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89 Cards in this Set
- Front
- Back
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acanthocyte
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spiky RBC
liver disease, abetalipoproteinemia |
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basophilic stippling
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blue dots in the RBC which are left over ribosomes (RNA)
Baste the ox TAIL Thalassemia anemia of chronic disease iron deficiency anemia Lead poisoning |
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Bite Cell
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like a bite mark in the cell
associated with G6PD deficiency |
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Ellipotcytes
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Elliptical cell
heredity elliptocytosis |
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Macro-ovalocyte
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enlarged oval cell
megaloblastic anemia, marrow failure |
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ringed sideroblast
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nucleated RBC with iron accumulation around perinuclear
sideroblastic anemia |
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schistocyte
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fragmented RBC - jagged edges
see in: DIC traumatic hemolysis (heart valves) thombotic thrombocytopenic papura hemolytic-uremic syndrome |
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spherocyte
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spherical cell
autoimmune hemolysis hereditary spherocytosis (spectrin, ankyrin mutation) |
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teardrop cell
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teardrop shape
bone marrow infiltration - myelofibrosis |
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target cell
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looks like a target
Thalassemia, Liver disease, HbC disease, Asplenia |
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heinz bodies
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dots in RBC
oxidation of iron -> hemoglobin precipitation sticks to RBC membrane - fragile RBC alpha-thalassemia G6PD def |
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howell-jolly bodies
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nuclear remnants (DNA) in RBC
see in asplenia or poorly functional spleen |
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von Wildebrand Factor
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mediates linking of platelet (via Gp1b) to subendothelial collagen - important for platelet adhesion
carries Factor VIII, releases factor VII due to thrombin |
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TxA2
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released by platelets to increase aggregation
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PGI2 and NO
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released by endothelial cells to decrease aggregation
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Aspirin
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decreases TxA2, thus decreasing aggregation
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Ticlodipine, Clopidogrel
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inhibit ADP expression of GpIIb/IIIa
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Abiximab
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antibody to GpIIb/IIIa - blocks
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vitamin K
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needed for production of factors II, VII, IX, X, C, S
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Warfarin
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inhibits expoxide reductase to prevent activation of vitamin K needed for coagulation factors
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antithrombin
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inactivates II, VII, IX, X, XI
induced by heprin |
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Protein C
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activated by protein S and thrombomodulin
cleaves and inactivates factor Va, VIIa |
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factor V leiden mutation
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prevents cleavage and inactivation by activated protein C
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tPA
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converts plasminogen to plasmin
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plummer vinson syndrome
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iron deficiency anemia
esophageal webs glossitis |
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alpha thalassemia
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defect in alpha globulin gene (4 total) - asia and africa
deletion of 4 genes - death - Hb Barts - hydrops fetalis deletion of 3 genes -> Hb H (beta4) disease deletion of 1-2 not associated with anemia |
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beta thalassemia
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mediterranian
minor - beta chain underprodction increases Hb A2 major - beta chain is absent - severe anemia, marrow expansion causes bone deformity especially face |
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Hb S/beta thalassemia
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milder version of sickle cell disease
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lead poisoning
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inhibits ferrochelatase and ALA dehydratase - prevents heme production
basophilic stippling |
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sideroblastic anemia
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defect in heme synth due to alcohol, lead, or defects in delta-ALA synthase
treat with B6 see increased serum iron, and ferritin |
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macrocytic, hypochromic anemias
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iron deficiency
alpha and beta thalaseemia lead poisoning sideroblastic anemia |
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macrocytic anemias
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megaloblastic anemia by B12 def or folate def - due to lack impaired DNA synthisis, normal RNA synth which allows cytoplasm to grow
nonmegaloblastic macrocytic anemia - DNA synthesis not impaired - don't see lots of megaloblasts or hypersegmented neutrophils |
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nonmegaloblastic macrocytic anemias causes
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liver disease, alcoholism (in absence of B12 or folate def), reticulocytosis, metabolic disorder, drugs (AZT, 5-FU)
