CLINICAL CASE

Presented by: Dr NJIANDOCK FOMENKY

Dr MEKONE NKWELE
Supervised by: Dr KAMGAING Nelly
Dr MONEBENIMP Francisca

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(-12); abdominal pains, moderate
diffuse.
- Automedication, Paracetamol
- D(-11); Persisting pain, jaundice of
skin and mucosa, progressively
increasing asthenia without fever
- Child taken to a clinic in Douala for
consultation
- An initial diagnosis of sickle cell
anemia and vaso-occlusive crisis was
9/13/16

HISTORY OF PRESENTING ILLNESS

Initial management:
Emergency blood transfusion (Hb of
3.2g/dl).
Ceftriaxone inj, folic acid tablets,
Paracetamol tablets.
Quinine IV
IV fluids
Ranitidine and
magnesium+aluminium hydroxide
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

7.6 g/dl 3,5 g/dl 4.1 g/dl 10,4

g/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.

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10

PAST MEDICAL HISTORY

PERSONAL
MEDICAL
Has never been hospitalized before
No known chronic illness
Epigastric pains (+)
Bronchiolitis vs. Asthma de novo at
19months
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11

PAST MEDICAL HISTORY

SURGICAL
- No surgery done, no trauma
IMMUNOLOGIC STATUS
- BG O Rh (+)
- El Hb AA
- HIV serology negative
- No known allergies
- Vaccines up to date till 18mths
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12

PAST MEDICAL HISTORY

ENVIRONMENTAL
No passive smoking, no contact with
tuberculosis patients, MILDA (+)
NUTRITION
No special regime
DEVELOPMENTAL
Psychomotor development normal for
age
9/13/16

13

PAST MEDICAL HISTORY

FAMILY HISTORY
MOTHER: 32y.o., Business woman, G3 P3
003,
BG O Rh (+), El HB not known. Apparently
in good health
FATHER: 36y.o. employee in a
multinational company, BG O Rh (+)
El Hb not known. Apparently in good health
SIBLINGS: 3/3 : 2 older brothers of 7y.o.
and 10y.o. all in apparently good health
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PAST MEDICAL HISTORY

PEDIGREE

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15

SYSTEMIC ENQUIRY
DIGESTIVE SYSTEM
Anorexia (+)
Constipation (+)
Abdominal pains(+), intense, diffuse
URINARY SYSTEM
Dark urine (+) coca cola
SKIN
Rash (-)
Pruritus (-)
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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
9/13/16

17

PHYSICAL EXAMINATION

HEAD AND NECK

Conjunctivae pale, jaundice of sclerae
Ears normal
Mouth: humid and clean, oropharynx :
tonsils are normal in size
Neck: supple, no cervical
lymphadenopathy

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
9/13/16

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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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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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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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
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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

No. of days of hospitalization

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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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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
9/13/16

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

ANKS FOR YOUR ATTENTI

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