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Criado por Jaimie Shah
quase 11 anos atrás
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Questão | Responda |
possible PE findings in different causes of anemia | pallor, flow murmur, pale conjunctiva, jaundice, scleral icterus |
anemia dx tests | CBC with perpheral smear, retic count, haptoglobin, LDH, total and direct bili, TSH with T4, B12/folate, iron studies; also order UA and u.micro |
patient has blood loss and elevated plt count what type of anemia does he have | Iron def anemia (Microcytic) |
Patient has hx of RA, ESRD and other chronic infection what type of anemia does he have | ACD (microcytic) |
patient has very small MCV with few or no symps and target cells on smear, what type of anemia does he have | thalasemia |
patient has hx of ETOH, INH or lead exposure what type of anemia does he have | Sideroblastic anemia |
labs for Fe def aneima | low ferritin, high TIBC, low Fe, Low Fe Sat, elevated RDW |
iron studies in ACD | high/nl ferritin, low TIBC, Low Fe, normal to low Fe sat, RDW nl |
thalassemia iron studies | normal |
sideroblastic aneima iron studies | high Fe |
most accurate test for thalassemia | Hg electrophoresis and if beta then elevated HgA2 and HgF; if alpha then normal |
most accurate test for sideroblastic aneima | prussian blue stain |
tx Fe def anemia | ferrous sulfate |
tx ACD anemia | tx underlying disease |
Tx of thalassemia | none |
tx of sideroblastic anemia | give pyridoxine and remove toxin |
Most accurate dx test of alpha thal is? | DNA sequencing |
will ferrous supplement give you a false positive FOBT? | No it will make the stools black though |
what type of anemia has an elevated RDW? | Iron def anemia (prog newer cells produced are smaller) |
what does the prussian blue stain show us? | in sideroblastic anemia it shows the build of Fe in the mitochondria of the RBCs |
what is HgH? | it is alpha thal when there is four beta units together due to a 3 gene deletion of the alpha units |
when is colonoscopy indicated with anemia? | any patient over 50, regardless of the FOBT shows you would go straight to this test if you see a microcytic anemia present on labs |
microcytic anemia with high retic count? | Hgh (alpha thal) |
Can you see extravascular hemolysis on the perph smear? | No it happens in the spleen and Liver |
what medication blocks B12 abs? | metformin |
Dx test for macrocytic aneima? | initially check CBC and smear--macrocytosis and hypersegmented neutrophils (more than 5% with more than 4 lobes); bili and LDH may be elevated and you may see oval cells on smear, and retic count is decreased |
most accurate test for macrocytic aneima | B12 and folate levels; also check a MMA and Homocystine level if these are normal (both elevated in only B12 def) |
what do you order to confirm etiology of B12 def | antiparietal cell abs and anti IF antibodies; if you miss B12 and only correct Folate you get improvement till you see the neuro symps present that are never seen in folate def |
what electrolyte do you need to replace with B12 def tx | low potassium |
Dx test findings just for hemolysis in general | inc indirect bili, inc retic count, inc LDH(IV hemolysis, nl to inc in EV), low Haptoglobin (normal to low in EV hemolysis) (also always check a periph smear--def abn cells in IV but if really bad can have abn cells in EV) |
what is in intravascular hemolysis spp? | abn periph smear (schistocytes, helmet cells, fragment cells), hemoglobinuria, Hemosiderinuria |
for SCC anemia do complete PE and if after tx still has fever repeat PE---findings on exam include? | retinal infarct, flow murmur, splenomegaly in kids, rales in lungs from infection or infarction, Skin ulcers and aseptic necrosis of hip, stroke hx and deficits |
tx of sickle cell crisis | O2, IVF, pain meds, if fever--ceftiraxone/levo/moxi (most key step with fever in this patient and is next step because they have no spleen)--on CCS order BCx, UA, UCx, retic, CBC and CXR but dont wait on results to give Abx. |
when do you do exchange transfusion in sickle cell crisis | retinal infarct, pulm infarct, Priapisim, stroke |
sudden drop in Hct and retic count in sickle cell patient or one with hemoglobinopathy? | check for parvo B19 and folate def especially |
what does parvo do to effect RBC | it invades BM and halts production of RBCs and you can check for it with PCR for DNA of parvo, more accuate than IGM and IGG for parvo (tx with transfusion and IGs) |
what do you send a sickle cell patient home on | folate, Pneumovax, Hydroxyurea if more than 4 crises a year |
Hemoglobin SC disease | a milder form of Sickle cell (two abnormal genes one from each parent, dont inherit Hgb A-normal), fewer crises, visual disturbances common, nonpainful crises, renal problems happen (hematuria, unable to concentrate urine, UTIs |
Sickle cell trait (AS/AC) | renal manifestations only findings (hematuria and cant concentrate urine) |
