Mastocytosis: Difference between revisions

Latest revision as of 12:11, 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

@@ Line 1: / Line 1: @@
-== Clinical Syndromes ==
+==Clinical Manifestations==
-=== Indolent systemic mastocytosis ===
+===Indolent Systemic Mastocytosis===
-* Most common cause of adult-onset mastocytosis
+*Most common cause of adult-onset mastocytosis
-* Usually associated with small monomorphic maculopapular lesions on thigh or trunk
+*Usually associated with small monomorphic maculopapular lesions on thigh or trunk
-* Anaphylaxis in 50%
+*Anaphylaxis in 50%
-=== Urticaria pigmentosa ===
+===Urticaria Pigmentosa===
-* Presents with maculopapular rash that can resemble freckles
+*Presents with maculopapular rash that can resemble freckles
-* Usually has systemic involvement
+*Usually has systemic involvement
-* Darier's sign: light rubbing or scratching of a lesion will cause erythema or urticaria
+*Darier's sign: light rubbing or scratching of a lesion will cause erythema or urticaria
-=== Unexplained or severe anaphylaxis ===
+===Unexplained or Severe Anaphylaxis===
-* Anaphylaxis, usually without urticaria or angioedema
+*Anaphylaxis, usually without urticaria or angioedema
-* Without a trigger, or in response to bee/wasp/etc sting
+*Without a trigger, or in response to bee/wasp/etc sting
-=== Other syndromes ===
+===Other Syndromes===
-* Adult with hematologic abnormalities, splenomegaly, and weight loss
+*Adult with hematologic abnormalities, splenomegaly, and weight loss
-== Investigations ==
+==Investigations==
-* CBC may show cyptopenias or cytoses, including eosinophilia and circulating mast cells
+*CBC may show cyptopenias or cytoses, including eosinophilia and circulating mast cells
-* Serum tryptase, at baseline (not during an attack), which should be elevated
+*Serum tryptase, at baseline (not during an attack), which should be elevated
-* Liver enzymes for possible liver involvement
+*Liver enzymes for possible liver involvement
-* Bone dexa for osteopenia or osteoporosis
+*Bone dexa for osteopenia or osteoporosis
 [[Category:Hematology]]