Presentation slides for CPCs paeds in 5th year M.B.B.S CUCMS academic session 2018/2019

1. Approach to a Child with
Pallor
Paediatrics Clinicopathological Conference:
5TH YEAR M.B.B.S COMMON SESSION
FACILITATOR: DR HANNAH WARDIAH ROSLAND & DR ASHIKIN MOHD NORDIN
PRESENTED BY:
GROUP 2
5TH YEAR M.B.B.S
ACADEMIC SESSION 2018/2019
3 0 N O V 2 0 1 8 | 2 . 3 0 P M – 4 . 3 0 P M | L E C T U R E H AL L 1 ( L E V E L 4 )
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2. Objectives
1. How to approach children presented with pallor.
• History taking
• Physical examination
• Investigation
2. How to manage children presented with pallor.
1

3. Chief complaint
FA, an 8 year old Malay boy complained of unresolved
fever since 5 days prior to admission associated with
cough and pallor.
2

4. What further history would
you like to know?
3

5. History of Presenting
Illness
FEVER
• Onset : 5/7
• high grade (39˚C)
• Continuous
• worsened at night
• with no chills and rigor
• Went to GP on day 2 of illness and was given
syrup PCM only, no antibiotic given
• temporarily resolved by syrup PCM taken from
GP.
PALLOR
• Onset : since early 2018
• Worsened since the onset of fever
• no complaint of easily fatigue
• patient still active as usual
• no palpitation
• no history of syncope before
• no complaint of easily bruising nor bleeding
tendency
• no recent history of vomiting of blood nor
bloody stool
• no complaint of joint nor bone pain
• no significant weight loss noted by mother
• no yellowish skin discolouration nor tea
coloured urine noted by mother
4

6. COUGH
• Onset : 5/7
• non productive
• on and off
• not related to food
• no post tussive vomiting
• no facial congestion nor cyanosis
• no wheezing nor rapid breathing.
• no sleep disturbance
• Patient also had reduced oral intake and become more lethargy since 1 day prior
to admission.
• Otherwise, patient had no runny nose, no rashes, no abdominal pain, no vomiting
nor diarrhea and no problem in passing urine and motion.
5

7. Past Medical History
• Kawasaki Disease 8 months old → given IVIG and Aspirin → claimed
echocardiography was normal but then defaulted at age of 3 year old.
Drugs History
• No known drugs allergies
6

8. Paediatric History
Antenatal History : anemia in pregnancy on haematinics
Birth History :
• Born at term via ELLSCS for cephalopelvic disproportion
• birth weight of 2.85 kg, G6PD, TSH screening normal
• no complication nor NICU admission
Postnatal :
• No history of neonatal jaundice nor prolonged jaundice
Immunization history : completed up to age with no optional vaccine
7

9. Developmental history :
• Height : 117cm
• Weight : 19 kg
8

10. Diet history
Breakfast : bread/cereal with 1 glass of milk
Brunch : nasi lemak/nasi goreng at school
Lunch : rice/pasta + chicken/fish/meat
Snacks at 4 pm
Dinner : occasionally eats rice
Takes minimal fruits and vegetables
* non-picky and can complete his meal.
9

11. Family History
• Non-consanguineous marriage.
• Paternal side :
➢patient‘s aunt has thalassemia
trait.
• Maternal side :
➢Grandmother has hypertension
➢Grandmother’s siblings has lung
ca.
• No family history of regular blood
transfusion nor bleeding
tendencies, no family history of
G6PD.
• Both parents never screened for
thalassemia.
33 y/o
Has business
in Johor
NKMI
33 y/o
Work in KPDN
Putrajaya
NKMI
2 y/o
NKMI
4 y/o
NKMI
10

12. Social History
• In standard 2 primary school
• In last ranking class but score 2nd out of total classroom.
• Able to read and write
• No complaints from teacher - no changes in school performances
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13. Problem List
12

14. 2. Pallor for 7 months
3. Family history of Thalassemia trait
1. Fever a/w cough for 5/7
13

