DEPAERTMENT OF PAEDIATRICS

AND CHILD HEALTH

TUTORIAL PRESENTATION
ROBERT KUMWENDA
HAZEL MUMPHANSHA

MODERATOR: Dr MULENDELE
DATE OF ADMISSION: 30TH APRIL, 2007

DATE OF INTERVIEW: 5TH MAY, 2007

INFORMANT: PATIENT AND AUNT

LANGUAGE: NYANJA
DEMOGRAPHIC DETAILS

 NAME : M.P
 AGE :10 YEARS
 SEX :FEMALE
 TRIBE :NGONI
 RES :CHUNGA
 RLGN :CHRISTIAN (pentecostal faith)
PRESENTING COMPLAINTS

– SWELLINGS IN THE NECK AND GROIN FOR 3/52

HISTORY OF PC

 Monica was well until 3/52 prior to admission

when she noticed a small swelling in the right
side of her neck.
 The swelling was not painful and hard and
said to be the size of a bottle top.
 A few days later, she noted that the swelling
had increased in size and number and were
present on the left side of her neck as well as
in the grion. They were painful at times
 The growth of the swellings was said to be fast both in number and
size.
 The patient denies any history of fever associated with these swellings
but said prior to the swelling she would experience some episodes of
fever with mild headache.
 she has also lost some weight but there has been no history of a
cough and night sweats.
 However, M.P would easily get tired but there is no hx of palpitations
and she was able to lie flat without having difficulties in breathing
 noted mild pain in her bones but no joint pain or swellings. No skin
rashes red discoloration.
 She also complained of abdominal pain which was non specific and
was not associated with diarrhea or vomiting. She was passing stool
normally. No hx of yellowing of eyes.
REVIEW OF SYSTEMS

 Haematopoietic, CVS, MSS and GIT

 GUS: no hx of pain on passing urine, no haematuria
 RS: no cough, chest pain, difficulties breathing
 CNS: no hx of LOC, convulsions or headache
PAST MEDICAL HISTORY

 2nd admission to UTH

 1st admission was january, 2006 for 3/12 with
the same symptoms. Tests were done and
mother was told she has cancer. Medication
was given up to march.
 Since been coming for reviews and
medication every month
 Rvt was done and was negative
PAST DRUG HISTORY

 Injections and pills which she took for first

presentation
 Currently not taking medication because its not
available in uth and is very expensive outside
 Has not shown any reaction to any medication taken
 BIRTH HISTORY – insufficient info
 DVPMENTAL Hx – has been developing well
since the age of three until onset of illness.
Grade 4, #19 in a large class
 IMMUNISATION – fully immunised
 NUTRITIONAL Hx – has 2-3 meals a day.
Whatever is available.
FAMILY HISTORY

 Fifth child in family of 5(2nd twin)

 All other siblings are alive and well
 No hx of similar illness in the family
 No hx of TB, epilepsy, asthma, scd. However
uncle is hypertensive.
SOCIO-ECONOMIC Hx

 uncle does not work due to illness (htn)

 aunt is a small scale business woman. Sells
tomatoes and parafin. Money is tight, barely
able to make ends meet especially since
M.P’s illness as she has neglected business
and medication is expensive
 Three bed roomed house with 9 occupants
 No power, communal tap water, pit latrine
SUMMARY

 MP, F/10 a known ALL pt on tx who px with

fast growing, painless swelling in the neck
and groin for 3/52. there is a hx of fever, wt
loss, easy fatiguability and bone pain
DIFFERENTIALS

 RELAPSE ALL
 Acute Lymphoma
 Tb lymphadenopathy
 ? PGL in RVD
PHYSICAL EXAMINATION

 GENERAL EXAMINATION
i examined a young girl lying comfortably in bed, not in
obvious respiratory distress
 General condition
Stable, oriented in TPP, well hydrated and well nourished.
Pale, no jaundice, cyanosis, clubbing
No pedal edema.
There was generalized lymphadenopathy
LYMPH NODE EXAMINATION

 There was, submental, submandibular,

cervical, post auricular, occipital
supraclavicular, axillary, and inguinal
lymphadenopthy.
 No discharge
 In all regions, the overlying skin was the
same colour and same temperature as rest
of body.
 Variable sizes in all regions ranging form 0.5
x 0.5cm to 5 x 5 cm and spherical in shape.
 They were multiple, distinct, rubbery and non
tender
 The were mobile
VITALS
– Temp: 36.3
– Pulse: 112, regular, full volume, synch
– BP:
– R.R: 22
Anthropometric measurements
– HC: 53cm
– Height: 130 cm
– Muac: 17cm
– Weight: 23kg
Respiratory system

 Chest symmetrical, moving with respiration

 No scars
 Trachea central
 Chest expansion equal bilaterally
 Tactile fremitus normal and equal bilaterally
 Resonant percussion note
 Vesicular breath sounds
cvs

