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209 Cards in this Set
- Front
- Back
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Source of energy for RBCs
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GLUCOSE
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How do RBCs metabolize glucose?
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90% anaerobicaly to LACTATE
10% by HMP shunt |
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What is the membrane of RBCs vital for?
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Maintaining the Chloride-Bicarb antiporter to allow for Chloride-Bicarb shift
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What is Cl-HCO3 shift for?
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Transporting CO2 from peripheral tissues back to the lungs for expiration - its just in the form of bicarb.
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3 major components of BASOPHIL granules; purpose of each:
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-Histamine - vasodilator
-Heparin - anticoagulant -LTD4 - bronchoconstrictor, vasoconstrictor, etc. |
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Cell that mediates ALLERGIC REACTION:
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MAST cell
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3 things released from Mast cell degranulation:
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-Histamine
-Heparin -Eosinophil chemotactic factors |
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What binds to the membrane of Mast cells?
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IgE - sensitization to antigen
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How are mast cells and basophils similar and different?
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Similar: structure/function
Different: Basos-blood Mastcells-tissue |
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What HSN are mast cells involved in?
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Type I
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What prevents Mast cell degranulation? For what condition?
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Cromolyn sodium - Asthma
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Eosinophils defend against what infections, with what substance:
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-Parasites
-Protozoa Major basic protein |
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2 things that Eosinophils produce other than MBP:
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-HistaminASE
-Arylsulfatase |
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5 'causes' of eosinophilia:
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NAACP
-Neoplasia -Asthma -Allergy -CT vasculitides (Churg Strauss) -Parasites |
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Primary granules of PMNS contain:
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Hydrolytic enzymes - Lysozyme, myeloperoxidase, lactoferrin
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Hypersegmented PMNs are seen in:
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-Vit B12 deficiency
-Folate defic |
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How do Macrophages arise?
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Monocytes migrate from blood and differentiate into macrophs in tissue
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What is the lifespan of macrophages in tissues like?
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LONG
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What are Macrophages activated by?
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Gamma interferon
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What disease do we give Gamma interferon to stimulate macrophages?
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Chronic granulomatous disease
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What are 2 functions of macrophages?
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-Eat stuff - phagocytosis
-APC - MHCII |
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2 B cell markers:
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CD19, CD20
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B cells do what when Ag is presented to them:
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Differentiate into plasma cells to produce antibody
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Bcells arise from:
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Bone marrow
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Bcells mature in:
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-Lymph node follicles
-Spleen white pulp -Unencapsulated lymphoid tissue |
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What can Bcells do in addition to make antibody?
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Present Antigen on MHC II
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Where do Plasma cells make antibody?
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Rough ER - abundant RER
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Neoplasm of Plasma cells:
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Multiple myeloma
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What are the main inducers of a primary antibody response?
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Dendritic cells - professional APCs
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What are dendritic cells in the skin?
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Langerhan's cells - birbeck granules; tennis racquet shape
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What is TENASE in the coag cascade?
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9a, 8a, and calcium/phospholipid
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What activates Prothrombin?
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Xa, 5a, and Calcium/phospholipid
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What does Thrombin do?
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Cleaves Fibrinogen I into Fibrin Ia
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What makes crosslinked fibrin clots?
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13
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What do Protein C and S do?
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Inactivate 5a and 8a
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What will resist Protein C?
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Factor 5 Leiden mutants
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What will make deficiency of Protein S/C?
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Vit K deficiency
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What does AT3 inactivate?
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2, 9a, 10a, and 11a
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What does AT3 need to be able to adequetely inactivate all these factors?
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HEPARIN
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What generates Plasmin from Plasminogen?
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tPA
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What does Plasmin do?
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Cleaves Fibrin clots
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What are 3 pathways that converge by the activity of one factor?
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-Coagulation
-Complement -Kinin pathway |
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What is the factor that brings them all together?
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HAGEMAN factor!
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What is Hagemen factor?
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Factor XIIa - 12a
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What is Factor 12 activated by?
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HMWK, collagen, basement membrane, and activated plts
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How does Hageman factor activate the Clotting cascade?
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12a activates 11a.. the intrinsic cascade
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How does Hageman factor activate the Kinin cascade?
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By activating Kallikrein from Prekallikrein
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What does Kallikrein activate?
