MASTOCYTOSIS

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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

  1. 1. SACRED HEART HOSPITAL DEPARTMENT OF PEDIATRICS Mortality and Morbidity Conference May 2008
  2. 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. 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. 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. 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. 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. 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. 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. 9. PERSONAL & 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. 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. 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. 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. 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. 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. 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 >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 >95%) </li></ul>
  16. 16. <ul><li>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 </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. 17. <ul><li>CHEST & LUNGS: equal chest expansion, no retractions, no rales, no wheeze </li></ul><ul><li>CVS: no bulging of precordium, distinct heart sound, normal rate & regular rhythm, no murmur </li></ul><ul><li>ABDOMEN: globular, NABS, soft, no organ enlargement </li></ul>
  18. 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 & lower, CRT < 2 sec. </li></ul>
  19. 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. 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. 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. 22. IMPRESSION: <ul><li>Cutaneous Mastocytosis </li></ul>
  23. 23. <ul><li>COURSE IN THE WARD </li></ul>
  24. 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. 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. 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. 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. 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. 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&L, submandibular area L, erupted bullae deltoid area R&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>
  30. 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>
  31. 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>
  32. 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>
  33. 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>
  34. 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>
  35. 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>
  36. 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>
  37. 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>
  38. 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>
  39. 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>
  40. 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>

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