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32 Cards in this Set
- Front
- Back
What precursors give rise to Mast Cells?
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CD34+ pluripotent cells
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What do mast cells precursors express?
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Express CD34, KIT & IgG receptors (FcγRII)
Do not express high affinity IgE receptors (FcεRI) |
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Tyrosine Kinase Receptor aka?
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CD117
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What is KIT?
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protein product of proto-oncogene c-kit located on chromosome 4q12 (belongs to the type III receptor tyrosin kinase subfamily
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What cells express KIT?
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mast cells, melanocytes, primitive hematopoietic stem cells, primordial germ cells, and interstitial cells of Cajal
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What is the ligand for KIT?
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SCF = stem cell factor
aka mast cell growth factor |
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SCF induces mast cell precursors to do with with their protein expression?
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In presence of SCF, peripheral blood mast cell precursors:
Become KIT+, CD34-, FcgammaRII- & FcepsilonRI+ Develop characteristic cytoplasmic granules |
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What is the most common KIT mutation in Mastocytosis?
What other codon mutations have been reported? |
somatic point mutations in codon 816
- activation mutation of KIT Other mutations: Codon 560 activating, Codon 820 activating, Codon 839 INactivating (NO MUTATIONS FOUND IN FAMILIAL CASES) Summary: Childhood Dz (no activating mutation in most, few 816, few 839) Adult Dz (816 most, 560 few, 820 few) Familial - none of either |
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What other mutations have been identified in Mastocytosis?
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Chromosomal deletion 4q12 - juxtaposition of platelet derived growth factor receptor alpha & FIP1L1 (fusion protein that activates cells)
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What is another name for Leder Stain?
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Chloroacetate esterase - mast cells are red
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What are some Mast cell stains?
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Toluidine blue, Giemsa (blue), labeled avidin, Leder, mAb to tryptase or CD117 (KIT)
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What is formed in primary hemostasis?
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The platelet plug
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What is formed in secondary hemostasis?
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The clot
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What are the names given to the two pathways utilized in coagulation, and which lab test is used to measure each?
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Extrinsic: measured via PT
Intrinsic: measured via PTT |
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What are the Vit K dependent coag factors and proteins
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Factors II, VII, IX, X and Proteins C & S
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Where does Vitamin K play a role?
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Factors are modified by a vitamin K-dependent post translational carboxylation of glutamic acid residues which allows these proteins to bind Ca and phospholipids. Allows them to function efficiently on plasma membranes.
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What two factors are inactivated by Protein C?
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Factors V and VIII
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Factor V leiden mutation leads to what?
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Unopposed thrombosis since the mutated Factor V is resistant to Protein C inactiviation
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What three labs are helpful in evaluating clotting d/o?
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Platelet count, PT, APTT
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High PT and normal PTT suggests?
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Factor VII deficiency OR coumadin use
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High PTT and normal PT suggests?
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von Willebrand Dz, lupus anticoagulant, heparin, acquired factor VIII inhibitor or deficiency of factor XII, XI, VIII, HMWK, or PK
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High PT and PTT suggests?
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Deficiency in fibrinogen, thrombin, factors V or X
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Name the test used to look for lupus anticoagulant activity?
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Russell viper venom test
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Name the five variants of the pigmented purpuras
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Schamberg's Disease
Purpura annularis telangiectodes of Majocchi Pigmented purpuric lichenoid dermatitis of Gougerot and Blum Eczematid-like purpura of Doucas and Kapetanakis Lichen Aureus |
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Schamberg's Dz -- aka? Describe.
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AKA, progressive pigmentary dermatosis of Schamberg
Most common pigmented purpura Most typical on lower legs in older men |
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Purpura annularis telangiectodes of Majocchi aka? Describe.
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AKA, Majocchi’s disease
Most common in adolescents or young adults (especially women) 1-3 cm annular plaques with telangiectasias and cayenne pepper petechiae in border Asymptomatic |
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PIgmented purpuric lichenoid dermatitis of Gougerot and Blum -- Describe
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Typically affect men 40-60 years of age
Lesions similar to Schamberg’s + lichenoid papules Asymptomatic |
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Lichen Aureus
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Usually solitary lesion on lower extremity
Patches with chronic rust- to purple-color and occasionally golden |
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What is: Hypergammaglobulinemic Purpura of Waldenstrom? What Immune complexes are usu seen? What dz is it assoc with? Describe the lesions. Tx?
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Characterized by presence of immune complexes of IgG or IgA RF
Associated with autoimmune diseases (Sjogren’s, RA, SLE) or rarely lymphocytic or plasma cell malignancies Recurring crops of macular hemorrhage (usually petechiae) or palpable purpura preceded by itch, tingling, or burning Treatment: Support stockings or ASA Avoid precipitating factors (ETOH & prolonged standing) |
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What is Gardner-Diamond Syndrome? Describe Lesions, length dz, histo? Clues to Dx?
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Clinical:
Psychiatric illness (depression, anxiety, personality disorders, etc.) Lesions characterized by sudden onset of painful, swollen and hemorrhagic bruises of variable sizes on any area of body Usually resolve in approx. 2 weeks (Original reports with these lesions at injection sites of patient’s own blood and other agents) Pathology: Edema with mild perivascular mononuclear infiltrate Clues to this diagnosis: atypical locations, geometric or peculiar morphology, lack of clear-cut precipitating factors |
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Mondor’s Syndrome of Superficial Thrombophlebitis: Describe clinical picture and assoc sxs. Etiology? Treatment?
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Chest pain followed by one or more visible or palpable cords over chest wall
Tender and painful with elevation of breast and ipsilateral upper extremity May see fever, arthralgias, erythema, and echymosis Etiology Trauma, excess activity, surgical procedure, infections, mastitis, or inflammatory conditions Treatment Heat, NSAIDs, rest, breast support Aching pain usually ends within 10 days (cord may persist for weeks or months and may be tender) |
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What CA is assoc with Mondor's Thrombophlebitis?
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Breast CA -- 12% of pts
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