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HEMATOLOGY CONFERENCE

HEMATOLOGY CONFERENCE. Maryann Chiombon Carmela D. Cho Joyce Chua Anne Cortez Jason Co. General data. SGF 5 y.o . female Single DOB: July 30, 2005 DOA: CC: pallor. CC: PALLOR. 3 mo. old. Mother noticed the patient to be pale N o other accompanying symptoms

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HEMATOLOGY CONFERENCE

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  1. HEMATOLOGY CONFERENCE Maryann Chiombon Carmela D. Cho Joyce Chua Anne Cortez Jason Co

  2. General data • SGF • 5 y.o. female • Single • DOB: July 30, 2005 • DOA: • CC: pallor

  3. CC: PALLOR 3 mo. old • Mother noticed the patient to be pale • No other accompanying symptoms • Consulted at USTH • Prescribed with multivitamins which she took for 1 month • No relief

  4. CC: PALLOR 4 mo. old • Persistence of symptoms • Sought consult at USTH • CBC was requested which showed anemia • Prescribed with multivitaminsand folic acid • Provided no relief

  5. CC: PALLOR 1 yr 1 mo. old • Persistence of symptoms • Sought consult at USTH • CBC was requested which showed anemia • Admitted • Hb gel electrophoresis • Diagnosed with B- thalassemia Major • Transfused with 1 PRBC • Provided relief of symptoms

  6. CC: PALLOR July 2006-2010 • monthly PRBC transfusion • Desferoxamine infusion 500mg/vial 5x/week was started • Serum ferritin every 6 months Admitted for desfuroxamine infusion

  7. Review of systems • General: (-) weight loss, (-) weight gain • Skin: (-) pruritus • HEENT: (-) visual changes, (-) dizziness, (-) hearing loss, (-) nasal discharge, (-) aural discharge • Pulmonary: (-) dyspnea, (-) shortness of breath • Cardiovascular: (-) chest pain, (-) palpitations, (-) cyanosis, (-) easy fatigability • Gastrointestinal: (-) nausea, (-) hematemesis, (-) melena, (-) dysphagia, (-) diarrhea, (-) constipation

  8. Review of systems • Genito-urinary: (-) frequency, (-)urgency, (-) dysuria, (-) hematuria, (-) nocturia • Musculoskelatal: (-) joint stiffness, (-) pain, (-) swelling • Endocrine: (-) heat or cold intolerance, (-) polyuria, (-) polydipsia • Hematopoietic: (-) abnormal bleeding, (-) easy bruising • Neurologic: (-) headache, (-) seizures, (-) speech disturbances • Psychiatric: (-) behavioral changes

  9. Feeding History • Exclusively breastfed until 10 months • 8x/day, 30minutes/feeding, every 2-3hours • Milk formula: Enfalac started at 10 months • 4:8 dilution, 8oz/feeding, 3-4x/day • Complementary feeding: 9 months • Rice gruel, rice with soup, chocolate drink • Patient eats 3 times in a day with occasional snacks • Multivitamins

  10. 24 Hour Food Recall

  11. Developmental & Birth History • Patient was born via NSD to 47 years old vendor, G5P5 (5-0-0-4), married to 48 year old father who is unemployed. • Had regular prenatal check up? • The mother is non-smoker and non-alcoholic beverage drinker prior to this pregnancy. • (-) exposure to viral exanthem diseases and radiation, (-) illicit, prohibited or abortifacient drugs. • The mother was advised multivitamins, ferrous sulfate and Folic acid however the mother did not comply.

  12. Immunization History • BCG; 3Hep B ; 3OPV; 3DPT • All done at a local health center

  13. Past medical history • Dengue Hemorrhagic Fever Gr. 3 – Sept. 2009 at USTH PICU • Blood transfusion – 11x at USTH • No injuries, allergies, drug or food sensitivities

  14. Family history • (+) Thalassemia – elder sister died at 7y/o; cousin, paternal side • (+) Stroke, HPN – father • (-) CA, asthma, thyroid d/o, autoimmune diseases

  15. Family Profile

  16. Socioeconomic/Environmental History • Patient lives with her family in a poorly ventilated and well-lit bungalow type house made of “yero” • Drinking water is tap water, not boiled • Garbage is collected 2x a week, not segregated • The family does not live near a factory • Has 1 pet dog • Patient is not exposed to cigarette smoke.

  17. Physical exam • VS: BP – 90/60, PR – 89bpm, regular, RR – 21, T: 36.7oC • General survey: awake, alert, not in cardiorespiratory distress, well-nourished, well-hydrated, ambulatory • Warm, dry skin, no active dermatoses, (+)jaundice • Symmetricall head, uniformly distributed black hair, (+) frontal bossing • Pale palpebral conjunctivae, anicteric sclera, isocoric pupils,

  18. Physical exam • No tragal tenderness, non-hyperemic EAC, intact TM, no aural discharge • Nasal septum midline, no nasal discharge • Moist oral mucosa, uvula midline, grade 1 tonsillar enlargement, non-hyperemic posterior pharyngeal wall, (+) mandibular prominence • Supple neck, no palpable cervical LN, thyroid not enlarged • Thorax: no chest wall deformities, symmetrical chest expansion, (-) intercostal retractions, (-) wheezes, crackles

  19. Physical exam • CV: Adynamicprecordium, AB 4th LICS MCL, normal S1, S2, no murmurs • Abdomen: Flat abdomen, NABS, tympanitic, soft, nontender, no palpable mass • Liver and splenic tip palpable at 2cm below the left and right subcostal area, respectively • Grossly female • Musculoskeletal: no tenderness, swelling, or limitation of motion • Pulses full and equal, no cyanosis, no edema

  20. Neurologic exam • Conscious, coherent, oriented to 3 spheres • Cranial nerves intact. • Can do FTNT and APST • No muscle atrophy, no fasciculations, no abnormal movements, MMT 5/5 on all extremities • Sensory: No sensory deficit • Reflexes: • Superficial: None • Deep Tendon: +2 on all extremities • No nuchal rigidity, no Kernig’s, no Brudzinski

  21. Salient Features • 5y/o • Female • Known case of B thalassemia major since 1y 1mo old • (+) jaundice • (+) frontal bossing • Pale palpebral conjunctivae • (+) mandibular prominence • Liver and splenic tip palpable at 2cm below the left and right subcostal area, respectively

  22. B- thalassemia major DIAGNOSIS:

  23. Course in the ward

  24. Day 1 • CBC w/ platelet requested, smear saved • Desferoxanine infusion • Vitamin C 5mL OD • Reserve 1 U PRBC properly typed and crossmatched

  25. Day 2 • 1 U PRBC was transfused (15mL/kg) over 4 hours • Vital signs was monitored • Pre BT meds: • Diphenhydramine 2.5mg/5mL (5mkday) 8mL PO q6 • Paracetamol 250mg/5mL (12mkd) 4mL PO q6 • (+)febrile episodes tmax 39C • Paracetamol 250mg/5mL (12.2mkd) 4mL PO now then q4 for fever

  26. Desferoxanine infusion • Infused 25 doses

  27. Thank you! 

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