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normocytic, normochromic, nonhemolytic anemia
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anemia of chronic disease, aplastic anemia, kidney disease (decreased erythropoietin)
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anemia of chronic disease
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inflammation increases hepcidin which prevents release of iron stores from macrophages
decreased iron, decreased TIBC, increased ferritin |
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apalstic anemia
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pancytopenia + replacement of bone marrow with fatty infiltrate
caused by radiation/drugs, viral (parvovirus, EBV, HIV), fanconi anemia, idiopathic |
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intrinsic hemolytic normocytic anemia
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intrinsic means inherent (genetic inheritance)
hereditary spherocytosis G6PD def Pyruvate kinase deficiency sickle cell disease hb C defect paroxysmal nocturnal hemoglobinuria |
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hereditary spherocytosis
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defect in ankrin or spectrin gene important in RBC cytoskeleton - see spherical cells small with no central palor
decreased RBC life, removal by spleen positive osmotic fagility test |
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G6PD deficiency
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defect in G6PD causes decrease in glutathione which is needed for elimination of oxidants
hemolysis after exposure to oxidant stress heinz bodies and bite cells |
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Pyruvate Kinase deficiency
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defective pyruvate kinase decreases the amount of ATP available to maintain RBC cell cytoskeleton - rigid cells
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Hb C disease
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different beta chain mutation than sickle cell disease - milder disease
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paroxysmal nocturnal hemoglobinuria
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complement mediated lysis = intravascular hemoylsis
due to impaired GPI anchor synthesis |
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extrinsic hemolytic normocytic anemia
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autoimmune - warm agglutinin, cold agglutinin, erythroblastosis fetalis
microangiopathic - dmg to RBC by passing through obstructed or narrow vessel infections - malaria, etc |
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microangiopathic anemia
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dmg to RBC by passing through obstructed or narrow vessel
see schizocytes DIC, TTP-HUS, SLE, malignant hypertension prosthetic heart valves can cause |
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cold agglutinin
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autoammune anemia
IgM mediated, triggered by cold seen with mycoplasma pneumonia or mono |
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warm agglutinin
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autoimmune anemia
IgG mediate due to SLE or CLL or drugs |
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erythroblastosis fetalis
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IgG to Rh factor
when Rh+ baby born to Rh- mother, mother makes antibodies to Rh cause hemolysis in fetus of SUBSEQUENT pregnancy |
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test for autoimmune hemolytic anemias
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direct coombs test - patient RBCs tested for already attached autoantibodies
indurect coombs test - patient serum is tested for autoantibodies |
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defective heme synthesis conditions
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lead poisoning
acute intermittent prophyria prophyria cutanea tarda |
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acute intermittent prophyria
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defect in porphobilinogen deaminase
see porphobilinogen, delta-ALA, uroprophyrin in urine 5 Ps symptoms Pain Pink urine Polyneuropathy Psych problems Precipitated by drugs treat with glucose, heme |
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Porphyria cutanea tarda
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defect in Uroprophyrinogen decarboxylase
uroporphyin - tea colored urine most common porphyria blistering cutaneous photosensitivity |
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Lead poisoning symptoms
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acronym: LEAD
Lead lines on gingivae and on epiphysis of long bones encephalopathy and erythrocyte basophilic stippling Abdominal colic and Anemia sideroblastic Drops of wrist and foot |
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treatment for lead poisoning
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1st line treat: dimercaprol and EDTA
succimer for kids |
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succimer
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treatment for lead poisoning in kids
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bernard soulier disease
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defect in platelet plug formation due to decreased Gp1b needed to bind collagen
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glanzamann thrombasthenia
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defect in GpIIb/IIIa - defect in platelet-platelet aggregation
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thrombotic thrombocytopenic purpura
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deficiency in ADAMTS13 - decreased vWF multimer degredation
increased multimers - increases platelet aggregation and thombosis - creation of microclots which occlude small vessels and shear blood cells - hemolysis and schistocytes consumes platelets - increased bleed time/decreases platelet count neurologic and renal symptoms, fever, low platelet count, hemolysis |
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idiopathic thrombocytopenic papura
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autoimmune antibodies to GpIIb/IIIa - platelet destruction
increased megakaryocytes |
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hemophilia A, B
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A - deficiency in factor VIII
B - deficiency in factor IX macrohemorrhage - joints bleeding, bruising, increased bleed time |
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DIC
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widespread activation of clotting factors leads to deficiency of clotting factors -> bleeding
