How to take a bleeding history Hemato update 2013 Hospital Ampang

Transcription

How to take a bleeding history Hemato update 2013 Hospital Ampang
How to take a
bleeding history
Hemato update 2013
Hospital Ampang
Toh See Guan
4 important points
4 important points I wish to obtain from
history taking:
1. Establish the presence of bleeding disorder
2. Assess the severity of bleeding
3. Congenital vs acquired
4. Looking for clues associated with specific
bleeding disorder
Point 1 : Establish the presence of
bleeding disorder
• Does my patient really has bleeding disorder?
• Patients with haemorrhagic disorders always have
significant abnormal bleeding histories
• Evaluate previous response to hemostatic
challenge, e.g. dental extraction, surgery, trauma,
childbirth, etc.
A significant bleeding history
• Epistaxis not stopped by 10 mins compression or
requiring medical attention
• Cutaneous haemorrhage or bruising without
apparent trauma (esp. multiple/ large)
• Prolonged (>15 mins) bleeding from trivial wounds,
or in oral cavity or recurring spontaneously within 7
days
• Post-operative bleeding
A significant bleeding history
• Menorrhagia (esp. from menarche) – clots > 1 inch
in diameter, changing a pad > frequent than
2hourly, or resulting in anemia.
• Bruising with minimal or no apparent trauma
• Heavy or prolonged bleeding after dental
extraction that required medical attention
Point 2 : Assess severity of the
bleeding
• Severe
• Minor
 Spontaneous haemorrhage  Haemorrhage
secondary to
 Early onset, usually from
major trauma/ surgery
infancy
 Rare spontaneous
 Frequent spontaneous
bleed
bleed required
intervention
Point 3 : Congenital vs acquired
• Congenital
• Acquired
 Platelet disorder –
Glanzmann
thrombasthenia, Bernard
Soulier syndrome
 Clotting factor deficiency
– Haemophilia A & B
 Von Willebrand disease
 Herediatry haemorrhagic
telangiectasia
ITP
APML/AA/MDS
Acquired haemophilia
Anticoagulant/
antiplatelet
medication
 Drug induced
thrombocytopenia
 Uraemia
 Liver disease
 DIC




