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Step 1 11.28.12
Hematology VIII
| Question | Answer |
|---|---|
| What is the mechanism of macrocyic anemias? | impaired DNA synth--> maturation of nucleus is delayed relative to cyto. can also be ineffective erythropoeis-->pancytopenia |
| What are some of the major etiologies of Folate deficiency macrocytic megaloblastic anemia? | malnutrition (esp alcholics), malabsorbtion, impaired metabolism (methotrexate, trimethoprim), incr requirement (hemolytic anemia, pregnancy) |
| What findings are seen in folate deficiency macrocytic megaloblastic anemia? | hyper segmented neutrophils, glossitis, decr folate, incr homocysteine but normal methylmalonic acid |
| What are the major etiologies of B12 cobalamin deficiency macrocytic megaloblastic anemia? | insufficent intake (vegans), malabsorbtion (Crohns), pernicious anemia, Diphyllobothrium latum (fish/tapeworm) |
| What are the key findings in B12deficiency macrocytic megaloblastic anemia? | hypersegmented neutrophils, glossitis, decr b12, incr homocysteine, incr methylmalonic acid. neurologic sx |
| What is subacute combined degeneration? When is it seen? | Neuro sx due to B12 anemia and deficiency: 1. peripheral neuropahty with sensory dysfx 2. posterior columns (vibration/proprioception) 3. lateral corticospinal (spasticity) 4. dementia |
| What causes a nonmegaloblastic macrocytic anemia? | macrocytic but DNA synth is not affected. caused by liver disease, alchoholism, reticulocytosis, metabolic disorders (orotic aciduria), congenital deficiencies of purine and pyrimadine synth |
| What drugs might cause a nonmegaloblastic macrocytic anemia? | 5-FU, AZT, hydroxyurea |
| What findings might be seen in a nonmegaloblastic macrocytic anemia? | macrocytosis and bone marrow surpression with normal folate/B12 |
| What findings are seen in normocytic, normochromic intravascular hemolytic anemia? | decr haptoglobin, incr LDH, hemoglobin in urine |
| What findings are seen in normocytic, normochromic extravascular hemolytic anemia? | macrophage in spleen clears RBCs. see incr LDH and incr UCB which causes jaundice |
| What types of diesease might cause normocytic, normochromic extravascular hemolytic anemia? | hereditary spherocytosis, G6PD deficiency, SCA |
| What diseases might cause normocytic, normochromic intravascular hemolytic anemia? | (paroxysmal nocturnal hemoglobinuria, mechanical destruction of aortic valve) |
| What are 3 types of settings for nonhemolytic, normocytic anemia? | anemia of chronic disease, aplastic anemia, kidney disease |
| What is the pathogenesis of anemia of chronic disease? | inflammation-->incr hepcidin (released by liver and inhibits iron transport)-->decr iron release from macrophages |
| What findings are seen in anemia of chronic disease? | decr iron, decr TIBC, incr ferritin. can become microcytic hypochromic in long standing disease |
| What is the pathogenesis of aplastic anemia? | failure or destruction of myeloid stem cells due to: 1. radiation and drugs 2. viral agents 3. fanconis anemia 4. idiopathic (maybe post acute hepatitis) |
| What drugs can cause aplastic anemia? | benzene, chloramphenicol, alkylating agents, antimetabolites |
| What viral agents can cause aplastic anemia? | parvovirus B19, EBV, HIV, HCV |
| What findings are seen in aplastic anemia? | pancytopenia with severe anemia, neutropenia, thrombocytopenia. normal morphology but hypocellular bone marrow with fatty infiltration |
| What sx are seen in aplastic anemia? | fatigue, malaise, pallor, purpura, mucosal bleeding, petichiae, infection |
| How do you tx aplastic anemia? | withdraw offending agent, immunosurpression, allogenic BMT, RBC and platelet transfusion, G-CSF |
| How can kidney disease cause an anemia? | decr erythropoetin--->decr hematapoesis |
| What type of anemia is hereditary spherocytosis? | intrinsic extravascular hemolytic normocytic anemia |
| What defect is present in hereditary spherocytosis? | defect in proteins interacting with RBC membrane skelton and plastma membrane ( ankirin, band 3, protein 4.2, or spectrin) |
| What do RBCs look like in hereditary spherocytosis? WHy? | less membrane causes small and round RBCs with no central pallor(incr MCHC, incr RDW)=premature removal of RBCs by spleen |
| What are the key findings seen in hereditary spherocytosis? | splenomegaly, aplastic crisis (B19 infection, Howell-Jolly bodies seen after splenectomy |
