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68 Cards in this Set
- Front
- Back
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Early HSC development
A. initial site of HSC B. common lymphoid progenitor gives C. morphology of bone marrow |
A. liver
B. T cell, B cell, NK C. thin walled sinusoids lined by endothelial cells - on discontinuous BM - Red cell precursors surround macrophages (nurse cells) that give iron |
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A. Normal wya to estimate marrow activity
B. hypoplastic state ratio C. in tumors, ratio |
A. fat cell to hematopoietic elements = 1:1
B. fat cells increase C. fat cells disappear |
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Disorders of White Cells
A. Proliferative disorders: 2 types B. leukopenia: most common type |
A. reactive; neoplastic
B. neutropenia |
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Leukopenia: neutropenia
A. def: neutroopenia B.. def: agranulocytosis C. sx of neutropenia D, sx of agranulocytosis E. morphology |
A. red in # of neutrophils
B. clinically signficant reduction in neutrophils C. malaise, chills, weakness, fever D. infection = agranulocytic angina (any affecting oropharynx) - deep fungal infxn (Candida and aspergillus) E. hypercellular marrow |
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Neutropenia
A. pathogenesis (2) |
A. inadq granulopoiesis: suppression of HSC
- suppression of committed granulocytic precursors - ineffective hematopoiesis - congenital conditions (Kostmann syn) B. Accelerated removal - immunologically mediated - splenomegaly - incr peripheral utilization |
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Reactive (Inflammatory) Proliferation
A. name two |
- leukocytosis
- lymphadenitis: acute & chronic nonspecific |
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Leukocytosis pathogenesis
A. incr production in marrow B. incr release from marrow stores C. decr margination D. decr extravasation into tissues |
A. chronic infection
- paraneoplastic (Hodgkin) - myeloproliferative (chronic myeloid leukemia) B. endotoxemia, infxn, hypoxia C. exercise, catecholamines D. glucocoritcoids |
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Leukocytosis: types
A. neutorphilic B. eosinophilic C. Bsophilic D. monocytosis E. lymphocytosis |
A. bacterial, pyogenic, tissue necrosis (MI)
B. allergic disorders (asthma); skin (pemphigus, dermatitis herpetiformis), malignancies (Hodgkin, non-Hodgkin) C. myeloprolifeative (myeloid leukemia) D. chronic infxn, SLE, mlcerative colitis E. viral infxn, Bordetalla pertusis |
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Reactive changes in neutrophils
A. toxic granulations B. Dohle bodies C. cytoplasmic vacuoles |
A. coarser, darker than normal primary granules
B. patches of dilated ER |
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Acute nonspeicifc lymphadenitis
A. morphology |
- primary follicles enlarge = germinal centers where B cells develop
- for pyogenic infxn: follicles necrosis |
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Chronic nonspecific lymphadenitis
A. morphology of Follicular hyperplasia B. "" paracortical hyperplasia C. "" sinus histiocytosis (reticular hyperplasia) |
A. germinal centers (secondary follicles) = light zone of centrocytes
- surrounded by resting naive B cell (centroblasts) = mantle zone B. stimuli trigger T cell mediated repsonses = T cellzone efface B cell folicles C. incr in # & size of cells that line lymphatic sinusoids = draining cancers (breast carcinoma) |
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Neoplastic WBC Proliferations
A. lymphoid neoplasms B. myeloid neoplasms C. histiocytoses |
A. B-cell, T-cell, NK cell origin
B. acute myeloid leukemia (immature progenitor accum in bone marrow) - myelodysplastic syndromes: ineff hematopoiesis = cytopenias - chronic myeloproliferative: incr prod terminally myeloid (granulocytes) C. prolif lesions of macrophges and dendritic cells |
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Lymphoid Neoplasms
A. leukemia def B. Lymphoma def C. Lymphoma categories D. Lymphoma features (2) |
A. neoplasms with widesprad involve of bone marrow
B. arise from discrete tissue masses C. Hodgkin lymphoma: spread in orderly fashion - plasma cell neoplasms D. most B cell origin - antigen receptor gene rearrange before transformation |
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Neoplasms of immature B and T cells
