PATIENT CARE MEETING

CASE BASED DISCUSSION

10200070825

Dr. Arsalan Saeed

Resident Pediatric Medicine
The Indus Hospital
• 12 years old girl, resident of Larkana, admitted with c/o:

Fever 1 month
Pallor 1 month
Gum bleeding (occasional) 1 month
Petechial Rash/ bruises 15 days
HOPI
• HX of low grade fever associated with bone pain, relieved by taking
antipyretic.

• No symptoms of cough, burning micturition, diarrhea, sore throat or

active bleed, but bruises and petechial rash.

• Progressive pallor associated with dyspnea, PRBCS transfused last

month.

• Occasional gum bleed in last month.

• CBC from Larkana revealed high TLC count with low platelets, so came
Karachi.
• Past history – Not significant past medical/surgical history.

• Birth HX: Not significant.

• Immunization: Vaccinated till 5 years according to EPI.

• Family HX: Not significant, other 3 siblings are healthy

• Nutritional HX: Take home made meal. However decreased

appetite for last one month.
 On Examination
Anxious looking, conscious, Anthropometric Measurements:
oriented child, sitting on bed • Weight 38 kg (25th centile)
comfortably in prop up position. • Height 155 cm (50th centile)
• BMI 16

GPE: A+, J-,E-,Cy-,CL- VITALS

Temp 98 F
Hear rate 121 b/min
BP 112/68
Generalized lymphadenopathy
R/R 24 br/min
(Ant / Post cervical, and bilateral
inguinal significant lymph nodes SPO2 98 % (room air)
palpable) CRT = < 2 sec sec
• Chest:
• Symmetrical wall & equal expansion
• Equal air entry bilaterally.
• Abdomen:
• Soft and non-tender, liver 2 cm below right costal margin, spleen palpable 4 cm below
left costal margin, smooth rounded border.

• CVS: No hyper dynamic precordium, S1 + S2 audible. No added sounds.

• CNS: Conscious , alert but anxious, well oriented.

• COVID PCR came out to be negative.

 MANAGEMENT

- Oxygen support given through face mask @ 10L/min.

- Intravenous line maintained followed by fluid @ 3L/m2.
- Baseline workup sent including CBC, flow cytometry, TLS
markers and RFT/LFT profile.
- CXR revealed mediastinal widening, so kept in sitting position.
- Intravenous antibiotic give, Allupurinol and calcium acetate
started considering TLS.
- Admitted in PICU for GCS, TLS, respiratory monitoring and
strict I/O & vital charting.
- Once out of TLS phase, chemotherapy started as per protocol
according to flow cytometry and markers.
HB: 9.4 (gm/dl) Na 140 RBS 76 mg/dl
PCV: 29.5 K 3.0 UA 7.6
TLC: 75.2 (N=4,L=6, Blast 90) Cl 104 Phos 3.6
PLT: 15 Bicarb 22 Cal 8.0
U 24
MP, Hep B & C – Neg Cr 0.46

PT 11.8 TB 0.70 U/S Abdomen: Spleen enlarged, rest

APTT 24.4 DB 0.20 normal.
INR 1.12 Alk P 176
SGPT 11 Flow cytometry: T-Lymphoblastic Leukemia
GGT 42
ECHO: Normal LBF, EF 60%, Trace pericardial
effusion.
 FINAL DIAGNOSIS

T-CELL ACUTE LYMPHOBLASTIC LEUKEMIA

DISCUSSION
Acute Leukemia
• Leukemia – most common malignancy in childhood (up to 9 years).

• ALL (Acute lymphoblastic leukemia) – Most common type (75% of all cases).

• AML (Acute Myelogenous leukemia) – neonatal/congenital, 11% of all childhood leukemia.

• ALL derived from progenitor B-cells (85%), T-cell (nearly 15%), B-cells (1%)

• Presence of hyperploidy is favorable, while Philadelphia chromosome t (9; 22); t (4;11) worse
prognosis.

• Most children with ALL (>80%) survive after 5 years.

What are the symptoms?
• Fever
• Anemia
• Bleeding
• Bone tenderness
• Lymphadenopathy
• Hepatospleenomegaly
• Headache
• Mass (Abdominal/Mediastinal)
Treatment
• Risk directed Therapy is the current standard of care.

