CRISBERT I. CUALTEROS, M.D. - mastocytosis case presentation
   
DR. CRISBERT I. CUALTEROS
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CRISBERT I. CUALTEROS, M.D. (+63919)8586556
Content of the new pag

SACRED HEART

HOSPITAL

DEPARTMENT OF PEDIATRICS

Mortality and Morbidity Conference

May 2008

GENERAL DATA:

A case of A. D., 9 mos. old, female child,

Filipino, Roman Catholic, from Lahug, Cebu

City. Admitted for the first time at SHH on

May 13, 2008 at 7:00 pm due to skin lesions.

PRENATAL HISTORY:

• Mother was 29 y.o.

• G

1Po.

• PNC at 2 mos. AOG at a private MD, with regular

visits

• Multivitamins was taken with good compliance.

• Non-smoker and non-alcoholic beverage drinker.

• Epigastric pain at 6 mos. AOG, no consult done, no

meds taken.

INTRANATAL HISTORY:

• Fullterm, via NSVD

• assisted by a doctor, good cry

• BW: 3.6 kgs.

• BR:1/1

• No complications

FEEDING AND NUTRITIONAL

HISTORY

:

• Exclusively breastfed up to 1 month old

• shifted to Mylac w/ 1:2 dilution until present

• semi-solid food at 8 mos

• solid food at 9 mos. old.

IMMUNIZATION:

• BCG- 1 dose

• Hep. B

• OPV - 3 doses

• DPT

• Measles

3 doses3 doses1 dose

DEVELOPMENTAL HISTORY:

• Social smile

• Roll over

• Sit w/ support

• Sit w/o support

2 mos.4 mos.7 mos.8 mos.

PAST MEDICAL HISTORY/ FAMILY

HISTORY:

• No previous hospitalization or any serious

physical injury, surgical procedure done.

• ( + ) HPN and DM- both

• ( - ) Allergies to food and drugs

• ( +) dermatographism- mother side

PERSONAL & SOCIAL HISTORY:

• Patient is a 9 mo child.

• stays with the parents.

• Playful and alert child.

HISTORY OF PRESENT ILLNESS:

• 5 mos. PTA, bullae on the forehead, pruritic

• asso w/ mild grade fever

• Sought consult to private physician.

• Fluocinolone Acetonide

prescribed with no relief noted.

• The condition was tolerated.

• The bullae usually lasted for 3 days

• Then bursting and development of a

yellowish-brown crust until complete healing

was noted.

• 4 mos. PTA, condition persisted and spread to

the scalp, face, trunk, and extremities.

• Sought consult again to the same private

physician, Fluocinolone Acetonide (Synalar

Cream) was discontinued

• Changed

cream) but still with no relief was noted.

• dermatologist, unrecalled cream was given,

still w/ no relief.

• Skin biopsy and Giemsa stain: revealed

mastocytosis.

(Synalar cream) wasPhysiological Lipid (Physiogel

PHYSICAL EXAMINATION

General Survey: alert, febrile, irritable, not in

respiratory distress

Vital Signs:

HR: 92 bpm RR: 55 cpm

Temp. 38.6oC

Wt. 8.5 kgs Ht. 69 cm. HC: 45 cm

CC: 47 cm AG: 52 cm

Waterlow Classification:

X100

• =8.5 kgs/9 X 100

• =94.4 % (normal >90%)

• =69/70 x 100

• =98.5 % (normal >95%)

Wasting= ABW/IBW for actual lengthStunting=actual ht/ideal ht for age

• SKIN: (+) bullae on right frontal area,

preauricular R&L, submandibular area L,

deltoid area R&L, upper thorax, upper

abdomen, and upper back. No jaundice, no

pallor, warm, good turgor

• HEENT: normocephalic, pinkish palpebral

conjunctiva, anicteric sclerae, pupils equally

reactive to light, intact TM, no nasal

congestion, no alar flaring, no TPC, no LAD

• CHEST & LUNGS: equal chest expansion, no

retractions, no rales, no wheeze

• CVS: no bulging of precordium, distinct heart

sound, normal rate & regular rhythm, no

murmur

• ABDOMEN: globular, NABS, soft, no organ

enlargement

• GENITALIA: grossly female, no vaginal

discharge, no lesions

• EXTREMTIES: no deformities, no edema,

strong peripheral pulses both upper & lower,

CRT < 2 sec.

NEUROLOGIC EXAM:

• Mental Status: alert

• Motor: normal

• Tone: normal

• CN I: NA

• CN II: pupils equally reactive to light

• CN III, IV, VI: (+) EOM

• CN V: NA

• CN VII: no facial asymmetry

• CN VIII: blink to loud sound

• CN IX, X: (+) gag reflex

(+) swallowing

• CN XI: can turn head side to side

• CN XII: tongue not deviated

• Reflex:

(+) babinsky

• Sensory: intact sensation to touch and pain

stimuli

• Cerebellar:

IMPRESSION:

• Cutaneous Mastocytosis

COURSE IN THE WARD

On admission:

D5 0.3% NaCl (MR+12%)

• Paracetamol 100mg/ml, 1.2 mL q4hrs prn

• Hydroxyzine

PO(AD:1.08mkD)

