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100 Cards in this Set
- Front
- Back
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neurofibromas, optic nerve gliomas, Lisch nodules(pigmented nodules of the iris), and café au lait spots(hyperpigmented cutaneous macules)
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neurofibromatosis type 1
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bilateral cranial nerve VIII schwannomas and multiple meningiomas
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neurofibromatosis type 2
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cutaneous facial angiomas + leptomeningeal angiomas; mental retardation, seizures, hemiplegia, skull radiopacities; "tram–track" calcifications
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Sturge–Weber syndrome(encephalotrigeminal angiomatosis)
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cappilary hemangioblastomas in the retina and cerebellum + congenital csts and neoplasms in the kidney, liver, and pancreas.
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Von Hippel–Lindau disease
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AD syndrome, characterized by cutaneous angiofibromas(adenoma sebaceum), visceral cysts,and variety of other hamartomas; renal angiomyolipomas and cardiac rhabdomyomas
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tuberous sclerosis
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short stature, short and thick neck(due to webbing), a broad chest, and shortened fourth metacarpals
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turner's syndrome
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most common congenital malformation affecting the heart in Turner's syndrome
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bicuspid aortic valve
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aortic ejection sound, which is an early systolic, high–frequency click heard over the cardiac apex and the right second interspace
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bicuspid aortic valve
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fixed splitting of the second heart sound; common with Down syndrome
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atrial septal defect
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mid–diastolic, low–pitched rumbling murmur that may begin with an opening snap
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mitral stenosis
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von Willebrand disease, lab findings
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Platlet count, PT, fibrinogen, blood smear – N;PTT – high
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immune thromb. purpura, lab findings
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Platlet count – low, PT, fibrinogen – N;PTT – N;blood smear – isolated thr.penia
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TTP–HUS lab findings
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low platelet count, N – PT, PTT, fibrinogen;smear: RBC fragmentation
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DIC, lab findings
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Low – platlet count and fibrinogen;RBC fragmentation;High PT, PTT
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DIC occurs due to...
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... exposure of blood to procoagulants that inititate intavasc. thrombosis, which is followed by compensatory thrombloysis.
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Result of DIC – ?
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Consumption of coagulation factors, coagulopathy, bleeding.
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ITP occurs secondary to..
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.. production of AB to platelets and megakaryocytes
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PT and PTT in ITP?
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normal
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spontaneous bleeding occurs when platlet count is less than..
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10000/ul
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thrombotic thrombocytopenic purpura and hemolytic uremic syndrome symptoms
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fever, CNS manifestations, renal failure, low thr count, microangiopathic hemolytic anemia.
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TTP occurs in..
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.. adults with predominant CNS symptoms.
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HUS occurs in..
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.. children with renal involvement
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ingerited bleeding disorder that presents with bruising and long bleeding from mucosal surfaces. impaired platlet adhesion
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von Willebrand d.
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cause of "chipmunk facies" in b–thalasemia
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Etramedullary hemopesis causes skeletal deformities and pathlogical fractures
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diagnostic test for sphrocytosis
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osmotic fragility test
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Splenic sequesration: cause. mechanism. findings;
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sickle cell d., splenic pooling of RBC due to vasooclusion; low Hg, splenomegaly, hypovolemic shock.
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Major basic protein. Where is found? function?
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in eosinophils.; defend against parasites
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TdT. (terminal deoynucleotidyl transferase).; Function?; What marker?
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adds nucleotides to the V, D, J regions of Ab gene.; marker of immature lymphocytes(B and T cells)
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Most common leukemia in children
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ALL TdT(+)
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Hairy cell leukemia; –stains?; –substrate?; –symptoms?
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–stains for tartrate resistant acid phosphatase(TRAP); –B–lymph precursor CD 20; –splenomegaly, fatigue, pancytopenia
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CD 20+ CLL
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Hairy cell leukemia
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Chronic myelomonocytic leukemia marker?
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Platlet derived growth factor receptor
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Hypersegmented neutrophils; Cause?
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B12/Folate dificiency
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Promyelocytic leukemia; FAB class?; morphology?; common complication?
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–M3; – promyelocytes with rod–shaped cytoplasm inclusions "Auer rods" and azurophillic granules; –DIC
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HER2/neu; function?;
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–oncogene, encodes 185–kD transmembrane glycoprotein that activates tyrosine kinase(growth factor receptor), leads to uncontolled epith. growth and differentiation
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Toll–like receptor function?
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induces cytokine release
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clathrin; function?;
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cytosol protein.; mediates endocytosis
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Follicular lymphoma morphlogy?
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B–cell tumor composed of centrocytes/blasts
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what t(14;18) gene expresess?
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bcl–2 overexpression – antiapoptotic agent
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BCR–ABL association
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t(9;22) – CML
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t(8;14) association
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c–myc translocation. Burckitt lymphoma.
