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MASTOCYTOSIS

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MASTOCYTOSIS:

MASTOCYTOSIS:
Is a group of rare disorders of both children and adults caused by the presence of too many mast cells (mastocytes) and CD34+ mast cell precursors in a person's body.

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MASTOCYTOSIS MASTOCYTOSIS Presentation Transcript

  • 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 1 P o.
    • 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.
    View slide
  • INTRANATAL HISTORY:
    • Fullterm, via NSVD
    • assisted by a doctor, good cry
    • BW: 3.6 kgs.
    • BR:1/1
    • No complications
    View slide
  • 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 – 3 doses
    • OPV - 3 doses
    • DPT – 3 doses
    • Measles – 1 dose
  • DEVELOPMENTAL HISTORY:
    • Social smile – 2 mos.
    • Roll over – 4 mos.
    • Sit w/ support – 7 mos.
    • Sit w/o support – 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 (Synalar cream) was 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 Physiological Lipid (Physiogel cream) but still with no relief was noted.
    • dermatologist, unrecalled cream was given, still w/ no relief.
    • Skin biopsy and Giemsa stain: revealed mastocytosis.
  • PHYSICAL EXAMINATION
    • General Survey: alert, febrile, irritable, not in respiratory distress
    • Vital Signs:
    • HR: 92 bpm RR: 55 cpm Temp. 38.6 o C
    • Wt. 8.5 kgs Ht. 69 cm. HC: 45 cm
    • CC: 47 cm AG: 52 cm
  • Waterlow Classification:
    • Wasting = ABW/IBW for actual length X100
    • =8.5 kgs/9 X 100
    • =94.4 % (normal >90%)
    • Stunting =actual ht/ideal ht for age
    • =69/70 x 100
    • =98.5 % (normal >95%)
    • 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 2mg/ml susp, 1.5ml TID PO(AD:1.08mkD)
    • Oxacillin (Wydox) 200mg, IV drip q 6 hours, ANST (AD:96.3 mkD)
    • Referrred to hema-onco
    • Labs Requested:
    • CBC: lymphocytosis and monocytosis
    • U/A : pyuria
    • Blood culture and sensi: No growth
  • LABS 42.6 lymph On admission CBC 367 plt 41.4 hct 14.4 hgb 10.5 mono 44.4 seg 12 wbc
    • 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: bullae on frontal area R, preauricular R&L, submandibular area L, erupted bullae deltoid area R&L, upper thorax, upper abdomen and upper back
    • 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)
    • Prednisone 10 mg , 2.5 ml BID, also started
    • Hydroxyzine
    • Ketotifen Drops, 0.4 ml BID was added
    • Oxacillin ( going day 5)
    • CBC: thrombocytosis and lymphocytosis
    • BMA: dysplastic changes in granulocytic and megakaryocytic cells and increased thrombopoiesis
  • 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 st day post BMA
    • HR: 129 RR: 36 Temp: 37.3
    • Skin: No bleeding on the BMA site
      • 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 Mastocytosis
  •  
  •