HEMATOLOGY CONFERENCE

Maryann ChiombonCarmela D. Cho

Joyce ChuaAnne Cortez

Jason Co

SGF 5 y.o. female Single DOB: July 30, 2005 DOA:

CC: pallor

General data

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 mo. old • Persistence of symptoms• Sought consult at USTH• CBC was requested which

showed anemia• Prescribed with multivitamins

and folic acid• Provided no relief

CC: PALLOR

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

CC: PALLOR

• monthly PRBC transfusion• Desferoxamine infusion

500mg/vial 5x/week was started

• Serum ferritin every 6 months

July 2006-2010

Admitted for desfuroxamine infusion

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

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

Review of systems

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

24 Hour Food RecallFood CHO (g) CHON (g) FATS (g) Calories

Breakfast 1 pc longganisa

- 8 20 212

½ cup rice 46 4 - 50

Water - - - -

Lunch 1 pc Chicken - 8 1 45

1 cup rice 92 8 - 100

Water - - - -

Snacks Hamburger - 8 20 187

Dinner 2 pc longganisa

- 16 40 424

1 cup rice 92 8 - 100

Water - - - -

TOTAL 384 118 60.1 1118

RENI 189 38 56 1410

% 79%

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.

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

 

Dengue Hemorrhagic Fever Gr. 3 – Sept. 2009 at USTH PICU

Blood transfusion – 11x at USTH No injuries, allergies, drug or food

sensitivities

Past medical history

(+) Thalassemia – elder sister died at 7y/o; cousin, paternal side

(+) Stroke, HPN – father

(-) CA, asthma, thyroid d/o, autoimmune diseases

Family history

Family ProfileName Age Relation Educational

AttainmentOccupation Health

SF 48 father HS graduate Unemployed (+) stroke, HPN

MF 47 Mother Elementary undergraduate

Vendor healthy

EF 19 Brother College Student Healthy

AF 12 Brother Elementary Student Healthy

RM 7 Sister - - Deceased

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.

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,

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

Physical exam

CV: Adynamic precordium, 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

Physical 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

Neurologic exam

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

Salient Features

B- thalassemia major

DIAGNOSIS:

Course in the ward

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

crossmatched

Day 1

11/09/10Hgb 68RBC 2.36HCT 0.20MCV 84.50MCH 29.10MCHC 34.50RDW 18.70MPV 9.0Platelet 352WBC 8.80Differential CountNeutrophils 0.45 -Metamyelocytes -Bands -Segmented 0.45Lymphocytes 0.43MonocytesEosinophils 0.12BasophilsRemarks: 02 nucleated

RBC/100 WBC seen

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

Day 2

Infused 25 doses

Desferoxanine infusion

Thank you!