path 2 exam 2 diseases Flashcards

Question

what is pigmented levi?

Answer 1

tan-brown, UNIFORMLY pig, small (<6 mm) lesion 3 morphologic structures - junctional (clustering at dermal papilla tips) - compound (spreading toward dermis from tips) - intradermal (in dermis)

Answer 2

deep dermal involvement nevus cells around adenxae

Answer 3

clear halo around nevus halo represented by intense infiltration of LYMPHOCTES at periphery or nevus

Answer 4

dark blue/black nodules histologically heavily pigmented lesions with DENDRITIC CELLS located in dermis spindle cell arrangement

Answer 5

neither benign or malig sporadic or familial larger than most benign nevi usually multiple ireg organization seen in sun and non-sun exposed areas seen in families with DYSPLASTIC NEVUS SYNDROME - AD (high incidence of malig melanoma) histo shows most compound nevi nevus cells fuse together at upper dermis, CYTOLOGIC ATYPIA present in nevus cells upper dermis has chronic inflam, melanin pigment in dermal macrophages (melanin incontinence) and fibrosis most do not progress into malig melanoma but can transform into melanoma, however familiar syndrome have lifetime risk close to 100%

Answer 6

growth phases are - radial = epi, at epi jxn - vertical = more likely to spread to other organs (lymph nodes) prognostic factors (know these) - depth of invasion - # of mitoses per square mm - evidence of tumor regression (bad bc it means tumor has moved) - ulceration - presence and degree of tumor infiltrating lymphocytes - gender (women do better than men) - location (more periphery, extremities better than trunk, head, and neck)

Answer 7

benign epi tumor most frequent in mid aged and older ppl round, flat plaques (coin shaped, stuck on lesions) tan to dark brown, UNIFORM in color in dark-skinned ppl, multiple small lesions on FACE are called DERMATOSIS PUPULOSA NIGRA can present as part of paraneoplastic syndrome --> LESER TRELAT SIGN histo shows KERATIN CYSTS, acanthosis

Answer 8

thickened, hyperpigmented zones commonly seem in axillae, neck, groin and anogenital area 2 types - benign (majority) - malig (often associated with underlying GI carcinoma) --> not actually malig histo shows papillomatous hyperplasia

Answer 9

rapidly growing cutaneous tumor that can mimic well diff squamous cell carcinoma MEN are more affected than women flesh colored, dome shaped nodules with CENTRAL CRATER

Answer 10

common seen in light skin races, older pxs usually to due chronic exposure to sunlight (DNA damage) usually <1 cm, tan brown or red lesions, may produce CUTANEOUS HORNS face, arms, and hands commonly affected

Answer 11

second most common tumor in sun exposed areas in older ppl sun exposure is major predisposing factor, others = chronic immunosuppression, chronic ulcers, industrial carcinogens, ingestion of arsenic (rice, wheat, legumes), ionizing radiation and tobacco, and BETEL NUT CHEWING

Answer 12

slow growing commonly seen in sun exposed areas in light skin races PEARLY, raised papules basal cell nevus syndrome --> in addition to BCC can have abnorms of bone, CNS, eye, and reproductive organs (Gorlin syndrome) on histo you see a CLEFT between the basal cells due to edema, palisading basal cell arrangement

Answer 13

nevoid basal cell carcinoma syndrome AD medulloblastoma, ovarian tumors and odontogenic keratocysts mut in PATCH gene sporadic BCC in abt 30% show PATCH gene mut

Answer 14

no single gene mut is detected but Tp53 mut has been seen in actinic keratosis that is a precursor of SCCA muts that increase RAS signaling and decrease Notch singling have been located in some of the tumors

Answer 15

tumors of dermis firm tan brown papules in adults, particularly young to mid aged WOMEN benign but recurs DERMAL HISTIOCYTE?FIBROBLAST origin

Answer 16

tumor of dermis slow growing locally aggressive tumor (hardly metastasize) firm tan nodules, most frequently seen in TRUNK translocation of genes encoding COL1A1 and PDGFB***

Answer 17

involving skin, and some involve other organs like GI (systemic mastocytosis) infiltration of dermis by variable numbers of mast cells special stains like GIEMSA help id metachromatic nature of mast cell cytoplasm histo make look liked fried egg with halo

Answer 18

also called cutaneous T cell lymphoma scaly red brown patches, raised plaque sometimes confused with psoriasis histo can see atypical lymphocytes beneath the epi focally invading into dermis (Pautrier microabscesses) these lymphocytes are atypical --> convoluted nuclei (SEZARY LUTZNER cells, looks like walnuts) T helper cells that are CD4 pos

Answer 19

decreased production = anemia increased production = polycythemia red cell morphology abnormalities red cell enzyme disorders disorders of Hb synthesis lytic disorders RBCs (PNH) RBC inclusions

Answer 20

spherocytosis elliptocytosis stomatocytosis acanthocytosis = spur cell echinocytosis = burr cell target cells schistocytes

Answer 21

release from marrow stores = infections decreased margination = EXERCISE, epinephrine decreased extravasation into tissue = CORTICOSTEROIDS increased marrow precursors = chronic INFECTIONS, TUMORS, MYELOPROLIFERATIVE disorders

Answer 22

ACUTE BACTERIAL INFECTIONS, esp those caused by PYOGENIC orgs STERILE INFLAM caused by TISSUE NECROSIS (MI, burns)

Answer 23

inadequate or ineffective production - suppression of pluripotent hematopoietic stem cell - suppression of committed granulocytic precursors - ineffective granulopoiesis - congenital/inherited disorders accelerated removal or destruction - overwhelming infections - immune mediated injury to neutrophils - splenic sequestration

