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190 Cards in this Set
- Front
- Back
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Anemia
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Causes of Anemia
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Symptoms of anemia
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[makes you tired, light-headed, pale, gives you a headache and racing heartbeat] |
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Pathology/etiology of sickle cell anemia
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Precipitating factors of sickle cell anemia
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Oral manifestations of sickle cell anemia
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Prognosis for sickle cell anemia
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-variable
-disability -decreased lifespan -90% survival age 20 -50% survival age 50 |
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Clinical features of sickle cell anemia
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List some ways in which sickle cell anemia can result in vasoocclusive crisis
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What causes rare and life-threatening aplastic anemia?
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Which type of anemia involves all hematopoietic lineages?
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aplastic anemia
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Etiology of aplastic anemia
(What causes pancytopenia and failure of hematopoietic stem cells to differentiate?) |
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Clinical findings associated with aplastic anemia
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pancytopenia -> total marrow shutdown no platelets = thrombocytopenia; bleeding disorders no WBC = bacterial/fungal infection no RBC= anemia;fatigue / weight loss |
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Management of aplastic anemia
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Mortality of aplastic anemia
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80%
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Pathological features of neutropenia
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Etiology of neutropenia
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Management of neutropenia
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-granulocyte colony-stimulating factor (G-CSF)
-antibiotics -oral hygiene: chlorohexidine rinses |
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Which hematologic disorder is managed with granulocyte colony-stimulating factor ?
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neutropenia
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What is thrombocytopenia?
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bleeding disorder characterized by a decreased number of circulating PLATELETS (prolonged bleeding time, normal PT and PTT)
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What causes thrombocytopenia?*
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What might cause increased destruction of platelets leading to thrombocytopenia?
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immune reaction
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What might cause reduced production of platelets leading to thrombocytopenia?
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malignancy (leukemia, for example, fills up an area with blood causing a DECREASE in other things), toxins, drugs
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What might cause increased consumption of platelets leading to thrombocytopenia?
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intravascular thrombi
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What might cause sequestration of platelets leading to thrombocytopenia?
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splenomegaly
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Clinical features of thrombocytopenia
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What is leukemia?
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malignant neoplasm of hematopoietic stem cells (white cells of lymphoid or myeloid origin)
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Leukemia can be either _________ or __________ and of _______ or ________ origin.
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acute; chronic
lymphoid; myeloid |
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What causes leukemia?
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Symptoms of leukemia
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Oral findings of leukemia
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What is Langerhans cell histiocytosis?
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idiopathic histiocytic proliferation common in children and young adult men!
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Clinical features of langerhans cell histiocytosis
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Radiographic ft of langerhans cell histiocytosis
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Histology of langerhans cell histiocytosis
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Tx of langerhans cell histocytosis
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surgical excision - curettage
radiotherapy chemotherapy |
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Systemic involvment of LCH
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Which hematopoietic disorder mimics periodontal disease and pulpal/periapical pathology?
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Langerhans cell histiocytosis
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Pt. presents with periodontal disease and eosinophils. Likely diagnosis?
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Langerhans cell histiocytosis
("floating teeth") |
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What is Hodgkin's lymphoma?
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Hodgkin's lymphoma exhibits what type of spread and presents where?
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contiguous spread - CONTINUOUS
CERVICAL NODES (70-75%); never oral cavity (non-tender enlargement) |
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What is the likely diagnosis of a child that presents with fever, night sweats, weight loss, nontender palpable cervical nodes and pruritus?
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Hodgkin's lymphoma
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Symptoms of Hodgkins lymphoma?
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Treatment for Hodgkin's lymphoma?
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Non-hodgkin's lymphoma constitutes a diverse group of__________ malignancies characterized by__________ proliferation; 85% are ____ neoplasms.
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lymphoid; monoclonal; B-cell
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Etiology of ________________ includes immunosuppression, autoimmunity (Sjogren's), and viruses.
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non-hodgkin's lymphoma
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Which two heme dx are characterized by floating teeth?
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1. Langerhan's cell histiocytosis
2. Burkitt lymphoma |
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_________ arise in bone marrow and circulate in peripheral blood while _________ arise in lymph nodes and may involve extranodal sites.
