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84 Cards in this Set
- Front
- Back
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Anisocytosis
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varying sizes of RBCs
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Poikilocytosis
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varying shapes of RBCs
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Life span of RBC
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120 days
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Life span of platelet
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8-10 days
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vWF receptor
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Gp1b
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Fibrinogen receptor
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GpIIb/IIIa
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Thrombocytopenia or platelet dysfunction results in
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Petechiae
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WBC differential from highest to lowest
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Neutrophils 40-60%
Lymphocytes 20-40% Monocytes inc lymphocytes 2-8% Eosinophils 1-4% Basophils 0.5-1% |
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Lactoferrin
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binds iron and inhibits growth of phagocytosed bacteria and some fungi
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Hypersegmented polys seen in
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vit B12/ folate deficiency
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Causes of eosinophilia
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NAACP
Neoplastic, Asthma, Allergic processes, Collagen vascular diseases, Parasites- invasive |
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Eosinophil
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produces histaminase and arylsulfatase- help limit rxn following mast cell degranulation
packed with large eosinophilc granules, defends against helminthic infxns with major basic protein |
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Basophil
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densely basophilic granules, containing heparin, histamine and other vasoactive amines, and leukotrienes- LTD4
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Mast cell
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med allergic rxn, degranulation- histamine, heparin and eosinophil chemotactic factors, can bind Fc portion of IgE to membrane
type I hs rxn |
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Cromolyn sodium
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prevents mast cell degranulation
used for asthma prophylaxis |
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Dendritic cells on skin are
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Langerhans cells
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Majority of circulating lymphocytes are
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T cells- 80%
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anti AB antibodies
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IgM- do not cross placenta
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anti Rh antibodies
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IgG- cross placenta
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AB blood group
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A and B antigens on RBC surface, no antibodies in plasma
universal recipient |
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O blood group
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no A or B antigens on RBC surface, both antiboies in plasma
univeral donor |
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Tx for Rh negative mothers
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Rho(D) immune globulin (Rhogam) for mother at first delivery to prevent future erythroblastosis
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Antithrombin
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inhibits thrombin and factors VIIa, IXs, Xa, XIa, XIIa
Activated by heparin |
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Protein C activated by
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Thrombomodulin- endothelial cells
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APC with protein S
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cleaves and inactivates Va, VIIIa
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tPA
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tissue plasminogen activator
plasminogen converts to plasmin, which cleaves fibrin mesh to fibrin degradation products used clinically as a thrombolytic! |
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Pro aggregation factors
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TXA2 released by platelets, dec blood flow and in platelet aggregation
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Anti aggregation factors
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PGI2 and NO released by endothelial cells, inc blood flow and dec platelet aggregation
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Platelets release
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ADP and Ca2+, necessary for coagulation cascade
ADP receptor on platelet irr blocked by clopidogrel and ticlopidine |
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inc ESR
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infxns, inflam dse, malignant neoplasms, gi dse, preg
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dec ESR
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polycythemia, sickle cell anemia, CHF, microcytosis, hypofibrinogenemia
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Macro-ovalocyte
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seen in megaloblastic anemia, marrow failure
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Basophilic stippling
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BASte the ox TAILS
seen in Thal, ACD, Iron def, Lead poisoning, Sideroblastic anemia |
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Schistocyte
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helmet cell
seen in DIC, TTP/HUS, traumatic hemolysis |
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Bite cell
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G6PD def
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Target cell
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HALT said the hunter to his TARGET
HbC dse, Asplenia, Liver dse, Thal |
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Plummer Vison syndrome
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triad of iron def anemia- microcytosis and hypochromia, esophageal web, and atrophic glossitis
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alpha thalassemia
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defect in alpha globin synthesis
4 gene deletion- Hb barts- gamma 4, incompatible with life, causes hydrops fetalis 3 gene deletion- HbH dse, beta 4 1-2 gene deletion- no significant anemia |
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Beta thal minor
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heterozygote, usually asympomatic, inc HbA2 >3.5% on electrophoresis
inc HgF! |
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Beta thal major
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homozygote, bea chain abset, severe anemia req transfusion, marrow expansion- crew cut on skull xray
inc HgF! |
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Lead poisoning sx
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Lead Lines on gingivae and epiphyses of long bones
Encephalopathy and Erythrocyte basophilic stippling Abdominal colic and sideroblastic Anemia Drops- wrist and foot drop |
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Sideroblastic anemia
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defect in heme synthesis, X linked defect in delta aminolevulininc acid synthase gene, ringed sideroblasts, inc iron, normal TIBC, inc ferritin
tx pyridoxine therapy |
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Intravascular hemolysis
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dec haptoglobin, inc LDH, hgb in urine
ex PNH, mechanical destruction |
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Extravascular hemolysis
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macrophage in spleen clears RBC, inc LDH, inc UCB, causes jaundice
ex hereditary spherocytosis, sickle cell anemia |
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ACD findings
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dec iron, dec TIBC, inc ferritin
normocytic nonhemolytic anemia can become microcytic in long standing dse inflammation causes release of hepcidin, binds ferroportin on intestinal mucosal cells and macrophages, inhib iron transport, dec release of iron from macrophages |
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Pyruvate kinase def
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AR, defect in pyruvate kinase leads to dec ATP, rigid RBCs, hemolytic anemia in newborn
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HbC defect
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glutamic acid to lysine mutation at position 6 in chain!
