger, 2013, pp 14–22 (DOI: 10.1159/000346914)
Table 2. Pediatric bleeding questionnaire scoring key
Symptom –1 0 1 2 3 4
Epistaxis – no or trivial >5 per year or consultation only packing, cauterization or blood transfusion, replacement
(≤5 per year) >10 min duration antifibrinolytics therapy or desmopressin
Cutaneous – no or trivial >1 cm and consultation only – –
(≤1 cm) no trauma
Minor wounds – no or trivial >5 per year or consultation only or surgical hemostasis or blood transfusion, replacement
(≤5 per year) >5 min duration steri-strips antifibrinolytics therapy or desmopressin
Oral cavity – no reported at consultation only surgical hemostasis or blood transfusion, replacement
least one antifibrinolytics therapy or desmopressin
Gastrointestinal – no identified cause consultation or surgical hemostasis, –
tract spontaneous antifibrinolytics,
Blanchette VS, Breakey VR, Revel-Vilk S (eds): SickKids Handbook of Pediatric Thrombosis and Hemostasis.
Basel, Karger, 2013, pp 14–22 (DOI: 10.1159/000346914)
disorders. Mucocutaneous bleeding suggests a dis 20 × 109/l). Pseudothrombocytopenia resulting
order of primary hemostasis, i.e. VWD or platelet from clumping of platelets collected in EDTA an-
dysfunction/deficiency, or a vascular disorder. In ticoagulant can be identified by examination of
males, deep hematomas, hemarthroses, or evidence the blood film, and confirmed by re-collecting a
of chronic joint abnormalities suggests hemophil- specimen in citrate anticoagulant in which clump-
ia. Acquired bleeding disorders may present in the ing will not occur. Examination of the peripheral
context of coexisting illness. Lymphadenopathy blood film is essential in the evaluation of a child
and/or organomegaly suggest an infiltrative pro- with a suspected platelet disorder (chapter 5). If
cess such as malignancy or a storage disease. Signs true thrombocytopenia is diagnosed, the next
of liver failure suggest acquired coagulation factor step would be to differentiate between new on-
deficiencies. Additional congenital anomalies may set acquired thrombocytopenia, chronic acquired
suggest the presence of a syndromic bleeding dis- thrombocytopenia and congenital thrombocyto-
order (table 1). penia (algorithm 1; chapter 5).
Blanchette VS, Breakey VR, Revel-Vilk S (eds): SickKids Handbook of Pediatric Thrombosis and Hemostasis.
Basel, Karger, 2013, pp 14–22 (DOI: 10.1159/000346914)
Child with bleeding symptoms
Blanchette VS, Breakey VR, Revel-Vilk S (eds): SickKids Handbook of Pediatric Thrombosis and Hemostasis.
– FII deficiency – FXI deficiency – Early Vitamin K
– Fibrinogen – FIX deficiency deficiency
deficiency/dysfunction – FVIII deficiency – Oral VKA
– Combined factor deficiencies – Severe VWD
– Vitamin K deficiency
19
1 See chapter 5. – Oral VKA excess
2 Not usually associated with bleeding. – Liver disease
Blanchette VS, Breakey VR, Revel-Vilk S (eds): SickKids Handbook of Pediatric Thrombosis and Hemostasis.
Basel, Karger, 2013, pp 14–22 (DOI: 10.1159/000346914)
A small sample of anticoagulated whole blood whole mount electron microscopy, flow cytomet-
(0.8 ml) is aspirated via a narrow-diameter cap- ric assessment of surface receptors and evaluation
illary through a microscopic aperture cut into a of platelet ultrastructure by transmission electron
membrane coated with the platelet agonists col- microscopy are discussed in chapter 5.
lagen and epinephrine or collagen and adenos-
ine 5′-diphosphate. The high shear rate gener- Fibrinolysis Inhibitors
ated under the standardized flow conditions and Abnormalities of fibrinolysis inhibitors (blood col
presence of the chemical stimuli result in platelet lected into citrate), such as α2-AP and PAI-1, can cause
adhesion, activation and aggregation at the aper- rare bleeding disorders because of increased fibrino-
ture, building a stable platelet plug. The time re- lysis [9].
quired to obtain full occlusion of the aperture is
reported as the closure time. The closure time is Genetic Testing
prolonged by low levels of VWF, thrombocytope- The genetic mutations associated with inher-
Blanchette VS, Breakey VR, Revel-Vilk S (eds): SickKids Handbook of Pediatric Thrombosis and Hemostasis.
Basel, Karger, 2013, pp 14–22 (DOI: 10.1159/000346914)
8 Harrison P, Mackie I, Mumford A, Briggs C, Liesner R, Winter M, FXI Factor XI
Machin S: Guidelines for the laboratory investigation of heritable FXII Factor XII
disorders of platelet function. Br J Haematol 2011;155:30. FXIII Factor XIII
9 Hayward CP: Diagnosis and management of mild bleeding disor- ICH Intracranial hemorrhage
ders. Hematology Am Soc Hematol Educ Program 2005;2005:423. INR International normalized ratio
ITP Immune thrombocytopenia
PAI-1 Plasminogen activator inhibitor type 1
PBQ Pediatric Bleeding Questionnaire
Abbreviations PFA-100® Platelet function analyzer-100
PT Prothrombin time
RBC Red blood cell
α2-AP α2-Antiplasmin
TT Thrombin time
aPTT Activated partial thromboplastin time
VKA Vitamin K antagonist
CBC Complete blood count
VWD von Willebrand disease
FII Factor II (prothrombin)
VWF von Willebrand factor
FV Factor V
VWF:Ag VWF antigen
FVII Factor VII
VWF:RCO VWF activity (ristocetin cofactor assay)
FVIII Factor VIII
Blanchette VS, Breakey VR, Revel-Vilk S (eds): SickKids Handbook of Pediatric Thrombosis and Hemostasis.
Basel, Karger, 2013, pp 14–22 (DOI: 10.1159/000346914)