A. B cell acute lymphoblastic leukemia/lymphoma B. T cell acute lymphoblastic leukemia/lymphoma |
A. in children -> pancytopenia
B. adolescent males --> thymic masses |
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Neoplasms of mature B cells
A. Burkitt lymphoma B. Diffuse large B cell lymphoma C. Follicular lymphoma D. Hairy cell leukemia E. Mantle cell lymphoma F. multiple myeloma G. Chronic lymphocytic leukemia |
A. t(8;14) -> c-MYC --> young adults with extranodal mass
B. rapidly growing masses C. t(14;;18) -> adults with generalized lymphadenopathy D. memory cells --> males w/ pancytopenia & splenomegaly E. t(11;14) -> males with disseminated disease F. lytic bone lesions, hypercalcemia, renal failure G. bone marrow, lymph node, spleen, liver disease |
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Neoplasms of mature T cells/NK cells
A Adult T cell leukemia/ lymphoma B. Sezary syndrome Large ranular lymphocytic leukemia |
A. HTLV virus --> cutaneous lesions, hypercalcemia
B. helper T-? cutaneous patches, plaques, gen erythema C. T and NK cells --> splenomegaly, neutropenia, anemia |
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Acute lymphoblastic Leukemia/Lymphoma (ALL)
A. B-ALL B. T-ALL C. vs AML |
A. childhood (3 yo) leukemia = loss-of-fxn (PAX5)
B. adolescent thymic lymphomas = gain of fxn (NOTCH1) C. lymphoblasts more condensed chromatin, smaller cytoplasm that lacks granules |
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ALL
A. sx B. dx |
A. sx related to decr marrow fxn: anemia fatigue, fever b/s neutropenia, bleeding due to thrombocytopenia
- sx of neoplastic infiltraion: bone pain, heptosplenomega B. Ig for B/T antigens. - lymphoblasts myeloperoxidase-neg adn acid-Schiff - pos cytoplasm (in contrast myeloblasts) |
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Chronic Lymphocytic Leukemia (CLL)
A. morphology B. immunophenotype |
A. small lymphocyte infiltrate mixed with proliferation centers = aggregates of activated lymphocytes
- smudge cells: lymphocytes disupted in making smears B. pan-B cell markers |
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CLL
A. sx |
- nonspecific: weight loss, anorexia, fatigue
- disrupt normal immune fxn - transform to more aggressive tumors - Richter syndrome: transform to diffuse large B cell lymphoma |
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Follicular Lymphoma
A. stat B. morphology C. moleuclar pathogenesis |
A. most common form of indolent NHL
B. 2 cel types: centrocytes = small cleaved cells + centroblasts = larger cells w/ cytoplsm, nuclear chromatin C. (14;18) translocation = overexpression of BCL2 = no apoptosis |
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Follicular lymphoma
A. sx |
- painless, generalized lymphadenopathy
- histo transf to diffuse large B cell lymphoma |
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Diffuse large B cell lymphoma
A. stat B. morphology C. immunophenotype D. moelcular pathogenesis |
A. most common form of NHL
B. large cell size with large nuclei, open chromatin - diffuse pattern of growth C. mature Bcell marker: CD19, CD20 D. BCL6 dysrg = silence expression of P53 |
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diffuse large b-cell lymphoma
A. subtypes B. sx |
A. immunodef - associated large Bcell lymphoma: B cells infected with EBV b/c T-cell immundef
- primary effusion lymphoma: malignant pleural/ascitic effusion = tumor cells infected with KSHV/HHV-8 B. rapidly enlarging mass at nodal or extranodal site - aggresstive tumors (tx w/ anti-CD20 Ig) |
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Burkitt Lymphoma
A. type(s) B. morphology C. molecular pathogenesis |
A. African (endemic) vs sporadic (nonendemic)
B. intermediate sized - high mitotic index with numerous apoptotic cells - starry sky pattern b/c of lots of macrophages (pale) C. translocations of c-MYC gene on chromo 8 [t(8;14)] - endemic: infxn with EBV |
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Burkitt lymphoma
A. sx B. prognosis |
- manifest at extranodal sites
- endemic: mandible, involving abdominal viscera (kidney, ovaries, adrenals) - sporadic: ileocecum, peritoneum B. aggressive but resonds well |
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Plasma Cell neoplasms = dyscrasias
A. def M component B. heavy and light chains production C. name types |
A. M component = monoclonal Ig in blood
B. usu balanced but in neoplastic plasma cells = excess light or heavy chaings along with complete Ig C. Multiple Myeloma - plasmacytoma, smoldering myeloma - MGUS, lymphoplasmacytic lymphoma |
