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9/13/16
IDENTIFICATION
M.N.
2years 9 months old
Female
Resident in Douala
9/13/16
PRESENTING COMPLAINT
Referred from Polyclinic Bonanjo
(Douala) for better management of
severe hemolytic anemia
9/13/16
HISTORY OF PRESENTING
ILLNESS
D(-10)
Developed a fever of 40.5C and an
inflammed throat.
Was put on Aztreonam and
Vancomycin IV
A series of work-ups and a total of 9
blood transfusions were done due to
repeated episodes of severe anemia
according to this chronology:
9/13/16
D(11)
D(-8)
D(-7)
Hb(g/dl)
3,2
g/dl
3g/dl
7.6g/dl
WBC/m
m
1250 9100
0
11000
14900
13 700
19 600
18 300
Platelet/
mm
4290 298
342
00/m 000/mm 000/m
m
m
386
000/m
m
327
000/m
m
301
000/m
m
384
000/mm
BG Rh
O
(+)
D(-4)
D(-3)
D(-2)
Hb - A
96.4%,
HbA2.6%, HbF
1.0%
El Hb
HP
Neg
CRP
96m
g/l
9/13/16
D(-5)
D(11)
D(-8)
D(-7)
D(-5)
D(-4)
D(-3)
D(-2)
SGOT
48IU/L
29
25
SGPT
25IU/L
141
32
Bilirubin
103.
Total
Conjugat 3
5.9
ed
HIV
negati
ve
Calcium
Urinalysi
s
Urea /
creatinin
e
9/13/16
Norma Norma
l
l
0.59/2
HISTORY OF PRESENTING
ILLNESS
ABDOMINAL ULTRASOND
Homogenous hepatomegaly. No ascitis,
no adenomegaly
CT SCAN OF THORAX AND ABDOMEN
Homogenous hepatosplenomegaly, no
deep adenopathy, no pulmonary
infection.
9/13/16
HISTORY OF PRESENTING
ILLNESS
The patient was referred to the YTHC
for proper management.
9/13/16
10
11
12
13
14
9/13/16
15
SYSTEMIC ENQUIRY
DIGESTIVE SYSTEM
Anorexia (+)
Constipation (+)
Abdominal pains(+), intense, diffuse
URINARY SYSTEM
Dark urine (+) coca cola
SKIN
Rash (-)
Pruritus (-)
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16
PHYSICAL EXAMINATION
Vital parameters:
T=37,9C; HR=130bpm; RR=40cpm SaO - 90%
(room air)
Anthropometric Parameters
Wt =16,40Kg
Ht = 92cm
MUAC = 16cm
Ht for Age= 0 to -1 Z-Score
Wt for age = +1 to +2 Z-Score
All normal for age
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17
PHYSICAL EXAMINATION
9/13/16
18
PHYSICAL EXAMINATION
CHEST
Symmetric, no increased work of
breathing
Lungs clear to auscultation bilaterally
CARDIOVASCULAR
Regular rate and rhythm, no murmurs,
rubs nor gallops. Radial, posterior tibial
and femoral pulses symmetric
bilaterally
CRT > 3s
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19
PHYSICAL EXAMINATION
ABDOMEN:
Slightly distended, mobile with breathing,
positive bowel sounds, tenderness of
epigastric region, splenomegaly Hackett 2,
tender hepatomegaly of 2cm below right
costal angle; no dullness on percussion
EXTREMITIES
No visible deformation, cold extremities, no
edema over knees or ankles, normal range of
motion without pain
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20
PHYSICAL EXAMINATION
NEUROLOGIC EXAM
Infant conscious, alert.
Reactive pupils bilaterally, no focal
signs.