Answer 24

ANC <1500 cells severe infections typical <500 cells sepsis/postinfection, drugs (many, including chemotherapy), aplastic anemia, SLE, and radiation

Answer 25

eosinophilic leukocytosis allergic disorders parasitic infections drugs rxns malignancies (Hodgkin and some non-Hodgkin lymphomas) autoimmune disorders and some vasculites

Answer 26

basophilic leukocytosis rare, often indicative of MYELOPROLIFERATIVE DISEASE (CML, basophilic leukemia) infections = influenza, flu, chickenpox, TB allergy or inflam = ulcerative colitis, utricarial autoimmune disorders = SLE, juvenile rheumatoid arthritis endocrinopathy = diabetes, estrogen therapy, hypothyroidism

Answer 27

chronic infections (TB) autoimmune disorders inflam bowel disease malignancy

Answer 28

inadequate or ineffective production - suppression of pluripotent hematopoietic stem cell - rare congenital/inherited disorders accelerated removal/destruction - infections - immune mediated - iatrogenic

Answer 29

accompanies MONOCYTOSIS in many disorders associated with CHRONIC IMMUNOLOGIC STIM viral ifnections bordetella pertussis infection lymphoproliferative diseases

Answer 30

ALC <1500 cells HIV, SLE, corticosteroids, radiation, sepsis, postop

Answer 31

decreased RBC count, hemoglobin, and hematocrit decreased oxygen carrying capacity leading to tissue hypoxia pale, weakness, malaise easy fatiguability, dyspnea on mild exertion cardiac failure can develop at a result of tissue hypoxia with acute blood loss and shock, oliguria and anuria can develop CNS hypoxia causes HA, dimness of vision, and faintness

Answer 32

smaller and stain more darkly than normocytes and LARGE POLYCHROMATOPHILIC RED CELLS muts leading to insufficiency of MEMBRANE SKELETAL components --> decreased RBC life span to 10-20 days AD most common RBC disorder in N European descent mut = ANKYRIN EXTRAVASC hemolysis, JAUNDICE, SPLENOMEGALY dx = osmotic fragility test differential dx = AUTOIMMUNE HEMOLYTIC ANEMIA --> DAT+ and increase MCV clinical feats = anemia, splenomegaly, jaundice, APLASTIC CRISIS (parvovirus infection) tx = symptomatic, splenectomy anemia resolves but spherocytes persist, HOWELL JOLLY BODIES seen in blood

Answer 33

causes oxidative stress (meds, FAVA BEANS, infection) --> INTRAVASC and EXTRAVASC hemolysis XLR high prevalence in Africa, S. Europe, Middle East clinical feats - hemolysis = hemoglobinemia, hemoglobinuria, self-limited - precipitated by oxidative stress - PROTECTS AGAINST P. FALCIPARUM INFECTION

Answer 34

hemolytic anemia in NEWBORNS AR PKD --> decreased ATP --> rigid RBCS --> EXTRAVASC hemolysis increase 2,3 BPG --> increase Hb affinity for O2

Answer 35

IHA dx requires detection of ABS and/or COMPLEMENT on red cells from px - done using DAT where px's RBCs are mixed with sera containing Abs that are specific for human Ig or complement - in INDIRECT COOMBS ANTIGLOBULIN test, px's serum is test for its ability to agglutinate commercially available red cells bearing particular Ags WARM AB TYPE --> MOST COMMON at 37 C most causative Ab = IgG idiopathic, CML, SLE, drugs see many SPHEROCYTES COLD AGGLUTININ TYPE caused by IgM that bind to red cells at low temps like 0-4 C cold agglutin Abs sometimes appear transiently following certain infections --> ***mycoplasma pneumoniae, EBV, CMV, or idiopathic, lymphoplasmacytic lymphoma cold agglutination can be reversed once warmed down and you can see very few spherocytes

Answer 36

RBCc damaged when passing thru narrowed or obstructed vessels intravasc hemolysis = DIC, TTP, HUS< HELLP syndrome, hypertensive emergency, SLE SCHISTOCYTES (helmets) on peripheral blood smear prosthetic heart valves and aortic stenosis hemolytic anemia due to mechanical destruction of RBCs

Answer 37

PNH acquired PIG-A muts on X chromosome deficiency in GPI ANCHOR in all blood cells --> GPI anchor porteins deflect complement mediated destruction increased complement sensitivity --> increased INTRAVASC hemolysis nocturnal bc blood becomes slightly more acidic during sleep --> increased complement activity --> more RBCs get destroyed clinical feats - hemolytic anemia and hemoglobinuria - noctural hemoglobinuria - decreased iron due to intravasc hemolysis - THROMBOSIS is leading cause of death from disease (hepatic v thrombosis or Budd-Chiari syndrome) dx = flow cytometry shows LACK OF CD55 (DAF) evolution to APLASTIC ANEMIA and LEUKEMIA

Answer 38

autoimmune hemolytic anemia where RBCs are destroyed because of Ab that reacts in cold most often seen in CHILDREN secondary to viral illnesses = measles, mumps, infectious mononucleosis paroxysmal episodes of hemoglobinuria associated COLD EXPOSURE IgG ABS***

Answer 39

pallor, tachycardia, systolic heart murmurs, irritably, restricted growth in children specific symps - ANGULAR CHEILOSIS or KOILONYCHIAS = iron deficiency - gallstones = chronic hemolytic anemia - bone deformities = severe thalassemia - hemoglobinuria in MORNING URINE = PNH

Answer 40

- iron deficiency (hypochromic) - anemia of chronic disease (normochromic) - thalassemia - sideroblastic anemia (often dimorphic) - lead poisoning (often normocytic, normochromic)

