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Oral pathology 3rd

Oral pathology midterm 3rd year

QuestionAnswer
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
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