 All pulses palpable

 Neck veins were not engorged
 Prechordium not hyperactive
 Apex in left 4th ICS MCL
 S1 and S2 were heard. Normal. No added
sounds
p/A

 Symmetrical, flat, moving with respiration

 No scars
 Light and deep palpation non tender
 Liver was not palpable below costal margin.
Liver span was…
 Spleen was 2-3cm palpable below costal
margin
 No other palpable masses
 Percussion note was tympanic
 Normal bowel sounds were heard
GUS
 No flank tenderness
 Genitalia consistent with that of girl her age
(tanner stage 1)
CNS

 Oriented in TPP
 Memory intact
 All cranial nerves intact
 Muscle bulk, tone, power normal
 Reflexes normal
 Coordination normal and so was gait
 Sensation intact
mss

 Skin was normal colour

 No rash
 No joint pain or joint tenderness
urinalysis

 Leukocytes –ve  Blood -ve

 Nitrites –ve  Bilirubin -ve
 Urobilinogen –ve  Glucose -ve
 Protein –ve  Specific gravity 1.020
 pH 7.0
summary

 I examined a young girl, tanner 1, OTPP and

not in respiratory distress. She had frontal
bossing.
she was mildly pale but not jaundiced and
she had generalised lymphadenopathy. She
also had a splenomegaly.
differentials

 Relapse acute lymphoblastic leukamia

 Acute Lymphoma
 PGL
management

 INVESTIGATIVE
 THERAPEUTIC
investigations

1. FBC
– Hb
– WBC and DC
– platelets
2. Peripheral smear
– Presence of blast cells
– reticulocytes
3. Bone marrow aspirate
– increased number of lymphoblasts
4. Biopsy
 U & Es
 LFTs
 CxR
 LP.
 Blood, urine and stool cultures if needed
 Abdominal U/S
Investigations done

 U & Es
 LFTs
 CxR
 ABD U/S
 RVT
treatment

Supportive treatment
– Packed cell transfusion
– Infections
– Platelet transfusion
– Allopurinol for prevention of hyperuricaemia
– Diet
– Emotional support – reassurance and honest answers both to
patient and family
Definitive treatment
– Chemotherapy
– Radiation
– BM transplant
Newly diagnosed

 Phase 1- induction
– Objective is to destroy the leukamia cells and
restore haematopoiesis. The peripheral blood and
bone marrow should be free of blast cells and
clinically, lymphadenopathy and
hepatosplenomegally should disappear
– Duration is 5 weeks
 Phase 2. CNS/ Intensification
– Objective is to eradicate microfoci from CNS
because BBB renders cns inaccessible to drugs
– Duration is 5 weeks
 Phase 3. interim maintenance
– To control the disease and to prevent BM and
extamedullary relapses.
– Duration is 8 weeks
Re induction/re intensification
if the blasts are still present in the blood smear or
on the bone marrow
 Phase 4. Consolidation
– Many regimes employ this therapy with periodic
(monthly/quarterly) of vincristine and prednisolone
Relapse ALL

 PHASE 1 – INDUCTION
– Prednisolone 100mg/ms po day1-7, 15-21
– Vincristine 1.5mg/ms iv day1,8,15,21
– Methotrexate 200mg/ms iv day1
– Cytarabine 300mg/ms iv day15,16
PHASE 2 CONSOLIDATION

 COURSE R1  COURSE R2
– Prednisolone – Dexamethasone
– 6 – mercaptopurine – 6 – thioguanine
– Vincristie – Methotrexate
– Methotrexate – Cyclophosphamide
– Cytarabine – doxorubicin
– IT methotrexate
PHASE 3 - MAINTENANCE

 thioguanine 50mg/ms/d 2 years

 Methotrexate 50mg/ms/wk 2 years
 Triple IT
– Mtx 15mg
– Cyt 30mg
– Hyc 15mg every 12wks 2 years
prognosis

 ALL has better than 95% chance of having the

leukemia go into remission if treated in a specialized
centre.
 Atleast 50% chance that the disease will remain in
complete remission for at least five years
 Prognosis is affected by:
– Infection (G-ve e.coli, pseudomonas, candida, pcp)
– Bleeding (gastro-intestinal, urinary. He from vital organs e.g.
brain)
– Iatrogenic (from cytotoxics which depress BM leading to
above complications
COMPLICATIONS

 Local infiltrations
– Drugs used do not cross the blood brain barrier
easily except for prednisolone. Therefore, cns
may be infiltrated with leukemia cells despite the
bone marrow being in remission
– Suspicion should arise if there are clinical signs of
raised icp and confirmed by lumbar puncture
– Testicular and kidney infiltrations
 Hyperuricaemia and renal shut down: due to
increased breakdown of cells during the induction of
remission. Allopurinol given prophylactically till wbc
returns to normal.
 SE of drugs
– BM depression
– Hepatotoxicity
– Alopecia
– Constipation
– Cystitis
– cardiotoxicity
Thank you