University of Santo Tomas Hospital Department of Pediatrics

1. University of Santo Tomas HospitalDepartment of Pediatrics Grand Rounds Ang.Ang.Aningalan. Antonio.Aramburo.

2. General Data • JPF • 6 year old, male • Birthdate: September 9, 2004 • Religion: Roman Catholic • Address: Nueva Ecija • Informant: Mother • Reliability: Good

3. CHIEF COMPLAINT: PALLOR

4. History of Present Illness:

5. History of Present Illness:

6. History of Present Illness:

7. History of Present Illness: Admission

8. Review of System: • Cutaneous: (-) rash, pigmentation, hair loss • HEENT: (-) lacrimation, (-) hearing loss, (-) aural discharge, (-) nasal discharge, (-) epistaxis, (-) toothache, (-) salivation, (-) sore throat • Respiratory: (-) dyspnea • Cardiovascular: (-) orthopnea, (-) cyanosis, • Gastrointestinal: (-) constipation, (-) jaundice, (-) pica

9. Review of System: • Genitourinary: (-) polyuria, (-) hematuria • Musculoskeletal: (-) bone pain, (-) limitation of movement • Nervous/Behavior: (-) tremors, (-) convulsions (-) mood/behavioral change • Endocrine: (-) breast asymmetry, (-) pain or discharge (-) heat/cold intolerance

10. 24 hour food recall

11. Developmental history • average prep student before he started getting absent from school due to easy fatigability • can read and write, draw a person with hands and clothes, knows morning and afternoon, and knows right and left sides • At par with age

12. Past Illnesses • No previous hospitalizations • No past surgeries • No food or drug sensitivities

13. Immunization History • Unrecalled

14. Family History • (-) HPN, DM, PTB, cancer, hematologic disorders

15. Family profile

16. Socioeconomic and Environmental History • lives in Nueva Ecija with his parents and siblings in a one-storey house made of wood and concrete, well-lit, well-ventilated • drink tap water and water supply at home is from NAWASA. • Garbage is collected every day, no segregation done • do not own any pets • no factories nearby • exposed to second hand cigarette smoke from his father

17. Physical Examination • General Survey • Conscious, coherent, ambulatory, not in cardiorespiratory distress, well-nourished and well-hydrated

18. Physical Examination • BP : 90/50 mmHg • HR : 120 bpm • RR : 24 cpm • Temp :36.9C • Wt : 17kg (WFA : z score below -1, normal) • Lt: 112 cm (LFA : z score below 0 normal) • BMI 13.6 kg/m2 (BFA: z score -1, normal)

19. Physical Examination • Skin • Warm, moist skin, no active dermatoses, good skin turgor, (+) pallor, no jaundice • HEENT • no unusual facies, no facial asymmetry • pale palpebral conjunctivae, anicteric sclerae, no tears, pupils 3-4 mm ERTL

20. Physical Examination • HEENT • no tragal tenderness, nonhyperemic external auditory canal, intact tympanic membrane, no discharge • nasal septum midline, no nasal discharge, turbinates nonhyperemic and not congested • moist buccal mucosa, no dental caries, non-hyperemic posterior pharyngeal wall, tonsils not enlarged

21. Physical Examination • Neck • Supple neck, no limitation of movement, (+) palpable cervical lymph nodes,< 1 cm firm, rubbery, nonmatted ,non tender • Lungs • symmetrical chest expansion, no retractions, equal vocal and tactile fremiti, resonant on percussion, clear breath sounds

22. Physical Examination • Heart • adynamic precordium, apex beat at 4th LICS MCL, no heaves/lifts, no thrills, normal rhythm, S1 louder than S2 at apex, S2 louder than S1 at the base, no murmurs • Abdomen • soft and flat, normoactive bowel sounds, tympanitic, no tenderness, no palpable masses, liver span 8 cm, spleen was palpable at 3cm below the subcostal margin

23. Physical Examination • Genitourinary • genitalia grossly male, no discharge • (+) inguinal lymphadenopathies • Extremities • pulses full and equal on all extremities, no edema, no cyanosis, no clubbing, no joint swelling or tenderness, no limitation of motion, capillary refill <2 seconds • Pale nail beds • no skin dimpling, no tufts of hair

24. Neurologic Examination • Mental Status: Conscious, coherent, oriented to time, place and person, follows commands • Cranial nerves: intact • Motor: no atrophy, no fasciculations, no spasticity or rigidity, MMT 5/5 on all extremities • Cerebellar: can do APST and FTNT with ease • Sensory: no sensory deficit • Reflexes: DTRs ++ on all extremities, (-) Babinski • Meningeal signs: (-) nuchal rigidty, (-) Brudzinski, (-) Kernig’s

