This is a case presentation at g level( FCPS)
It is all about the diagnosis of ALL. practical first hand experienced case at Lady Reading Hospital Peshawar.
By: Dr. Shah Abdar Khan
2nd Year PGR pediatrics LRH
Date: 28th March 2023
• 9 Years old school going child named XYZ from Swabi
presented to our OD with the complaint of ;
• Fatigability 45 days
• Fever 45 days
• Pain in both ankles 30days
• 4 episodes of epistaxis in last 5 days
o According to the mother of the child, this otherwise
healthy child experienced tiredness while doing minimal
routine activity like going to school, carrying his bag, and
while climbing on stairs which he previously used to do
with any comlaint.
• Cont….
In addition to this she told that
the boy has fever since last 45
days which is non documented,
episodic and nocturnal in
nature, relieves with
paracetamol, and is not
associated with rigors, chills,
sweating or weight lose.
• The mother further added to the history that along with
above mentioned complaints, the child experiences pain
in both lower limbs, are tender to touch, were not swelled
in the beginning but swelled with the passage of time and
has disturbed his sleep on several occasions due to
increase in its severity at night..
• There was no history of trauma, lumping, migration of
pain to other joints, hematuria, hotness of joints, rash ,
alopecia , photophobia , abdominal distention or any
changes in nails color.
• The child recently had 4 episodes of nasal bleeding in
previous 5 days that stopped by applying pressure at
home. No history of blood in urine, stools, vomits and any
heavy bleed with minor traumas or circumcision.
•
• Upon inquiry the mother told that there are several bluish
patches on his body more on his legs and back. She also
confirmed that his color became paler and paler over the
previous month although change in the color of his eyes,
stools and urine has not ben noted.
Systemic review Unremarkable
Vaccination was up to date
No significant past medical or surgical hx was resent
Consanguinity negative
No worth mentioning issue in antenatal, post natal or natal
hx.
• Examination
• Upon examination the child had fever of 102 F , was ale
looking, with pulse rate of 80/minute and BP of 110 mm
Hg , has anterior cervical and inguinal lymphadenopathy.
The nodes were about v.5cmm, hard and mobile.
Petichea were present on both shins, buttocks and back.
Rest of the examination was unremarkable.
• On lower limb examination both the shins just superior to
ankle joints were noted to be mildly swelled with no
redness or increase warmth and intact range of motion.
No abnormality in the gait of the child was noted.
• On peripheral smear lymphocytosis with few predominant
blast cells having increased Nuclear to cytoplasmic ratio
were noted along with normochromic/ normocytic red
blood cells and decreased number of platelets.
• The bone marrow report showed 34% of blast cells with
suppressed erythropoiesis and mrgakaryopoiesis .
• For further investigations to know the sub type and do the
treatment accordingly the attendants were advised to
consult pediatric oncology at Lahore Children Hospital as
they had no affordability to bear the cost here by
themselves.
• ALL is the most common malignancy of childhood
accounting for % of all the cancers in this age group.
• It a
• I a heterogeneous group of malignancies result from
distinctive genetical abnormalities with varying clinical
manifestation.
• Fortunately it is the first disseminated cancer shown to be
curable
• All most commonly effect the pediatric population with
peak at the age of 2 to 3 years with male predominance
• 85 % of ALL are B ALL , 15% T ALL and 1% are derived
from mature B cells( Burkett lymphoma
• Th disease has close association with certain
chromosomal abnormalities , such as Bloom syndrome,
Down's syndrome, Ataxia telangiectasia and fanconi
anemia.
• The risk of ALL in identical twin to develop ALL is at least
twice as that of other population.
• Symptoms
• Anorexia malaise irritability
• Intermittent , non specific , low grade fever
• Bones and often joints pain and joints swelling
• Pallor, fatigue, bruising , oral mucosa bleeding and
epistaxis.
• Pallor
petechial skin lesions
• Lymphadenopathy
• Splenomegaly
• Hepatomegaly less often
• Signs of increased IC and CNS nerves palsies in case of
CNS dissemination
• The differentials of ALL differs on the basis of signs and
symptoms the patient present with.
• If pancytopenia is the only presentation then the main
differentials are aplastic anemia, myelofibrosis, familial
HLH.
• The differentials of fever range from various bacterial and
viral infections to autoimmune and connective tissue
diseases.
• If there is joints involvement then JIA, SLE, psoriatic
arthritis and septic arthritis must be excluded.
• The treatment options varies in correspondence to
several factors like age of the patient, age of diagnosis,
type of ALL, WBC count at diagnosis and response of
patient to initial chemo.
• As a general role the treatment has following stages
• Remission induction
• Consolidation
• Interim Maintenance phase
• Delayed Intensification phase
• Maintenance
• Treatment of relapse
• In this phase chemo is given foe 4 weeks aimed to
achieve remission , which is defined as less than 5%
blast cells in marrow.
• Ideally on 29th day bone marrow test is performed which
is called Minimal Residual Disease (MRD ) test to know
the effectiveness of treatment and progression of disease
Various chemotheraputic options are availed but the most
common for ALL is
Inj vincristine weekly
A corticosteroids
Single dose of pegylated asparaginase
High risk patients also receives daunomycin at weekly
intervals
• After eradication of blasts from the patients blood and
marrow the patient take rest from chemo for a week and
the another phase of chemo starts called consolidation.
• This phase lasts for 4 to 8 weeks
• In this phase the patient receives intravenous as well as
intrathecal chemo which aims to prevent CNS relapse.
• Methotrexate, cytarabine, vincristine,cyclohoshamide and
mercatapurins are the prominent drugs used in this hase.
• These are the relatively non toxic phases of
chemotherapy , each lasting for 8 weeks and aim to
eradicate residual malignant cells if any.
• The same drugs as mentioned in previous slides are the
options to be used in these phases.
• The final phase in treatment of ALL is the maintenance
hae , the failure of completing this phase is the most
common cause of treatment's failure.
• This duration of this phase is 2 years for girls and 3 years
for boys.
• In this phase the patient take most of his her medications
at home and comes to hospital for iv vincristine and
course of steroids every 4 weekly, cbc monthly an
intrathecal chemo once in 3 months.
• Relapse occurs in bone marrow in 15 % cases, in CNS in
5% cases and in testis in about 25 cases.
• It predicts poorer prognosis and then needs more
aggressive course of chemo.
• The prognosis of ALL varies from patient to patient
• It depends upon several factors like
• Age and gender of the patient
• Time of diagnosis
• Chromosomal makeup of the patient
• CNS involvement at the time of diagnosis
• WBC count at the time of diagnosis
• Male ender, age less than 1 year or greater than 1 years,
initial WBC count of greater than 50,000, CNS
involvement at the time of diagnosis, philadelphia
chromosomes and failure to induce remission are the
common factors that indicates poorer prognostic factors.