15. What is your differential
diagnosis?
14

16. Physical Examination
15

17. General
• Alert, lying comfortably, no respiratory distress, pale, growth appropriate to
age and no dysmorphism.
• Conjunctiva pallor, no sclera jaundice, good hydration
• Hand : pale, warm peripheries, no finger clubbing, CRT <2 seconds
• Vital signs :
➢Bp : 96/61 mmHg
➢Pulse rate : 120 beats/min, regular with good volume
➢Temperature : 38 ˚C
➢SPO2 : 100 under room air
➢RR: 28 breath/min
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18. What system would you like to
examine and what signs are
you looking for ?
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19. Systemic Examination
Throat Examination
• Tonsils grade 2
• No exudates
• Throat not injected
Respiratory Examination
• Inspection : no scar, normal chest shape, equal chest expansion, no signs of
distress
• Palpation : lung expansion equal bilaterally
• Auscultation : vesicular breath sound, air entry equal bilaterally, no
additional sound
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20. Cardiovascular Examination
• Palpation : apex beat not displaced
• Auscultation : S1, S2 normal, no murmur
Abdominal Examination
• Inspection : Not distended, umbilicus centrally located, no scar, no
prominent vessels nor pulsation.
• Palpation & Percussion : soft, non-tender, liver palpable 7 cm below
costal margin (liver span 12cm),firm, non tender, smooth surface
with regular margin and spleen palpable (7cm), no ballotable kidney
• Auscultation : normal bowel sound, no aortic nor renal bruit.
19

21. Lymph Node Examination
Multiple non tender lymph node palpable (0.5cm x 0.5cm) :
• Bilateral paracervical
• Right supraclavicular
• Bilateral cervical
• Bilateral inguinal
• Bilateral axillary
20

22. Problem List
21

23. 4. Hepatosplenomegaly
5. Lymphadenopathy
2. Pallor for 7 months
3. Family history of Thalassemia trait
1. Fever a/w cough for 5/7
22

24. Differential Diagnosis
23

25. Pallor
Reduced in production of RBC Increased in destruction of RBC Blood loss
Abnormal bone marrow
- Aplastic anemia
- Leukemia
- Myelodysplastic syndrome
Essential factor deficiency
-Deficiency anemia
Iron, vitamin B12, Folic acid
Ineffective erythropoiesis
-Thalassemia syndrome
-Sideroblastic anemia
Hemolytic anemia
Intracapsular defect
-Membrane : Hereditary spherocytosis
-Enzyme : G6PD deficiency
-Hemoglobin : Thalassemia
Extracapsular defect
-Mechanical
Microangiopathic hemolytic anemia
-Infections
-Antibodies
Acute blood loss
-Gastro-intestinal loss
Chronic blood loss
-Parasitic infestation
24

26. Hepatosplenomegaly
Infection Primary liver malignancy Blood disorder
Sepsis
Malaria
Typhoid
viral hepatitis
Hepatocellular carcinoma
Hepatoblastoma
Haemolytic anemia
Leukemia / Lymphoma
25

27. Generalized lymph nodes
Infectious ● Viral (most common): URTI, measles, varicella,
rubella, hepatitis, adenovirus, HIV, EBV
● Bacterial: syphilis, brucellosis, tuberculosis,
typhoid fever, septicemia
● Fungal: histoplasmosis
● Protozoal:toxoplasmosis
Non Infectious ● Sarcoidosis
● Rheumatoid arthritis
● SLE
Malignant ● Leukemia
● Lymphoma
● Neuroblastoma
Drug reaction ● Phenytoin
● Allopurinol
26