Answer 41

decrease serum iron decrease marrow iron store increase TIBC (transferrin) <10% sat decrease ferritin increase transferrin recep

Answer 42

decrease serum iron INCREASE marrow iron store DECREASE TIBC >15% sat INCREASE ferritin normal to increase transferrin recep

Answer 43

INCREASE serum iron INCREASE marrow iron store DECREASE TIBC sig increased % sat sig increased ferritin DECREASE transferrin recep

Answer 44

B12 deficiency folate deficiency acquired defects in DNA synthesis inherited aplastic anemias acquired aplastic anemias direct alcohol effect

Answer 45

hypercellular - MDS hypocellular - aplastic anemias normocell - chronic disease - early iron deficiency - erythropoietin deficiency

Answer 46

due to decreased dietary intake, impaired absorption, increased requirement, or chronic blood loss iron deficiency in adult men and postmenopausal women in W world must be attributed to GI blood loss until proven otherwise most common nutritional deficiency Fe is absorbed in DUODENUM transferrin transports iron in blood and takes it to liver and bone marrow macrophage for storage stored intracell iron is bound to ferritin (ferritin in erythroblasts and hemosiderin in macrophages)

Answer 47

nutritional deficiency anemia of chronic disease aplastic anemia other forms of bone marrow failure

Answer 48

Vit B12 or folate deficiency CBC and PBS = macrocytic anemia, HYPERSEGMENTED NEUTROPHILS bone marrow = megaloblastic hyperplasia, giant metamyelocytes increase HOMOCYSTEINE in both, but increase in MMA only in vit B12 deficiency*** sig increased LDH

Answer 49

inadequate uptake (vegetarianism) increased absorption = intrinsic factor deficiency (PERNICIOUS anemia, gastroectomy), malabsoprtion, ileal resection, intestinal disease, bacterial overgrowth, fish tapeworm increased required = pregnancy, hyperthyroidism, cancer clinical feats = megaloblastic anemia, atrophic glossitis, NEURO ABNORMALITIES tx = folate B12 supplements

Answer 50

inadequate uptake (alcoholism, infancy) decreased absorption (malabsorption, intestinal disease, drugs like anticonvulsants, oral contraceptives) increase loss (hemolysis) increased requirement (pregnancy, infancy, cancer, increased hematopoiesi) impaired use - folic acid antagonist (METHOTREXATE)

Answer 51

pernicious anemia due to autoimmune attack on GASTRIC MUCOSA chronic atrophic gastritis due to loss of PARIETAL CELLS, prominent infiltrate of lymphocytes and plasma cells, and megaloblastic change about 75% of pxs have Ab that blocks binding of vit B12 to intrinsic factor other Abs prevent binding of intrinsic factor-vit B12 complex to its ILEAL RECEPTOR see HYPERCELLULAR bone marrow that, resembles AML seem megaloblastic erythroblasts, granulocytes, resembling MDS

Answer 52

anemia commonly associated with many systemic inflam conditions, including infection, rheumatologic disorders, and cancer ACD is anemia of UNDERPRODUCTION that is NORMOCYTIC, NORMOCHROMIC, mild, with Hb level >10 g/L bone marrow looks NORMAL PRUSSIAN BLUE IRON STAIN shows INCREASED IRON STORES w/ increased histiocytic iron but decreased sideroblastic iron marked by LOW SERUM IRON total IRON STORES ARE NORM OR ELEVATED

Answer 53

CBC, smear, bone marrow = anemia, BASOPHILIC STIPPLING, RING SIDEROBLASTS causes - MDS with ring sideroblasts - genetic --> X-linked sideroblastic anemia (XLSA) shows microcytic anemia, responsive to PYRIDOXINE (B6) tx - toxic = lead, alcohol - meds = isoniazid, chloramphenicol, chemotherapy

Answer 54

failure of multipotential stem cell = anemia, THROMBOCYTOPENIA, NEUTROPENIA idiopathic, inherited defects = fanconi anemia, defects in telomerase, VIRAL (EBV, HIV, hepatitis), RADIATION, DRUGS HYPOCELLULAR BONE MARROW clinical feats = weakness, pallor, dyspnea, bleeding, recurrent infections

Answer 55

congenital - DIAMOND BLACKFAN ANEMIA - CDA acquired - TEC - PARVOVIRUS B19 APLASTIC CRISIS - thyoma, spindle cell type - autoimmune = older children, adults - lymphoproliferative disease = CLL, LGL

Answer 56

caused by parvovirus B19 humans are the only known host clinical feats = erythema infectiosum in CHILDREN (5th disease = SLAPPED CHEEK RASH), arthalgias and arthritis in adults bone marrow = GIANT PROERYTHROBLASTS

Answer 57

shows circulating normoerythroblasts and RED CELL FRAGS, GIANT PLATELET can be associated with METASTATIC ADENOCARCINOMA

Answer 58

sub-saharan Africa, SE Asia PRESENT AT BIRTH CBC = microcytosis, RBC >5.5 x 10^12, MCV 65-75** NORMAL Hb A2 Hb quantitative defect silent carrier (minor)(-a/aa) = asymp with norm or decrease A2 alpha thal trait (major) (-a/-a, or --/aa) = microcytosis, mild anemia with norm or decrease A2 --> --/aa (asian) and -a/-a (black african and asian) HbH disease (--/-a) = CHRONIC HEMOLYTIC ANEMIA --> resembles beta thal intermedia Hb Bart's (--/--) = lethal, hydrops fetalis

Answer 59

hydrops fetalis MOST SEVERE alpha thal caused by DELETION OF ALL 4 ALPHA GLOBIN GENES excess gamma globin chain form TETRAMERS

