Heme Flashcards

1
Q

What is Packed RBC’s? how much will they change Hct?

A

RBC’s - plasma.

PRBC will raise Hct by 3pts/unit

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2
Q

Microcytic anemia’s? what does the reticulocyte count look like?

A

Thalassemia, Anemia of Chronic Dz, Iron Def, Lead Poisoning, Sideroblastic

*all but A-thal have low reticulcyte count

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3
Q

Ferritin, TIBC, Fe, RDW in iron def anemia?

A

low: ferritin, fe
High: RDW & TIBC

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4
Q

Ferritin, TIBC, Fe, RDW in anemia of chronic dz

A

HIGH: ferritin

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5
Q

Ferritin, TIBC, Fe, RDW in thalaseemia

A

all normal

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6
Q

Ferritin, TIBC, Fe, RDW in sideroblastic anemia?

A

All normal but high iron

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7
Q

what is sideroblastic anemai?

A

RBCs are unable to properly utitilize Fe due to toxins

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8
Q

causes of siderblsatic anemia?

A

alcohol, isoniazid, lead exposure

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9
Q

B12 vs folate deficiency symptoms

A

b12: peripheral neuropahty, glossitis, diarrhea, NEUROLOGICAL FINDINGS + hypersegmented neutrophils
Folate: ^ but NO neuro shit

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10
Q

whats differnent with B12 def due to alcohol vs other causes of B12 def.

A

B12 def due to alcohol with no have hypersegmented neutrophils

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11
Q

tx of B12/folate def anemia? what must u look out for? y?

A

replace b12/folate! watch out for hypokalemia = vitamins given = new cells made = K is taked up into new cells dropping serum K.

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12
Q

haptoglobin, LDH, reticulocyte, bili, smear of hemolytic anemia

A

decreased: hyptoglobin(recycles Fe, gets used up with hemolysis)
elevated: LDH, Reticulocyte, bili

*smear shows spherocytes, schistocytes

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13
Q

SSD mutation

A

glut –> val

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14
Q

sx of acute SSD crisis? what must you always be on the look out for?

A

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

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15
Q

Reticulocyte count in pt wiht SSD

A

very high all the time. if you see it low = aplastic crisis

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16
Q

can you tell SSD from SST via smear?

A

yes! only SSD with have SS, SST wont have SS.

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17
Q

pt in ss crisis with a reticulocyte count of 34 3 days ago that is now 2. what happpened?

A

prob got parvo infection. parvo freezes the bone marrow and can cause aplastic crisis

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18
Q

when do you exchange for ssd?

A

visual distrubances, pulmonary infarction, priapism, stroke

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19
Q

tx of parvo with ssd?

A

transfusions and IVIG

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20
Q

what meds do you give for SSD with fever?

A

Ceftriaxone, levofloxacin or moxifloxacin

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21
Q

sx of SST?

A

inabiltiy to concentrate or dilute the urine, hematuria, UTI

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22
Q

when will you get spherocytes?

A

anythign that attacks the RBC membrane will give you spherocytes!

  • AI hemolytic anemia, cold agglutins, hereditary spherocytosis
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23
Q

warm vs cold agglut which will respond to steroids?

A

warm! = IgG!

cold = IgM & will not respond to steroids

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24
Q

Cold Agglutinins

cause? coombs? complement tests? sx?