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HMWK -> Bradykinin
Plasminogen -> Plasmin |
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So Kallikrein has 2 activities:
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-Kinin cascade activation
-Fibrinolysis activation! |
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How does Hageman factor get the COMPLEMENT cascade going?
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Plasminogen activates it
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What are the 3 effects of Bradykinin that gets produced by the action of Kallikrein on HMWK?
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-Vasodilation of arterioles
-Vasoconstriction of veins -Increased vasc permeability - leads to edema -PAIN |
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What is the universal blood donor?
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Type O packed cells
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Acanthocytes are a sign of:
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Abetalipoproteinemia
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What should you do if you see TARGET CELLS?
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HALT
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HALT is:
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-HbC disease
-Asplenia -Liver disease -Thalassemia! |
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When would you see Poikilocytes and Burr cells?
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TTP/HUS and DIC - microvascular damage
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What are laminated concentric calcific spherules called?
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Psamomma bodies
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What are 4 conditions in which you find Psammoma bodies?
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-Papillary adenocarcinoma of the THYROID
-Serous cystadenocarcinoma ovary -Meningioma -Malignant mesothelioma |
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What are 2 drugs that can cause Megaloblastic anemia due to decreased DNA synthesis?
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-Sulfa drugs
-AZT |
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How to differentiate Fe deficiency anemia from anemia of chronic disease when both are hypo/micro:
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Iron studies:
-Increased serum Ferritin and iron stores in chronic disease - you're just not using it! |
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What is the TIBC in Fe defic anemia and Anemia of Chronic disease compared to normal?
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Fe defic anemia: HIGH TIBC
ACD: Lower TIBC than normal - the excess unused Fe takes up space on Transferrin |
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What are 6 drugs/treatments that can cause APLASTIC anemia?
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RBC - AA - T
Radiation Benzene Chloramphenicol Alkylating agents Antimetabolites (azathioprine) expired Tetracycline |
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How does expired tetracycline cause aplastic anemia?
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By causing Fanconi's syndrome
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What are 4 viral causes of aplastic anemia?
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-Parvovirus B19
-CMV -HIV -Acute hepatitis |
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What will be symptoms of aplastic anemia?
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Everything that needs blood cells - RBC, WBC, platelets - anemia, infections, petechiae
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How is aplastic anemia diagnosed?
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Bone marrow biopsy
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What will the bone marrow look like in Aplastic anemia?
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Hypocellular with FATTY infiltration
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What will the PBS look like in aplastic anemia?
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Hypocellular but NORMOcells!
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How is Aplastic anemia treated?
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-Transfusions
-BMT -G-CSF or GM-CSF |
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What is recombinant G-CSF?
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Filgrastim
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What is GM-CSF?
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both grans and macrophage CSF
-Sargramostim |
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In what Hb chain is the AA substitution that makes Sickle cell anemia? What is the mutation?
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Beta - Val replaces Glutamic acid at pos 6
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What % of AA's have the trait? What % have the disease?
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8% are trait AS
only .2% have the disease SS |
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What is a common finding on Xray of patients skulls in SS disease?
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Crew-cut appearance from marrow expansion
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When else is crew cut appearance seen?
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In thalassemias
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What precipitates sickling of red cells in SS disease?
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Low O2 or dehydration
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What is special about people with AS trait?
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They resist P. falciparum due to a BALANCED POLYMORPHISM
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Why do homozygotes with SS disease get aplastic crises?
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Due to Parvovirus B19 infections
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What happens to their spleens?
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Autosplenectomy
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What bacteria are likely to infect patients with SS disease?
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Encapsulated organisms
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What causes osteomyelitis in patients with SS disease?
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SALMONELLA
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What are SS patients treated with?
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Hydroxyurea to increase HbF
BMT |
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Where are alpha-Thals prevalent?
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Asia/Africa
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Where is B-thal prevalent?
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Mediterranean
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What is Hb H and in what disease is it produced?
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Hb H is Beta-4 tetramers; produced in 3-gene deletion a-thalassemia
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What is Hb-Barts and in what disease is it produced?
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y-4 gamma tetramers; produced in a 4-gene deletion a-thalassemia
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What does a 4-gene deletion of alpha globin chains result in?
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Hydrops fetalis and intrauterine fetal death
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What is the difference between Beta-thal MINOR vs MAJOR?
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Minor = heterozygote
Major = homozygous lack of B-globin chains |
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What is required for B-thal major to be compatible with life?