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leukemias; lymphomas
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Chronic lymphocytic leukemia (CLL) involves ________ blood
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peripheral
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Small lymphocytic lymphoma (SLL) involves _____ ______
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lymph nodes
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Clinical presentation of lymphoma
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B-cell malignant lymphoma
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Burkitt lymphoma
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Burkitt's lymphoma is associated with ...
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histopathology of Burkitt's lymphoma
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Diffuse, small non-cleaved lymphocytes with macrophages in a starry sky pattern |
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Agressive malignancy, death 4-6 mo. requires chemotherapy; Associated W/ HIV infection & has the fastest doubling time of any known tumor
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Burkitt lymphoma
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______ ________ is a ___________ plasma cell malignancy mainly involving _______ _______.
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multiple myeloma; multicentric; bone marrow
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ASL of multiple myeloma
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Black male
60-70 multicentric bone-marrow involvement |
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Signs/symptoms of multiple myeloma in older black men*
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* |
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__________ is a ________plasma cell malignancy usually in spine and is _______________ with 90% occuring in ___________.
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plasmacytoma; UNIFOCAL; extramedullary; SOFT TISSUE OF HEAD AND NECK
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A "localized form" of myeloma...
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plasmacytoma
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If a __________ presents as a single lesion in bone (usually spine) 50% will become ________.
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plasmacytoma --> myeloma
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For which is the prognosis better: extramedullary (soft-tissue) plasmacytoma or unifocal bone plasmacytoma?
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osseous ... 50% disseminate in 2-3 years (compared to only 30% in soft-tissue)
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___________ is characterized by deposition of extracellular proteinaceous material and comes in two forms: __________ and __________.
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amyloidosis: (1) organ-limited and (2) systemic
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What are the 4 subtypes of systemic amyloidosis?
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1. primary (myeloma-associated)
2. secondary 3. hemodialysis-associated 4. heredofamilial |
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Primary/myeloma-associated amyloidosis primarily affects ______ ______ and is characterized by skin/oral lesions.
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older men
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What biochemical entity is associated with primary amyloidosis?
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AL amyloid light chain protein
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What are the clinical features of primary amyloidosis?
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Elderly male presents with nonspecific symptoms (weight loss, fatigue, paresthesia)
-skin and oral lesions -macroglossia -dry eyes and mouth -AL amyloid light chain protein |
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What causes dry mouth and eyes in primary amyloidosis?
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amyloid deposits in the connective tissue constricts the glands associated with moistening our eyes and mouths
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________ amyloidosis is associated with chronic inflammatory diseases: osteomyelitis, tuberculosis and sarcoidosis and Amyloid-associated antibody (AA)
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Secondary amyloidosis
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Hemodialysis associated amyloidosis is characterized by an accumulation of _____________ in ______ and _______.
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beta-2-microglobulin in bone and joints
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Patients with heredofamilial amyloidosis usually present with
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polyneuropathies
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Histology of amyloidosis
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extracellular eosinophilic material stains with congo red
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Death from primary amyloidosis is usually from __________ failure
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cardiac
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Death from secondary amyloidosis is usually from _________ failure
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renal
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Most common cause of anemia in the US
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iron deficiency
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Iron deficiency is most commonly due to
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-increased blood loss (menstruating women)
-increased demand (childhood/pregnancy) -decreased iron intake -decreased absorption of iron |
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Clinical ft. of anemia
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What are the lab findings of iron-deficient anemia?
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Plummer-Vinson (Paterson Kelly) Syndrome is a triad of
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Classic pt. presentation of Plummer-Vinson syndrome
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middle-aged women
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Plummer-Vinson Syndrome is considered a _________________ process due to an association with increased incidence of ____________
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premalignant; oral and esophageal squamous cell carcinoma
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What are the oral manifestations of Plummer-Vinson Syndrome?
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burning mucosa, angular cheilitis, atrophic glossitis
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______________ is a type of megaloblastic anemia due to lack of _________. Autoimmune reaction that destroys parietal cells in the stomach.