No neck stiffness, no signs of
meningeal irritation
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21
SUMMARY
A 2yr 9mth old female infant with PMH
of uncompleted vaccination, good
growth and psychomotor development
for age, referred from Polyclinic
Bonanjo, Douala for better
management of acute severe
hemolytic anemia having necessitated
9 blood transfusions in the past
2weeks
9/13/16
22
SUMMARY
Physical examination revealed:
An altered general state by asthenia and
anorexia
Signs of Collapse (cold extremities,
CRT>3s)
An anemic-hemolytic syndrome: jaundice,
pallor of conjunctivae, palms and soles of
feet, HPM, SPM, dark urine (coca cola)
A SIRS: fever, tachycardia (110bpm),
tachypnea (36cpm)
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23
DIAGNOSIS
1. POSITIVE DIAGNOSIS
Severe sepsis complicated by
severe clinical hemolytic anemia
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24
DIAGNOSIS
DIFFERENTIAL DIAGNOSIS:
Severe malaria (jaundice, severe anemia,
dark coca cola urine)
Severe hemolytic anemia due to an
erythrocyte enzymopathy (deficit in G6PD,
pyruvate kinase)
Severe hemolytic anemia of immunologic
origin (Autoimmune, Allo-immune, immuno
allergic)
Acute Viral hepatitis (HBV,HCV)
Malignant hemopathy (leukemia)
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25
MANAGEMENT
GOALS:
Stabilize hemodynamic state
Identify and treat the cause
Prevent repeated episodes of
hemolytic anemia
Prevent blood reactions and
infections such as HIV, HCV
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26
MANAGEMENT
WORK UP
Infectious workup
Hemoculture
HP
AgHBs
AcHBC
Dipstick+Urinalysis
9/13/16
Immuno-hematologic
work-up:
FBC (emergency):
Hb=3,5g/dl +
Reticulocytes
Coombs test Direct
Indirect
Rhesus phenotype
Consultation with a
hematologist
27
MANAGEMENT
TREATMENT
1.General measures :
Oxygen therapy 2L/min
Urinary catheter to quantify diuresis
Feed child regularly
2.Therapeutic measures:
a. Fluid intake
Volume expansion : 2.5L/m/24h
{SA = (4P+7)/(P+90)} 2.5 x 0.68 = 1.7L of
liquids/24h
Emergency blood transfusion of whole blood :
6xWt(Hb d Hb a) = 300ml in 4hrs
1000ml mixed serum/24h
400ml of liquids per os
9/13/16
28
MANAGEMENT
b. Antibiotics:
Ceftriaxone : 100mg/kg/24h IV
Amikacine IV : 7,5mg/kg/24h
c. Anti-malarial:
Arthemeter inj 1.6mg/kg/24h
c. Anti-pyretic:
Paracetamol 60mg/kg/24h
(15mg/kg/6h) if T>38.5
3. Survey: T/6h, HR,RR/4h, SaO/4h,
icterus,
diuresis + colour of urine/24h
9/13/16
29
EVENT
Day 3
Day 7
Day 8
Day 10
Paramet
er
T 40C
T 37,0C
T 37,3C
T 37,5C
P/E
Pallor, Jaundice
Dark urine (coca
cola)
Workup
s
Coombs test
(direct): Neg
AgHBs negative
Phenotype :
cc ee k- HP Neg
Creatinine:14.76m
g/L
Fine crackles at
base of lungs
bilaterally,
abdominal pains
(HPM=4cm,
SPM=3cm)
FBC + peripheral
blood
smear:Numerous
Heinz bodies
Few
schizocytes
Reticulocytes
0.8% (N: 0.2-1.5)
Coombs
test(direct): Neg
133000/mm
Erythroblasts-23%
Chest x-ray:
alveolar
condenstation of
right lung base.
Bilateral peribronchial
thickening
Coombs test
(direct) Positive to
IgG
-FBC: nucleated
RBCs -16.64
WBC 13 520 (N76%; L-17%)
Hemoculture
1,2,3 : Sterile
Alloimmunization?
Immuno-allergic?
Working
diagnosi
s
Anemic-hemolytic
syndrome
Pneumonia
Plan
Collegial
discussion with
hematology and
nephrology
services
Abdominal
Ultrasound
9/13/16
Treatme
Hyperhydration
Day 24
Amikacine
Stop Arthemeter
Cotrimoxazole
30
Referre
9/13/16
31
DYNAMICS OF Hb
Hb(g/dl)
10
9
8
7
6
5
Blood
transfusions
4
3
2
1
0
1
10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25
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32
GB.10 15. 12. 11. 10. 9.1 14. 16. 11. 10. 13. 14. 11. 10. 20. 20. 12. 20. 38. 14. 19.
/mm 7 3 1 7
4 3 1 4 9 0 2 5 9 1 7 2 9 5 7 1 9.3
PN%
55.
42. 33.
59 52 69 50 72 55 3 60 58 70 66 61 50 54 55 68 8 5 84
34.
6
L%
45. 51.
37 41 27 44 24 36 37 32 36 25 32 34 46 38 37 27 9 3 13
46.
1
Plt x
10/m
m
188148 73 64 68 86136100107133 40 68 68 65111 85 88 23109118131
9/13/16
33
DAY 24
Blood transfusion
Referred to Tunisia (Amen clinic of Tunis )
on request of parents for diagnostic and
appropiate therapeutic measures
RESULT OF ABDOMINAL SCAN
Multi nodular splenomegaly which evokes
a lymphoproliferative pathology
associated with pelvic ascitis of mild
abundance
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34
EVOLUTION
MEDICAL REPORT FROM TUNISIA
RETAINED DIAGNOSIS: inaugural
autoimmune hemolytic anemia of
systemic lupus erythematosus origin
WORKUP :
Antinuclear antibody: 1/400
Anti-Sm antibody: positive
MANAGEMENT:
Prednisone 2mg/kg/day for 3-6mths
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35
CONCLUSION
We have presented the clinical case of
a 2yr 9mth old child with systemic
lupus erythematosus whose inaugural
manifestation was severe hemolytic
anemia.
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36
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37