Heme Flashcards
What is Packed RBC’s? how much will they change Hct?
RBC’s - plasma.
PRBC will raise Hct by 3pts/unit
Microcytic anemia’s? what does the reticulocyte count look like?
Thalassemia, Anemia of Chronic Dz, Iron Def, Lead Poisoning, Sideroblastic
*all but A-thal have low reticulcyte count
Ferritin, TIBC, Fe, RDW in iron def anemia?
low: ferritin, fe
High: RDW & TIBC
Ferritin, TIBC, Fe, RDW in anemia of chronic dz
HIGH: ferritin
Ferritin, TIBC, Fe, RDW in thalaseemia
all normal
Ferritin, TIBC, Fe, RDW in sideroblastic anemia?
All normal but high iron
what is sideroblastic anemai?
RBCs are unable to properly utitilize Fe due to toxins
causes of siderblsatic anemia?
alcohol, isoniazid, lead exposure
B12 vs folate deficiency symptoms
b12: peripheral neuropahty, glossitis, diarrhea, NEUROLOGICAL FINDINGS + hypersegmented neutrophils
Folate: ^ but NO neuro shit
whats differnent with B12 def due to alcohol vs other causes of B12 def.
B12 def due to alcohol with no have hypersegmented neutrophils
tx of B12/folate def anemia? what must u look out for? y?
replace b12/folate! watch out for hypokalemia = vitamins given = new cells made = K is taked up into new cells dropping serum K.
haptoglobin, LDH, reticulocyte, bili, smear of hemolytic anemia
decreased: hyptoglobin(recycles Fe, gets used up with hemolysis)
elevated: LDH, Reticulocyte, bili
*smear shows spherocytes, schistocytes
SSD mutation
glut –> val
sx of acute SSD crisis? what must you always be on the look out for?
pain in chest, back, thighs that is very very severe. WATCH OUT FOR FEVER! if there is a fever give abx now!
others: OM, autosplectomy, bilirubin gallstones, retinopathy, stroke, enlarged heart, skin ulcers, avascular necrosis, CHILDREN WITH DACTYLITIS
Reticulocyte count in pt wiht SSD
very high all the time. if you see it low = aplastic crisis
can you tell SSD from SST via smear?
yes! only SSD with have SS, SST wont have SS.
pt in ss crisis with a reticulocyte count of 34 3 days ago that is now 2. what happpened?
prob got parvo infection. parvo freezes the bone marrow and can cause aplastic crisis
when do you exchange for ssd?
visual distrubances, pulmonary infarction, priapism, stroke
tx of parvo with ssd?
transfusions and IVIG
what meds do you give for SSD with fever?
Ceftriaxone, levofloxacin or moxifloxacin
sx of SST?
inabiltiy to concentrate or dilute the urine, hematuria, UTI
when will you get spherocytes?
anythign that attacks the RBC membrane will give you spherocytes!
- AI hemolytic anemia, cold agglutins, hereditary spherocytosis
warm vs cold agglut which will respond to steroids?
warm! = IgG!
cold = IgM & will not respond to steroids
Cold Agglutinins
cause? coombs? complement tests? sx?
IgM fixing compliment and fucking up RBC
*assoc with mycoplasma or EBV
SX: compliment +, Coombs -, bilateral infiltrate on CXR+dry cough(EBV or mycopla infections), numbness or MOTTLING of the ears, nose, toes and fingers
tx of cold agglutinins
will not respond to steroids!
*Rituximab + keep warm +/- cyclophosphamide
G6PD
inheritance? sx? smear?
XL deficiency of G6PD, smear: heinz bodies & bite cells
sx: AA with sudden onset of anemia, normal sized spleen w/recurrent infection or drug use, fava bean use
Pyruvate Kinase Deficiency
looks just like G6PD but seems to be UNPROVOKED
Hereditary spherocytosis
sx?
recurrent episdoes of hemolysis, spherocytes, splenomegaly, bilirubin gallstones, ELEVATED MCHC(each cell has more hg than it is suppose to have)
Hereditary spherocytosis
dx?
