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MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
MASTOCYTOSIS
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MASTOCYTOSIS
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MASTOCYTOSIS
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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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  • 1. SACRED HEART HOSPITAL DEPARTMENT OF PEDIATRICS Mortality and Morbidity Conference May 2008
  • 2. GENERAL DATA: <ul><li>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. </li></ul>
  • 3. PRENATAL HISTORY: <ul><li>Mother was 29 y.o. </li></ul><ul><li>G 1 P o. </li></ul><ul><li>PNC at 2 mos. AOG at a private MD, with regular visits </li></ul><ul><li>Multivitamins was taken with good compliance. </li></ul><ul><li>Non-smoker and non-alcoholic beverage drinker. </li></ul><ul><li>Epigastric pain at 6 mos. AOG, no consult done, no meds taken. </li></ul>
  • 4. INTRANATAL HISTORY: <ul><li>Fullterm, via NSVD </li></ul><ul><li>assisted by a doctor, good cry </li></ul><ul><li>BW: 3.6 kgs. </li></ul><ul><li>BR:1/1 </li></ul><ul><li>No complications </li></ul>
  • 5. FEEDING AND NUTRITIONAL HISTORY : <ul><li>Exclusively breastfed up to 1 month old </li></ul><ul><li>shifted to Mylac w/ 1:2 dilution until present </li></ul><ul><li>semi-solid food at 8 mos </li></ul><ul><li>solid food at 9 mos. old. </li></ul>
  • 6. IMMUNIZATION: <ul><li>BCG- 1 dose </li></ul><ul><li>Hep. B – 3 doses </li></ul><ul><li>OPV - 3 doses </li></ul><ul><li>DPT – 3 doses </li></ul><ul><li>Measles – 1 dose </li></ul>
  • 7. DEVELOPMENTAL HISTORY: <ul><li>Social smile – 2 mos. </li></ul><ul><li>Roll over – 4 mos. </li></ul><ul><li>Sit w/ support – 7 mos. </li></ul><ul><li>Sit w/o support – 8 mos. </li></ul>
  • 8. PAST MEDICAL HISTORY/ FAMILY HISTORY: <ul><li>No previous hospitalization or any serious physical injury, surgical procedure done. </li></ul><ul><li>( + ) HPN and DM- both </li></ul><ul><li>( - ) Allergies to food and drugs </li></ul><ul><li>( +) dermatographism- mother side </li></ul>
  • 9. PERSONAL &amp; SOCIAL HISTORY: <ul><li>Patient is a 9 mo child. </li></ul><ul><li>stays with the parents. </li></ul><ul><li>Playful and alert child. </li></ul>
  • 10. HISTORY OF PRESENT ILLNESS: <ul><li>5 mos. PTA, bullae on the forehead, pruritic </li></ul><ul><li>asso w/ mild grade fever </li></ul><ul><li>Sought consult to private physician. </li></ul><ul><li>Fluocinolone Acetonide (Synalar cream) was prescribed with no relief noted. </li></ul>
  • 11. <ul><li>The condition was tolerated. </li></ul><ul><li>The bullae usually lasted for 3 days </li></ul><ul><li>Then bursting and development of a yellowish-brown crust until complete healing was noted. </li></ul><ul><li>4 mos. PTA, condition persisted and spread to the scalp, face, trunk, and extremities. </li></ul>
  • 12. <ul><li>Sought consult again to the same private physician, Fluocinolone Acetonide (Synalar Cream) was discontinued </li></ul><ul><li>Changed Physiological Lipid (Physiogel cream) but still with no relief was noted. </li></ul>
  • 13. <ul><li>dermatologist, unrecalled cream was given, still w/ no relief. </li></ul><ul><li>Skin biopsy and Giemsa stain: revealed mastocytosis. </li></ul>
  • 14. PHYSICAL EXAMINATION <ul><li>General Survey: alert, febrile, irritable, not in respiratory distress </li></ul><ul><li>Vital Signs: </li></ul><ul><li>HR: 92 bpm RR: 55 cpm Temp. 38.6 o C </li></ul><ul><li>Wt. 8.5 kgs Ht. 69 cm. HC: 45 cm </li></ul><ul><li>CC: 47 cm AG: 52 cm </li></ul>
  • 15. Waterlow Classification: <ul><li>Wasting = ABW/IBW for actual length X100 </li></ul><ul><li>=8.5 kgs/9 X 100 </li></ul><ul><li>=94.4 % (normal &gt;90%) </li></ul><ul><li>Stunting =actual ht/ideal ht for age </li></ul><ul><li>=69/70 x 100 </li></ul><ul><li>=98.5 % (normal &gt;95%) </li></ul>