• According to National cancer Centre (NCI), there are two group:

• Standard Risk – Age 1-10 years, WBC < 50,000/uL

• High Risk – Age <1 or > 10 year, WBC > 50,000/uL

• Standard treatment involves chemotherapy for 2-3 years.

• Patient in clinical remission can have MRD (indicative of burden of leukemic cells).
• MRD of >0.01% on marrow on day 29 of induction is a significant risk factor.
Initial Therapy
• Remission induction – Given for 4 weeks. Consist on vincristine
(weekly), corticosteroid (dexamethasone/prednisone),Pegylated
asparginase preparation (single dose), Daunomycycin for high risk
(weekly).

• 95% achieve remission (<5% blast in marrow, near normal cell line in
4-5 weeks).

• Intrathecal therapy given at the start, and also once during induction.
Second Phase
• Consolidation – Intense CNS therapy alone with continued systemic therapy (to prevent CNS
relapse later - <5%).

• Intrathecal chemotherapy is given repeatedly by LP.

• 14-28 weeks therapy with various regimen and schedule based on risk group – Intensification
period.

• Its phases include delayed intensification (aggressive treatment) & interim maintenance
(nontoxic phases of treatment).

• Drugs include Cytarabine, Methotrexate, Asparginase & Vincristine (for residual disease).
Maintenance Phase
• It last 2-3 years, depending on the protocol used.

• Mercaptopurine (daily), oral methotrexate (weekly), with intermittent

dose of corticosteroid and vincristine.

• Side effects by chemotherapy include ulcers in mucosa, intestine, liver

damage, myelosuppression, pancreatitis, cardiomyopathy,
constipation, seizures and long list.
Complication & Management
• Tumor Lysis Syndrome – (in Acute Leukemia, NH Lymphoma)
• Hyperuricemia (gout/uric acid nephropathy), treated with allopurinol, hydration, diuresis,
alkaline urine.

• Hyperkalemia (arrhythmia/cardiac arrest), treated with Kayexalate, sodium bicarbonate,

glucose & insulin.

• Hyperphosphatemia (hypocalcemic tetany, pruritis, photophobia, metastatic

calcification), treated with hydration, forced diuresis, oral alluminium hydroxide, and stop
alkalization.

• Hypocalcemia – if symptomatic (carpopedal spasm, seizures), then give treatment.

• Increased ICP – confusion, headache, vomiting, seizures, CN III & VI palsy
 CT head or MRI  Treatment Phenytoin, corticosteroid, chemotherapy.

• Hyponatremia (asymptomatic/lethargy, seizures)  Decreased serum Na+

and Cl- level  Replace sodium (true hyponatremia) or fluid restriction
(increased ADH).

• Superior Vena Cava Syndrome - edema of head & neck, distended neck
veins, proptosis; Horner’s syndrome  CT/MRI chest  Treatment
chemotherapy/radiation.
Tumor Lysis Syndrome
• Article published in January 2020

• Tumor lysis syndrome (TLS) - Most common life-threatening emergency in childhood

malignancies.

• Severe electrolytes derangements include hyperuricemia, hyperkalemia,

hyperphosphatemia, and hypocalcemia.

• It increase the risk of acute kidney injury, cardiac arrhythmias, seizures, and even death.

• TLS most commonly occurs in rapidly dividing hematologic malignancies.

• TLS management centers  a high index of suspicion  identify higher
risk for TLS Aggressive prophylactic strategy to prevent TLS.

• Patient with new malignancy  Up-front measurement of serum uric acid,

lactate dehydrogenase, potassium, phosphorus, creatinine and calcium
levels.

• Consideration of aggressive volume repletion and/or expansion to

optimized renal function. 
• Patient at intermediate or high risk for TLS  intravenous hydration to optimize kidney
function.

• Achieved with hyperhydration (without K+ )  urine output of 3-5 mL/kg/hr.

• Close monitoring of laboratory TLS is also a key strategy.

• Prophylaxis (with allopurinol) and treatment of hyperuricemia  normal serum levels of

uric acid.

• If signs or symptoms of TLS  prompt multidisciplinary approach Treat any TLS-

induced metabolic derangements and maximize outcomes.