clues patient has autoimmune Hemolysis | SLE, RA, CLL, pencillin use, alpha-methyldopa, quinine or sulfa |
Dx tests for autoimmune hemolysis | indirect bili and retic count elevated, Haptoglobin and LDH vary if IV-complement med or EV hemolysis- IG mediated |
warm antibodies causes hemolysis what is tx | so postive coombs finds IGG to RBC so this will respond to splenectomy and steroids (only if IGG) |
Cold agglutinins | (complement med) so negative coombs and complement test positive, will not respond to other tx, you need RITUXIMAB (look for EBV and mycoplasma in history) |
Agents that can cause G6PD exacerbation | sulfa meds, primaquine, dapsone, fava beans |
dx test for G6PD def | blood smear with Heinze bodies and bite cells; most accurate test is level of G6PD after 2 mos have passed |
tx G6PD def | avoid oxidant agents |
Pyruvate Kinase def note | presents as hemolysis like in G6PD but no ppt agents are known |
Presentation of Hereditary Spherocytosis | recurrent episodes of hemolysis, splenomegaly, bili gallstones, elevated MCHC |
Dx and tx of hereditary spherocytosis | most accurate test is osmotic fragility test, and tx is splenectomy since this is EV hemolysis |
HUS triad | IV hemolysis with abnormal smear (autoimmune hemolysis), elevated BUN and Cre (renal failure), thrombocytopenia |
TTP pentad | HUS + Fever and Neuro abn |
tx of TTP and HUS | most resolve on own but if not than need plasmapheresis, steroids wont help...ABX and Platelets transfusion will make it WORSE! |
Presentation with PNH | pancytopenia and recurrent episodes of dark urine esp in morning; common cause of death is large vessel thrombosis (Portal vein); can turn into aplastic anemia and AML |
Dx testing for PNH | CD 55 and CD 59 antibodies (low levels of these proteins so they cannot prevent complement activation aganist the RBC membrane) |
tx of PNH | prednisone and transfusion but if severe eculizumab (inhibits C5 and so complement doesnt get activated and no hemolysis) |
difference between DIC and HELLP syndrome | DIC has abnormal coagulation studies |
Methemoglobinemia presentation | SOB for no clear reason, clear lungs on exam, normal CXR, Here Hgb locked so cant pick up O2 or drop it off, look for drug exposure (nitro, nitrates nitropursside, dapsone, anesthetics end in -caine), look for brown blood in case and tx with methylene blue |
TRALI presentation | acute SOB from antibodies in donor blood aganist recipient WBC. No tx and resolves on own |
IGA def and transfusions | presents with anaphylaxis use washed RBC |
ABO incompatibility | presents with acute hemolysis with transfusion |
Minor blood group incompatibility | presents with delayed jaundice (no tx) |
Febrile nonhemolytic reaction to transfusion | small rise in temp and no tx, rxn aganist donor WBC antigens. Prevented by using filtered blood |
Leukemia presentation | pancytopenia, fatigue, bleeding, infection, functionally immunodef |
Dx testing for leukemia | best initial is perp smear showing blasts, cytogenics tell you will who will relapse and pronosis if at high risk then need BM transplant ASAP in remission after chemo |
What disease do you see Auer rods? | AML |
tx of leukemia | chemo idarubicin/donarubicin, cytosine arabinoside, add ATRA if APML and add intrathecal MTX for ALL |
What disease is APML associated with? | DIC |
what do you do if there is acute leukocytosis in leukemia that block BV in brain to cause neuro symps? | leukapheresis and Hydroxyurea (lowers WBC) |
Myelodysplasia basics | see elderly, pancytopenia, elevated MCV, low retic count, macroovalocytes, bilobe PMN (pelger Huet cells), normal B12, blast but not enough for leukemia so preleukemic--can progress. most die of bleeding or infection |
tx myelodysplasia | support with transfusions as needed and Azacitadine or lenalidomide |
CML presentations | elevated WBC with most neutrophils and splenomegaly frequently; highest risk to transform to AML |
Dx of CML | LAP score (low) and inc neutrophils, in normal ppl with infection LAP is elevated with increased WBCs; most accurate test is philadelphia chromosome |
tx of CML | imatinib (best initial with 90%remission), BM transplant (cure) |
Busulfan SE | pulm fibrosis |
CLL presentation | patient over 50, elevated WBC normal appear lymphs, Asx and found on routine testing. |
CLL dx | initially get perp smear and see smudge cells; stage 0 elevated WBC alone, stage 1 enlarged LN, stage 2 spleen enlarged, stage 3 anemia, stage 4 low plts |
CLL tx | stg0-1 no tx, later stages fludarabine (extendes survival) and rituximab |
Hairy cell leukemia basics | pancytopenia, massive splenomegaly, mid 50s pt; most accurate test is TRAP and smear of hairy cells; best initial test cladribine |
Myelofibrosis | present same as HCL with normal TRAP, tear drop cells on smear; tx lenalidomide or thalidomide and cure can be BM transplant |