  • 16. <ul><li>SKIN: (+) bullae on right frontal area, preauricular R&amp;L, submandibular area L, deltoid area R&amp;L, upper thorax, upper abdomen, and upper back. No jaundice, no pallor, warm, good turgor </li></ul><ul><li>HEENT: normocephalic, pinkish palpebral conjunctiva, anicteric sclerae, pupils equally reactive to light, intact TM, no nasal congestion, no alar flaring, no TPC, no LAD </li></ul>
  • 17. <ul><li>CHEST &amp; LUNGS: equal chest expansion, no retractions, no rales, no wheeze </li></ul><ul><li>CVS: no bulging of precordium, distinct heart sound, normal rate &amp; regular rhythm, no murmur </li></ul><ul><li>ABDOMEN: globular, NABS, soft, no organ enlargement </li></ul>
  • 18. <ul><li>GENITALIA: grossly female, no vaginal discharge, no lesions </li></ul><ul><li>EXTREMTIES: no deformities, no edema, strong peripheral pulses both upper &amp; lower, CRT &lt; 2 sec. </li></ul>
  • 19. NEUROLOGIC EXAM: <ul><li>Mental Status: alert </li></ul><ul><li>Motor: normal </li></ul><ul><li>Tone: normal </li></ul><ul><li>CN I: NA </li></ul><ul><li>CN II: pupils equally reactive to light </li></ul><ul><li>CN III, IV, VI: (+) EOM </li></ul><ul><li>CN V: NA </li></ul>
  • 20. <ul><li>CN VII: no facial asymmetry </li></ul><ul><li>CN VIII: blink to loud sound </li></ul><ul><li>CN IX, X: (+) gag reflex </li></ul><ul><li>(+) swallowing </li></ul><ul><li>CN XI: can turn head side to side </li></ul><ul><li>CN XII: tongue not deviated </li></ul>
  • 21. <ul><li>Reflex: (+) babinsky </li></ul><ul><li>Sensory: intact sensation to touch and pain stimuli </li></ul><ul><li>Cerebellar: </li></ul>
  • 22. IMPRESSION: <ul><li>Cutaneous Mastocytosis </li></ul>
  • 23. <ul><li>COURSE IN THE WARD </li></ul>
  • 24. On admission: <ul><li>D5 0.3% NaCl (MR+12%) </li></ul><ul><li>Paracetamol 100mg/ml, 1.2 mL q4hrs prn </li></ul><ul><li>Hydroxyzine 2mg/ml susp, 1.5ml TID PO(AD:1.08mkD) </li></ul><ul><li>Oxacillin (Wydox) 200mg, IV drip q 6 hours, ANST (AD:96.3 mkD) </li></ul>
  • 25. <ul><li>Referrred to hema-onco </li></ul><ul><li>Labs Requested: </li></ul><ul><li>CBC: lymphocytosis and monocytosis </li></ul><ul><li>U/A : pyuria </li></ul><ul><li>Blood culture and sensi: No growth </li></ul>
  • 26. LABS 42.6 lymph On admission CBC 367 plt 41.4 hct 14.4 hgb 10.5 mono 44.4 seg 12 wbc
  • 27. <ul><li>URINALYSIS </li></ul><ul><li>Transparency yellow </li></ul><ul><li>Spec. Gravity 1.010 </li></ul><ul><li>Albumin (-) </li></ul><ul><li>Glucose (-) </li></ul><ul><li>Ketone/Blood (-) </li></ul><ul><li>WBC 3 - 6 </li></ul><ul><li>RBC 0 - 3 </li></ul>
  • 28. 1 st Hospital Day: <ul><li>Afebrile, still with blisters on forehead, temporal area, chest and back </li></ul><ul><li>With good appetite, playful, alert </li></ul><ul><li>HR:120’s RR:30’s temp:36.5 – 37.1C </li></ul><ul><li>Skin: blisters on her forehead, temporal area, with multiple brownish crusted lesions on chest and back </li></ul><ul><li>Oxacillin was increased to 250 mg IV drip q6hrs (AD:120.4 mkD) </li></ul><ul><li>Hydroxyzine </li></ul>
  • 29. &nbsp;
  • 30. 2 nd Hospital Day: <ul><li>Afebrile, new bullous formation seen at the back </li></ul><ul><li>HR: 120 RR:30 TEMP: 36.5 </li></ul><ul><li>SKIN: bullae on frontal area R, preauricular R&amp;L, submandibular area L, erupted bullae deltoid area R&amp;L, upper thorax, upper abdomen and upper back </li></ul><ul><li>Mastocytosis </li></ul><ul><li>Hydroxyzine </li></ul><ul><li>Oxacillin ( going day 2) </li></ul>
  • 31. &nbsp;
  • 32. 3 rd Hospital Day: <ul><li>Patient was scheduled for BMA </li></ul><ul><li>Brownish crust noted on errupted bullae </li></ul><ul><li>New bullae was also noted </li></ul><ul><li>HR: 118 RR: 30 Temp: 36 </li></ul><ul><li>D5 IMB (MR) </li></ul><ul><li>Hydroxyzine </li></ul><ul><li>Ketotifen Drops, 0.4 ml BID was added </li></ul><ul><li>Oxacillin ( going day 3) </li></ul>
