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Old 10-20-2011
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Skin Red brown macules on back

A 40-year-old woman is referred to a dermatologist with more than 100 oval or round red-brown macules on her back. There is a positive Darier's sign. The dermatologist takes a skin biopsy, which is stained with toluidine blue. There are an excessive number of the metachromatically- stained cells labeled with the arrows and shown in the inset to the lower left in the photomicrograph below. Which of the following would be the most likely expected symptom in the patient?

Red brown macules on back-image1.jpg
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A. Inhibition of HCl production by parietal cells
B. Darkening of the skin
C. Osteoporosis
D. Anemia
E. Edema


I NEVER KNEW ABOUT THIS AILMENT TILL I WATCHED HOUSE LATEST SEASON
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Old 10-20-2011
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e) edema?

tol.blue -> mast cells
Darier's sign-> mast cells degranulation


3rd clue -> something dramatic must happen in House (bleeding, chocking..edema ...)
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Old 10-21-2011
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i go for E too but idk what is this thing
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Mastocytosis is a disease in which there is excessive production of mast cells by the bone marrow. The cells in the biopsy are mast cells that stain metachromatically (change color of the stain from blue to purple). Like basophils, they synthesize and secrete heparin and histamine. The result of mastocytosis is an excessive release of the bioactive products contained in mast cell granules: histamine, heparin, eosinophil chemotactic factor of anaphylaxis (ECF-A), slow-reacting substance of anaphylaxis (SRS-A), and leukotrienes. Mastocytosis induces urticaria pigmentosa (the skin condition from which the patient in the scenario suffers), including edema (caused by the increased vascular permeability induced by histamine and SRS-A). In mastocytosis, there is infiltration of eosinophils (attracted by ECF-A), which causes itching. Excessive production of acid by the parietal cells of the stomach (answer a) occurs because of the overstimulation of histamine receptors on these cells. This can result in peptic ulcers and gastritis. Lower GI tract symptoms include increased motility and diarrhea due to the stimulation by mast cell contents. Periportal fibrosis of the liver often occurs in systemic mastocytosis due to the extensive infiltration of mast cells into the liver. Melanocytes (answer b) are not affected. Anemia and osteoporosis (answers c and d) would occur in either multiple myeloma or plasmacytosis, where there are excessive numbers of plasma cells. The excessive production of plasma cells in the bone marrow disrupts normal hematopoiesis including the production of RBCs, causing anemia. Plasma cells release interleukins (IL-1 and IL-6) and tumor necrosis factor-alpha (TNF-) that stimulate osteoclastic activity and induce osteoporosis (see clinical case questions in the chapter on bone and cartilage).
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