Answer 60

in Mediterranean seen 6-9 MONTHS after birth (after birth, begin to switch from gamma to beta) CBC = microcytosis, RBC >5.5x10^12, MCV 55-65** Hb quantitative defect beta thal minor (b/b0, b/b+) = mild anemia beta thal intermedia (b/b0, b+/b+) = SEVERE ANEMIA, no transfusion needed beta thal major (b0/b0, b+, b0) = SEVERE ANEMIA, REQUIRES TRANSFUSION b0 = absent beta globin synthesis b+ = reduced beta globin synthesis normal synthesis of alpha chains increased production of gamma and theta chains bone marrow HYPERPLASIA extramedullary hematopoiesis PROTECT AGAINST FALCIPARUM MALARIA

Answer 61

deoxy --> aggregation and polymerization of HbS --> irreversible sickling --> hemolysis homozygous HbS/HbS --> sickle cell anemia heterzygous HbS/HbA --> sickle cell trait (8% of AAs) moderators of sickling = HbF, HbA decreases sickling while dehydration and lower pH increases sickling pt mut of valine for glutamic acid at 6th residue microvasc occlusion --> thrombosis infarction, autosplemectomy bone marrow HYPERPLASIA, extramedullary hematopoiesis, pigment gallstones tx = HYDROXYUREA to increase HbF

Answer 62

EXTRAVASC hemolysis, milder than HbSS MUTATION IN BETA CHAIN homozygotes (HBCC)form CRYSTALS in RBCS, target cells HbSC = milder disease than HbSS pxs

Answer 63

vasculitis - infections - drugs impaired collagen formation - scruvy - ehler danlos syndrome - cushing syndrome immune complex deposition (hypersensitivity) - henoch schonlein purpura hereditary hemorrhagic telangectasia amyloidosis

Answer 64

decreased plt survival - immunologic destruction - ITP, SLE, drug (heparin), infection (HIV) - non-immuno destruction - TTP, DIC, MAHA decreased production - bone marrow failure, drug, infection, megaloblastic anemia, PNH, MDS, marrow infiltration sequestration - hypersplenism

Answer 65

plt clumping due to EDTA dependent-ab (lavendar top tube) --> abs to GPIIb/IIIa causes plt to clump plt satellitism = plt adherence to leukocytes in EDTA sodium citrate anticoagulant (blue top tube) instead of EDTA to correct

Answer 66

ITP autoAbs against HPIb-IX or IIb-IIIa usually IgG anti-plt autoabs --> plt opsonization --> plt destruction in spleen --> thrombocytopenia findings - megathrombocytes - prolonged BT/PFA-100 - NORM PT/PTT - anti-plt abs - splenic congestion and lymphoid hyperplasia - increased bone marrow megakaryocytes clinical feats chronic ITP = more common in WOMEN (<40), mucocutaneous bleeding tx = steroids, splenectomy, immunosuppression acute ITP common in CHILDREN follows viral infection self-limited

Answer 67

immunologic destruction of plts quinine, quinidine, sulfonamides heparin (HIT-heparin induced thrombocytopenia) - severe thrombocytopenia due to Abs against herparin-PF4 complex - paradoxical risk of thrombosis due to plt activation HIT aka HIT type II heparin-dependent Abs non-induced heparin associated thrombocytopenia aka HIT type I other factors causing thrombocytopenia

Answer 68

type I no ab 5% incidence in heparinized pxs onset as eaerly as day i plt count = abt 100k, >50% of baseline rarely thrombosis tx = no contraindication to future heparin type II anti-PF4 abs 1% incidence in heparinized pxs onset around day 5-15 plt count = <50k, <50% basline thrombosis forms often, arterial > venous tx = STOP HEPARIN, WARFARIN, or PLTS consider argatroban

Answer 69

giant plts ADHESION problem, AR norm PT/PTT, INCREASED BLEEDING TIME norm plt aggregation to agonists ABNORMAL RISTOCETIN diff dx = von willebrand disease = norm plt count, increased PTT tx = plt transfusion

Answer 70

most common inherited bleeding disorder carrier for factor VIII, protecting it from inactivation and clearance AR or AD, norm PT and plt count, INCREASED aPTT AND BLEEDING TIME, ABNORMAL RISTOCETIN diff dx = bernard soulier syndrome = norm PT/aPTT, increased bleeding time, thrombocyopenia, and giant plts production endo cells and megakaryocytes --> large multimers cleaved by vWF cleaving protease (ADAMTS 13) fxns - plt adhesion --> binds plt GPIb and collagen - stabilization of FVIII --> non-covalent linkage prolongs FVIII half life from 2.4 to 12 hrs lab measurements quantitative = immunoassay qualitative = ristocetin co-factor activity clinical feats most cases are type 1 w/ mild or no bleeding mucocutaneous bleeding excessive bleeding from wounds tx = DDAVP (increase vWF and FVIII), vWF replacement

Answer 71

deficiency of GPIIb-IIIa AR plt aggregation defect norm PT/PTT prolonged BT/PFA-100 no GP for plt to bind to fibrinogen and aggregate to form clot tx = plt transfusion

Answer 72

aspirin and other NSAIDS - irreversible inhibition of COX 1 - plt aggregation defect uremia - both adhesion and aggregation impaired --> plt dysfxn in uremia --> tx = dialysis, DDAVP, or estrogen prolonged bleeding time, NORM PT/PTT

Answer 73

thrombocytopenia and plt dysfxn depletion of alpha and delta granules prolonged bleeding time abnorm plts fxn test