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pernicious anemia; intrinsic factor
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What age group is usually affected by pernicious anemia
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old people
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What is a distinguishing feature of pernicious anemia?
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paresthesia - tingling, numbness of the extremities
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Lab findings of pernicious anemia
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macrocytic anemia (decreased B12)
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What anemia is associated with increased risk of gastric carcinoma?
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pernicious anemia
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__________ is due to excess production of growth hormone by a functioning _________ __________after closure of the epiphyseal plate
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acromegaly; pituitary adenoma
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Oral manifestations of acromegaly
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-mandibular prognathism
-anterior open bite -spacing between teeth -macroglossia |
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Hypothyroidism is called _______ during childhood and _________ during adulthood.
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childhood: cretinism
adulthood: myxedema |
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What's the difference between primary and secondary hypothyroidism?
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primary - thyroid gland isn't secreting enough hormone
secondary - pituitary gland isn't secreting TSH hormone |
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Clinical ft. of hypothyroidism
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-goiter
-lethargy -cold intolerance |
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Oral manifestations of hypothyroidism
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-swollen lips
-macroglossia (GAG deposition) -failure of tooth eruption |
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Laboratory findings for hypothyroidism
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T3 and T4 decreased
primary: TSH is increased secondary: TSH is decreased |
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Tx for hypothyroidism
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levothyroxine
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Excess production of thyroid hormone that increased BMR
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hyperthyroidism
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65% of cases of hyperthyroidism are due to ___________ disease
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Graves
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In hyperthyroidism, auto antibodies directed against _______ receptors on thyroid cells cause excess release of ___.
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TSH; T4
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Causes of hyperthyroidism
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1) functioning thyroid tumor
2) pituitary adenoma that produces TSH |
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Laboratory findings for hyperthyroidism
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T4 increased
TSH decreased |
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Primary hyperparathyroidism occurs due to uncontrolled production of ____ by functioning cells.
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PTH
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Secondary hyperparathyroidism is due to __________ from chronic renal disease.
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hypocalcemia
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Classic pt. affected by hyperparathyroidism
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older female
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"stones, bones, and abdominal groans"
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clinical triad of hyperparathyroidism
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Manifestation of hyperparathyroidism bone
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ground glass opacification; loss of lamina dura due to calcium leaching
brown tumor |
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What is a 'brown tumor'?
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large, multilocular radiolucency leading to cortical expansion; associated with hyperparathyroidism
called "osteitis fibrosa cystica" if multiple bones are affected |
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This histology of hyperparathyroidism involves vascularized fibrous CT interspersed with __________________
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multinucleated giant cells
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What are the two forms of Addison's disease?
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primary - decreased corticosteroid production due to destruction of adrenal cortex
secondary - decreased ACTH due to pituitary abnormality |
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Clinical ft. of Addison disease
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90% of adrenal cortex must be destroyed before symptoms occur
-fatigue, depression, weakness, hypotension -bronzing of the skin |
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Oral manifestations of Addison disease
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-brown macular pigmentation (gingiva and labial mucosa)
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Treatment for Addison's disease:
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increase steroid dose before a stressful event such as perio/oral surgery
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Inflammatory disease that affects distal portion of small bowel and proximal colon
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Crohn's disease
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Teenager with apthous-like ulcerations in the depth of the vestibule; cobblestone appearance and metallic taste
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Crohn disease
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Describe the vestibular ulcers associated with Crohn disease
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apthous-like
linear hyperplastic, elevated borders (hotdog) |
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Inherited condition in which 2+ ectodermally-derived structures fail to develop
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ectodermal dysplasia
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Ectodermal dysplasia is ___ linked
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x-linked inheritance
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Clinical ft. of ectodermal dysplasia
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Due to defect in normal keratinization of oral mucosa
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white sponge nevus
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inheritance of white sponge nevus
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auto dominant
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Baby or young child presents with bilateral hyperparakkeratosis and acanthosis on the buccal mucosa
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white sponge nevus
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Histology of white sponge nevus
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- hyperparakeratosis and acanthosis
-eosinophilic consdensation in perinuculear region of epithelial cells |
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___________ is a type ___ hypersensitivity/ autoimmune disease that affects the skin and mucous membranes
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pemphigus; type 2
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Autoantibodies in pemphigus are directed against ....
