1

Coagulation : Case based approach

2nd Basic hematopathology course, TMH,
Mumbai
Saturday, 11th June 2011
r! M!B! "gar#al, M, M$"MS
Head, ept o% Haematology, Bombay Hospital &nst o% Med Sc, Mumbai
2
Bleeding disorders
'
Case 1
(
Jigar, 1)*year old boy %rom +adodara #ith
post*traumatic nasal , oral bleeding : 2 hours
$o %amily h-o bleeding disorder
)
Jigar : .ab! in/estigations
Tests 0esults Control
Hb 1'!' g-dl
Haematocrit (1!( 1
2BC 3400-cmm
5latelets '67,000-cmm
5T 11 s 11 s
5TT (6 s 27 s
6
i%%erential diagnosis
3
8actor de%iciency
or
&nhibitors
7
Test 0esult Control
5TT (6 s 27 s
5TT mi9 :1:1; '0 s
2hat does this mean <
4
8actor de%iciency
10
Test 0esult Control
5TT (6 s 27 s
5TT mi9 :1:1; (' s
2hat does this mean <
:(6 = 27 > 3( ? 2 > '3;
11
&nhibitors
8actor speci%ic .upus type
12
&nhibitors
8actor speci%ic .upus type

@CT

d0++T

.1 - .2 A 1!'

8actor assay
1'
$ormal coagulation cascade
1(
&ntrinsic path#ay de%ect
8actor +&&& de%iciency including
/on 2illebrand isease
8actor &B de%iciency
8actor B& de%iciency :rare;
8actor B&& de%iciency :non*bleeder;
1)
Test 0esult $ormal
8actor +&&& : C 4 1 )0 * 1)0 1
8actor &B 33 1 )0 * 1)0 1
2hat does this mean <
16
Classical haemophilia "
or
/on 2illebrand isease
13
/2 has eCual pre/alence in
both se9es :"utosomal;
17
Coming bacD to Jigar
Bleeding %rom nose
0aised 5TT
Eood correction on mi9ing studies
.o# %actor +&&& : C
14
Jigar : 8urther #orD up
Test 0esult $ormal
+&&& : C 41 )0*1)01
/28 : "g 12 1 60*1)0 1
/28 : 0Co8 10 1 )0*1)0 1
20
Jigar : 8inal diagnosis
21
+on 2illebrand isease
22
/2 : 5rimary classi%ication
Subtypes /28
Type 1 5artial de%iciency :";
Type 2 Fualitati/e de%ect :";
Type ' Total de%iciency :"0;
2'
/2 type 2 : sub classi%ication
Subtypes Characteristics
2"G
High mol #t /28
multimers absent
/2 : Type 1 ,
Type 2M
2BG
.o# and High mol #t /28 H
I 0&5"
Thrombocytopenia
2MG
High mol #t /28 multimers normal
H /28 : CB
/2 : Type 2"
2$GG MarDedly H a%%inity %or %actor +&&& Haemophilia
G/28 : "g A /28 : 0co, GG"0
2(
/2 : .aboratory tests
CBC, platelet count, 5T : $ormal
5TT : o%ten normal
8actor +&&& :coagulometer;
/28 : 0Co8 :aggregometry;
/28 : CB :J.&S";
/28 : "g :J.&S";
0&5" : "ggregometry
/28 : Multimer analysis :gel electrophoresis;
2)
+ariations in /28 le/els :K;
J9ercise and stress :adrenaline;
&n%ection and in%lammation
.i/er disease
5regnancy
Jstrogen and progesterone therapy
Hyperthyroidism
26
Eenetic /ariations in /28 le/els
Blood group L has lo#est le/el
BlacD race has higher le/el
BlacD #ith non " may ha/e double the le/el o%
a Caucasian #ith group L
egree o% proteolysis /aries
""MTS*1' acti/ity /aries
23
5latelet*type /2
&t is not /2
" genetic platelet disorder :";
" gain*o%*%unction mutation
K a%%inity o% platelet E51b %or /28
K 0&5"
Thrombocytopenia
5henotype : similar to type 2B
Treatment : platelet trans%usion and not /28
27
"cCuired /2
$ot an uncommon disease
5ts #ith autoimmune disorders, hypothyroidism,
lymphoma, 2M
"ntibodies are present only in a minority
J9cessi/e proteolysis or consumption :"S;
24
"ssociations
"ngiodysplastic lesions in gut
M "therosclerosis :pigs;
iagnosis may be totally missed during pregnancy
and early puerperium
8actor +&&& le/el may /ary #ithin the %amily
0ole o% 58"*100
'0
'1
Case 2
'2
Case study
Mr! 0aN, 64y %rom 0aNDot
SC hematomas , easy bruising : 2 mths
5latelet count : ','2,000-cmm
5T : ('-12 secs, &$0 '!3
5TT : )6-'0 secs
''
2hat is the
di%%erential diagnosis <
'(
2hene/er 5T , 5TT
both are raised OO<
')
8irst,
#e must e9clude
di%%icult collection
:partially clotted blood;
'6
2e must also e9clude
e%%ect o% high haematocrit
:polycythemia;
'3
Common path#ay de%ect
+itamin @ de%iciency
Chronic li/er disease
Consumpti/e coagulopathy
"nticoagulant therapy
'7
.i/er %unction : normal

&C pro%ile : normal

'4
0aN : Mi9ing studies
Test 5T 5TT
0aN (' )6
Control 12 '0
( : 1 mi9 1) ')
(0
8actor & : 240 mg-dl
8actor && : 731
8actor + : 371
8actor B : 1!21
(1
2hat is
the aetiology o%
selecti/e %actor B de%iciency
at the age o%
64 years <
(2
('
"myloidosis
((
0aN : "myloidosis
Macroglossia
S! 5rotein electrophoresis : 8aint M band present
S! &mmuno%i9ation : .ambda monoclonal gammopathy
Bone marro# : 5lasma cells : 2*'1
Bone marro# biopsy : "myloidosis
"bdominal %at pad biopsy : "myloidosis
()
5art '
5ictorial CuiP
(6
(3
(7
(4
2hat is this <
)0
)1
)2
)'
Blue toe syndrome
)(
))
)6
Conclusion
)3
B+&&&th $ational CMJ in Haematology ,
Haemato*oncology on
26th * 24th o% January 2012 :Thurs * Sun;
at
Bombay Hospital &nst o% Med Sc, Mumbai
: Subsidised registration to participants submitting
registration %orm today;
)7
Thank
You
)4
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