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Oral pathology midterm 3rd year

Quiz yourself by thinking what should be in each of the black spaces below before clicking on it to display the answer.
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Question
Answer
Fibroma is a reactive hyperplasia of what type of tissue?   fibrous  
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Fibroma's most common location in mouth   along the bite line  
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Fibroma - surface texture and color   smooth and pale  
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Fibroma common size   1.5cm  
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Fibroma usually occurs in what decades?   4-6th  
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How is fibroma treated?   surgery  
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Giant cell fibroma clinical appearance and size   sessile or pedunculated, often papillary, usually less than 1cm  
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Giant cell fibroma represents what percent of oral proliferations   5%  
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Giant cell fibroma may be mistaken for what other lesion?   papilloma  
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Giant cell fibroma is most common in what decades?   first three  
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About 1/2 of all giant cell fibroma's occur where?   gingiva  
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Microscopic appearance of giant cell fibroma?   stellate fibroblasts and elongated rete pegs  
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Treatment of giant cell fibroma   surgery  
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Epulis fissuratum caused by?   inflammatory fibrous and epithelial hyperplasia or denture injury tumor  
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epulis fissuratum most common population affected?   older people and women  
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epulis fissuratum treatment   excision and denture modification  
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Inflammatory papillary hyperplasia is most commonly related to what three things?   1. denture doesn't fit 2. not cleaning denture or mouth 3. never taking dentures out  
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Inflammatory papillary hyperplasia looks like what other lesion under the microscope   SCC  
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How is inflammatory papillary hyperplasia treated?   surgically remove excess tissue, fix denture so it fits properly, make sure pt leaves denture out at night  
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Pyogenic granuloma most commonly seen on what tissue?   gingiva  
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Most common cause cause of pyogenic granuloma?   trauma  
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Appearance of pyogenic granuloma   red, usually ulcerated  
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Pyogenic granuloma's are most commonly seen in what population of people?   women, usually pregenant women  
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How is phogenic granuloma usually treated?   surgery  
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Peripheral giant cell granuloma - most common location and appearance   gingiva (always) and anterior to molars (usually), dark red, usually ulcerated  
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Peripheral giant cell granuloma is most common in what population of people?   women  
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What is the treatment for peripheral giant cell granuloma and how frequently do they recur?   surgery, 10%  
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Why are peripheral giant cell granuloma's so red?   they are very vascular, (contain osteoclast-like multineucleated giant cells)  
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Peripheral ossifying fibroma is only seen on what tissue? (just like what other lestion)   gingiva same as peripheral giant cell granuloma  
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What age group is affected most by peripheral ossifying fibroma?   teens and young adults  
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What sex is most commonly affected by peripheral ossifying fibroma?   women 2/3  
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What is the treatment and recurrence of peripheral ossifying fibroma   surgery, 16%  
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What is the clinical appearance of peripherial ossifying fibroma?   usually ulcerated, fibrous proliferation with reactive bone or other calicified tissue  
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A lipoma is a tumor of what tissue? benign or malignant?   fat, benign  
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Most common locations in the mouth for lipoma   buccal mucosa and vestibule, tongue, floor of mouth and lips  
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Common age for lipoma to occur?   40 and over  
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treatment of lipoma?   surgery  
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What causes a traumatic neuroma?   body attempts to repair a nerve  
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What symptom is frequently associated with traumatic neuroma?   pain  
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Where is the most common location for traumatic neuroma?   mental foramen  
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What is the treatment for traumatic neuroma?   surgey  
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Neurilemoma is made of what cell type?   Schwann cells  
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Clinical symptoms and appearance of a neurilemoma include?   painless with a well circumscribed border  