| What labs are seen in hereditary spherocytosis? | positive osmotic fragility test |
| How is hereditary spherocytosis treated? | splenectomy |
| What type of anemia is caused by G6PD deficiency? | intrinsic intravascular hemolytic normocytic anemia |
| What is the defect an pathogenesis of G6PD anemia? | X linked, deficiency in G6PD. decr glutathione causes incr RBC suceptibility to oxidant stress. see hemolytic anemia following oxidant stress |
| What are 3 things which could ellicit an oxidant stress situation in a G6PD defieicny? | sulfa drugs, infections, fava beans |
| What are the findings seen in G6PD defieicny anemia? | back pain, hemoglobinuria a few days later |
| What labs are seen in G6PD deficiency anemia? | blood smear showing RBCCs with heinz bodies and bite cells |
| What type of anemia is pyruvate kinase deficiency anemia? | intrinsic extravascular hemolytic normocytic |
| What is the defect, mech and inheritance of pyruvate kinase deficiency anemia? | autoR. defect in pyruvate kinase-->decr ATP--> rigid RBCs |
| What is the major setting in pyruvate kinase deficiency anemia? | hemolytic anemia in a newborn |
| What is the defect in HbC defect anemia? | glutamic acid to lysine mutation at position 6 in the chain . |
| What can be said about the varying severity of HbC defect in different patients? | patients with HbSC (1 of each mutant) have milder disease than those with 2 genes |
| What type of anemia is paroxysmal nocturnal hemoglobinuria anemia? | intrinsic intravascular hemolytic normocytic anemia |
| What is the pathogenesis of paroxysmal nocturnal hemoglobinuria anemia? | incr in complement mediated RBC lysis ( impaired synth of GPI anchor/decay accelating factor in RBC membrane) |
| What labs are seen in paroxysmal nocturnal hemoglobinuria anemia? | incr urine hemosiderin |
| What is a major complication of paroxysmal nocturnal hemoglobinuria anemia? | thrombosis |
| What is the derangement in Sickle cell anemia? | HbS point mutation causes a single amino acid replacement in the beta chain (subs glutamic acid with valine) at position 6 |
| What type of anemia is SCA? | intrinsic extravascular hemolyic normocytic anemia |
| What is the pathogenesis of SCA? | low O2 or dehydration precipitates sickling (deox HbS polymerizes) which results in anemia and venoocclusive disease |
| Why are newborn initially Asx in SCA? | have incr HbF and less HbS, this changes as the child develops |
| What are SCA heterozygotes called? Why might it be advantageous? | heterozygotes= sickle cell trait. they can reist malaria. 8% of AA have sickle cell trait |
| What is seen on a skull XR in SCA? | "crew cut" due to marrow expansion due to incr eryhtropoesis( also seen in thalassemias) |
| What virus might cause an aplastic crisis in SCA? | parvovirus B19 |
| An autosplenectomy occuring during SCA puts the pt at higher risk for? | infection with encapsulated organisms (Howell-Jolly bodies), functional splenic dysfunction in early childhood |
| Salmonella osteomyelitis ia a complication of? | SCA |
| What types of painful crises can happen in SCA? | painful crises ( vasoocclusive), dactylitis(hand), acute chest syndrome |
| How can SCA cause renal papillary necrosis and microhematuria? | necrosis due to low O2 in the papilla, and microhematuria due to medullary infarcts |
| How do you treat SCA? how does it work? | hydroxyurea (incr HbF) and bone marrow transplatation |
| What are 4 types of extrinsic hemolytic normocytic anemia? | autoimmune hemolytic anemia, microangiopathic anemia, macroangiopathic anemia, infectious anemia |
| What is warm agglutin autoimmune hemolytic anemia? Which Ab is involved? | IgG. chronic anemia seen in SLE, CLL, or with alpha methyldopa use |
| What is cold agglutin autoimmune hemolytic anemia? What Ab is involved? | IgM. acute anemia triggered by cold, seen in CLL, mycoplasma pneumoniae infection, or infectious mononucleosis |
| What type of anemia is erythroblastosis fetalis? What happens? | autoimmune hemolytic anemia in newborns due to Rh or blood incompatability. mothers Abs attack fetal RBCs |
| Are autoimmune hemolytic anemias usually Cooomb's positive? | yes |
| What is the Direct Coomb's test? | anti-Ig Ab are added to patients RBCs, they will agglutinate if RBCs are covered with Ig |
| What is the indirect Coomb's test? | normal RBCs are added to patients serum, they will agglutinate if the serum has anti RBC surface Ig |