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Transfusions for life
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What complication can blood transfusions for life lead to?
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Cardiac failure from HEMOCHROMATOSIS
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How does hemochromatosis lead to cardiac failure?
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From liver cirrhosis due to iron deposits; nutmeg liver -> Right heart failure
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What compensatory mechanism is seen in Beta-thal but not in Alpha thal?
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Increased fetal hemoglobin - HbF and HbA2
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What is another major systemic manifestation of Beta thal?
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Bone deformity due to marrow expansion
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What are 2 things that all the Hemolytic anemias will cause?
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-Increased serum bilirubin
-Increased reticulocytes |
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What are 2 complicatinos of increased serum bilirubin?
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-Jaundice
-Pigmented gallstones |
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What are increased retics a sign of?
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BM compensating for anemia
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What type of hemolysis occurs in Autoimmune hemolytic anemias?
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mostly EXTRAvascular
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Where is Extravascular hemolysis?
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In the RES - macrophages of the liver and spleen (Kupffer cells)
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What type of antibody are Warm agglutins vs Cold?
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Warm = IgG
Cold = IgM |
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What lab test is positive in all autoimmune hemolytic anemias?
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DAT and IAT coombs pos
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What does a DAT tell you?
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If a patient's cells have auto-Ab on them already
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What does an IAT tell you?
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If a patient's serum has AutoAb floating in it
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What are 3 conditions in which WAIHA develops?
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-Systemic lupus
-CLL -Drugs - alpha-methyldopa!!! |
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When does Cold AIHA develop?
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During recovery from M. pneumonia or infectious mono
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What is the onset of CAIHA like?
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Acute anemia triggered by cold
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What AIHA occurs in newborns?
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Erythroblastosis fetalis
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Is the COOMBs test positive in Hereditary spherocytosis?
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nope
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What are 2 RBC parameters abnormal in Hered spherocytosis?
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-Increased RDW due to the little spherocytes
-Increased MCHC - they're denser |
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What is PNH caused by?
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A somatic mutation of the PIG-A gene causing RBCs to lack CD59
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What does lack of CD59 mean?
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Increased sensitivity to Complement - RBCs lyse
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What is the finding in PNH?
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-All cells are lysed - pancytopenia
-Hemoglobinuria in the morning |
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4 conditions in which Microangiopathic anemia can occur:
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-DIC
-TTP/HUS -SLE -Malignant hypertension |
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What is DIC?
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Activation of the coag cascade leading to microthrombi and consumption of platelets, fibrin, and clotting factors
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How do you remember what causes DIC?
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STOP Making New Thrombi
-Sepsis, Trauma, Obstretic complications, Pancreatitis, Malignancy, Nephrotic syndrome, Transfusions |
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What is the most common cause of DIC?
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OBSTRETIC COMPLICATIONS
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4 Lab findings in DIC:
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-Prolonged PT, PTT
-Increased d-dimer (FSP) -Low platelets |
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How low does the Plt count have to go before generalized bleeding?
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15-20,000
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Reed-sternberg cells:
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Hodgkin's lymphoma!
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2 cell markers on RS cells; what are they derived from?
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CD15, CD30
-From Bcells |
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What are Non-hodgkins lymphomas typically associated with?
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Immunosuppression and HIV
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what type of nodal involvement is typically seen in
-Hodgkins lymphoma -NHL |
HL = localized, single groups of nodes; INTRAnodal (extranodal is RARE)
NHL = Multiple, peripheral nodes; Extranodal involvement is common! |
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What type of 'spread' is exhibited by HL vs NHL?
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HL = contiguous
NHL = noncontiguous |
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Which type of lymphoma typically exhibits 'B' signs?
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Hodgkins lymphoma
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What are B symptoms?
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Constitutional
-Fevers -Night sweats -Weight loss |
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Which lymphoma causes Mediastinal lymphadenopathy?
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HODGKINS lymphoma
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In what sex are almost all subtypes of Hodgkins lymphoma seen? What is the exception?
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MEN
-except Nodular sclerosing which is in young females |
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What indicates GOOD PROGNOSIS in hodgkins lymphoma?
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-More LYMPHOCYTES
-Few REED-STERNBERG Cells |
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what is the MOST COMMON type of Hodgkin's lymphoma? %? Sex/age?
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Nodular sclerosing
70% Young women |
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What is the prognosis like for Nodular sclerosing HL? Why?
