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