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desmoglein 3 and and desmoglein 1 (the glue that holds the epithelial cells together
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Oral manifestation of pemphigus:
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blister -> ulcer -> crust
desquamative gingivitis positive Nikolsky sign |
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Histopathologic features of pemphigus
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Treatment for pemphigus
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systemic corticosteroids + immunosupressive drugs
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Cicatricial pemphigoid is chronic blistering, mucocutaneous autoimmune disease that is a type ___ hypersensitivity that
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II
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Which is most common in middle aged WOMEN, pemphigus or pemphigoid?
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pemphigoid
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Which has ocular manifestations in addition to desquamative gingivitis, pemphigus or pemphigoid?
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pemphigoid (symblepharon, entropion, trichiasis)
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What is the pattern of IgG and C3 in pemphigus? pemphigoid?
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pemphigus: chicken wire pattern
pemphigoid: linear band at BM |
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________ ________ is a blistering, ulcerative mucocutaneous condition of uncertain pathogenesis with 50% of cases being associated with herpes simplex or mycoplasma pneumoniae and is NOT autoimmune in nature
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erythema multiform
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Who is most likely to present with eythema multiform?
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young adult male!
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'Erythema multiform major' (AKA ______-_______ syndrome) is usually triggered by a _____ rather than by __________; _________ may occur.
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Stevens-Johnson; drug not infection; symblepharon
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What is the most severe form of erythema multiform?
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Toxic epidermal necrolysis
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Who's most likely to present with toxic epidermal necrolysis?
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OLD WOMAN with severe penicillin allergy (almost always triggered by drug)
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_________ _________ (geographic tongue) is a common, benign condition that may represent a hypersensitivity to an an environmental factor and usually affects ________
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Erythema migrans; women
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Clinical ft. of erythema migrans
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Histopath of erythema migrans
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Munro abscesses - neutrophils in the superficial epithelium
hyperparakeratosis, spongiosis, acanthosis, elongation of rete ridges |
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_________ _______ is a common, chronic, dermatologic disease that often affects oral mucosa and is an autoimmune, T-cell mediated process and has a female predilection
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Lichen Planus
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Cutaneous lichen planus is characterized by what 3 P's?
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Purple
Pruritic Polygonal papules |
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Wickham's striae
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white line associated with cutaneous lichen planus or reticular oral lichen planus
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3 forms of oral lichen planus
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1. reticular (asymptomatic - thick keratin)
2. erosive (painful; white, radiating bands) 3. desquamative gingivitis |
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Most common location for lichen planus
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buccal mucosa
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lichen planus can resemble ....
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tertiary syphilis
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Histopathology of lichen planus
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- orthokeratosis or parakeratosis
-"Saw tooth" rete pegs - hydropic degeneration of the basal cell layer |
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immunologically mediated multisystem disease; a type III hypersensitivity
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Lupus erythematosus
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3 types of lupus erythematosus
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1. systemic lupus erythematosus
2. chronic cutaneous lupus erythematosus 3. subacute cutaneous |
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Characteristics of systemic lupus
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- 31 yo female
- butterfly rash over malar/nose area - kidney failure |
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oral lesions associated with chronic cutaneous lupus erythematosus are similar to those seen in what other dermatological disease?
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erosive lichen planus ; but lupus is NOT bilateral whereas lichen planus IS
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the black patches associated with chronic lupus arise to the destruction of _______ ______ _____ and subsequent melanin incontinence
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basal cell layer
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diagnosis of lupus erythematosus
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- shaggy or granular band of IgM , IgG or C3 at the basement membrane zone
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What are the direct immunofluoresence findings for Lichen Planus?