A

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

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25
tx of cold agglutinins
will not respond to steroids! *Rituximab + keep warm +/- cyclophosphamide
26
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
27
Pyruvate Kinase Deficiency
looks just like G6PD but seems to be UNPROVOKED
28
Hereditary spherocytosis | sx?
recurrent episdoes of hemolysis, spherocytes, splenomegaly, bilirubin gallstones, ELEVATED MCHC(each cell has more hg than it is suppose to have)
29
Hereditary spherocytosis | dx?
1. eosin-5-maleimide | 2. osmotic fragility test
30
Hereditary spherocytosis | tx?
splenectomy + folic acid + vaccines
31
HUS triad?
ART! | AI hemolysis, Renal Failure(elevated BUN & Cr), Thrombocytopenia
32
Coombs and PT/PTT with HUS and TTP
normal PT/PTT & negative coombs
33
TTP pentad
FAT RN: | Fever, AI hemolysis, Thrombocytopenia, Renal Failure, Neurological abnormalites
34
What test can you do to dx TTP?
ADAMTS-13 level is decreased
35
Paroxysmal Nocturnal Hemoglobinuria(PNH) | sx?
pancytopenia, recurrent episodes of dark urine, large vessel venouse thrombosis(MC is portal vein thrombosis)
36
Paroxysmal Nocturnal Hemoglobinuria(PNH) | increased risk of what?
aplastic anemia & AML
37
Paroxysmal Nocturnal Hemoglobinuria(PNH) | dx?
CD55 & CD59 antibodies aka decay accelerating factor antibodies
38
Paroxysmal Nocturnal Hemoglobinuria(PNH) | tx?
steroidis or Eculizumab
39
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
40
methemoglobinemia | tx?
methylene blue
41
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
42
Type of transfusion reaction? | as soon as a pt receives transfusion they bc hypotensive, SOB and tachycardic. LDH & bilirubin are normal
IgA deficiency - anaphylaxis!
43
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
44
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
45
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
46
Sx of acute leukemia
fatigue, bleeding, infections
47
which leukemia has auer rods
AML, APL
48
which leukemia is associated with DIC
M3 APL
49
Tx of ALL
intrathecal methotrexate
50
tx of APL
ATRA & arsenic trioxide
51
tx of AML
-rubicin & cytosine, arabinoside
52
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
53
tx of myelodysplasia
lenalidomide, azacitidine(decrease the need for transfusions)
54
CML | sx?
elevated WBC that is mostly neutrohpils, splenomegaly, puritis, LUQ pain(enlarged spleen), abdominal fullness and early saity
55
CML mutation
BCR-ABL 9:22t
56
tx of CML
imatinib
57
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
58
mechanism of hemolysis with CLL?
lymphocytes produce abnormal of insufficieny Ig = IgG made is directed against RBCs = hemolysis & thrombocytopenia
59
Hairy Cell Leukemia | sx?
pancytopenia, MASSIVE SPLENOMEGALY, M > F, middle aged, HAIRY CELLS ON SMEAR +/- myelofibrosis
60
tx of Hairy cell leukemia
cladribine(2CDA)
61
mutation associated with myelofibrosis?
JAK2
62
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
63
mutation associated with Polycythemia Vera?
JAK 2
64
tx of polycythemia vera?
phlebotomy > hydroxyurea | *daily aspirin
65
``` Essential Thrombocytopenia(ET) sx? tx? ```
>1 million platelets with no other shit. sx: HA, visual distrubances, pain in hairs tx: hydroxyurea, daily ASA
66
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
67
MCC of death in MM?
infection & renal failure(hyperCa, uricemia, amyloid + bence Jones proteins)
68
what are bence jones proteins?
light chains = deposit in kidneys causing dmg = seen in MM
69
tx of MM
thalidomide
70
Waldenstrom's Macroglobulinemia | sx?
hyperviscosity from IgM overproductions = blurry vision, confusion, HA, enlarged nodes and spleen tx: plasmapheresis
71
Chronic infection with ----can cause aplastic anemia
hep B/C
72
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) ```
73
dx of lymphoma
excisional lymph node bx
74
tx of hodgekin lymphoma?
ABVD: adriamycin/doxorubicin, bleomycin, vinblastine, dacarbazine
75
tx of nonhodgekin lymphoma?
CHOP: Cyclophosphamide, hydroxyadriamycin, oncovin/vincristine, prednisone
76
Platelet bleeding vs factor bleeding
platelet: superficial/petechiae, nose, mucosal, gums factor: deep/joints & muscles
77
Von Willebrand's Disease(VWD)
``` platelet dysfunction = superficial bleedings +/- elevated PTT due to VWF interfearing with F8 ```
78
Von Willebrand's Disease(VWD) | dx?
Ristocetin cofactor assay
79
Von Willebrand's Disease(VWD) | tx?
desmopressin(DDAVP) & F8 replacement
80
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
81
tx of ITP
>50k = no tx; <50k w/minor bleeding = prednisone; <10-20k = IVID or rhogam
82
Heparin-Induced Thrombocytopenia(HIT) | tx? sx?
drop in platlets a few days after starting heparin tx: stop heparin & start fondaparinux
83
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
84
Protein C deficiency | sx?tx?
skin necrosis with the use of warfarin tx: heparin then warfarin
85
Factor V Leiden Mutation | sx? tx?
MCC of thrombophilia; venous thrombosis | tx: heparin then warfarin