Point 3 : Congenital vs acquired
• Congenital
• Acquired
 Family history – blood
relative with bleeding
problem; consanguinous
marriage; autosomal/Xlinked inheritance
 Onset since small/young
 Medication history –
on anticoagulant/
antiplatelet?
medication/
traditional medicine
a/w thrombocytopenia
 Late/recent onset
 Underlying
lymphoproliferative
d/o, CTD, HIV, HCV,
CKD, liver disease,
sepsis, etc
Inheritance : X-linked recessive
Carrier Woman
Healthy Man
XH X
XH X
Carrier Girl
X
X X
Healthy Girl
XH Y
Haemophilic Boy
Y
X Y
Healthy Boy
Point 4 : Looking for clues associated with
specific bleeding disorder
• Mucocutaneous bleed – thrombocytopenias, plt
dysfunction, vWd
• Cephalhematomas in newborns, hemarthroses,
intramuscular, retroperitoneal hemorrhages –
severe hemophilias A & B, severe FVII def, severe
type 3 vWd, afibrinogenemia
Point 4 : Looking for clues associated with
specific bleeding disorder
• Bleeding from stump of umbilical cord – FXIII def,
afibrinogenemia
• Recurrent epistaxis & chronic iron def anemia –
hereditary hemorrhagic telangiectasia
Primary haemostasis
EC
platelets
TF
vWF
Platelets adhere to vWF-collagen
M. Laffan
Secondary haemostasis
X
Xa
EC
VIIa
TF
TF-VIIa triggers Xa production
Thrombin generation proceeds on PL (platelet) surface
M. Laffan
Stable clot formation
fibrin
platelets
Stable fibrin-platelet clot is formed
M. Laffan
Bleeding
• Immediate bleeding
– Defects in primary haemostasis
– Vascular abnormality
• Delayed bleeding
– Defects in secondary haemostasis
A good bleeding history is the
best screening test
Bleeding disorders not detected by
routine coagulation screen
• Mild factor deficiencies
• von Willebrand disease
• Factor XIII deficiency
• Platelet disorders
• Excessive fibrinolysis
• Vessel wall disorders
• Metabolic disorders (e.g. uraemia)
Case 1
• 1o year old boy with chronic tonsillitis
• Planned for tonsillectomy
• FBC, PT, APTT sent
• Mother c/o that son has easy bruising and
recurrent epistaxis and she herself has
menorrhagia
• Hb 12 Plt 243
• PT 12.5s (12- 16s) APTT 38s (30- 42s)
Case 1 – cont’d
• Since platelet count and PT, APTT all normal
• Mother reassured and proceeded with
tonsillectomy
• During surgery, excessive bleeding noted
but controlled with local measures
• 2 hours post-op, further significant bleeding
Case 1 – cont’d
• Returned to OT, cauterization done
• 2 Packed RBC and 2 FFP transfused; bleeding
controlled
• Repeat PT, APTT the following day- normal
• Refer hematologist
Case 1 – cont’d
• Further bleeding history taken
• Mother’s blood sample sent
• FBC normal PT 13s (12-16) APTT 40s (3042)
• FVIII 34% (40-150) vWF 30% (50-150)
• Son’s results similar to mum’s (on f/u)
• Diagnosis: von Willebrand disease type 1
Limited investigation of a patient with
a bleeding history
is as inappropriate as
Extensive investigation of a patient
with no bleeding history
Limitations of PT, APTT
• Lack sensitivity and specificity
• Tests a very limited portion of haemostasis
• Can only detect factor levels below 30%
Case 2
• 11 year-old boy
• Blunt abdominal trauma by bicycle handle
bar on 30th June 2013
• 3 days later on 3rd July – abdominal pain
• Hospitalised on 5th July 2013
S. Krishnan 2013
Case 2 – cont’d
• In pain
• Stable circulation
• Tender LHC
• Bicycle handle bar
impression
S. Krishnan 2013
CT scan 05/07/13
S. Krishnan 2013
CT scan 05/07/13
S. Krishnan 2013
CT scan 05/07/13
S. Krishnan 2013
Case 2 – cont’d
• Hb 11.6
TW 11.8
• PT 12 s
APTT 38.7 s
Plt 208
TT 15.7 s
• Fibrinogen 2.6 g/L
• D-Dimers detected
• Serum amylase: not elevated
• RP, LFT normal
Case 2 – cont’d
• 48 hours later …
• Increasing abdominal
distension and pain
• Hb 6.4 g/dL
S. Krishnan 2013
CT scan 07/07/13
S. Krishnan 2013
CT scan 07/07/13
S. Krishnan 2013
CT scan 07/07/13
S. Krishnan 2013
CT scan 07/07/13
S. Krishnan 2013
Case 2 – further tests
• Repeat tests on 8th July
• PT 14 s
• APTT 43.4 s
• Fibrinogen 4 g/L
• Platelet 106
Case 2 – clinical suspicion
• Diagnosis: DIC
• Rx: Transfusions
– Packed red cells
– FFP
– Platelets and
– Cryoprecipitate
Case 2 – bleeding history
• Normal SVD
• No umbilical stump bleeding
• Vaccinations – OK
• No mucocutaneous bleeds
Case 2 – bleeding history
• Age 6 years old
– delayed expanding haematoma R shin after hit by a
stone
– Had some tests to investigate bleeding tendency but no
abnormalities detected
– Surgical evacuation done. No specific therapy
– Wound healing by secondary intention
S. Krishnan 2013
Arrows mark the linear surgical scar resulting from operative evacuation of an expanding traumatic right shin haematoma
at the age of 6 years at Hospital Sultanah Aminah JB
Case 2 – family history
• 3rd of 5 children; 1 sister and 3 brothers
• Non-consanguinous parents
• No family history of easy bruising or h/o
haemophilia
Case 2 – specific factor assays
• Factor VIII 13%
• Factor IX 150%
• vWF Ag 144%
• Diagnosis: mild haemophilia A
Case 2 – progress
• Factor replacement: 100%
• Double J stent inserted in L ureter
• Planned for evacuation of haematoma
Case 3
• 5 year-old boy
• Admitted for upper GI haemorrhage
• h/o recurrent epistaxis and easy bruising
• No f/h of bleeding
• Hb 4.5 g/dL TW 4.5 Plt 398
Case 3 – cont’d
• PT
12.o (11.5- 14.4) sec
• APTT
102.0 (36.9- 45.5) sec
• 4 PRBC & 4 FFP transfused
• Factor VIII 2.5%
• Diagnosis: Moderate Haemophilia A
Case 3 – cont’d
• Bleeding stopped with FFP x 3 doses
• OGDS: pangastritis
• Switched to hemofil M (high purity FVIII)
• 3 days later, re-bled
• Hb fell from 11.o to 5.0 g/dL
• APTT 98 sec Mixing studies 48 sec
Case 3 - cont’d
• Suspected inhibitor; switched to PCC
• Unable to do inhibitor assay
• Sample sent to reference laboratory
– FVIII 3%
No inhibitor detected
– vWF Ag < 1%
• Diagnosis: severe type 3 vWD
A good bleeding history is the
best screening test
Thank you