Answer 74

plt dysfxn due to coating with IgA, IgM, or IgG

Answer 75

FVIII deficiency XLR males affected, females are carriers prolonged PTT norm plt count and PT clinical feats - massive hemorrhage after trauma - easy bruising - hemarthrosis (recurrent and spontaneous) tx - FVIII replacement --> recombo or plasma-derived comps = anti-FVIII abs in 15% and infectious disease transmission if plasma-derived product used

Answer 76

FIX deficiency XLR males affected, females are carriers prolonged PTT norm plt count, PT clinical feats - variable severity of bleeing tx = FIX replacement (recombo or plasma-derived)

Answer 77

MULTIPLE factor deficiency PROLONGED PT/PTT norm plt count, BT/PFA4-100

Answer 78

PROLONGED PT/PTT norm plt count, BT/PFA4-100 vit K depedent factors deficient II, VII, IX, X, protein C, protein S WARFARIN produces vit K deficient state

Answer 79

TTP norm PT/PTT clinical PENTAD - fever - anemia (microangiopath hemolytic) - thrombocytopenia - renal failure - neuro deficit more common in WOMEN PBS = SCHISTOCYTES decreased vWF cleaving protease (ADAMTS 13) --> ultra high molecular weight vWF multimers --> plts activation and consumption --> thrombocytopenia and microvasc thrombi tx = plasma exchange with FFP +/- steroids and IVIG

Answer 80

typically in CHILDREN shiga-like toxin from EHEC (serotype O157:H7) infection TRIAD = thrombocytopenia, microangiopath hemolytic anemia, AKI and blood diarrhea no neuro symps

Answer 81

systemic activation of coagulation system --> gen and deposition of fibrin microvasc thrombi in various organs --> multiple organ dysfxn syndrome ongoing activation of coag --> consumption and subsequent exhaustion of coag proteins and plts --> severe bleeding may present with SIMULTANEOUS THROMBOSIS AND BLEEDING clinical feats - bleeding diathesis - microagniopathic hemolytic anemia - multisystem involvement - circulatory failure and shock tx = treat underlying disorder, balanced use of anticoags and procoags based on clinical manifestations causes = obstetric comps, infections, neoplasms, massive tissue injury, other PROLONGED PT/PTT reduced fibrinogen ELEVATED D-DIMERS thrombocytopenia SCHISTOCYTES

Answer 82

inflam of lymph nodes caused by reactive processes like - infectious mononucleosis - HIV - cat scratch disease - hemophagocytic lymphohistiocytosis (HLH) - rheumatoid arthritis - toxoplasmosis on histo, see tingible body macrophages B cells located in the center, T cells located in periphery

Answer 83

EBV infection TRIAD = tonsillitis, fever, enlarge lymph nodes (neck, axilla, groin) transmitted by saliva + hepatosplenomegaly + MONOSPOT TEST + HETEROPHIL Ab + CD8+ cytotoxic cells in peripheral blood PBS shows ATYPICAL LYMPHOCYTES

Answer 84

caused by B henselae self-limited, primarily in childhood 2 wks after cat scratch regional lymphadenopathy - axilla, neck histo shows IRREG STELLATE NECROTIZING GRANULOMA

Answer 85

viral infection or other proinflam exposures trigger activation of MACROPHAGES throughout the body - phagocytosis of blood cells - cytopenias - symps related to systemic inflam and organ dysfunction inherited defects in genes that reg fxn of immune cells (perforins) or in T cell lymphomas defect of killer lymphocytes cytokines (TNF, IL-6) causing symps and signs similar to those seen in sepsis and SIRS FEVER, SPLENOMEGALY, PANCYTOPENIA, INCREASE FERRITIN, INCREASE TGs

Answer 86

caused by toxoplasma gondii enlarge lymph nodes usually in neck by CONSUMING MEAT CONTAMINATED BY PARASITE or being in contact with CAT FECES preg women and immunocomp ppl are at risk

Answer 87

clonal prolif of lymphocytes usually arise from lymph nodes but can also be other organ of body some primarily involve peripheral blood of bone marrow like CLL variable clinical behavior

Answer 88

2nd most common lymphoma in US mid to older adults generalized lymphadenopathy, hepatosplenomegaly indolent = chronic course, medial survival = 7-9 years nodal or extra-nodal, slow growing CD10+ t(14,18) IgH, BCL-2

Answer 89

most common lymphoma in US mid age to older adults lymphadenopathy, hepatosplenomegaly frequent EXTRANODAL presentaiton diffuse pattern of infiltration by large cells CD20+, CD10 +/-, BCL6+, CD5- molecular = BCL6 gene rearrangement, BCL2 rearrangement - t(14,18)

Answer 90

adults EBV related associated w/ HIV/AIDS (considered AIDS defining illness) confusion, memory loss, serizures CNS mass, RING ENHANCING LESION on MRI ddx = toxoplasmosis

Answer 91

PMLBCL usually affects YOUNGER ppl 25-40, more common in WOMEN than men neoplastic cells express B cell markers and CD30 oftens shows heterogeneous staining

Answer 92

endemic = jaw sporadic = intestine adults = nodal disease AGGRESSIVE CD19+, CD10+, kappa or lambda light chain t(8,14), t(2,8), t(8,22) c-myc, 8q24 on histo, see STARRY SKY in lymph nodes endemic form in equartorial Africa --> primarily children, >95% associated with EBV, extranodal sites = mandible, ab sporadic in US --> children and adults, 15-20% associated with EBV, extranodal sites = ab HIB associated --> 25% associated with EBV highly aggressive course but high cure rates with chemotherapy