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What % of neurilemoma's occur in the head and neck   25-48%  
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Where do neurilemoma's most commonly occur?   tongue but can also occur in bone  
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Neurilemoma may be associated with what other lesion?   neurofibromatosis  
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What is the microscopic appearance of a neurilemoma   Encapsulated with Antoni A tissue and Verocay bodies  
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What is the treatment for neurilemoma?   surgery  
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Neurofibroma is composed of what type of nerve elements? Where is it located?   all nerve elements(antoni A tissue not seen), location - peripheral nerves  
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How does a neurofibroma present clinically?   Unencapsulated, painless, slow growing tumor  
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neurofibroma may be associated with what other lesion?   neurofibromatosis  
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What is the most common location for neurofibroma?   Skin, may also be seen in tongue, buccal mucosa and bone  
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What is the treatment for neurofibroma?   surgery  
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How is neurofibromatosis inherited?   autosomal dominant (1 in 3,000 births)  
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What is another name for neurofibromatosis?   von Recklinghausen disease of skin  
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What is the primary presenting factor for neurofibromatosis and why?   Deafness due to 8th cranial nerve involvement  
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What % of neurofibromatosis become malignant?   5%  
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What are the clinical signs/sx of neurofibromatosis?   multiple neurofibromas, colored spots, skeletal problems, 8th nerve problems (deaf)  
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Granular cell tumors are likely from what cells?   Schwann cells  
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What % of granular cell tumors occur in the tongue?   40%  
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What % of granular cell tumors are incompletely removed? recur? are multifocal?   50%, 10%, 10%  
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What % of granular cell tumors show pseudoepitheliomatous hyperplasia?   10%  
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Why is pseuroepitheliomatosus hyperplasia a concern with granular cell tumors?   Because it looks like SCC under the microscope  
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What sex is most commonly affected by congenital epulis?   women 90%  
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Where does congenital epulis usually occur?   maxillary gingiva (twice as common), usually anterior gingiva  
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What % of congenital epulis is multiple? recur? show pseudoepitheliomatous hyperplasia?   10%, Do not recur, Do not show pseudoepitheliomatous hyperplasia  
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Define hemangioma   benign proliferation of blood vessels, many are most likely hamartomatous  
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What % of kids have hemangioma's   12%  
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Where are hemangiomas most commonly found?   head and neck  
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What are the treatments for henangiomas?   may resolve on own, surgery, laser, criotherapy, embolization, sclerosing agents, pressure  
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Encephalotrigeminal angioatosis is also known as   Sturge-Weber angiomatosis  
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What areas are affected in sturge weber angiomatosis?   one side of face and meninges  
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What symptoms may appear in sturge weber angiomatosis?   convulsions, spastic hemiplegia  
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What is the radiographic appearance of sturge weber angiomatosis?   "tram-track" calcifications on skull films  
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What tissues are commonly involved in sturge weber angiomatosis?   oral mucosa and jaws  
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Vascular gingival proliferations of sturge weber angiomatosis can resemble what other lesion?   pyogenic granulomas  
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Lymphangiomas are tumors of? malignant or benign?   lymph vessels, benign  
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What % of lymphangiomas occur in the head and neck? what specific location?   75%, posterior triangle  
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What % of lymphangiomas develop by 2y/o   90%  
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Oral lesions of lymphangiomas are most common in what areas?   anterior 2/3 of tongue  
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What is the treatment for lymphangioma   surgery  
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Arbitrary classifications of lymphangiomas include   lymphangioma simplex, cavernous lymphangioma, cyctic lymphangioma  
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Cystic hygroma is caused by   markedly dilated lymph vessels  
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Cystic hygroma is most common in what areas?   head and neck  
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What is the mortality rate for cystic hygroma and common size?   2-6% mortality. No common size (may become massive)  