25. Salient Features: Subjective • 6 year old male • Pallor • Intermittent low grade fever • easy fatigability • Decreased level of activity • loss of appetite • Weight loss • Chest X-ray: Primary Tuberculosis Infection, on treatment

26. Salient Features: Objective • (+) pallor • Persistent anemia • pale palpebral conjunctivae, nail beds • Palpable CLN, inguinal lymphadenopathies • hepatosplenomegaly

27. Approach To Diagnosis

31. Course in the Ward 1st Hospital day Request for: CBC with platelet Reticulocyte count Peripheral blood smear

32. Peripheral Smear WBC: presence of immature cells (blast cells) RBC: Anisochromia with Anisocytosis and Poikilocystosis Platelet decrease ANC=561

33. 2nd Hospital day Patient was scheduled for BMA Transfusion of 1 satellite bag properly typed and cross matched over 4 hours Started on IVF D5 0.3% NaCl 14-15 gtts/min

34. 3rd Hospital day Transfused with 1 satellite bag PRBC properly typed and cross matched over 4 hours after Bone marrow aspiration

35. BMA was done. Obtained 2 ml bone marrow aspirate. Specimen was sent for flow cytometry

36. 4th Hospital day Request for: CBC with platelet Total bilirubin BUN, Creatinine Na, K Total Calcium, iPO4, BUA Alkaline phosphatase, LDH CXR (PA, Lat)

37. ANC=770

38. CXR: There is haziness over the right lung bases and the retrocardiac region Confluent densities are seen over the paratracheal and peribronchial regions Impression: Above findings may be suggestive of Primary Koch’s Infection

39. 2D echo was requested as baseline studies prior to Doxorubucin Initial reading: Ejection fraction 69; mild pericardial effusion

40. 5th Hospital day Patient was started Prednisone 10 mg/5 ml, 10 ml BID on full stomach Patient was scheduled for intrathecal chemotherapy (Methotrexate 25 mg/ml) Started on NaHCO3 325 mg/tab, 1 tab now then TID; Allopurinol 100 mg/tab, 1 tab BID For 5 days only 6th Hospital day Transfuse with 2 ‘u’ type specific Platelet concentrate

41. Intrathecal chemotherapy was done 2 ml CSF fluid was obtained Cell cytology CSF differential count

42. ANEMIA Reduction below normal in the concentration of hemoglobin or RBCs in the blood

43. Changes in Normal Hemoglobin/Hematocrit Values with Age and Pregnancy • Age/Sex Hemoglobin g/dl Hematocrit % At birth 17 52 Childhood 12 36 Adolescence 13 40 Adult man 16(+2) 47(+6) Adult woman 13(+2) 40(+6) (menstruating) Adult woman 14(+2) 42(+6) (postmenopausal) During pregnancy 12(+2) 37(+6)

44. Anemia is not a diagnosis in itself, but merely an objective sign of disease.First step in its diagnosis is detection of its presence.

45. 3 FUNCTIONAL CATEGORIES OF THE ANEMIAS • Disorders of Proliferation • Disorders in Erythrocyte Maturation • Disorders due Primarily to Erythrocyte Destruction or Red Cell Loss

47. PROBLEM: ANEMIA Subjective Data Objective Data

48. Introduction Acute Lymphocytic Leukemia -The leukemias are the most common malignant neoplasms in childhood: 41% of all malignancies that occur in children [<15 yrs]. -Acute lymphoblastic leukemia (ALL) 77%. -Acute myelogenous leukemia (AML) 11%. -Chronic myelogenous leukemia (CML) for 2–3%. -Juvenile chronic myelogenous leukemia (JCML) for 1–2%.

49. Introduction Acute Lymphocytic Leukemia Leukemias may be defined as: -a group of malignant diseases in which genetic abnormalities in a hematopoietic cell give rise to a clonal proliferation of cells.  -Increased rate of proliferation, a decreased rate of spontaneous apoptosis, or both.  -Disruption of normal marrow function and, ultimately, marrow failure.

50. Acute Lymphoblastic Leukemia Acute Lymphocytic Leukemia Epidemiology: -It has a striking peak incidence between 2–6 yr of age. -Occurs slightly more frequently in boys than in girls. -More common in children with certain chromosomal abnormalities such as Down syndrome, Bloom syndrome, ataxia-telangiectasia, and Fanconi syndrome.