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Step 1 11.28.12

Hematology VIII

QuestionAnswer
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
Created by: tjs2123