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Name a benign neoplasm of smooth muscle and one of striated muscle   smooth - leiomyoma, striated - rhabdomyoma  
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Name a malignant neoplasm of smooth muscle and one of striated muscle   smooth - leiomyoscrcoma, striated - rhabdomyosarcoma  
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How common is leiomyoma in the oral cavity? What areas are most commonly affected?   rare, uterus and GI tract  
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Most oral cases of leiomyoma are derived from what tissues?   blood vessels (vascular leiomyomas)  
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Are leiomyomas painful?   usually not but can be  
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What is the treatment for leiomyoma?   surgery  
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Leiomyoma is a benign neoplasm of what type of muscle?   smooth  
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Rhabdomyoma is a benign neoplasm of what type of muscle?   striated  
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Where are the most common locations for rhabdomyoma?   head and neck  
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What are the most common oral locations for rhabdomyoma?   Floor of mouth, tongue, soft palate, buccal  
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How does rhabdomyoma present clinically   multinodular or multifocal  
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What is the treatment of rhabdomyoma?   surgery  
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Fibrosarcoma is a malignant tumor caused by what cell type   fibroblasts  
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Where is the most common location for fibrosarcomas to occur?   extremities (10% occur in head and neck)  
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What area of the head is most frequently affected by fibrosarcoma?   nose and paranasal sinus (fibrosarcomas can occur anywhere)  
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What age group is most commonly affected by fibrosarcoma?   any age can be affected (most common in kids and young adults)  
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How does a fibrosarcoma usually present?   as a slow growing mass  
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What is the microscopic appearance of a fibrosarcoma?   spindled cells usualy in a "herringbone" pattern  
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What is the treatment for fibrosarcoma? recurrance? 5 yr survival   surgery, 20-60% recur, 40-70% survival at 5 yrs  
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Malignant fibrous histiocytoma is also known as?   pleomorphic sarcoma  
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What type of differentiation is present in malignant fibrous histiocytoma?   fibroblastic and histiocytic  
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What was the most common soft tissue sarcoma in adults at one time?   malignant fibrous histiocytoma (pleomorphic sarcoma)  
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What age group is affected by malignant fibrous histiocytoma?   usually older people  
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What areas are affected by malignant fibrous histiocytoma?   extremities and retroperitoneum. Rare in head and neck  
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How does malignant fibrous histiocytoma appear under a microscope? What is the treatment?   Several subtypes under microscope, tx /c surgery  
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What % of malignant fibrous histiocytoma results in recurance? mets?   40%, 40%  
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What tissue does a liposarcome come from?   fat  
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What is the 2nd most common malignancy of adults?   Liposarcoma  
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Where are the most common areas for liposarcoma?   thighs, retroperitoneum, inguinal. Rare in head and neck. (cheek most common area if it does make it to the head and neck)  
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How does a liposarcoma present clinically?   soft, ill defined, slow growing mass, without pain or tenderness  
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What is the treatment, recurrence and 5 yr survival for liposarcoma?   treatment - surgery, recur 50%, survival at 5 yrs 57-70%  
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Neurogenic sarcoma is a malignancy of what type of tissue?   nerve  
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Neurogenic sarcoma are associated 50% of the time with what other lesion?   neurofibromatosis  
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Where are the most common areas for neurogenic sarcoma?   proximal portions of extremities and trunk  
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Where are the most common sites for neurogenic sarcoma?   Mandible, lips and buccal mucosa  
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What is the average age of neurogenic sarcoma associated with neurofibromatosis? de novo?   neurofibromatosis - 29 y/o, de novo 40 y/o  
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What is the treatment for neurogenic sarcoma?   surgery, sometimes chemo and rad  
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What is the 5 yr survival for neurogenic sarcoma in neurofibromatosis? all others?   16%, for others 53%  
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How common is angiosarcoma? where does it occur?   rare, vascular endothelium  
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Where do 50% of cases for angiosarcoma appear?   head and neck  
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What age group is affected by angiosarcoma?   old people  