- eosin-5-maleimide
2. osmotic fragility test
Hereditary spherocytosis
tx?
splenectomy + folic acid + vaccines
HUS triad?
ART!
AI hemolysis, Renal Failure(elevated BUN & Cr), Thrombocytopenia
Coombs and PT/PTT with HUS and TTP
normal PT/PTT & negative coombs
TTP pentad
FAT RN:
Fever, AI hemolysis, Thrombocytopenia, Renal Failure, Neurological abnormalites
What test can you do to dx TTP?
ADAMTS-13 level is decreased
Paroxysmal Nocturnal Hemoglobinuria(PNH)
sx?
pancytopenia, recurrent episodes of dark urine, large vessel venouse thrombosis(MC is portal vein thrombosis)
Paroxysmal Nocturnal Hemoglobinuria(PNH)
increased risk of what?
aplastic anemia & AML
Paroxysmal Nocturnal Hemoglobinuria(PNH)
dx?
CD55 & CD59 antibodies aka decay accelerating factor antibodies
Paroxysmal Nocturnal Hemoglobinuria(PNH)
tx?
steroidis or Eculizumab
Methemoglobinemia
sx?
SOB for no clear reason + clear lungs on exam/CXR, BROWN BLOOD, look for exposure to: NO, amyl nitrate, Nitroprusside, dapson, -caine
*blood is locked in oxidized state = cannto pick up O2
methemoglobinemia
tx?
methylene blue
Type of transfusion reaction?
20 min after pt recieves a blood transfusion they bc SOB, transient infiltrates on teh CXR, all sx respond spontaneously
transfusion-related acute lung injury(TRALI) or leukoagglutination reaction
Type of transfusion reaction?
as soon as a pt receives transfusion they bc hypotensive, SOB and tachycardic. LDH & bilirubin are normal
IgA deficiency - anaphylaxis!
Type of transfusion reaction?
during transfusion pt becomes hypotensive, tarchycardic. she has back and chest pain, there is dark urine, LDH & bilirubin are elevated and the haptoglobin is low.
ABO incompatability - acute sx of hemolysis during transfusion
Type of transfusion reaction?
a few days after a transfusion a pt becomes jaundice. Hct does not rise with transfusion, and they are generally w/o symptoms.
Minor blood group incompatibility - incompatility to kell, duffy, lewis or kidd antigens or Rh incompatibility presentingwith delayed jaundice
Type of transfusion reaction?
few hours after transfusion a pt becomes febrile with a rise in temp of about 1 degree. there is no evidence of hemolysis
Febrile nonhemolytic reaction - small rise in temperature. tx are against donor WBC antigens. prevented by using FILTERED BLOOD TRANSFUSIONS
Sx of acute leukemia
fatigue, bleeding, infections
which leukemia has auer rods
AML, APL
which leukemia is associated with DIC
M3 APL
Tx of ALL
intrathecal methotrexate
tx of APL
ATRA & arsenic trioxide
tx of AML
-rubicin & cytosine, arabinoside
Myelodysplasia
sx? mutation?
“5q-“ elderly pt with pancytopenia, elevated MCV(+/- ringed siderblasts), low reticulocyte count, macroovalocytes, “pelger-huet cells”(bilobed neutrophil), normal b12, small number of blasts
tx of myelodysplasia
lenalidomide, azacitidine(decrease the need for transfusions)
CML
sx?
elevated WBC that is mostly neutrohpils, splenomegaly, puritis, LUQ pain(enlarged spleen), abdominal fullness and early saity
CML mutation
BCR-ABL 9:22t
tx of CML
imatinib
CLL sx?
pt>50 yoa with elevated WBC full of “normal appearing lymphocytes”, fatigue, lymphadenopathy, spleen/liver enlargement, increased infections, + SMUDGE CELLS, thrombocytopenia with decreased megakaryocytes
*5% convert to lymphoma
mechanism of hemolysis with CLL?
lymphocytes produce abnormal of insufficieny Ig = IgG made is directed against RBCs = hemolysis & thrombocytopenia
Hairy Cell Leukemia
sx?
pancytopenia, MASSIVE SPLENOMEGALY, M > F, middle aged, HAIRY CELLS ON SMEAR +/- myelofibrosis
tx of Hairy cell leukemia
cladribine(2CDA)
mutation associated with myelofibrosis?