causes: STOP making new thrombi Sepsis Trauma Obstretric copmlications Pancreatitis (acute) Malignancy Nephrotic syndrome Transfusions labs: inc d-dimers, dec fibrinogen, dec factor V and VIII |
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most common inherited bleeding disorder
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vonWildebrand disease
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most common inherited hypercoaguability
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Factor V Leiden - factor V mutation that cannot be degraded by factor C
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hereditary thrombosis syndromes leading to hypercoagulability
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factor V leiden
prothrombin gene mutation ATIII deficiency Protein C or S deficiency |
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leukemia vs lymphoma
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leukemia - lymphoid neoplasm with widespread bone marrow involvement - see tumor cells in blood
lymphoma - descrete tumor masses |
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hodgkin vs nonhogkin lymphoma
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hodgkin - reed-sternberg cells, localized, contiguous and predictable spread from LN to LN, more constitutional symptoms, mediastinal lymphadenopathy, bimodal distribution (young and old), assocated with EBV
nonhogkin - multiple nodes, noncontiguous spread, usually B-cell, less constitutional symptoms, 20-40yo, associated with HIV/immunosuppression, |
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reed-sternberg cell
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associated with hodgkin lymphoma
owl eyes look bilobed or binucleus - mirror images CD30+, CD15+ |
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hodgkin's lymphona types
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nodular sclerosing (most common)
mixed cellularity lymphocytes predominant lymphocyte depleted |
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T/F: increased lymphocyte to RS ratio is worse prognosis for Hodgkin lymphoma
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False - want increased lymphocyte to RS ratio
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non-hodgkin lymphoma types - Bcells
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burkitt
diffuse large b-cell mantel cell follicular |
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non-hodgkin lymphoma types - Tcells
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adult t-cell
mycosis fungoides/sezary syndrome |
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burkitt's lymphoma
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young
t(8,14) c-myc(8) next to heavy chain Ig(14) starry sky appearance - lymphocytes with macrophage intersperce EBV |
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diffuse large b cell lymphoma
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most common adult Nonhodgkin lymphoma
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mantle cell lymphoma
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adults
t(11;14) - cyclin D1/bcl-1 overexpression CD5+ |
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follicular lymphoma
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t(14;18) bcl-2 next to heavychain Ig
bcl-2 overexpression inhibits apop |
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adult t-cell lymphoma
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HTLV-1 caused
cutaneous lesions adults - japan, africa, carribean |
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mycosis fungoides/sezary syndrome
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cutaneous patches
adults |
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multiple myeloma
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monoclonal plasma cell secretes IgG or IgA
M protein spike bence jones proteins (Ig light chain) rouleux formation associated with amyloidosis and punched out bone lesions hypercalcemia, increased infection, anemia, renal failure |
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acute lymphoblastic leukemia (ALL)
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children
bone marrow replaced with lymphocytes TdT+, CALLA+ t(12;21) - better prognosis |
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leukemia types
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acute/chronic lymphoblastic (chronic lymphoblastic leukemia = small lymphocytic lymphoma)
acute/chronic myelogenous hairy cell leukemia (lymphoid origin) |
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small lymphocytic lymphoma (SLL), chronic lymphocytic leukemia (CLL)
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elderly
smudge cells warm antibody autoimmune hemolytic anemia |
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hair cell leukemia
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elderly
Bcells with hair like projections stains with TRAP |
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Acute myelogenous leukemia
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Adults
Auer rods circulating myeloblasts M3 treat with vit A |
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chronic myelogenous leukemia
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philadelphia chromosome t(9,22), bcr-abl fusion
increased neutrophils, metamyelocytes, basophils, splenomegaly, can turn into AML or ALL low leukocytes alk phos |
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auer rods
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peroxidase positive cytoplasmic inclusions in granulocytes and myelocytes
see in acute promyelocytic leukemia. treatment can release auer rods -> DIC |
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langerhans cell histocytosis
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proliferative disorder of dendritic cells (langerhans cells)
expresion of S-100, CD1a, birbeck granules (tennis racket in EM) aka eosinophlic granuloma |
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chronic myeloproliferative disorders types
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polycthemia vera
essential thrombocytosis myelofibrosis CML |
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polycthyemia vera
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clone of hematopoietic stems cells sensitive to growth factors
see increased RBC, WBC, Platelets JAK2 mutation+ |
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essential thrombocytosis
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megakaryocytes sensitive to growth factors - increased platelets
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myelofibrosis
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bone marrow replaced by fibrosis
decreased RBC variable effects on WBC, Platelet |