2mg/ml susp, 1.5ml TID

• Oxacillin

ANST (AD:96.3 mkD)

• Referrred to hema-onco

(Wydox) 200mg, IV drip q 6 hours,

Labs Requested:

• CBC:

lymphocytosis and monocytosis

• U/A :

pyuria

Blood culture and sensi: No growth

LABS

lymph 42.6

CBC On admission

plt 367

hct 41.4

hgb 14.4

mono 10.5

seg 44.4

wbc 12

URINALYSIS

Transparency yellow

Spec. Gravity 1.010

Albumin (-)

Glucose (-)

Ketone/Blood (-)

WBC 3 - 6

RBC 0 - 3

1

st Hospital Day:

• Afebrile, still with blisters on forehead, temporal

area, chest and back

• With good appetite, playful, alert

• HR:120’s RR:30’s temp:36.5 – 37.1C

• Skin: blisters on her forehead, temporal area,

with multiple brownish crusted lesions on chest

and back

• Oxacillin was increased to 250 mg IV drip q6hrs

(AD:120.4 mkD)

• Hydroxyzine

 

2

nd Hospital Day:

• Afebrile, new bullous formation seen at the back

• HR: 120 RR:30 TEMP: 36.5

• SKIN:

submandibular area L, erupted bullae deltoid area

R&L, upper thorax, upper abdomen and upper back

bullae on frontal area R, preauricular R&L,

• Mastocytosis

• Hydroxyzine

 

Oxacillin ( going day 2)

3

rd Hospital Day:

• Patient was scheduled for BMA

• Brownish crust noted on errupted bullae

• New bullae was also noted

• HR: 118 RR: 30 Temp: 36

• D5 IMB (MR)

• Hydroxyzine

• Ketotifen

Drops, 0.4 ml BID was added

 

Oxacillin ( going day 3)

4

th Hospital Day:

• Afeb, still with new bullae formation noted

• SKIN: Brownish crust was also noted

• HR: 121 RR: 29 Temp: 36.5

• Mupirocin ointment started

• D5 IMB (MR)

• Hydroxyzine

• Ketotifen Drops, 0.4 ml BID

 

Oxacillin ( going day 4)

5

th Hospital Day:

• afebrile

• HR: 124 RR: 28 Temp: 36.8

• Skin: decrease in the appearance of new bullae was

noted

• D5 IMB (MR)

• Hydroxyzine

• Ketotifen Drops, 0.4 ml BID was added

Prednisone 10 mg, 2.5 ml BID, also started

Oxacillin ( going day 5)

• CBC: thrombocytosis and lymphocytosis

• BMA:

and increased thrombopoiesis

dysplastic changes in granulocytic and megakaryocytic cells

BMA Result:

• Cellular bone marrow, w/ 20% increased fat cells

• There is erythroid hyperplasia

• Toxic changes are seen about 40% of cells

• Increased number of mast cells which appear as

round to oval cells about the same size as a

segmented neutrophil; they have round to

spindle-shaped nucleus surrounded by

cytoplasm with blue and black granules

6

th Hospital Day:

• 1

• HR: 129 RR: 36 Temp: 37.3

• Skin: No bleeding on the BMA site

st day post BMA

– NO new blister formation seen on any part of the body

of patient

– Still w/ brownish crust on the errupted bullae

• D5 IMB (MR)

• Hydroxyzine

• Ketotifen Drops, 0.4 ml BID was added

Oxacillin ( going day 6)

• Prednisone 10 mg, 2.5 ml BID

7

th Hospital Day:

• HR: 121 RR: 33 36.4

• Skin: NO blister formation. Healing of the blister

formation was noted

• D5 IMB (MR)

• Hydroxyzine

• Ketotifen

Oxacillin ( going day 7)

• Prednisone

• Peripheral Blood Smear: thrombocytosis and

lymphocytosis

8

th Hospital Day:

• Afebrile, no bullae formation noted

• Continued healing of previously errupted

bullae on patient’s back, face and neck

• D5 IMB (MR)

• Hydroxyzine

• Ketotifen

Oxacillin ( going day 7)

• Prednisone

9

th Hospital Day:

• Afebrile,

• NO bullae formation noted, continued healing of

the remaining bullae and brownish crusts

• Oxacillin was discontinued

• Shifted to Flucloxacin 125 mg/ 5ml, 5ml q 6hrs

(AD:60.2 mkD)

• D5 IMB (MR)

• Hydroxyzine

• Ketotifen

• Prednisone

10

th Hospital Day

• HR125 RR: 33 Temp: 36.6

• Skin: (+) whitish scar noted on the affected

areas. NO more blisters noted.

• IVF discontinued

• Hydroxyzine, Ketotifen, Prednisone

• Flucloxacin (going Day 2)

• Repeat CBC: leukocytosis (lymphocytosis)

 

• MGH

Take Home Meds:

• Pred 10 syrup, 2.5ml BID X 10 days

• Flucloxacin 125/5 ml, 5 ml q6hrs x 6 days

• Hydroxyzine 2 mg/ml, 1.5 ml TID PRN for

pruritus

• Ketotifen 0.4 ml BID

• Follow up 1 week after discharge

 

Final diagnosis: Systemic Mastocytosise
   

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