| What is the pathogenesis of microangiopathic anemia (intravascular)? | RBCs are damaged when passing through a obstructed or narrow vessel lumina |
| When is microangiopathic anemia seen? | DIC, TTP-HUS, SLE, maligngant hyperTN |
| What is seen on blood smear in microangiopathic anemia? | Schistocyteshelmet cells due to mechanical destruction of RBCs |
| What settings can cause macroangiopathic anemia? | prostetic heart valves. and aortic stenosis |
| What is seen on a blood smear of macroangiopathic anemia? | schistocytes ( helmet cells) |
| What types of infections cause anemia? | ones which destroy RBCs, esp malaria, Babesia |
| What are the key lab values seen in Iron deficiency anemia? | decr serum iron, incr tranferrin or TIBC, decr ferritin, very decr tranferrin saturation |
| What are the key lab values in anemia of chronic disease? | decr serum iron, decr transferrin, incr ferritin, no change to transferrin saturation |
| What are the key lab values in hemochromotosis? | incr serum iron, decr tranferrin, incr ferritin, very incr tranferrin saturation |
| What are the key lab values in pregnancy/OCP use anemia? | no change to serum iron, incr tranferrin, no change to ferritin, decr tranferrin saturation |
| What are the key lab values in lead poisoning"? | incr serum iron, decr tranferrin, no change to ferriitin, incr tranferrin saturation |
| What is the role of tranferrin? | transports iron in the body |
| What is the role of ferritin? | primary store of iron in the body |
| What are the general mech of porphyrias? | defective heme synthesis leads to accumulation of heme precursors |
| What enzyme is affected in lead poisoning? | ferrochelatase and ALA dehydrase |
| What substrate accumulates where in lead poisoning? | protoporphyrin in the blood |
| What are the presenting sx of lead poisoning? | microcytic anemia, GI, kidney disease |
| What happens to kids exposed to lead pain? | mental deterioration due to lead paint |
| What happens to adults with lead poisoning? how do they get it? | battery/ammunition/radiator factory--> see HA, memory loss, demyelination |
| What is the affected enzyme in acute intermittent porphyria? | porphobilinogen deaminase (uroporphyrinogen I synthase) |
| What substrate accumulates where in acute intermittent porphyria? | porphobilinogen, delta ALA, uroporphyrin in urine |
| What Sx are seen in acute intermittent porphyria? | painful abdomen, red wine urine, polyneuropathy, psychological distrubances, can be precipated by drugs |
| What is the treatment for acute intermittent porphyria? | glucose and heme, which inhibit ALA synthase |
| What is the affected enzyme in porphyria cutanea tarda? | uroporphyrinogen decraboxylase |
| What substrate accumulates where in porphyria cutanea tarda? | uroporphyrin in urine ( tea colored) |
| What is the presentation of porphyria cutanea tarda? | blistering cutaneous photosensitivity |
| What enzymes are affected in sideroblastic anemia? Where are they? | delta sminolevulinic acid synthase in mitochondria |
| What does incr Heme mean with regard to aminolevulinic acid (ALA) synthase? | incr heme= decr ALA synthase activity |
| What does PT test? | tests functions of the extrinsic pathway (I,II,V,VII,X) defect incr PT |
| What does PTT test? | tests all factors except VII, XIII (intrinsic pathway). defect = incr PTT |
| What are the PT and PTT in hemophilia A and B? | normal Pt, incr PTT |
| What cuaes an incr PTT in hemophilia A? | defiecincy of factor VIII |
| What causes an incr PTT in hemophilia B? | deficiency of facotr IX |
| What are some clinical signs of hemophilia A or B? | macrohemmorage into joints: hemarthroses, easy bruising |
| What are the PT and PTT seen in vitamin K deficiency? | Pt and PTT are both prolonged |
| What factors and proteins are affected in a vitamin K deficiency? | decr synthesis of factors II, VII, IX, X, protein C and S |
| What lab is increased in defects of platelt plug formation? | bleeding time (BT) |
| What are some clinical signs of platelets disorders? | mucous membrane bleeding, epistaxis, petichiase, purpura, incr bleeding time |
| What are the PC (platelet count) and BT ( bleeding time) of Bernard-Soulier disease? | decr PC, incr BT |
| What is the problem in Bernard-Soulier disease? | decr Gp1b causes a defect in platelet to collagen adhesion=defect in plug formation |
| What is the Pc and Bt in Glanzmann's thrombasthenia? | no change in PC, incr BT |