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Multiple Myeloma
A. molecular pathogenesis B. sx |
A. somatichypermutation = post-germinal center B cell home to bone marrow & differentiated into plasma cell
- survival of myeloma cell dep on IL-6 - neoplastic plasma cells mediate bone destruction B. bone resorption --> fractures, chronic pain, hypercalcemia - recurrent bacterial infxn b/c sup of humoral immunity - renal insufficiency b/c Bence Jones proteinuria |
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Multiple Myeloma
B. dx findings |
- XRAY punched out defects
- pathologic fractures (vertebral column) - diffuse demineralization (osteopenia), not focal defect - plasma cells w/ multiple nuclei, prominent nucleoli, cyoplasmic droplets containing Ig - globular inclusions = Russell bodies (ctoplasmic ) or Dutcher bodies (if nuclear) - rouleaux formation - myeloma kidney (from Bence Jones protein = light chain) |
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Multiple myeloma
A. tx |
- inhibitors of proeasome
- biphosphonates: inhibit bone resorption - prognosis poor |
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Solitary Myeloma (plasmacytoma)
A. defintion B. progression |
A. solitary lesion of bone or soft tissue
B. takes 15-20 yrs to progress to MM |
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Smoldering Myeloma
A. definition |
A. asymp but M protein level is ? 3 gm/dL
- takes 15 years to progress to MM |
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Monoclonal Gammopathy of Uncertain Significance (MGUS)
A. stat B. progression |
A. most common dyscrasia. mostly in elderly
B. 1% develop MM per year |
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Lymphoplasmacytic lymphoma
A. vs CLL B. vs MM C. morphology D. tx |
A. B cell neoplasm = cells undergo dif to plasma cells
B. heavy & light chain syn balanced - no bone destruction and complication from secretion of free light chain is rare C. periodic acid-Schiff pos inclusions continaing Ig = Russell bodies if cytoplasmic or Dutcher bodies D. plasmapheresis |
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Lymphoplasmacytic lymphoma
A. clinical sx |
- nonspecific: weakness, fatigue, wt loss
- lymphadenopathy, heptosplenomegaly - Waldenstrom macroglobulinemia: hyperviscosity from excess monoclonal IgM = viusla impairment, neurologic problems, bleeding, cryoglobulinemia (precipitation of macroglobulins at low temp = Raynaud phenomena) |
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Mantle Cell Lymphoma
A. morphology B. immunophenotype/pathogenesis C. vs CLL and follicular lymphoma D. prognosis |
A. small lymphoid cells w/ irreg nuclear outlines, cleaved membrane, condensed chromatin, scant cytoplasm
B. high cyclin D1 cased by (11;14) translocation C. large cells (centroblasts) and proliferation centers absent. D. no curable with chemo -> succumb to organ dysfxn |
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Marginal Zone lymphomas
A. location B. pathogenesis point |
A. mucosa-assocaited lymphoid tumors (maltomas)
B. arise from chornic inflam areas and can regress if agent removed |
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Hairy Cell leukemia
A. epidemio B. morphology C. sx |
A. middle-aged white male
B. fine hairlike projections from cells that enmeshe din ECM = dry tap (not aspirated) - splenic red pulp infilted --> no white pulp C. splenomegaly, pancytopenia |
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Anaplastic large-cell lymphoma (ALK positive)
A. molecular pathogenesis B. morphology C. prognosis |
A. rearrangement of ALK gene on chromo 2p23
--> activate tyrosine kinases B. horsehowe-liek nuclei and abundate cytoplasm C. good prognosis |
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Adult T-cell leukemia/lymphoma
A. hallmark B. cell morphology |
A. infected w/ HTLV-1 (human Tcell leukemia retrovirus)
B. multilobated nuclei |
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Mycosis Fungoides/Sezary sndrome
A. def B. phases of mycosis fungoides C. Sezary syndrome sx |
A. tumor of CD4 helper T cells with CLA, CCR4, CCR10 markers tha thome to skin
B. inflam premycotic phase, plaque phase, tumor phase C. generalized exfoliative erythroderma that rearely proceed to tumefaction - Sezary cells w/ cerebriform nuclei |
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Large Granular Lymphocytic leukemia
A. morphology of tumor cells B. hallmarks sx |
