Mastocytosis: Difference between revisions
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+ | ==Clinical Manifestations== |
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+ | ===Indolent Systemic Mastocytosis=== |
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+ | *Most common cause of adult-onset mastocytosis |
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+ | *Usually associated with small monomorphic maculopapular lesions on thigh or trunk |
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+ | *Anaphylaxis in 50% |
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+ | ===Urticaria Pigmentosa=== |
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+ | *Presents with maculopapular rash that can resemble freckles |
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+ | *Usually has systemic involvement |
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+ | *Darier's sign: light rubbing or scratching of a lesion will cause erythema or urticaria |
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+ | ===Unexplained or Severe Anaphylaxis=== |
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+ | *Anaphylaxis, usually without urticaria or angioedema |
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+ | *Without a trigger, or in response to bee/wasp/etc sting |
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+ | ===Other Syndromes=== |
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+ | *Adult with hematologic abnormalities, splenomegaly, and weight loss |
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+ | ==Investigations== |
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+ | *CBC may show cyptopenias or cytoses, including eosinophilia and circulating mast cells |
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+ | *Serum tryptase, at baseline (not during an attack), which should be elevated |
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+ | *Liver enzymes for possible liver involvement |
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+ | *Bone dexa for osteopenia or osteoporosis |
[[Category:Hematology]] |
[[Category:Hematology]] |
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Latest revision as of 08:11, 2 August 2020
Clinical Manifestations
Indolent Systemic Mastocytosis
- Most common cause of adult-onset mastocytosis
- Usually associated with small monomorphic maculopapular lesions on thigh or trunk
- Anaphylaxis in 50%
Urticaria Pigmentosa
- Presents with maculopapular rash that can resemble freckles
- Usually has systemic involvement
- Darier's sign: light rubbing or scratching of a lesion will cause erythema or urticaria
Unexplained or Severe Anaphylaxis
- Anaphylaxis, usually without urticaria or angioedema
- Without a trigger, or in response to bee/wasp/etc sting
Other Syndromes
- Adult with hematologic abnormalities, splenomegaly, and weight loss
Investigations
- CBC may show cyptopenias or cytoses, including eosinophilia and circulating mast cells
- Serum tryptase, at baseline (not during an attack), which should be elevated
- Liver enzymes for possible liver involvement
- Bone dexa for osteopenia or osteoporosis