28. Differential diagnosis in this patient
1. Iron Deficiency Anaemia
2. Thalassemia
3. Acute leukemia
27

29. Investigations
28

30. What Investigation would
you like to do?
29

31. Serial Full Blood Count
Parameters Normal
Range
26/8/2018 27/8/2018 28/8/2018 29/8/2018
Haemoglobin 11.0 – 16.0 4.4 5.4 5.8 8.1
MCV 76.0-96.0 60.2 - - 71.0
MCH 27.0-32.0 19.3 - - 24.1
Total White
Blood Cell
(Differentials)
4.5 – 13.5 4.0 3.8
(N 12%,
L 71%)
4
(N 10.3 %,
L 74.3%)
2.7
(N 19.9%,
L 56.4%)
Platelet 150 - 400 65 115 65 46
Haematocrit 38.8 - 50 16.3 13.9 23.8
Absolute
Neutrophil
Count
- 0.4 0.7 0.4 0.5
Interpretation: pancytopenia with
haemodilution
1 PINT BLOOD
TRANSFUSION
1 PINT BLOOD
TRANSFUSION
30

32. Full Blood Picture
Red Blood Cell: Microcytic, hypochromic cells.
Target Cells seen. Occasional tear drop.
Platelets: Low platelet count. Occasional large
and giant platelet seen. No platelet clumping 31

33. Full Blood Picture
White Blood Cell: Reduce with neutropenia.
Reactive lymphocyte seen. No left shift or
blast cells seen. 32

34. Full Blood Picture
• Microcytic hypochromic anaemia
• Pancytopenia
33

35. Renal Profile
COMPONENT RESULT NORMAL RANGE UNIT INTERPRETATION
Urea 3.5 2.5 – 6.4 mmol/L Normal
Sodium 135 136 - 145 mmol/L Mild hyponatremia
Potassium 3.4 3.5 - 5.1 mmol/L Mild hypokalemia
Chloride - 98 - 107 mmol/L -
Creatinine 26 71 - 115 umol/L Low creatinine
34

36. Other Serum Electrolyte Level
COMPONENT RESULT NORMAL RANGE INTERPRETATION
Calcium 2.04 2.20 – 2.68 mmol/L Hypocalcemia
Corrected calcium 2.08 2.20 – 2.68 mmol/L Hypocalcemia
Magnesium 0.81 0.65 – 1.05 mmol/L Normal
Phosphate 1.03 1.45 – 1.78 mmol/L Hypophospahtaemia
35

37. Liver Function Test
COMPONENT RESULT NORMAL RANGE INTERPRETATION
ALP 48 53-128 u/L Low ALP
ALT
AST
16
45
<54 u/L
<40 u/L
Normal
High AST
Total Bilirubin 11.7 <205 umol/L Normal
Conjugated bilirubin
Unconjugated bilirubin
3.3
8.4
<5.1 umol/L
<21 umol/L
Normal
Normal
Total Protein
Albumin
66
38
66-87 g/L
35-50 g/L
Normal
Normal
36

38. Coagulation Profile and Lactate
Dehydrogenase
COMPONENT RESULT NORMAL RANGE INTERPRETATION
Prothrombin Time 12 11 – 13 seconds Normal
International Normalized
Ratio (INR)
1.1 < 1.1 Normal
Activated Partial
Thromboplastin Time
(aPTT)
38.3 22 – 33 seconds Prolonged aPTT
Lactate Dehydrogenase 576 110 – 295 U/L Elevated LDH
37

39. Iron Study
COMPONENT RESULT NORMAL RANGE INTERPRETATION
Iron 17.9 60 – 170 ug/dL Low Iron
Total Iron Binding
Capacity
N/A - -
Serum Ferritin 978.7 7-140 mcg/L Highly elevated
38

40. • Infectious disease:
➢Mycoplasma Pneumoniae
Serology = POSITIVE
➢Hepatitis B Surface Antigen
➢Antibody to Hepatitis C Virus
➢Epstein Barr Virus Serology
➢Parvovirus Serology
➢Dengue Serology IgM/IgG
• Hb Electrophoresis: HbE B-
Thalassemia
• Coombs Test: Negative for direct
and indirect
Non-Reactive
39

41. Plain Chest Radiograph
40

42. Working diagnosis
1. Haematological malignancy
2. Thalassemia
with underlying Iron deficiency anemia
41

43. Bone Marrow Aspiration Test
• Conclusion by HUKM Haematopathologist:
• Hypercellular marrow with marked trilineage dysplasia. Secondary
cause of myelodysplastic syndrome need to be excluded (eg:
infection)
• Hypochromic microcytic red cells (from FBP) with adequate iron
store in marrow suggestive of ineffective utilisation of iron
secondary to underlying chronic infection.
42