A. large lymphocytes w/ abundant blue cytoplasm + few coarse azurophilic granules
B. neutropenia and anemia but paucity of marrow infiltration |
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Extranodal NK/T cell lymphoma
A. morphology B. sx |
A. tumor cell (NK markers) surround and invade small vessels = ischemic necrosis. cell is invaded with EBV
B. destructive nasopharygeal mass |
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Hodgkin lymphoma vs NHL
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A. localized to single axial group of nodes; mulitple peripheral nodes
B. contiguity spread; noncontinguous C. mesenteri nodes/Waldeyer ring not involved; involved D. extra-nodal rare; common |
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Hodgkin lymphoma
A. hallmark morphology B. Reed-sternberg cell variants C. diff from large-cll NHL or mononucleuosis |
A. Reed-Sternberg cells surrounded lymphocytes, macrophages, granulocytes
B. mononuclear variants: single nucleus w/ large inclusion-like nucleolus - Lacunar cell: abundant cytoplasm disrupted durign sections = nucleus sitting in empty hole C. HL = reed-sternberg in background of non-neoplastic inflam cells |
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Hodgkin lymphoma
A. nodular sclerosis B. lymphocyte predominance |
A. lacunar variant + collagen in bands that divide lymph nodes into circumscribed nodules
- cells postive for PAX5 (B-cell) B. L&H variants (lymphocytic and histiocytic) express B cell markers typical of germinal -center B cells |
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Hodgkin lymphoma
A. MixedCellularity type B. Lymphocyte-rich type C. lymphocyte depletion |
A. RS cells + mononulclear variants infected w/ EBV
B. reactive lymphocytes infiltrates. C. paucity of lymphocytes but abundant RS cells - immunophenotyping essential to distinguish from large-cell NHL |
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Hodgkin lymphoma
A. molecular pathogenesis B. spread of HL C. sx |
A. RS cell undergone VDJ recombo and somati chypermutation but fail to express B cell specific genes
- activation of NF-kB by EBV rescues crippled germinal center B cells form apoptosis B. nodal -> splenic dz --? hepatic --> marrow C. painless lymphadenopathy, fever, night sweats |
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Myeloid neoplasms
A. types |
- acute myeloid leukemia: accum of immatur emyeloid forms (blasts) = suppresses hematopoiesis
- myelodysplastic syn: ineffective hematopoiessi --> cytopenias - myeloproliferativce disorder: incr prod of >1 blood cell |
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Acute myeloid leukemia
A. def B. sx C. morphology |
A. accumulation of immature myeloid blasts in marrow
B. anemia, thrombocytopenia, neutropenia B. >20% myeloid blasts in bone marrow - myeloblasts: delicate nuclear chromatin, > 2 nucleoli, > cytoplasm than lymphoblasts - Auer rods: needle-like azurophilic granules in t(15;17) - mono-blasts: foldednuclei lack Auer rods |
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Acute myeloid leukemia
A. molecular apthogenesis B. t(15;17) tx |
A. mutated tyrosine kinases collaborate w/ transcription factor aberratiosn = AML
B. t(15;17) creats fusion gene that encodes part of retinoic aicd recptor (RARalpha) - ATRA (all-trans retinoic acid) binds to PML-RARalpha fusion protein and antagonizes inhibitory effect on transcritpion of target genes |
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Myelodysplastic syndrome
A. hallmark B. molecular pathogenesis |
A. bone marrow replaced by clonal progeny of neoplastic multipotenet stem cell
B. progenitors undergo apoptosis at incr rate but ineffective hematopoiesis |
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Myelodysplastic syndrome
A. morphology |
- disordered (dysplastic) differentiation
- ringed sideroblasts: erythr w/ iron-laden mitochondria - megaloblastoid maturation (like VitB12 def) - nuclear budding abnl - pseudo-pelger-Huet cells: neutrophils w/ 2 nuclear lobes - pawn ball megakaryocytes: multiple nuclei not single - myeloid balsts <20% of overall marrow cellularity |
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Myeloproliferativ edisorders
A. molecular pathogenesis B. name types |
A. activated tyrosine kinase = incr prod of mature blood elements
B. chronic myeloid leukemia (BCR-ABL) - polycythemia vera (JAK2) - essential thrombocythemia (JAK2) - primary myelofibrosis (JAK2) |
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Chronic Myeloid leukemia