Answer 93

CLL or SLL neoplasm of mature lymphocytes - accum of mature B lymphocytes epidemiology - CLL = predom blood and bone marrow (MOST COMMON LEUKEMIA IN US) - SLL = predom lymph nodes older adults (median age 70) lymphocytosis >5000 per mL anemia, thrombocytopenia, bone marrow (diffuse and nodular infiltrate), lymph node (diffuse infiltrate of small lymph nodes) SMUDGED CELLS in PBS origin = B cells CD20+, CD5+, CD23+, CD10- prognosis = generally good, bad in 1-2% due to RICHTER TRANSFORMATION (2-8%, diffuse large B cell lymphoma)

Answer 94

>50 years asymp lymphocytosis lymphadenopathy, hepatosplenomegaly CD19+, CD5+, CD23+, clonval kappa or lambda light chain trisomy 12, 13q-

Answer 95

adults nodal or extranodal, PAINLESS MORE AGGRESSIVE than CLL/SLL CD5+, cyclinD1+ t(11,14)

Answer 96

adults extranodal, nodal, SPLENIC STOMACH, lung, intestine, eye H. pylori t(11,18) most common

Answer 97

adults pancytopenia, splenomegaly CD19+, CD11c+, CD25+, CD103+, Annexin A1+ (MOST SPECIFIC) good prognosis neoplasm of mature lymphocytes --> mature B-lymphocytes with villous cytoplasmic projections 2% of all leukemias mid to older adults (M>F) BRAF V600E muts are found in almos all cases on histo, looks like fried egg and dry tap

Answer 98

neoplasms of terminally differentiated, immunoglobulin secreting B lymphocytes plasma cell prolif and M-component mid and older adults plasma cell myeloma (multiple myeloma) - 10% or >10% neoplastic plasma cells (PC) - multifocal destructive infiltration of plasma cells in bone marrow monoclonal gammopathy of undetermined significant (MGUS) = <10% neoplastic PC solitary osseous plasmacytoma extraosseous plasmacytoma Waldenstrom macroglobulinemia = hyperviscosity

Answer 99

abnormal plasma cells hyperCalcemia Renal disease Anemia Bone lesions on X-ray, see PUNCHED OUT LYTIC BONE LESIONS blood - paraprotein = monoclonal Ig or light chain forms spike on serum protein electrophoresis (M SPIKES) - ROULEAUX FORMATION urine - Bence Jones protein (monoclonal free light chain)

Answer 100

monoclonal IgM symps related to monoclonal IgM protein are attributalbe to - characteristics in circ - interaction with various body tissues when depositied - autoab activity

Answer 101

- neoplasm of B cells - REED STERNBERG CELLS (OWL EYES) in a mixed cell background --> derive from B cells nodular sclerosis, lymphocyte rich, mixed cellularity, lymphocyte depleted - bimodal age distribution (15-35 and >50) - can be related to EBV infection - CD30+, CD15+, PAX5+ in nodular sclerosis type --> broad collagen bands clinical feats = lymphadenopathy, splenomegaly, constitutional B symps (fever, night sweats, weight loss), immune dysfxn prognosis = excellent at early age (90% cure rate) risk of second malig in long-term survivors

Answer 102

CD30-, CD15- NO RS CELLS, eosinophils, plasma cells, or fibrosis L&H cells (POPCORN CELLS) with multilobed, folded nucleus in a background of lymphocytes

Answer 103

- EBV associated - older adults - abrupt onset with constitutional symps (fever, night sweats, weight loss) - gen lymphadenopathy, pruritic skin rash - diffuse nodal effacement with prominent vessels - ABSENCE OF FOLLICLES - CD4***, CD8-, CD10+ clinical feats = high grade fever, rash, anemia lymph node architecture = effaced by a cell infiltrate and marked vascular prolif

Answer 104

PTCL prolif of MATURE T lymphocytes clinical feats - gen lymphadenopathy - FEVER, PRURITUS, weight loss - EOSINOPHILIA prognosis worse than similar B cell lymphomas majority are CD4+ (lack one or more of T cell markers, most often CD7) norm T lymphocytes = CD2, CD3, CD4, CD5, CD7, CD8 path findings - architectural effacement - vasc prolif immunophenotype - CD2, CD5, CD3, TCR alpha beta or gamma delta genetics = T cell receptor gene rearrangement

Answer 105

ATLL SKIN LESIONS, LYTIC BONE LESIONS, gen lymphadenopathy, hepatosplenomegaly, lymphocytosis, HYPERCALCEMIA S. Jana, Caribbean regions mid to older adults VERY AGGRESSIVE FLOWER CELLS CD3, CD4, CD25, CD7- HTLV-1 associated poor prognosis, esp in leukemic phase

Answer 106

in CHILDREN and YOUNG ADULTS nodal or extranodal sinusoidal/subcapsular infiltration LARGE ANAPLASTIC CELLS (HALLMARK) CD30, EMA+, CD25+, T cell markers variable t(2,5) ALK POSITIVE nuclear staining, good prognosis ddx = HODGKIN LYMPHOMA cells look like HORSESHOE in older age ALK NEG, no t(2,5), WORSE prognosis

Answer 107

older adults CUTANEOUS PATCH, PLAQUE, and NODULE stages lymphadenopathy UNUSUAL lymphocytes CD3, CD4, CD7- indolenet, chronic course = 90% 5 year survival EXTRACUTANEOUS INVOLVEMENT indicates worse prognosis see epidermotropism, papillary dermal fibrosis, PAUTRIER MICROABSCESSES sezary syndrome --> circulating tumor/lymphoid cells (CEREBRIFORM NUCLEI), unfavorable prognosis leukemic phase of mycosis fungoides