| What is the problem in Glanzmann's thrombasthenia? | decr GpIIb/IIIa decr platelet to platelet adhesion |
| what would a blood smear in Glanzmann's thrombasthenia show? | no platelet clumping |
| What is the PC and BT in idiopathic thrombocytopenic purpura? | decr PC, incr BT |
| What is the problem in thromboctopenic purpura? | decr platelet survival due to presence of anti-GpIIb/IIIa Ab= peripheral platelet destruction |
| What would be a key lab finding in idiopathic thrombocytopenic purpura? | incr megakaryocytes |
| What is the PC and BT in thrombotic thrombocytopenic purpura? | decr PC, incr BT |
| What is the deficiency in thrombotic thrombocytopenic purpura? | decr platelet survival due to deficiency of ADAMTS 13 (vWF mettalloprotease)---> degradation of vWF multimers |
| What is the pathogenesis of thrombotic thrombocytopenic purpura? | incr in large vWF multimers increase platelet aggregation and causes thrombosis |
| What labs are seen in thrombotic thrombocytopenic purpura? | schistocytes, incr LDH |
| What sx are seen in thrombotic thrombocytopenic purpura? | pentad: neurologic, renal sx, fever, thrombocytopenia, microangiopathic hemolytic anemia |
| What are the PC, BT, PT, and PTT in von Willebrand's disease? | no PC change, incr BT, no PT change, none or incr PTT |
| What is the mechanism of von Willebrand's disease? | decr vWF = normal or incr PTT depends on severity. VWF carries/protects factor VII. decr vWF causes = decr in platelet to collagen adhesion |
| What is the severity and inheritance of von Willebrand's disease? | autoD. mild but most common inherited bleeding disorder |
| What is the Tx for von Willebrand's disease? | DDAVP (desmopressin), releases vWF stored in the endothelium |
| What are the PC, BT, PT, and PTT in DIC? | decr PC, incr BT, PT, PTT |
| What happens in DIC? | widespread activation of clotting factors leads to deficiency of clotting factors causing a bleeding state |
| What is mnemonic for the causes of DIC? | STOP Making New Thrombi: sepsis (g-), trauma, obstetric complications, acute Pancreatitis, malignancy, nephrotic syndrome, Transfusion |
| What labs are seen in DIC? | schistocytes, incr fibrin split products (d-dimers), decr fibrinogen, decr factors V and VIII |
| What is the problem and what is seen in Factor V Leiden? | production of a mutant factor V which can't be degraded by protein C. most common cause of inherited hypercoagulability |
| What is the problem and what is seen in prothrombin gene mutation? | mutation in 3' UTr associated with venous clots |
| What is the problem and what is seen in antithrombin defieciency? What can be given to help? | deficiency of antiuthrombin, reduced incr in PTT if heparin given |
| What is the problem and what is seen in protein S or C deficiency? What medicine causes major problems? | decr abitlity to inactivate V and VIII. incr risk of thrombotic skin necrosis with hemorrhage following warfarin administration |
| what is the dosage effect and use of packed RBCs? | effect: incr Hb and O2 carrying capacity use: acute blood loss, severe anemia |
| what is the dosage effect and use of platelets? | effect: incr PC, usally 6 or more per. use: stop bleedeing in thrombocytopenia, qulitative platelet defects |
| what is the dosage effect and use of freash frozen plasma? | incr coagulation factor levels by 20%. use: DIC cirrosis, warfarin over anticoagulation |
| what is the dosage effect and use of cryoprecipatate? | dosage: contains fibrinogen, factor VIII, factor XIII use: tx factor deficiencies invovling fibrinogen, factor VIII |
| What are some of the major risks of blood transfusions? | infection transmission, transfusion reaction, iron overload, hypocalcemia (citrate can chelate), hyperkalemia |
| What is a leukemia? Where does it arise and where are its tumor cells found? | lymphoid neoplasm with widespread involvement of bonemarrow. tumor cells in peripheral blood |
| What is an lymphoma? | discrete tumor masses arising from lymph nodes, |
| What is a leukemoid reaction? | incr WBC count with left shift (80% bands) and incr leukocyte phosphatase usually due to an infection |
| What disease are Reed-Sternberg cells found? | Hodgkin's lymphoma |
| Which lymphoma is associated with HIV or immunosurpression? | NHL |
| What is the localization and spread of HL? | localized in a single group of nodes, contiguous spread with stage being best predictor of prognosis |