A. molecular pathogenesis B. morphology |
A. BCR-ABL fusion gene self activates
B. hypercellular in granulocytes, megakaryocytes - macrophages = sea-blue histiocytes b/c cytoplasm - leukocytosis |
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chronic Myeloid leukemia
A. sx B. tx |
A. dragging sesation in abdomen = splenomegal b/c extramedullary hematopoisesis
- ULQ pain = splenic infarct - anemia, thrombocytopenia B. imatinib decr # of BCR-ABL cells but not kill CML stem cell |
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Polycythemia Vera
A. hallmark B. morphology C. sx |
A. panmyelosis (incr prod of everything) but incr RBC most trouble
B. extensive marrow fibrosis - incr extramedullary hematopoiesis - oranomegaly C. plethoric & cyanotic due to stagnation of blood - headache, HTN, GI sx - major bleeding and thrombotic episodes |
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Essential thrombocytosis
A. vs PCV vs primary myelofibrosis B. lab dx C. sx |
A. vs PCV (absence of polycythemia)
- vs primary myelofibrosis (absence of fibrosis) B. peripheral smear: abnl large platelets C. thrombosis, hemorrhage - erthromelalgia (thrombing/burning of hands by occlusion of small arterioles) - DVT, portal vein thrombosis, MI |
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Primary myelofibrosis
A. hallmark B. molecular pathogenesis C. sx |
A. obliterativ emarrow fibrosis --> cytopenia + neoplastic extramedulalry hematopoiesis
B. neoplastic megakaryocytes release fibrogenic factors C. > 60 yo, hyperuricemia, secondary gout - anemia, splenomegaly (fullness in ULQ) |
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Primary myelofibrosis
A. morphology |
- megakaryocytes large, dysplastic, abnl clustered
- fibrotic obliteration --> exramed hematopoiesis - leukoerythroblastosis: premature release of nucleated erythroid - teardrop-shaped red cells (dacryocytes) |
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Langerhas cell histiocytosis
A. hallmark B. multifocal multisystem LCH C. eosinophilic granuloma D hand-schuller-christian triad |
A. birbeck granules in cytoplasm: pentalminal tubule with dilated terminal end = teniis racket appearance
B. cutaneous lesions rembling seborrheic eruption C. prolif LC mixed with eosinophils, lympho, plasma, neutrophils D. triad of calvarial bone defect, DI, exophthalmos |
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Spleen
A. anatomy B. two routs to splenic veins |
A. red pulp traversed by thin-walled vascular sinusoids separted by cords of Billroth
- white pulp follicules = arter with collar of T lymphocytes = periarteriolar lymphati cshealth - sheat expands to form lymphoid nodules of B lymph B. RBC into cords, squeezing throgh gpas to reach = open circuation - blood directly into splenic viens |
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Spleen
A. 4 fxns B. infectious caveat |
A. phagocytosis of blood cells
- Ig production - hematopoiesis - sequestion of platelet (htrombocytopenia) or WBC if splenomegaly B. susceptibility to sepsis by encap bacteria (H. flu, penmunoccus, meningocccus) |
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Splenomegaly
a. hypersplenism def B. morphology |
A. anemia, leukopenia, thrombocytopenia b/c incr sequestration and phagocytosis
B.. congestion of red pulp |
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Congestiv esplenomegaly
A. causes B. morphology |
A. right side heart failure
- cirrhosis - schistosomiasis - spontaneous portal vein thrombosis - pylephlebitis B. thickened fibrous capsule with firbotic red pulp |
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A. types of congenital anomalies
B. spleen rupture consequences |
A. hypoplasia, accessory spleens
B. caused by blunt trauma --> rapidly enlarging capsule that is thin = easy to rupture --> intraperitoneal hemorrhage |
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Thymus
A. anatomy B. Thymic hyperplasia |
A. medullary epithelia cells create Hassall corpuscles (whorls)
B. appearance of B cell germinal centers (seen in myasthenia gravis) |
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Thymomas
A. morphology B. noninvasive tymomas C. invasive thymoma D. thymic carcinoma E. sx |
A. lobulated, gray-white masses
B. medullary type epithelial cells = elongated or spindle shaped C. corticla epithelial cell = abundant cytoplasm + vesicular nuclei. penetrate through capsule to surrounding structures D. squamous cell carcinomas E. impingement on mediastinal structures |