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N–MYC association
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–neuroblastoma; –small cell carcinoma of the lung
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Myeloblasts; what stains for?
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auer rods stains for meyloperoxidase
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B–lymph marker
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CD 20
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T–lymph marker
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CD 3
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Vimentin?
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intermediate fillament in mesenchymal tissue
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Chromogranin A
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marker for neuroendocrin tumors
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cytokeratin
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intermediate fillament in epithelial tissue
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mycosis fungoides–?
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manifestation?; cutaneous T–cell lymphoma
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... lower risk of ovarian/endometrial cancer
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–oral contraceptives ;–multiparity; –breastfeeding
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glycogen stains for?
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schiff reaction
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most common indolent non–Hodgkin lymphoma in adults?
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follicular lymphoma.
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Schilling test need for?
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ID B12 deficiency
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Ovarian cancer risk factors?
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–infertility; –nulliparity; –miscarriage; –clomiphene citrate
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Burkitt lymphoma virus association
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EBV
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locations of diffuse large cell lymphoma?
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–Waldeyer ring; –GI tract
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most common leukemia in children
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ALL
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ALL findings?
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–lymphadenopathy; –hepatosplenomegaly; –vefer; –bleeding; –bonepain
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ALL subtrate?
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preB and preT lymphoblasts
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JAK2 mutation leads to?
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Polycythemia vera
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p53 mutation leads to?
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Li–Fraumeni syndrome, AD disorder; – predispostion to cancers
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K–ras?; Ca association?
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proto–oncogen. leads t high response to mitogenic stimuli.; bladder lung colon pancreas kidney cancer
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n–myc association
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neuroblastoma; small cell carcinoma
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ERB–B1 association
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squamous cell carcinoma of the lung
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ERV–B2 ass?
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breast/ovarian cancer
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TGFalpha ass?
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astrocytoma; hepatocellular carcinoma
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sis association?
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astrocytoma; osteosarcom
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BRCA–1/2 inactivation
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breast/ovarian CA
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NF–1 m. ass.
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neuroblastoma; neurofibromatosis type 1; sarcomas
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APC/b–cafenin m.ass.
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gastric, colonic, pancreatic CA; familial adenomatous poluposis coliDD
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DCC m.ass.
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colon cancer
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p–53?; function?
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anti–oncogen.;ass. Li–Fraumeni sydrome; blocks proliferation of cells with genetic abnormalities
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RB;function?
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anti–oncogen.;retinoblastoma;osteosarcoma;F: blocks G1>S phase of cell cycle
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WT
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anti–oncogen. wilms tumor
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VRCA–1 function?
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DNA repair
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High PTT = low ? factor
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VIII f
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High PT = low ? factor
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VII
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vit. K dependent factors?
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2 7 9 10
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coarse basophilic stippling on a background of hypochromic microcytic anemia
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lead poisoning
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lead inhibits …
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ALA dehydratase nad the resultant reduced incorporation of iron into heme; deceased Hg synthesis
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myeloblasts containing cytoplasmic Auer rods on peripheral blood smear are the characterisitc finding in
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acute myelogenous leukemia
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kaposi's sarcoma is strongly associated with
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human herpes virus type 8 infection in HIV+
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gastric adenocracinoma and muosa–asscoiated ymphoid tissue tumor are associated with
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helicobacter pylori infection
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cervical carcinoma is strongly associated with
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HPV type 16, 18, 31, 33 infection
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EBV infections is associated with
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Burkitt lymphoma and nasopharyngeal carcinoma
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main mediator of paraneoplastic cachexia
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TNF–alpha
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in bacterial infections, … produces fever along with IL –1, mediates many of the symptoms of septic shock and causes hepatic release of acute–phase reactants such as C–reactive protein and fibrinogen
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TNF–alpha
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synthsized by leukocytes and has antiviral and anti–tumor activity
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interferon–alpha
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mechanism of action of organophosphates
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inhibit cholinesterase ==> decrease degradation of ACh and increase concentration in the synaptic cleft, leading to overstimulation of the Rs
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clinical presentation of organiophosphate poisoning
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diarrhea; urintaion; miosis; bronchospasm; emesis; lacrimation; salivation
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pralidoxime use; mechanism of action
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oraganophsophate intoxication; restores cholinesterase from its bonds with organophosphates
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zolpidem –? mech of act
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short–acting hypnotic agent; bind to GABA–a R and enhance action of GABA on the CNS
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Zolpidem use
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short–term Tx of insomnia
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Zolpidem metabolized by ...
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liver P450 microsomal oxidases
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temazepam–?
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benzodiazepine
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estazolam–?
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benzodiazepine
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triazolam –?
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short–acting benzodiazepine
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flurazepam –?
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long–acting benzodiazepine
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mood stabilizers in bipolar disorder
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Li; Valproate; carbamazepine; lamotrigine
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indications for lithium in bipolar disroder
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manic and depressive episodes, maintence
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