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How does angiosarcoma appear clinically?   bruise like to a ulcerated mass, may appear multifocal  
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How does angiosarcoma appear microscopically   crowded or piled up endothelial cells  
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What is the treatment for angiosarcoma?   surgery and or rad  
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What is 5 year survival for angiosarcoma?   12%  
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Define Kaposi's sarcoma?   unusual vascular neoplasm associated with HHV 8, usually multicentric  
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What are the four classical presentations of Kaposi's sarcoma?   classic, endemic, iatrogenic immunosuppression associated, AIDS related  
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What three stages does Kaposi's sarcoma evolve through?   Patch, Plaque, Nodular  
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What is the microscopic appearance of Kaposi's sarcoma?   granulation tissue type appearance to fibrosarcoma type spindle cell proliferation  
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What is the prognosis for Kaposi's sarcoma?   varies with clinical subtype and stage of disease  
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What subtype of Kaposi's sarcome is the chronic form? When does it occur? what sex is affected?   Classic, late in adult life, 90% men  
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What nationalities are most affected by Classic Kaposi's sarcoma?   Italian, Jewish, Slavic descent  
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How does Classic Kaposi's sarcoma present clinically?   slow growing red plaques with nodules on the skin of the lower extremities  
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People with classic Kaposi's sarcoma often get what malignancy?   lymphoreticular malignancy  
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What is the 8-13 year mortality rate for Kaposi's sarcoma?   10-20%  
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What are the four subtypes of African endemic type Kaposi's sarcoma?   benign nodular, agressive or infiltrative, florid, lymphadenopathic  
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How is benign nodular Kaposi's sarcoma of the African endemic type different from the Classic type   similar but benign nodular is found in young adults  
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What features define agressive african endemic type of Kaposi's sarcoma?   progress invasive development of the vascular neoplasm  
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What features define florid african endemic type of Kaposi's sarcoma?   rapidly progressive with wide spread, often with organ involvement  
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What group of people are primarily affected by lymphadenopathic subtype of Kaposi's sarcoma of the african endemic type?   young black children - generalized rapid growth of tumors of lymph nodes and organs  
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What groups of people are most affected by iatrogenic type Kaposi's sarcoma?   organ transplant patients (0.4% kidney transplant patients)  
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What causes the symptoms of iatrogenic type Kaposi's sarcoma?   probably loss of cellular immunity  
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What nationalities are affected most by iatrogenic type Kaposi's sarcoma?   similar to classic form (Italian, Jewish and Slavic)  
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Which is more aggressive classic or iatrogenic type Kaposi's sarcoma?   Iatrogenic  
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What is the most common type of Kaposi's sarcoma in the USA?   AIDS related  
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How does AIDS related Kaposi's sarcoma usually start? What areas are commonly involved?   lesion on skin or mucosa, -- trunk, arms, head and neck are commonly involved  
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What % of AIDS Kaposi's patients had oral lesions?   50%  
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What is the treatment for AIDS Kaposi's?   HAART and sometimes local chemo. The disease is progressive with wide distribution to lymph nodes and organ systems  
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Rhabdomyosarcoma is a malignant neoplasm of what muscle type?   striated  
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What is the most commmon soft tissue sarcoma of children?   Rhabdomyosarcoma  
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What are the three microscopic patterns of rhabdomyosarcoma?   embryonal (first decade), alveolar (16 y/o), pleomorphic (50's y/o)  
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Rhabdomyosarcoma is most common in what decade of life?   first  
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How does rhabdomyosarcoma present clinically?   painless, rapidly growing mass, may be botryoid (appear like a cluster of grapes)  
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What is the 5 year prognosis of rhabdomyosarcoma?   90% fatal before 1960, 63% survival rate at 5 years  
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Leiomyosarcoma is a malignant tumor of what type of tissue?   smooth muscle  
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The most common areas that leiomyosarcoma affects are?   uterus and GI tract, uncommon in other locations, rare in oral cavity  
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What age group is affected by leiomyosarcoma?   middle aged to older adults  
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How does leiomyosarcoma appear clinically?   nonspecific mass  