JAK2
Polycythemia Vera
sx? labs?
sx: HA, blurred vision, dizziness, fatigue, splenomegaly
labs: elevated Hct in the absence of hypoxia & low MCV + low erythropoietin + elevated WBC count, LAP(increased bone activity) & B12 elevated
mutation associated with Polycythemia Vera?
JAK 2
tx of polycythemia vera?
phlebotomy > hydroxyurea
*daily aspirin
Essential Thrombocytopenia(ET) sx? tx?
> 1 million platelets with no other shit.
sx: HA, visual distrubances, pain in hairs
tx: hydroxyurea, daily ASA
Multiple Myeloma
sx?
- bone pain caused by fracture occuring under normal use!
sx: punched out osteolytic lesions, elevated SPEP/Mspike(IgG), UPEP + for bence-jones proteins, Rouleaux on peripheral smear, elevated Ca levels, Bun/Cr elevated
MCC of death in MM?
infection & renal failure(hyperCa, uricemia, amyloid + bence Jones proteins)
what are bence jones proteins?
light chains = deposit in kidneys causing dmg = seen in MM
tx of MM
thalidomide
Waldenstrom’s Macroglobulinemia
sx?
hyperviscosity from IgM overproductions = blurry vision, confusion, HA, enlarged nodes and spleen
tx: plasmapheresis
Chronic infection with —-can cause aplastic anemia
hep B/C
Hodgkin lymphoma vs non-hodgekin lymphoma
which is widespread?
hodgkin = starts in neck then spread centrifugally non-hodgkin = usually presents as widespread(& in late stages)
dx of lymphoma
excisional lymph node bx
tx of hodgekin lymphoma?
ABVD: adriamycin/doxorubicin, bleomycin, vinblastine, dacarbazine
tx of nonhodgekin lymphoma?
CHOP: Cyclophosphamide, hydroxyadriamycin, oncovin/vincristine, prednisone
Platelet bleeding vs factor bleeding
platelet: superficial/petechiae, nose, mucosal, gums
factor: deep/joints & muscles
Von Willebrand’s Disease(VWD)
platelet dysfunction = superficial bleedings \+/- elevated PTT due to VWF interfearing with F8
Von Willebrand’s Disease(VWD)
dx?
Ristocetin cofactor assay
Von Willebrand’s Disease(VWD)
tx?
desmopressin(DDAVP) & F8 replacement
ITP
sx? dx? tx?
sx: platelet type bleeding with <10-30k platelets
dx: ab to 2b/3a receptor
tx: >50k = no tx; <50k w/minor bleeding = prednisone; <10-20k = IVID or rhogam
tx of ITP
> 50k = no tx; <50k w/minor bleeding = prednisone; <10-20k = IVID or rhogam
Heparin-Induced Thrombocytopenia(HIT)
tx? sx?
drop in platlets a few days after starting heparin
tx: stop heparin & start fondaparinux
Antiphospholipid syndrome
sx? dx? tx?
sx: thrombosis, eleavted PTT but normal PT, false + VLDL
dx: russel viper venom test is most accurate for lupus anticoagulant
tx: heparin then warfarin
Protein C deficiency
sx?tx?
skin necrosis with the use of warfarin
tx: heparin then warfarin
Factor V Leiden Mutation
sx? tx?
MCC of thrombophilia; venous thrombosis
tx: heparin then warfarin