  • 33. &nbsp;
  • 34. 4 th Hospital Day: <ul><li>Afeb, still with new bullae formation noted </li></ul><ul><li>SKIN: Brownish crust was also noted </li></ul><ul><li>HR: 121 RR: 29 Temp: 36.5 </li></ul><ul><li>Mupirocin ointment started </li></ul><ul><li>D5 IMB (MR) </li></ul><ul><li>Hydroxyzine </li></ul><ul><li>Ketotifen Drops, 0.4 ml BID </li></ul><ul><li>Oxacillin ( going day 4) </li></ul>
  • 35. &nbsp;
  • 36. 5 th Hospital Day: <ul><li>afebrile </li></ul><ul><li>HR: 124 RR: 28 Temp: 36.8 </li></ul><ul><li>Skin: decrease in the appearance of new bullae was noted </li></ul><ul><li>D5 IMB (MR) </li></ul><ul><li>Prednisone 10 mg , 2.5 ml BID, also started </li></ul><ul><li>Hydroxyzine </li></ul><ul><li>Ketotifen Drops, 0.4 ml BID was added </li></ul><ul><li>Oxacillin ( going day 5) </li></ul><ul><li>CBC: thrombocytosis and lymphocytosis </li></ul><ul><li>BMA: dysplastic changes in granulocytic and megakaryocytic cells and increased thrombopoiesis </li></ul>
  • 37. BMA Result: <ul><li>Cellular bone marrow, w/ 20% increased fat cells </li></ul><ul><li>There is erythroid hyperplasia </li></ul><ul><li>Toxic changes are seen about 40% of cells </li></ul><ul><li>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 </li></ul>
  • 38. 6 th Hospital Day: <ul><li>1 st day post BMA </li></ul><ul><li>HR: 129 RR: 36 Temp: 37.3 </li></ul><ul><li>Skin: No bleeding on the BMA site </li></ul><ul><ul><li>NO new blister formation seen on any part of the body of patient </li></ul></ul><ul><ul><li>Still w/ brownish crust on the errupted bullae </li></ul></ul><ul><li>D5 IMB (MR) </li></ul><ul><li>Hydroxyzine </li></ul><ul><li>Ketotifen Drops, 0.4 ml BID was added </li></ul><ul><li>Oxacillin ( going day 6) </li></ul><ul><li>Prednisone 10 mg, 2.5 ml BID </li></ul>
  • 39. 7 th Hospital Day: <ul><li>HR: 121 RR: 33 36.4 </li></ul><ul><li>Skin: NO blister formation. Healing of the blister formation was noted </li></ul><ul><li>D5 IMB (MR) </li></ul><ul><li>Hydroxyzine </li></ul><ul><li>Ketotifen </li></ul><ul><li>Oxacillin ( going day 7) </li></ul><ul><li>Prednisone </li></ul><ul><li>Peripheral Blood Smear: thrombocytosis and lymphocytosis </li></ul>
  • 40. 8 th Hospital Day: <ul><li>Afebrile, no bullae formation noted </li></ul><ul><li>Continued healing of previously errupted bullae on patient’s back, face and neck </li></ul><ul><li>D5 IMB (MR) </li></ul><ul><li>Hydroxyzine </li></ul><ul><li>Ketotifen </li></ul><ul><li>Oxacillin ( going day 7) </li></ul><ul><li>Prednisone </li></ul>
  • 41. 9 th Hospital Day: <ul><li>Afebrile, </li></ul><ul><li>NO bullae formation noted, continued healing of the remaining bullae and brownish crusts </li></ul><ul><li>Oxacillin was discontinued </li></ul><ul><li>Shifted to Flucloxacin 125 mg/ 5ml, 5ml q 6hrs (AD:60.2 mkD) </li></ul><ul><li>D5 IMB (MR) </li></ul><ul><li>Hydroxyzine </li></ul><ul><li>Ketotifen </li></ul><ul><li>Prednisone </li></ul>
  • 42. 10 th Hospital Day <ul><li>HR125 RR: 33 Temp: 36.6 </li></ul><ul><li>Skin: (+) whitish scar noted on the affected areas. NO more blisters noted. </li></ul><ul><li>IVF discontinued </li></ul><ul><li>Hydroxyzine, Ketotifen, Prednisone </li></ul><ul><li>Flucloxacin (going Day 2) </li></ul><ul><li>Repeat CBC: leukocytosis (lymphocytosis) </li></ul>
  • 43. &nbsp;
  • 44. <ul><li>MGH </li></ul><ul><li>Take Home Meds: </li></ul><ul><li>Pred 10 syrup, 2.5ml BID X 10 days </li></ul><ul><li>Flucloxacin 125/5 ml, 5 ml q6hrs x 6 days </li></ul>
  • 45. <ul><li>Hydroxyzine 2 mg/ml, 1.5 ml TID PRN for pruritus </li></ul><ul><li>Ketotifen 0.4 ml BID </li></ul><ul><li>Follow up 1 week after discharge </li></ul><ul><li>Final diagnosis: Systemic Mastocytosis </li></ul>
  • 46. &nbsp;
  • 47. &nbsp;

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