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What is the microscopic appearance of leiomyosarcoma?   spindle shaped cells with pink cytoplasm  
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What is the treatment for leiomyosarcoma?   surgery, sometimes chemo and rad  
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What is the prognosis for oral lesions of leiomyosarcoma?   poor  
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What is the most common nonepithelial tumor of major salivary glands?   hemangioma  
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Most common salivary gland tumor in what age group?   under 1 year  
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Most common site for hemangioma?   parotid gland  
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Average age for hemangioma   10 y/o  
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Sex most affected by hemangioma   female  
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Site where hemangioma commonly seen   soft mass in earlobe  
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What are the two histological types of hemangiomas?   juvenile 85%, cavernous 15%  
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What is the treatment for hemangioma?   90% resolve by age 7, postpone tx until school age, compression tx may work, surgery, laser, cryotherapy  
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Name two mesenchymal neoplasms   hemangioma, lymphoid lesion  
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benign epithelial neoplasm - mixed tumor is the most common __________ tumor?   salivary gland  
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What % of parotid and minor gland tumors are mixed?   60-70% parotid, 40-70% minor gland  
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What are the most common sites for mixed tumors?   palate 54%, upper lip 18%, buccal mucosa 11%  
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What is the average age for mixed tumors   41.2 y/o (30-50 y/o)  
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What is the female to male ratio for mixed tumors   1.9 to 1  
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How does a mixed tumor present clinically?   painless slow growning firm mass  
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What cells are considerer neoplastic in mixed tumors?   myoepithelial cells  
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What two types of microscopic components are present in mixed tumors?   epithelial and mesenchymal  
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What is the treatment for mixed tumors?   surgery, will recur if it isn't completely removed, may become malignant  
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Benign epithelial neoplasm - basal cell adenoma is most commonly found at what location?   parotid gland  
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What is the average age for basal cell adenoma?   57.7 y/o (peaks in 7th decade)  
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What is the F:M ratio for basal cell adenoma?   2:1  
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Basal cell adenomas have a clinical appearance similar to what other tumor?   mixed tumor - well circumscribed with capsule  
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Basal cell adenoma may be associated with what other tumors?   dermal appendage tumors (especially dermal cylindroma  
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What is the microscopic view of basal cell adenoma?   regular cell population in a broad rounded lobular pattern  
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What type of cells are found in basal cell adenoma?   cuboidal to slightly columnar with amphophilic cytoplasm and ovoid nuclei  
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What is the treatment for basal cell adenoma?   surgery  
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Benign epithelial neoplasms - papillary cystadenoma lymphomatosum is also known as   Warthin's tumor  
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Average age for papillary cystadenoma lymphomatosum to occur? F:M ratio? Race affected?   Age 57.3 y/o, 1.2:1 ratio, whites  
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What is the most frequent benign epithelial salivary gland tumor thatis commonly synchronous with another type of salivary gland neoplasm?   papillary cystadenoma lymphomatosum (Warthin's tumor)  
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How does Warthin's tumor usually present?   painless nodule at inferior pole of parotid next to angle of mandible  
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What cell types are found in Warthin's tumor?   tall columnar, eosinophilic, oncocytic, epithelial  
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What is the best treatment for Warthin's tumor   superficial parotidectomy  
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What is the recurrence for Warthin's tumor?   6-12% approximately  
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Name a benign neoplasm composed of a distinctive single cell type?   oncocytoma  
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Oncocytoma's make up what % of all salivary gland neoplasms? Where are they commonly located?   1%, most common in parotid, rare in minor glands  
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What is the average age of occurence for oncocytomas?   64 y/o  
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What sex do oncocytomas predominate   almost the same rate of occurence  
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What is the histological appearance of oncocytoma?   large well definded polyhedral or round cells with eosinophilic granular cytoplasm, a clear cell variant exists  
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What is the treatment for oncocytoma?   superficial parotidectomy with facial nerve preservation  
