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Acute Lymphoblastic Leukemia

  1. By: Dr. Shah Abdar Khan 2nd Year PGR pediatrics LRH Date: 28th March 2023
  2. • 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
  3. 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….
  4. 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.
  5. • 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..
  6. • 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.
  7. • 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. •
  8. • 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.
  9. 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.
  10. Developmental hx normal Socioeconomic hx The child belonged to a poor family, was the son of a labor.
  11. • 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.
  12. • 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.
  13. • CBC • Blood c/s • peripheral smear • Bone Marrow Biopsy
  14. • TLC = 33600 • Hb= 3.3 g/dl • MCV=83 • MCH=28 • Platelets= 8000
  15. • 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.
  16. • The bone marrow report showed 34% of blast cells with suppressed erythropoiesis and mrgakaryopoiesis .
  17. • 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.
  18. • 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
  19. • 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.
  20. • 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.
  21. • Pallor petechial skin lesions • Lymphadenopathy • Splenomegaly • Hepatomegaly less often • Signs of increased IC and CNS nerves palsies in case of CNS dissemination
  22. • 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.
  23. • Baseline investigations • Peripheral smear • Bone marrow biopsy • Flow cytometry • Immunohistochemistry
  24. • 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
  25. • 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
  26. 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
  27. • 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.
  28. • 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.
  29. • 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.
  30. • 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.
  31. • 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
  32. • 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.
  33. THANK YOU!!!