Polycythemia vera presentation | HA, blurry vision, dizziness, fatigue, puritis after hot shower, splenomegaly, high Hct and no hypoxia and low MCV, low EPO, WBC and plt may be elevated, high HCt can cause thrombosis |
Dx polycythemia vera | CBC, ABG, EPO level, JAK 2 mutation, B12 and LAP elevated here |
tx of P vera | phlebotomy, hydroxyurea, ASA, Anagrelide if there is low plts |
essential thrombocytopenia basics | inc platlets, HA, visual distub, pain in hands; common cause of death bleeding and thrombosis; tx is hydroxyurea, ASA, anagrelide |
what cells do you get from the myeloid lineage hence the myeloproliferative d/o | RBC, Platlets, mast cell, basophil, eosinophil, neutrophil, marcophage |
cells that come from lypmphoid precurrsors | plasma cells and NK cells |
common causes of death in MM | infection and RF |
Dx test of MM | skeletal survery, SPEP, UPEP, Perp smear, elevated Ca, Beta 2 microglobulin level (prognostic factor), inc BUN/Cre; Most accurate test is Bone Marrow Bx (more than 10% plasma cells) |
Tx of MM | Melphalan and steroids, can add thalidomine/lenalidomide/bortezomib; most eff tx is autologous BM transplant; support: hydration and diuresis, bisphosphonates, EPO, vaccinations |
MGUS | incidental increase in total serum protein so you see increased IGG on SPEP, no tx |
Waldenstrom's presentation | hyperviscosity from IGM overproduction, you see blurry vision, confusion, HA, and enlarged LN and spleen |
Waldenstrom's dx and tx | serum viscosity level, SPEP; plasmaphersis, fludarabine/chlorambucil |
aplastic anemia basics | pancytopenia w/o etiology, tx BMT or antithymocyte globulin and cyclosporine |
b symptoms of lymphoma | fever, weight loss, night sweat, indicate more wide spread disease |
lymphoma presentation | enlarged LN (HD presents from center Neck LNs and out; non HD is wide spread LAD) |
Dx testing of Lymphoma | Best initial test for HD and non HD is excisional LN bx (HD-reed sternberg cells); then staging--stage 1 is one LN group, stage 2 is two LN groups on same side of diaphragm, stage 3 LN groups on both sides of diaphragm, stage 4 wide spread diseaes |
lymphoma presentation stage | most HD present stage 1/2, most non HD present stage 3/4 |
staging test in lymphoma | CXR, CT with contrast, BM bx |
tx of lymphoma | stage 1-2 w/o b syms get radiation; stage 3-4 treated with chemo (HD-ABVD, NHL- CHOP + anti CD 20 antigen + rituximab) |
VWD presentation | bleed from plt dys from skin and mucosal surface, nose bleeds, and plt count normal +/- petechia |
labs of VWD | most accurate is ristocetin cofactor assay and VWD level and if later normal the cofactor will tell you if if works appropirately |
tx of VWD | DDAVP if doesnt work use factor 8 replacement (has both VWF and factor 8 in it) |
S/S of a platelet d/o | Petechiae, Epistaxis, Purpura, Gingival bleed, vaginal bleed increase |
s/s of factor def bleed | Hemarthrosis and hematoma |
ITP presentation and dx | platelet type bleed if plt<50,000; check antiplt abs, US shows normal spleen size, BM increased megakaryocytes, abs to GLP 2b/3a receptors |
what is the most important thing to do when you think you have ITP on hx? | dont delay tx for further testing |
tx of ITP | plt count>50000 no tx; count<50000 with minor bleed tx with prednisone; count<20000 with serious bleed give IVIG (not platelets); recurrent episodes tx with splenectomy, in no response add Romiplostim/eltrombopag (thrombopoetin analogs) |
tx of uremia induced plt dys | DDAVP |
Mixing study | used to differentiate clot factor def and factor inhibitor antibodies, the former will correct with mixing with normal plasma |
factor 8 def | joint bleeds and hematoma in male kids (x linked def); mix test and get the level; <1% level needs replacement and minor def use DDAVP |
factor 9 def | x linked and less common, same dx tests, and need replacement of factors |
factor 11 def | rare bleeding with trauma or surgery, same dx, and give FFP with bleeds |
factor 12 def | no bleeding, same dx, and no tx |
HIT presentation | drop in plts by 50% after a few days started on any amount heparin, Thrombosis is common presentation (venous 3 x more than arterial), LMWH less likely to cause this |
HIT dx and tx | platelet factor 4 antiboides or HIT platlets abs; stop heparin (all forms) and add lepirudin or argatroban |
Lupus anticoagulant/anticardiolipin abs basics | Venous thrombus, elevated aPTT with normal PT, spontaneous abortions, false pos VDRL; Dx with mixing study, russel viper venom test; tx heparin followed by warfarin |
Protein C def basics | skin necrosis with use of warfarin, check protein C level, tx is heparin followed by warfarin |
factor 5 leiden mutation basics | most common cause of thrombophilia; test for the mutation; tx is heparin followed by warfarin |
Antithrombin def basics | no change in the aPTT with a bolus of IV heparin; check AT3 level; large amounts of heparin or direct thrombin inhibitors followed by warfarin |
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