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Name a distinctive benign salivary gland neoplasm that is seen almost exclusively in minor salivary glands, especially the upper lip 73.5%   canalicular adenoma  
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What is the average of onset for canalicular adenoma?   65 y/o  
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psammona bodies are associated with what benign salivary gland neoplasm?   canalicular adenoma  
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Hemosideran may be seen in epithelial cells of patients who have what benign salivary gland neoplasm?   canalicular adenoma  
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Canalicular adenoma may be misdiagnosed as?   tubular variant of adenoid cystic carcinoma  
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Name the second most common salivary gland tumor and the most common malignant salivary gland tumor   mucoepidermoid adenocarcinoma  
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What age group is affected by mucoepidermoid adenocarcinoma?   Average age 43 y/o, under 20 y/o (44%), can affect anyone in 2nd to 8th decades  
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Where do mucoepidermoid adenocarcinomas occur?   54.4% major glands, 46.5% parotid, 41.7% minor glands, 4.3% jaws  
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What is the female predilection for mucoepidermoid adenocarcinoma?   64%  
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Mucoepidermoid adenocarcinoma may mimic what common oral lesion?   Mucoceles  
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What is the treatment ofr mucoepidermoid adenocarcinoma?   surgery, partial maxillectomy not indicated for low grade tumors  
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Where are adenocarcinoma NOS found?   66.2% major glands  
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What is the clinical presentation for adenocarcinoma?   single asymptomatic mass but 25% may complain of nerve pain, paralysis or numbness  
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What is the average age for adenocarcinoma?   55.6 y/o (75% occur in 4th to 8th decade)  
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What is the female predilection for adenocarcinoma?   55%  
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What is the prognosis for adenocarcinoma   varies on stage and grade of tumor (easily cured to lethal, low grade tumors are less common  
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Name a low grade adenocarcinoma?   actinic cell adenocarcinoma  
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acinic cell adenocarcinoma represents what % of all salivary gland neoplasms?   6.5%  
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Where do acinic cell adenocarcinomas occur? what is the F:M ratio   parotid, 59% Female  
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What is the average age of occurence for actinic cell adenocarcinoma   44 y/o (may occur at any age)  
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Histoligical presentation of polygonal cells with dark staining basophilic cytoplasmic granules represents what malignant tumor   acinic cell adenocarcinoma, usually well circumscribed  
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What is the treatment for acinic cell adenocarcinoma   superficial parotidectomy  
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What is the recurrence rate and % chance of malignancy of acinic cell adenocarcinoma?   12% recurrence, 8% mets  
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What tumor has the best prognosis for a malignant salivary gland neoplasm?   Acinic cell adenocarcinoma  
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Name a low grade adenocarcinoma that microscopically appears very invasive, only recognized since 1983, occurs almost exclusively in minor glands   polymorphous low grade adenocarcinoma  
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Where does polymorphous low grade adenocarcinoma usually occur   minor glands (58.6% palate, 18.7% upper lip, 16% cheek)  
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When are patient most likely to be affected by polymorphous low grade adenocarcinoma   about 60 y/o, most common in 6th, 7th and 8th decade  
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Polymorphous low grade adenocarcinoma must be distinguished microscopically from what other lesions?   adenoid cystic carcinoma and mixed tumor  
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name a common feature of polymorphous low grade adenocarcinoma?   neurotropism  
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What is the treatment for polymorphous low grade adenocarcinoma?   surgery, may recur, mets is unknown  
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Where do adenoid cystic carcinomas occur?   evenly split between major and minor glands, (major - parotid and submandibular evenly)(minor - palate most common)  
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What are the clinical symptoms of adenoid cystic carcinoma? what sex is most affected?   slow growing swelling often with pain and tenderness ; fixation to surrounding structures ; paralysis of the facial n. , 62% female  
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What are the three patterns of adenoid cystic carcinoma?   cribiform, tubular and solid  
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What is the treatment for adenoid cystic carcinoma?   surgery, difficult to get clean margins, add rad tx, distant mets after 15-20 yrs  
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Where do carcinoma ex mixed tumors develop?   Develop in a pre-existing benign mixed tumor  
