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Mast Cell Dysfunction

Mast Cell Dysfunction. Hyeyoon Boo, Nicholas Chan, Jason Lo, Christina Ma. PHM142 Fall 2013 Coordinator: Dr. Jeffrey Henderson Instructor: Dr. David Hampson. What are Mast Cells?. Part of the immune system – allergies Contain granules with histamine and heparin

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Mast Cell Dysfunction

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  1. Mast Cell Dysfunction Hyeyoon Boo, Nicholas Chan, Jason Lo, Christina Ma PHM142 Fall 2013 Coordinator: Dr. Jeffrey Henderson Instructor: Dr. David Hampson

  2. What are Mast Cells? • Part of the immune system – allergies • Contain granules with histamine and heparin • Derived from haematopoietic stem cells (ie. bone marrow) • Located in connective tissue

  3. Mast Cell Activation

  4. Mast Cell Dysfunctions 1) Excessive proliferation of mast cells (Mastocytosis) • Cutaneous • Systemic • Localized mast cell proliferations 2) Normal number of cells but abnormal activity (MCAS)

  5. Cutaneous Mastocytosis • Accumulation of mast cells in the skin 3 types: 1) Urticariapigmentosa (Most common) - Brown patches of the skin where mast cells have accumulated 2) Diffuse cutaneous mastocytosis (Most rare) - Generalized erythroderma and blisters 3) Mastocytoma of skin - Round tumor consisting of mast cells on the skin

  6. Systemic Mastocytosis • Accumulation of mast cells in different tissues (Ex. Organs, bones, skin) • Mainly affects adults 3 types: 1) Indolent mastocytosis • 46% of all cases 2) Aggressive mastocytosis • Invade organs such as spleen, liver and digestive system 3) Systematic mastocytosis with associated haematological disease - May develop conditions affecting blood cells (ex. Chronic leukaemia)

  7. Localized mastocytosis • Dense infiltration of mast cells in upper dermis • Appears in early childhood • Brownish-red or yellow nodules

  8. Mast Cell Dysfunctions 1) Excessive proliferation of mast cells (Mastocytosis) • Cutaneous • Systemic • Localized mast cell proliferations 2) Normal number of cells but abnormal activity (MCAS)

  9. Mast cell activation syndrome (MCAS) • Causes mast cells to degranulate excessively and release histamine, heparin and other chemicals 3 types 1) Primary MCAS • Mutated clonal mast cells are found 2) Secondary MCAS • An underlying allergy disorder induces mast cell activation, but no clonal mast cells are detected 3) Idiopathic MCAS • No underlying allergy disorder, and no clonal mast cells are detected, but MCAS criteria are still fulfilled

  10. Summary • Mast cells - granule filled white blood cells that are part of the immune system that regulate allergic reactions. • Mastocytosis: excessive proliferation of mast cells • Cutaneous: accumulation of mast cells in skin • Urticariapigmentosa: brown patches • Diffuse cutaneous mastocytosis: erythroderma and blisters • Mastocytoma: round tumors • Systemic: accumulation of mast cells in different tissues • Indolent Mastocytosis: benign • Aggressive Mastocytosis: active and invasive • Systemic Mastocytosis with associated haematological disease: may affect blood cells • Localized Mast Cell Proliferations: dense infiltration of mast cells in upper dermis (extracutaneous) • Mast Cell Activation Syndrome (normal amount of cells, abnormal activity) • Primary: Result of mutated clonal mast cells • Secondary: Result of underlying allergy disorder • MCAS criteria fulfilled but no clonal mast cells no underlying allergy disorder.

  11. References • "About Mast Cells." MastCellAware. N.p., n.d. Web. 19 Nov. 2013. <http://www.mastcellaware.com/about.html>. • "DermIS - Localized Mastocytoma (information on the Diagnosis)." DermIS - Localized Mastocytoma (information on the Diagnosis). N.p., n.d. Web. 19 Nov. 2013. <http://www.dermis.net/dermisroot/en/41716/diagnose.htm>. • "Diffuse Cutaneous Mastocytosis." Orphanet. N.p., n.d. Web. 18 Nov. 2013. <http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en>. • "Mastocytosis ." Mastocytosis. N.p., n.d. Web. 19 Nov. 2013. <http://emedicine.medscape.com/article/1057932-overview>. • "Mastocytosis ." Mastocytosis. N.p., n.d. Web. 19 Nov. 2013. <http://www.nhs.uk/conditions/Mastocytosis/Pages/Introduction.aspx>. • "Mastocytosis Society Canada: Mastocytosis & Mast Cell Disorders." Mastocytosis Society Canada: Mastocytosis & Mast Cell Disorders. N.p., n.d. Web. 19 Nov. 2013. <http://www.mastocytosis.ca/masto.htm>. • "Patient.co.uk - Trusted Medical Information and Support." Patient.co.uk. N.p., n.d. Web. 19 Nov. 2013. <http://www.patient.co.uk/doctor/Mastocytosis-and-Mast-Cell-Disorders.htm>. • "Systemic Mastocytosis." — Diagnosis and Treatment at Mayo Clinic. N.p., n.d. Web. 19 Nov. 2013. <http://www.mayoclinic.org/systemic-mastocytosis/>.

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