| What is the localization and spread of NHL? | multiple, peripheral nodes with common extranodal invovlement. non contiguous spread |
| What are constitutional B signs and which are they seen in NHL or HL? | low grade fever, night sweats, weight loss. Seen in HL and NHL except thos of lymphoblastic T cell origin |
| Which has mediastinal lymphadenopathy HL or NHL? | HL |
| What causes HL, what is the epidemiology? | 50% associated with EBV. bimodal distrobution in the young and old. more common in men except nodular sclerosing type |
| What is the prognosis in HL? | good in high lymphocytes and decr RS |
| When is the peak incidence of NHL? | 20-40 years of age for certain subtypes |
| What is a Reed-Sternberg cell? What are some biomarkers? | tumor cell of HL. binucleate "owl's eyes". Cd30 and Cd15 + b/c they are B cell origin. neccessary but not sufficient HL dx |
| What is the RS, lymphocytes, and prognosis of nodular sclerosing HL? | a little RS, many lymphocytes, excellent prognosis |
| what cells are seen in nodular sclerosing HL, who gets it? | see lacunar cells, collagen banding. women=men, primarily young adults |
| What Rs lymphocytes and what is the prognosis in mixed cellularity HL? | very many RS, very many lymphocytes, intermediate prognosis |
| What RS, lymphocytes, and what is the prognosis in lymphocyte predominant HL? | a few RS, many lymphocytes, excellent prognosis |
| Who gets lymphocyte predominant HL? | <35 y/o males |
| What RS, lymphocytes, and what is the prognosis in lymphocyte depleted HL? | RS will be high compared to lymphocytes = poor prognosis |
| Who gets lymphocyte depleted HL? | older males with disseminated disease |
| What are 4 B cell NHLs? | Burkitt's, diffuse large B cell lymphoma, mantle cell lymphoma, follicular lymphoma |
| Who gets Burkitt's lymphoma? | adolescents and YA |
| What is the genetic component of Burkitt's lymphoma? | t(8;14), c-myc gene moves next to heavy chain Ig gene (14) |
| What is seen on biopsy in Burkitt's lymphoma? | "starry sky" appearance of sheets of lymphocytes with interspearsed macrophages |
| What virus is Burkitt's lymphoma assoicatied with? | EV |
| What is a common sign of endemic Burkitt's lymphoma? | jaw lesion in Africa, also pelvis or abdomen in the sporadic form |
| Who gets diffuse large cell B cell lymphoma? | usually older adults but 205 children |
| What is the most common adult NHL and what is its origin? | diffuse large B cell lymphoma but can be of T cell origin |
| Who gets mantle cell lymphoma? | older males |
| What is the genetic component of mantle cell lymphoma? | t(11;14) deactivation of cyclin D tregulatory gene |
| What is the prognosis and a useful biomarker in mantle cell lymphoma? | poor prognosis, these are CD5+ |
| Who gets follicular lymphoma? | adults |
| What is the genetic component of follicular lymphoma? | t(14;18) bcl-2 gene expression (inhibits apoptosis) |
| What are 2 types of mature T cell NHLs? | Adult T cell lymphoma, mycosis fungoides/Sezary syndrome |
| What causes adult T cell lymphoma? | HTLV-1 |
| How do people with adult T cell lymphoma present and where is it most common in the world? | adults present with cutanous lesion esp in Japan, west Africa, Caribbean |
| Who gets mycosis fungoides/Sezary syndrome? | adults |
| What is the presentation of mycosis fungoides/Sezary syndrome and what biomarker? | cutaneuous patches/nodules, CD4+, indolent course |
| What does a multiple myeloma cell look like? | monoclonal plasma cell with a fried egg appearane that produces large anounts of IgG and IgA |
| What is the most common primary bone tumor in elderly? | multiple myeloma |
| What is the mnemonic for the presentation of mutliple myeloma? | CRAB: hyperCalcemia, renal insufficiency, anemioa, Bonelytic lesion/back pain |
| What are 6 major assoications of multiple myeloma? | 1. incr susceptibility to infection 2. primary amyloidosis 3. punched out lytic bone lesion on xray 4. M spike protein on electrophoresis 5. Ig light chains in urine (Bence Jones protein) 6. Rouleaux formation (RBCs like poker chips in blood smear) |
| What allows you to distinguish b/w multiple myeloma and Waldenstrom's macroglobinemia? | MM has an M spike. in addtion, Waldenstroms wont have lutic bone lesion |
| What is MGUS? | monoclonal plasma cell expansion without the Sx of multiple myeloma |
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