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How common is carcinoma ex mixed tumor? average age or occurence, F:M ratio   2.2% of all salivary gland tumors, 56.4 y/o (64% occur in 6th to 8th decade, 55% Female  
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What are the clinical symptoms of carcinoma ex mixed tumor?   painless or painful mass, may develop facial palsy and fixation  
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Where do carcinoma ex mixed tumors occur?   80% major salivary glands (64.4% parotid) 11% in palate  
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What is the histological presentation of carcinoma ex mixed tumor?   poorly differentiated adenocarcinoma or undifferentiated carcinoma with residual benign mixed tumor, may also see the other forms of adenocarcinoma (epidermoid, mucoepidermoid and adnoid cystic)  
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What is the treatment for carcinoma ex mixed tumor?   wide surgical excision. high % of recurrence and distant mets  
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Lymphoid hyperplasia is caused by?   reactive enlargement of lymphoid tissue usually do to infection  
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What areas are affected by lymphoid hyperplasia?   lymph nodes, waldeyer's ring, oral cavity lymphoid aggregates  
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Tingible body macrophages are associated with what hematologic disorder?   lymphoid hyperplasia  
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What is the treatment for lymphoid hyperplasia?   not tx needed usually  
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Reactive lymph nodes usually affect what areas?   anterior cervical chain, parotid gland and buccal node  
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How do acutely inflamed lymph nodes usually present? Chronically inflamed?   Acute - enlarged, mobile, tender, soft. Chronic - enlarged, rubbery firm, nontender  
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Reactive lymph Nodes may be tough to distinguish from what?   lymphoma, HIV infection may also have similar nodes  
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What symptoms occur in hyperplasia of lymphoid aggregates of Waldeyer's Ring?   Enlarged tonsils - may touch midline. Tonsils should be symmetrical. They usually very in size. Largest size is in 2nd decade then atrophy begins  
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What areas is commonly affected by hyperplasia of oral lymphoid aggregates?   posterior lateral tongue (foliate papillitis), may also occur on floor of mouth and soft palate  
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What are the symptoms of hyperplasia of oral lymphoid aggregates? What disease can it mimic?   usually bilat symmetry, may be tender, not ulcerated. May mimic carcinoma due to location  
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Hemophilia A is a deficiency in what factor? How is it aquired? What test is used to confirm Dx?   Factor 8 deficiency, X-linked recessive, abnormal PTT  
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Hemophilia B is a deficiency in what factor? How is it aquired? What test is used to confirm Dx?   Factor 9 deficiency, X-linked recessive, abnormal PTT  
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von Willebrand's disease is aquired by? What tests are used to confirm?   autosomal dominant, abnormal platelets, bleed time and PTT  
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What is the most common type of anemia?   hemophilia A  
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How many people are affected by hemophilia A? How is it expressed? What level of function is OK?   1 in every 8,000 to 10,000 men. Variable expression, 25% normal factor 8 allows normal function, less than 10% factor 8 become symptomatic  
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What symptoms are seen in hemophilia A. How is it corrected?   severe bleeding. replace clotting factor 8.  
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Which bleeding disorder is most frequently inherited? How many people are affected?   von Willebrand's disease, 1 in every 800 to 1000 people  
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How severe is von Willebrand's disease? Which sex is most commonly affected?   usually mild; not clinically significant. Seen in men and women; autosomal dominant  
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What does von Willebrand's factor do?   aids adhesion of platelets at bleeding site and acts as a transport molecule for factor 8  
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What are the two causes of anemia?   Decrease in blood cell volume or decrease in Hgb  
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What can cause cell volume or Hgb to decrease?   altered iron metabolism, megaloblastic anemia, hemolytic anemia, Hgb disorders  
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What are the symptoms of anemia?   Decreased ability of blood to carry O2, may see pallor of the mucous membranes  
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Sickle Cell anemia is a genetic disorder that affects what?   beta globin chains of Hgb causing sickling of RBC during time of low O2 tension  
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In heterozygous patients for sickle cell, what % are carriers?   50%, homozygous patients have the disease  
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What % of blacks are carriers of sickle cell? How many blacks are born with the disease   8%, 1 in every 350  
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What causes the damage in sickle cell disease?   vascular obstruction  
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What characterizes thalassemia?   reduced production of alpha or beta globin chains of Hgb, normal amount of Hgb can't be made  
