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Anaphylaxis and Mastocytosis Flashcards

1
Q

Classification of mast cells disorders

A
  1. Primary - MC more activatible either spont. or unknown trigger
  2. Secondary - MCs are activated by an ext. trigger like an allergen and antigens
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2
Q

MCAS definition (AAAAI)

A

A primary clinical condition with spont. episodic signs and symptoms of systemic anaphylaxis concurrently affecting two or more organ systems, resulting from secreted MC mediators

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3
Q

List the Anaphylaxis Criteria (WAO)

A

Criteria 1: Acute onset of illness involving skin/mucosal tissue with either a. resp sx or b. reduced BP

Criteria 2 - two or more of the following after exposure to a likely allergen

  • skin/mucosal tissue
  • resp sx
  • reduced BP
  • persistent GI sx

Criteria 3 - reduced BP after a known allergen
- in adults: <90 or 30%

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4
Q

What is Carmine allergy?

A
  • red food dye
  • from dried bodies of cost insects
  • used in food like candy, ice cream etc
  • confirm with skin testin
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5
Q

What is Anato allergy?

A

Yellow food colouring

- produced from fruit of a tropical annatto tree, Bixa orellana

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6
Q

Man develops anaphylaxis after eating a muffin and jumping into a pool, whats your ddx?

A
  1. FDEIA
  2. CIU
  3. EIA
  4. Local heat, cold or cholinergic urticaria
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7
Q

6 ways to differentiate exercise anaphylaxis vs cholinergic urticaria

A
  1. wheal size (cholinergic are small and pinpoint, EIA are larger)
  2. triggers - cholinergic triggers include warm baths, sweating, strong emotions
  3. Occlusive body suit test - CU have a drop in FEV1, EIA does not
  4. CU is responsive to hydroxyzine, EIA is not
  5. Hyperthermic blanket test or submersion into warm water
  6. Systemic symptoms are present in EIA not in CU
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8
Q

DDx for idiopathic anaphylaxis

A
  1. Food allergy - overlooked foods include spices, food containing aeroallergens, mislabeled foods, and food ingested with cofactors
  2. Medication allergy - NSAIDs
  3. Mast cell disorders
  4. Carcinoid syndrome
  5. Pheo
  6. CU
  7. Flushing reactions
  8. Restaurant syndromes: MSG, sulfites, scromboid
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9
Q

Work up for idiopathic anaphylaxis

A
  1. history and exam
  2. SPT, and rast
  3. tryptase, 24 hr urine histamine, 5-HIAA, VMA, and urinary catecholamines
  4. AI work up- lytes, TSH, T4
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10
Q

risk factors for severe anaphylaxis

A
  1. asthma
  2. CV disease
  3. mast cell disorder
  4. older age
  5. acute infections
  6. meds - BB, Alpha blocks, Acei
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11
Q

Criteria for mastocytosis

need 1 major and 1 minor OR 3 minor

A

Major
- presence of multifocal, dense mast cell infiltrates (>15 in aggregates in BM or other exogenous organs)

Minor

  • > 25% mast cells spindle shaped or with abnormal morphology in bone or other exo organs
  • detection of c-kit point mut at codon 816 in BM
  • mast cells that co express CD117 with CD2 or CD25
  • persistent serum tryptase> 20
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12
Q

Criteria for mast cell disorders

A
  1. Episodic multi system symptoms consistent with anaphylaxis
  2. Increase in validated markers of mast cell activation (serum or urine)
  3. response to medications that target mast cell activation syndrome
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13
Q

Classify mastocytosis

A
  1. systemic mastocytosis
  2. cutaneous mastocytosis
  3. solid organ tumours
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14
Q

pathogenesis of mastocytosis

A
  • SCF (stem cell factor) is a growth factor which controls the production of mast cells
  • this mutation is in KIT (CD117) receptor on codon 816 which is a receptor for SCF
  • usually GOF CD 117
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15
Q

clinical features of mastocytosis

A
  1. dariers sign
  2. UP or MPCM
  3. diffuse cut. mastocytosis (DCM)
  4. mastocytomas
  5. telengectasia macularis eruptive perstand (TMEP)
  6. Anaphylaxis
  7. GI disturbance
  8. Neurophysiatric - mixed organic brain syndrome
  9. Heme
  10. MSK
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16
Q

lab evaluation in mastocytosis

A
  1. CBC with differential to look for cytopenias, eosinophils, circulating mast cells and any abnormal myeloid or lymphoid leuks
  2. LFTs
  3. Bone density
  4. KIT mutation evaluation on peripheral blood or bone marrow
  5. Serum total tryptase at baseline on two occasions
    - subtypes if possible
    - would expect total tryptase to mature tryptase ratio to be very high
17
Q

differential for mastocytosis

A
  1. MCAS - monoclonal
  2. MCAS - idiopathic
  3. Anaphylaxis
  4. HAE
  5. Pheo
  6. VIP secreting GI tumors
  7. Carcinoid tumours
  8. medullary thyroid cancer
  9. Zollinger Ellison syndrome
18
Q

differential for a high tryptase

A
  1. HES
  2. AML
  3. MDS
  4. Refractory anemia
  5. ESRD
  6. famililal hypertrypsinemia
  7. transient or reactive
19
Q

Management of mastocytosis

A
  1. Non pharma
    - avoid triggers - heat, cold, stress, alcohol, exercise, infections, surgery
    - medic alert bracelet
  2. Pharm
    - EpiPens
    - VIT if indicated
    - H1 and H2 blockers can be used
    - Ketotifen (can be sedating)
    - Singulair
    - Flushing - try ASA which reduced PGD2 released by mast cells
    - for GI sx - try cromolyn which gradually increases to 200 mg QID
    - for osteo and #s - Ca, Vit D, pamidotronate
  3. SM- AHNMD tx the underlying heme disorder
  4. Agressive SM - INFa, Cladribine, Steroids, Hydroxyurea, TK inhibitors
20
Q

factors associated with poor px in mastocytosis

A
  1. older age
  2. low albumin
  3. high LDH
  4. HSM
  5. Ascites
  6. BM blasts
  7. cytopenias
21
Q

What is hereditary a-trypsinemia

A
  • an AD disorder with a phenotype which can include dysautonomia with POTs, flushing, GI hypomotility, joint hyperextensibility, vibratory urticaria
  • due to 1 or more extra copies of a tryptase gene encoded by TBSAB1 resulting in over expression of a tryptase and increased mast cells in BM
22
Q

surgical considerations in mastocytosis

A

Pre-medication prior to procedures - can consider administering 1 hr prior, but efficacy not studied:
○ Diphenhydramine 25-50mg
○ Famotidine 20 mg
○ Montelukast 10mg
○ Prednisone (preferred if pt has frequent hypoTN episodes or problems with anesthesia in the past) 25-50mg PO 12 hrs and 1-2 hrs prior

23
Q

classify MCAS

A
  1. monoclonal mast cell activation disorder (MMAS)

2. idiopathic mast cell activation disorder (MCAS)

24
Q

algorithm for diagnosis MCAS

A
  1. recurrent sx consistent with mast cell activation with involvement of two organs
  2. evaluation of one or more validated mast cell mediators in associated with symptoms
  3. response to targeted therapeutic interventions
  4. peripheral blood or bone marrow GOF KIT mutation or buccal swab increased TPSAB1 a tryptase CNV
    - if positive: then primary MCAS with somatic or germline mutation
    - if negative: then MCAS without a known mutation

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