HbSC milder than HbSS |
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PNH
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impaired syn of GPI anchor/ decay accelerating factor in RBC membrane, inc complement mediated RBC lysis
thrombosis! inc urine hemosiderin |
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Sickle cell anemia
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HbS point mutation causes single AA replacement in bet chain- substitute glutamic acid with valine at position 6
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tx Sickle cell anemia
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hydroxyurea- inc HbF
bone marrow transplant |
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Autoimmune hemolytic anemias are usually Coomb's...
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POSITIVE
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Direct Coomb's test
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anti Ig antibody added to pts RBCs agglutinate if RBCs coated with Ig
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Indirect Coomb's
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normal RBCs added to patients serum agglutinate if serum has anti-RBC surface Ig
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Acute intermittent porphyria
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porphobilinogen deaminase affected, get painful abd, red wine colored urine, polyneuropathy, psych disturbances, precipitated by drugs, tx glucose and heme, which inhibits ALA synthase
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Porphyria cutanea tardad
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uroporphyrinogen decarboxylas, affected, tea colored urin, blistering cutaneous photosensitivity, most common porphyria
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Increase bleeding time
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defects in platelet plug formation
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Bernard Soulier disease
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defect in platelet plug formation- dec Gp1b- defect in platelet to collagen adhesion
dec platelet count, inc bleeding time |
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Glanzmann's thrombasthenia
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dec GpIIb/IIIA- defect in platelet to platelet aggregation
inc BT |
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ITP
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dec platelet survival, anti GpIIb/IIIA antibodies- peripheral platelet destruction, inc megakaryocyte
dec PC inc BT |
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TTP
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def of ADAMTS13- vWF metalloprotease- dec degradation of vWF multimers
inc large multimers- inc platelet aggregation and thrombosis dec PC inc BT |
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tx von Willebrand's dse
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DDAVP- desmopressin- releases vWF stored in endothelium
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Causes of DIC
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sepsis from gram neg, trauma, obstetric complications, acute pancreatitis, malignancy, nephrotic syndrome, transfusion
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Cryoprecipitate
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contains fibrinogen, factor VIII and XIII
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FFP
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inc coagulation factors levels by 20%
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Leukemoid rxn
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inc WBC count with left shift- 80% bands, and inc leukocyte alk phos
usually due to infxn |
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Reed Sternberg cells
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tumor giant cell in Hodgins
CD30+ and CD15+ B cell origin |
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Most common adult NHL
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Diffuse large B cell lymphoma
tx R CHOP |
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Mantle cell lmphoma
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B cell NHL
t(11,14) overexpression of cyclin D1 CD 5+ Poor prognosis |
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Follicular lymphoma
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Indolent course, difficult to cure
2nd more common adult NHL B cell t(14,18) bcl-2 expression |
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Most common primary tumor arising within bone in elderly
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Multiple Myeloma
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MM
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CRAB
hyperCalcemia Renal insufficiency Anemia Bone lytic lesions/Back pain monoclonal M protein spike produces large amounts of IgG 55% and IgA 25% |
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Bence Jones protein
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Ig light chains in urine
seen in MM |
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ALL
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MC in children
b replaced by lymph TdT+, CALLA+ (CD10+) Most responsive to tx May spread to CNS and testes t(12,21) better prognosis |
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SLL/CLL
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smudge cells in peripheral blood smear- artifact
CD 5+ |
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Hairy cell leukemia
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mature B cell tumor in elderly
cells have filamentous hair like projections stains TRAP positive |
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M3 AML subtype (APML) responds to what tx
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all trans retinoic acid (vit A)
induces differentiation of myeloblasts see Auer rods! t(15,17) |
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CML
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Philadelphia chromosome
t(9,22) bcr-abl myeloid stem cell prolif can accelerate and transform to AML or ALL- blast crisis very low leukocyte alk phos responds to Imatinib! |
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Tx of AML M3 can release
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Auer rods
can cause DIC! |
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Langerhans cell histocytosis
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proliferative dso of dendritic langerhans cells from monocyte lineage
immature cells, express S100 and CD1a, birbeck granules- tennis rackets on EM |
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Production of inappropriate ectopic erythropoietin can occur in
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RCC, WIlm's ymor, cyst, HCC, hydronephrosis
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Beta thal minor
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heterozygote, beta chain underproduced, dx confirmed by inc HbA2 on electrophoresis
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ACD
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liver produces inc amounts of hepcidin in response to inflammatory cytokines, which stops ferroportin from releasing iron stores, allows body to keep iron away from bacterial pathogens while producing more leukocytes
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HIT
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heparin induced thrombocytopenia
heparin binds to platelet factor IV, causing antibody production that binds to and activates platelets, results in thrombocytopenia, hypercoag |
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tx tPA (alteplase), a thrombolytic, toxicity
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Aminocaproic acid, an inhibitor of fibrinolysis
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