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What is the clinical progression of thalassemia?   Excess globin chains accumulate in RBCs, the abnormal cells are destroyed in spleen results in hypochromic microcytic anemia  
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What is the benefit for sickle cell anemia and thalassemia   may be more resistant to malaria  
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What is the difference between beta thalassemia minor and major?   Minor - one defective gene; no significant clinical problems. Major - cooley's anemia; major clinical problems, extremely fragile RBCs, Mx and Md enlargement, "hair on end" skull films, die by one year due to infection or heart failure  
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How do the number of genes affected in alpha thalassemia affect clinical presentations?   One gene - no problem, Two genes - mild anemia; not significant, three genes - hemolytic anemia and spleenomegally, Four genes - hydrops fetalis, die within a few hours at birth  
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How many genes affected in hydrops fetalis, Hb H disease, alpha-thalassemia trait   4,3,2  
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What lab value is consistant with neutropenia? Causes?   1500/mm3 neutrophils, malignancy affecting bone marrow, drugs, viral and bacterial infection  
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How does neutropenia affect the oral cavity?   pus and abscess formation may not happen, oral lesions or ulcers usually on the attached gingiva  
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Name a condition where the cells of the granulocytic series are missing?   agranulocytosis  
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What usually causes agranulocytosis?   drugs, bacterial infection  
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How does agranulocytosis usually present in the oral cavity?   "punched out" ulcerations and necrotizing gingivitis  
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How common is cyclic neutropenia? What are the lab symptoms and when to they start?   rare and idopathic, cyclic reduction in neutrophils (21 days), very low counts for 3-6 days in cycle, symptoms begin in childhood  
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What are the physical symptoms of cyclic neutropenia? When do symptoms improve?   fever, anorexia, cervical lymphadenopathy, malaise, pharyngitis, oral ulcers and severe periodontal bone loss. Symptoms deminish in 2nd decade but cycling does not  
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What is the normal count for platelets? What is the name for the condition with below normal platelets?   200,000-400,000/mm3 platelets, thrombocytopenia, symptoms begin below 100,000/mm3  
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What causes thrombocytopenia?   reduced production, increased distruction (spleen)  
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What are the physical symptoms of thrombocytopenia?   petechiae, ecchymosis and/or hematoma, spontaneous gingival hemorrhage often occurs  
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What is the name for the overproduction of RBCs at 2-3 times the normal rate?   Polycythemia Vera  
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How common is polycythemia vera? Age affected? sex predilection?   Rare, idiopathic hematologic disease, 60 y/o, no sex predilection  
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What are the physical symptoms of polycythemia vera?   Increased blood viscosity, thrombus formation, ruddy complection, generalized puritus, erythromelagia (buring sensation in extremities), 2-10% develop acute lukemia  
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What is the treatment for polycythemia vera?   Blood letting  
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Leukemia is a malignancy of?   bone marrow stem cells, may be acute or chronic  
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How do acute and chronic lukemias differ?   Acute = aggressive, chronic = mild  
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What causes the symptoms of lukemia?   normal blood components are displaced by malignant cells = anemia, thrombocytopenia and increased infections  
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Reed sternberg cells are associated with what malignancy?   hodgkins disease  
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What sites are most commonly affected in the initial presentation of hodgkins disease? Age?   supraclavicular nodes and cervical nodes (70-75%), 15-35 y/o and after 50 y/o  
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Which is more common, non hodgkins or hodgkins?   Hodgkins is about 1/5 as common  
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How many subtypes are there for hodgkins?   four  
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non hodgkin's lymphoma is a diverse group of ____________ malignancies?   lymphoreticular  
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How do non Hodgkin's lymphomas present clinically? Which series is most common?   Form in lymph nodes as solid masses, B-lymphocyte series is most common  
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How many cases of non Hodgkin's lymphoma every year?   40,000 cases a year (half that # will die), usually seen in adults, most common in patients with immune problems, prognosis depends on grade  
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oral lymphoma may affect what tissues?   soft tissue or bone  
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What is the clinical presentation of oral lymphoma?   nontender diffuse swellings at buccal vestibule, gingiva and posterior hard palate, vague pain, jagged or ill defined radiolucenty, uniformly neoplastic lymphocytes.  
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Who is the most cuteist girl ever in the WWW   Rhonda  
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