Answer 108

LGLL unexplained sustained increase in peripheral blood LGLs may be T cytotoxic or NK cells CD4-/CD8+ NEUTROPENIA, SPLENOMEGALY, HYPERGLOBULINEMIA OLDER MALES (median age = 60) incidence higher in RHEUMATOID ARTHRITIS usually indolent

Answer 109

nasal type more prevalent in ASIA NASAL LESIONS, but may present at non-nasal sites AGGRESSIVE clinical courses ANGIOINVASIVE, MARKED VASCULATURE CD16, CD56, CD57, granzyme, TIA1, perforin+ EBV+ most cases often in adults (M>F) site of involvement - upper aerodigestive tract (NASAL CAVITY) - extranasal including skin, soft tissue, GI tract prognosis = aggressive lymphoma poor response to chemotherapu medial survival 1 year high local recurrence rate

Answer 110

neoplasm of lymphoid stem cell, leukemia, lyphoma path findings - leukocytosis with lymphoblasts - norm or decreased WBCs - anemia, thrombocytopenia bone marrow - hypercell - sheets of lymphoblasts - high mitotic index lymph nodes - architectural effacement by sheets of lymphoblasts clinical feats = anemia, neutropenia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, bone pain B cell = CD10, CD19, TdT+, PAX5 T cell = CD2, CD5, CD7, cCD3, TdT+ genetics - favorable prognosis = hyperdiploidy (51-65 chr), t(12, 21) - poor prognosis = t(4,11) and t(9,22)

Answer 111

85% of ALL children > adults anemia, infections, bleeding, adenopathy, hepatosplenomegaly TdT+, CD19+, CD10 +/- t(12, 21)

Answer 112

15% of ALL MEDIASTINAL mass and NECK node enlargement TdT+, CD3, CD5, CD7, (CD4+/CD8+ or -) TCR gene rearrangement

Answer 113

spleen >1000 g moderate splenomegaly = 500-1000 g -chronic congestive splenomegaly (portal HTN as in cirrhosis, splenic v obstruction) - acute leukemias - diseases with extramedullary hematopoiesis mild splenomegaly = <500 g - acute splenitis - acute splenic congestion - infectious mononucleosis - septicemia - SLE - intraab infections hypersplenism = chronically enlarged spleen that removes blood cells --> anemia, leukopenia, thrombocytopenia

Answer 114

enlargement of thymus contains lymphoid follicles or germinal centers (reactive B cells) within medulla in px with myasthenia gravis, autoimmune diseases removal of hyperplastic thymus is beneficial in early MG

Answer 115

tumors of thymic epi cells, mostly in mid aged adults benign (encapsulated thymoma) = cytologically and biologically benign malig type I = cytologically benign but infiltrative and locally aggressive type II (thymic carcinoma) = cytologically and biologically malig

Answer 116

ineffective proliferation absent splenomegaly cytopenia (unilineage or multilineage) with dysplasia hypercell present in one or more lineages high risk of AML peripheral pancytopenia despite of hypercell marrow due ineffective hematopoiesis blasts <20% cytogenic prognosis favorable = norm karyotype, or isolated Y-, 5q-, 20q- intermediate = 8+, mostly in males, higher risk for acute leukemia unfavorable = COMPLEX KARYOTYPE, 7- clinical feats = anemia, thrombocytopenia, neutropenia related to pancytopneia variable course - may smolder for years, some transfusion depedent, 10-40% transform to AML, therapy related MDS has worse prognosis path findings blood - variable cytopenias - macrocytic anemia - granulocytes --> hypolobulated nuclei, hypogranular cytoplasm - PSEUDO PELGER HUET CELL bone marrow - hypercell - variable increased blasts - dysplastic maturation

Answer 117

elderly women, 2:1 female predominance pxs have MACROCYTIC ANEMIA, norm or increased plts, MONOLOBATE or SMALL MONONUCLEAR MKCs, and norm granulopoiesis and peripheral counts survival in this subset of MDS is exceptional, approaching that of age-matched peers

Answer 118

congenital and acquired form (including reversible) 65-70 years at presentation (acquired) DIMORPHIC ANEMIA with anisocytosis (increased RDW) RING SIDEROBLASTS >/= 15% of BM erythroid precursors leukocytes and plts are normal associated with SF3B1 mutation in most cases prolonged survival (5-10 years)

Answer 119

pxs with AML present with anemia, thrombocytopenia, and WBC dysfunction also extramedullar tumor prolif (myeloid sarcoma, more common in childhood AML) dx morphology, immunohistochem, immunophenotypic (flow cytometry), cytogenetics (karyotype, FISH, molecular studies) most AMLs have mutations in genes encoding transcription factors required for norm myeloid cell diff fine/immature/smooth nuclear chromatin large in size and high N/C ratio neoplasm of myeloid stem cell or early lineage-specific precursor cell >/= 20% blast in bone marrow patho = acquired genetic alterations - disrupt maturation = PML-RARalpha - activate transcription factors = FLT3 peak incidence = after 6 years of age, also in older adults and infants

Answer 120

with t(15,17)(q22,q12) (PML-RARA) t(15,17) due to fusion of promyelocytic gene (PML) on chr 15 with retinoic acid recep (RARA) gene on chr 17 usually has an abrupt onset, and constitutes 5-8% of AML cases most common in YOUNG ADULTS, rarely occurring before 1- y/o and diminishing in incidence after 60 y/o prompt dx is ESSENTIAL bc of high freq of LIFE THREATENING DIC blasts are highly sensitive to ANTHRACYCLINE BASED THERAPY and differentiate in response to ATRA and ARSENIC TRIOXIDE TX characteristic myeloid lineage immunopehnotype, w/ weak or ABSENT HLA-DR and ABSENT CD34 common association w/ DIC favorable prognosis = FLT3 neg and treated w/ combo chemotherapy that includes ATRA hypergranular type exhibits abundant cytoplasmic granules and bundles of AUER RODS