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EXCELLENT
-Many lymphos -Few RS cells |
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What are the 2 main histologic findings in Nodular sclerosing HL?
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-Collagen banding forms nodules
-Lacunar cells |
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What is not common in Nodular sclerosing HL?
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EBV is not really associated with it
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What is the 2nd most common HL? What %? Age/sex? Prognosis? Why?
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Mixed cellularity - 25%
-Older males -Intermed prognosis -More RS cells than Lymphos |
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What % of Mixed cellularity HL cases are associated with EBV?
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MOST - 70%!
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What cells are mixed in with Lymphos and RS cells in Mixed Cellularity type HL?
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-Eos
-Histiocytes -Plasma cells |
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What % of HL cases are Lymphocyte predominant? What age group/sex? Prognosis?
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Only 6% is Lymph predominant
-Younger males <35 -Excellent prognosis - it's lympho predominant! |
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What is the least common but most SEVERE Hodgkin lymphoma type?
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Lymphocyte depleted
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In what patients is Lympho depleted HL mostly seen?
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Older males with disseminated disease
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What does Lympho depleted HL show the highest association with?
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EBV
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What is used for prognostic indication in HL?
PS: what are lacunar cells? |
Ann arbor stage
Lacunar cells are variants of RS cells |
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What cell type is involved in the MAJORITY of Nonhodgkin lymphomas?
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Bcells - except for those of T-lymphoblast origin of course..
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What is the peak age of onset for NHL?
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20-40 YRS OLD
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How does the presentation of NHL differ from HL most markedly?
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No constitutional symptoms
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What is the most common NHL form?
-What age -What cell type -Hallmark feature |
Follicular lymphoma
-Adults -Bcells -t14;18 |
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What is the t14;18 translocation?
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Ig heavy chain promoter on 14 moves near Bcl2 on 18; Bcl-2 is an Anti-apoptotic protein; overexpression means too much cell growth
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What is an indolent smoldering NHL that is closely related to CLL?
-What age -What cell type |
Small cell lymphoma
-Adults -B cells |
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Which NHL can be seen in either children or adults? What age is it MORE common in?
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Diffuse Large Cell lymphoma
-More in adults; only 20% children |
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What type of cells are seen in Diffuse large cell lymphoma?
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80% B cell
20% T cell |
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What does Diffuse large cell lymphoma behave like? Prognosis?
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Aggressive but 50% are curable
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What NHL is usually disseminated, very aggressive and incurable and occurs in older men?
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Mantle cell lymphoma
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What sets Mantle cell lymphoma apart from Follicular lymphoma?
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FL = CD5-
Mantle cell = CD5+ |
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And what is the prognosis for Mantle cell lymphoma like?
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POOR
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What is the hallmark translocation associated with MCL?
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11;14
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What does the t11;14 result in?
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Overexpression of Cyclin-D1 - overactive Bcl-1
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What is Bcl-1?
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Like Bcl-2 - an antiapoptotic protein
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What 2 Nonhodgkin lymphomas are most often seen in CHILDREN? What type of lymphos in both?
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-Lymphoblastic lymphoma - T cell
-Burkitt lymphoma - B cell |
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What are the Tcells that cause lymphoblastic lymphocytes? What is a hallmark histologic feature?
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Precursor T-cells; IMMATURE LYMPHOBLASTS; Convoluted nuclei
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What do most children have when they present with Lymphoblastic lymphoma?
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ALL (blood form) and a Mediastinal mass (tissue form)!!!
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What is the translocation in Burkitt lymphoma? What is the effect?
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t8;14 - c-myc moves from 8 to 14 and gets over-expressed
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What is c-myc?
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A protooncogene
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Hallmark histologic finding in Burkitt lymphoma:
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Starry sky - macrophages eat all the turnover degraded products
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Where is endemic vs sporadic Burkitt lymphoma and what do patients with each get?
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Endemic - Africa - jaw lesions
Sporadic - US - abdominal form |
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And what are Reed-sternberg cells associated with?
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HODGKIN'S lymphomas - NOT NHL
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Are RS cells necessary or sufficient to diagnosis HL?
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-Necessary
-Not sufficient |
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t9;22
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Philadelphia Ch' - CML; BCR-Abl hybrid - increased tyrosine kinase activity
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t8;14
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Burkitt lymphoma overactive c-myc oncogene
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t14;18
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Follicular lymphoma; Bcl2 overactivation; too much inhibition of apoptosis
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t15;17
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M3 promyelocytic AML - responsive to ATRA
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t11;22
|
Ewings Sarcoma!