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linear band of fibrin at BM (t-cell mediated)
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IF findings in lupus
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shaggy band of IgG, IgM, C3 similar to pemphigoid
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An immunologically mediated disease characterized by dense collagen deposition in tissues; typically in adult females
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systemic sclerosis
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Sclerodactyly /clawlike fingers associated with
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systemic sclerosis
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oral manifestations of systemic sclerosis
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-microstomia
-xerostomia -diffuse widening of the PDL space |
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Diagnosis of systemic sclerosis
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Anti-SCl 70 (topoisomerase)
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Treatment for systemic sclerosis
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D-penicillamine (inhibits collagen production)
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CREST usually affects ...
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eldery women
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CREST is an acronym for ...
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Calcinosis cutiss
Raynaud's phenomenon Esophageal dysfunction Sclerodactyly Telangectasia |
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Lab finding of CREST
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anticentromere antibodies
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Degenerative condition associated with atrophic changes on one side of the face; a localized form of scleroderma
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progessive hemifacial atrophy
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"Coup de sabre"
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"strike of sword" linear scleroderma on midline of forehead; associated with progressive hemifacial atrophy
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Clinical presentation of progressive hemifacial atrophy
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Oral manifestation of PHFA
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unilateral atrophy of the tongue
mouth/nose deviation unilateral posterior open bite |
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premature closure of the cranial sutures
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Crouzon syndrome
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Crouzon syndrome (premature closure of the cranial sutures) exhibits __________ __________ inheritance and is positively correlated with increasing____________ _____.
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autosomal dominant; paternal age
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Ocular protrusion, hearing loss, occlusal abnormalities, crowding, midface hypoplasia all characterize ________ syndome.
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Crouzon
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Defects in structure derived from 1st and 2nd branchial arches; autosomal dominant trait associated with increased paternal age
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Treacher Collins syndrome
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Clinical features of Treacher-Collins
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narrow face
downward slanting eyes hearing loss hypoplastic zygomas coloboma (75%) -notch in lower eyelid |
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Oral manifestations of TC syndrome |
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Auto-recessive trait associated with infection with A. actinomycetemcomitans
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Papillon-LeFevre syndrome
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Derm. manifestations of Papillon-LeFevre syndrome
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diffuse keratosis on the palms, soles, dorsa of hands and feet, elbows and knees
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Oral manifestations of papillon-lefevre
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advanced periodontitis (primary and secondary dentitions)
floating teeth exfoliation of teeth |
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Tx for papillon-lefevre
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antibiotics: amoxicillin + clavulanate potassium
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Sturge-Weber Angiomatosis is a devlopmental condition characterized by _________ ___________ of face and brain.
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vascular hamartomas
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Clinical ft. of Sturge-Weber
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unilateral port wine stain or nevus flammeus on the face distributed along one or more segments of the trigeminal nerve
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Sturge-Weber syndrome is associated with ________ ________ in ipsilateral cerebral cortex and can lead to mental retardation if not detected
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leptomeningeal angiomas
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defects that primarily involve the skull and clavicles
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cleidocranial dysplasia
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occular hypertelorism (wideset eyes), broad nose base, midface hypoplasia, mobile shoulders due to hypoplasia/absence of clavicles
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cleidocranial dysplasia
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oral manifestations of cleidocranial dysplasia
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-high palatal arch
-retained primary teeth -unerrupted secondary teeth - supernumerary teeth |
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painless bilateral expansion of the posterior mandible is characteristic of which syndrome in children?
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Cherubism
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Radiographic ft. of cherubism
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bilateral multilocular expansile radiolucencies*
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Histology of cherubism
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Freckle-like lesions in periorificial areas are associated with ...
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Peutz-Jegher's syndrome
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Hamartomatous intestinal polyps in illeum and jejunum can lead to intestinal obstruction in which syndrome ? (1-2% of pt. develop gastric carcinoma)
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Peutz-Jegher's syndrome
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Hereditary Hemorrphagic telangiectasia is a mucocutaneous disorder that resembles which other syndrome?
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resembles Peutz-Jegher's but the macules are red and not blue-brown
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Hereditary Hemorrphagic telangectasia is usally initially diagnosed due to
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epistaxis
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A group of inherited CT disorders; 10 types all characterized by production of abnormal collagen
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Ehlers-Danlos
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clinical ft. of Ehlers-Danlos
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hypermobility of joints, easy bruising, elasticity of skin, poor wound healing
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