Answer 121

frequently has myelomonocytic or monocytic feats de novo in older adults with norm karyotype good prognosis if not associated with FLT3-ITD mut

Answer 122

previous MDS = very poor w/o previous MDS = poor

Answer 123

alkylating agent related = very poor epipodophyllotoxin related = very poor

Answer 124

effective and over prolif splenomegaly is present with extramedullary hematopoiesis cytosis (uni or multilineage), NO DYSPLASIA hypercell with prolif of one or more lineages no overt dysplasia in granulocytes and erythroid precursors AML risk is low

Answer 125

chronic myeloid leukemia (CML) polycythemia vera (PV) essential thrombocythemia (ET) primary myelofibrosis (PMR)

Answer 126

adults, 25-60 years with peak around 4th and 5th decade nautral history of untreated CML is bi- or triphasic = initially indolent chronic phase (CP) followed by accelerated phase (AP) or blast phase (BP) or both chimeric BCR-ABL1 gene derived from BCR gene (chr 22) and ABL1 gene (chr 9) t(9,22), BCL/ABL1 fusion gene encodes abnorm oncogene and constitutively active tyrosine kinase activity gene is present in granulocytic, erythroid, megakaryocytic B cell precursors +/- T cells, tumor origin is TRANSFORMED HEMATOPOIETIC STEM CELL BCR-ABL does NOT inhibit differentiation --> early disease course, excessive porlif of norm cells Ph chr is highly characteristic of CML, present in 25% of ALL and in small sub of AML clinical feats = fatigure, weight loss, fever, splenomegaly (can cause ab symps), 20-40% pxs are asymp at initial dx peripheral blood = increased WBC, often exceeding 100k cells (predom neutrophils, metamyelocytes, myelocytes, basophils, and eosinophils) bone marrow = hypercell, increased MYELOID to ERYTHROID RATIO, blases usually <5%, always <10%, megakaryocytes are norm or increased with DWARF morphology genetics = 100% have Ph chr or BCR-ABL1 fusion gene

Answer 127

leukemoid rxn = increased WBC in response to - infection - stress - chronic inflam - certain neoplasms BCR-ABL fusion gene detection by karyotyping, FISH, or PCR assay

Answer 128

rare disease clonal prolif of erythroid precursors, granulocytes, and megakaryotes in bone marrow (PANMYELOSIS), in addition to POLYCYTHEMIA there's also LEUKOCYTOSIS and THROMBOCYTOSIS strongly associated with activating pt muts in TYROSINE KINASE JAK2 (causes panmyelosis) clinical feats = increased RBC mass, splenomegaly, thrombosis or bleeding, low or norm serum erythropoietin clinical manifestations are usually due to increased red cell mass or thrombosis and hemorrhage 3 phases of PV - mild erythrocytosis (with prominent thrombocytosis) - overt polycythemic phase - postpolycythemic phase characterized by cytopenias and bone marrow fibrosis tx = phlebotomy, hydroxyurea or INFalpha as cytoreductive therapy, Ruxolitinib JAK2 inhibitor

Answer 129

involves megakaryocytic lineage characterized by sustained THROMBOCYTOSIS and episodes of THROMBOSIS OR HEMORRHAGE JAK2 mut is present in abt 50% of cases, and MPL mut in 1-2% these muts lead to constitutive activation of paths that stimulate megakaryocyte prolif and plt production

Answer 130

PMF clonal myeloid neoplasm characterized by neoplastic prolif of predom MEGAKARYOCYTES and GRANULOCYTES, accompanied by increase in bone marrow CT and often by EMH --> basically neoplasm of myeloid stem cell with reactive fibrosis patho = pro-fibrotic growth factors from megakaryocytes --> PDGF and TGF-beta blood = leukoerythroblastosis with prominent DACROCYTES bone marrow = hyper cell in early phase and hypocell in late phase (marked reticulin and collagen fibrosis and osteosclerosis) spleen = expanded red pulp with EMH genetics - JAK2 V617F adults >60 medial survival = 3-5 years --> transformation to AML 5-20% 2 phases - PREfibrotic stage, often accompanied by peripheral blood thrombocytosis - fibrotic stage (HYPOCELL bone marrow with marked RETICULIN or COLLAGEN FIBROSIS, often OSTEOSCLEROSIS) on histo, see TEAR DROPS

Answer 131

prolif disorders of DCs or MACROPHAGES, derived from bone MARROW MONOCYTES most of histiocytic prolifs in lymph nodes are BENIGN and REACTIVE rarely highly malig LANGERHAN CELLS are DCs found in SKIN and multiple ORGANS --> capture Ags and present them to T cells

Answer 132

in CHILDREN LYTIC BONE LESIONS skin rash, recurrent OTITS MEDIA BIRBECK GRANULES (TENNIS RACKET) Letterer-Siwe disease - malig prolif of Langerhans cells = SKIN RASH, cystic skeletal defects in INFACTS <2 years old, involves MULTIPLE organs and is FATAL IF NOT TREATED eosinophilic granuloma (SOLITARY OSTEOLYTIC BONE LESION IN CHILDREN) - benign prolif of Langerhans cells in bone = path fracture in an adolescent, NO SKIN involvement, biopsy with Langerhan cells and lots of eosinophils Hand-Schuller-Christian disease - malig prolif of Langerhans cells, SCALP RASH, LYTIC SKULL DEFECTS, DIABETES INSIPIDUS, EXOPHTHALMOS in child

path 2 exam 2 diseases Flashcards

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