Step 3- Hematology

Frage	Antworten
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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	Erstellt von Jaimie Shah vor etwa 11 Jahre