44. Immunophenotyping
• No evidence of abnormal blast population.
43

45. Final Diagnosis?
Myelodysplastic Syndrome with concurrent atypical pneumonia
44

46. Breaking Bad News
45

47. SPIKE Model of Breaking Bad News (Baile et al., 2000)
•Setting
•Perception
•Invitation
•Knowledge
•Emotion & Empathy
•Strategy & Summary
46

48. Setting
• comfortable, quiet, and private room
• Ensure both you and the Pt./relative are
sitting down
• Avoid physical barriers
• Uninterrupted time during the meeting
(NO phones, beepers, etc)
• KIV tissues
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49. Perception
• Events leading up to now
• Any symptoms the patient may have been experiencing
up to this point
• Establish what the patient already knows or is expecting
• Patient’s current emotional state, anything particular on
their minds (FIFE)
“Could you tell me what’s happened so far?”
48

50. Invitation
• Check is the Pt. wants to receive their
results today
• Pt. may put it off until family are
present, family occasion etc.
“I have the result here today, would you
like me to explain it to you now?”
49

51. Knowledge
• Chunk the Dx, pausing after each piece of
information
• Check regularly for understanding
• Use a warning shot to indicate that you
have unfortunate news, Eg:
“As you know we took a biopsy/did a scan,
and unfortunately the results were not as we
hoped”
• Then provide the Dx. in simple language
• Make sure tone is respectful, slow paced,
and clear
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52. Emotion
• Recognise and respond to emotions with acceptance, empathy, and
concern
• Acknowledge and reflect their emotions and body language
• DO NOT LIE about prognosis (no giving false hope)
• If you don’t know, tell them you don’t know
“I can see this is a huge shock for you”
“I can see that this is not the news that you expected, I’m so sorry”
51

53. Strategy & Summary
• Make a plan together to meet again
• Inform Pt. what the next step is
• Reassure the Pt. that they are going to/have been
referred to the appropriate team of specialist who
are best equipped to come up with a plan going
forwards
• Try to not rush Pt. into making decisions about Tx.
• Summarise by respectfully repeating key points
• Answer any question or address concerns
• Highlight where Pt. can gather more info (support
groups, websites etc.
52

54. Management
53

55. Anemia
Blood transfusion
•Pre transfusion Hb: 4.4
•Transfuse 1 pint
•Post transfusion Hb: 5.4 (no
improvement in Hb).
•Transfuse another 1 pint
•Post transfusion Hb: 5.8
•In between transfusion, given
IV Frusemide 20mg
Syrup folic acid
•5ml OD for 2 weeks
• Fever
• Syrup Paracetamol 285mg PRN for 1
week
To cover for pneumonia
Syrup Azithromycin
• 290mg OD on day 1
• 145 mg OD day 2-5
Syrup Augmentin
• 1100mg TDS for 1 week
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56. TAKE HOME MESSAGE
1. Fever, anaemia & hepatosplenomegaly: think about MALIGNANCY !
2. When is BMA needed?
In this patient :
• bilirubin normal (unlikely thalassemia)
• hepatosplenomegaly
• serum ferritin high (unlikely IDA)
3. When do we refer to a tertiary center?
In this patient :
• anemia, hepatosplenomegaly, not responding to transfusion as expected, no
increase in bilirubin, prolong fever, lymphadenopathy, infection.
55

57. Acknowledgement
• Dr Ashikin Mohd Nordin
• Dr Hannah Wardiah Rosland
• Assoc Prof Dr Roswati Mohd Noor (Haematopathologist CUCMS)
• Dr Asmiati Arbi (Consultant Pathologist and Head of Pathology
Department, Hospital Putrajaya)
• Prof Dr Raja Zahratul Azma (Haematopathologist, Hospital Universiti
Kebangsaan Malaysia)
56

58. Thank You
57