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t11;14
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Mantle cell lymphoma - overactive Cyclin D1 and Bcl-1
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What is the monoclonal plasma cell cancer?
|
Multiple myeloma!
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Classic appearance of MM cells on PBS:
|
Fried egg (dont confuse this with oligodendroglioma)
|
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What are the 2 types of Ig that can be produced in Multiple Myeloma?
|
IgG - usually, or IgA - less
NOT IgM though you'd think M goes with M. |
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What is interesting about multiple myeloma?
|
It is the MOST COMMON PRIMARY BONE TUMOR arising within the bones of adults (children its osteosarcoma).
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What lab finding shows the destruction of bone in multiple myeloma?
|
Hypercalcemia
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What are the 3 main complications of Multiple myeloma?
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-Renal insufficiency
-Susceptible to infections -Anemia |
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What are 2 additional hallmark findings in patients with Multiple myeloma?
|
-Primary amyloidosis
-Punched out lytic bone lesions on Xray |
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What 2 lab tests are highly indicative of multiple myeloma?
|
SPE - shows M spike in the gammaglobulins
-Urinalysis shows bence jones proteins |
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What does the PBS show that is highly indicative of MM?
|
Rouleaux
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In what condition is there an M-spike made up from IgM immunoglobulins?
|
WALDENSTROM MACROGLOBULINEMIA!!!
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What is the disease of malignant circulating leukocytes?
|
Leukemia
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What are the 4 forms of leukemia?
|
ALL
AML CLL CML |
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Which form is most responsive to therapy? In what age does this occur?
|
ALL - children
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What can ALL in children spread to?
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Testes and CNS
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What patient age tends to get AML? 2 major findings?
|
Adults - Auer rods and Myeloblasts
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What age patients get CLL?
|
Older adults
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What are the 2 most common presenting symptoms in CLL?
|
-Lymphadenopathy
-Hepatosplenomegaly |
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What is the course of CLL like typically?
|
Indolent and smoldering
|
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Hallmark morphologic finding in CLL:
|
Smucge cells
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What RBC problem develops secondary to CLL?
|
Warm AIHA
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And what is CLL very similar and closely related to?
|
Small lymphocytic lymphoma - Nonhodgkins lymphoma
|
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Which leukemia presents with increased neutrophils, metamyelocytes, and splenomegaly?
|
CML
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Hallmark feature of CML:
|
Philadelphia chromosome; t9;22 - Bcr-Abl fusion product
|
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How is CML differentiated from a leukemoid reaction?
|
Very low LAP despite the extremely high WBC count
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What can CML transform into if it enters a blast crisis?
|
AML
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Hallmark finding that tells you something is MYELOID leukemia:
|
Auer rods
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What ARE Auer rods? What do they stain positive with?
|
Peroxidase positive cytoplasmic inclusions in grans/myeloblasts
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In what form of leukemia are Auer rods seen primarily? What can treatment of this lead to?
|
M3 - promyelocytic; ATRA releases Auer rods and causes DIC!!!
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What is HISTIOCYTOSIS X?
|
Leukemic proliferation of HISTIOCYTES from the Langerhan's lineage infiltrating the lung.
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What patients are primarily affected with Histiocytosis X and what makes the condition worse?
|
Young adults - SMOKING EXACERBATES IT
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What are the 3 FAB classifications of lymphocytic leukemia?
|
L1, L2, L3
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What does L1 correspond with?
|
ALL - null
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What is L2?
|
Acute ALL-T type
or Chronic CLL-T which is Sezary syndrome |
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What is Sezary syndrome?
|
The leukemic form of mycosis fungoides
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What is L3?
|
Bcell leukemias - ALL or CLL of the B type.
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So what is each cell type?
L1 L2 L3 |
L1 = null
L2 = T L3 = B |
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What WHO classes correspond with the FAB M1?
|
Acute AML
Chronic Myeloproliferative syndromes - CML, PV, ET, IMF |
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What are M2, M3, M4, and M5?
|
M2 = AML
M3 = Promyelocytic M4 = Myelomonocytic M5 = Acute monocytic ONLY |
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What is M6?
|
Acute erythroleukemia
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What is M7?
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Acute megakaryocytic leukemia
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