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
Q

tx of cold agglutinins

A

will not respond to steroids!

*Rituximab + keep warm +/- cyclophosphamide

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26
Q

G6PD

inheritance? sx? smear?

A

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

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27
Q

Pyruvate Kinase Deficiency

A

looks just like G6PD but seems to be UNPROVOKED

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28
Q

Hereditary spherocytosis

sx?

A

recurrent episdoes of hemolysis, spherocytes, splenomegaly, bilirubin gallstones, ELEVATED MCHC(each cell has more hg than it is suppose to have)

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29
Q

Hereditary spherocytosis

dx?

A
  1. eosin-5-maleimide

2. osmotic fragility test

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30
Q

Hereditary spherocytosis

tx?

A

splenectomy + folic acid + vaccines

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31
Q

HUS triad?

A

ART!

AI hemolysis, Renal Failure(elevated BUN & Cr), Thrombocytopenia

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32
Q

Coombs and PT/PTT with HUS and TTP

A

normal PT/PTT & negative coombs

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33
Q

TTP pentad

A

FAT RN:

Fever, AI hemolysis, Thrombocytopenia, Renal Failure, Neurological abnormalites

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34
Q

What test can you do to dx TTP?

A

ADAMTS-13 level is decreased

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35
Q

Paroxysmal Nocturnal Hemoglobinuria(PNH)

sx?

A

pancytopenia, recurrent episodes of dark urine, large vessel venouse thrombosis(MC is portal vein thrombosis)

36
Q

Paroxysmal Nocturnal Hemoglobinuria(PNH)

increased risk of what?

A

aplastic anemia & AML

37
Q

Paroxysmal Nocturnal Hemoglobinuria(PNH)

dx?

A

CD55 & CD59 antibodies aka decay accelerating factor antibodies

38
Q

Paroxysmal Nocturnal Hemoglobinuria(PNH)

tx?

A

steroidis or Eculizumab

39
Q

Methemoglobinemia

sx?

A

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
Q

methemoglobinemia

tx?

A

methylene blue

41
Q

Type of transfusion reaction?

20 min after pt recieves a blood transfusion they bc SOB, transient infiltrates on teh CXR, all sx respond spontaneously

A

transfusion-related acute lung injury(TRALI) or leukoagglutination reaction

42
Q

Type of transfusion reaction?

as soon as a pt receives transfusion they bc hypotensive, SOB and tachycardic. LDH & bilirubin are normal

A

IgA deficiency - anaphylaxis!

43
Q

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.

A

ABO incompatability - acute sx of hemolysis during transfusion

44
Q

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.

A

Minor blood group incompatibility - incompatility to kell, duffy, lewis or kidd antigens or Rh incompatibility presentingwith delayed jaundice

45
Q

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

A

Febrile nonhemolytic reaction - small rise in temperature. tx are against donor WBC antigens. prevented by using FILTERED BLOOD TRANSFUSIONS

46
Q

Sx of acute leukemia

A

fatigue, bleeding, infections

47
Q

which leukemia has auer rods

A

AML, APL

48
Q

which leukemia is associated with DIC

A

M3 APL

49
Q

Tx of ALL

A

intrathecal methotrexate

50
Q

tx of APL

A

ATRA & arsenic trioxide

51
Q

tx of AML

A

-rubicin & cytosine, arabinoside

52
Q

Myelodysplasia

sx? mutation?

A

“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
Q

tx of myelodysplasia

A

lenalidomide, azacitidine(decrease the need for transfusions)

54
Q

CML

sx?

A

elevated WBC that is mostly neutrohpils, splenomegaly, puritis, LUQ pain(enlarged spleen), abdominal fullness and early saity

55
Q

CML mutation

A

BCR-ABL 9:22t

56
Q

tx of CML

A

imatinib

57
Q

CLL sx?

A

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
Q

mechanism of hemolysis with CLL?

A

lymphocytes produce abnormal of insufficieny Ig = IgG made is directed against RBCs = hemolysis & thrombocytopenia

59
Q

Hairy Cell Leukemia

sx?

A

pancytopenia, MASSIVE SPLENOMEGALY, M > F, middle aged, HAIRY CELLS ON SMEAR +/- myelofibrosis

60
Q

tx of Hairy cell leukemia

A

cladribine(2CDA)

61
Q

mutation associated with myelofibrosis?

A

JAK2

62
Q

Polycythemia Vera

sx? labs?

A

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
Q

mutation associated with Polycythemia Vera?

A

JAK 2

64
Q

tx of polycythemia vera?

A

phlebotomy > hydroxyurea

*daily aspirin

65
Q
Essential Thrombocytopenia(ET)
sx? tx?
A

> 1 million platelets with no other shit.

sx: HA, visual distrubances, pain in hairs
tx: hydroxyurea, daily ASA

66
Q

Multiple Myeloma

sx?

A
  • 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
Q

MCC of death in MM?

A

infection & renal failure(hyperCa, uricemia, amyloid + bence Jones proteins)

68
Q

what are bence jones proteins?

A

light chains = deposit in kidneys causing dmg = seen in MM

69
Q

tx of MM

A

thalidomide

70
Q

Waldenstrom’s Macroglobulinemia

sx?

A

hyperviscosity from IgM overproductions = blurry vision, confusion, HA, enlarged nodes and spleen

tx: plasmapheresis

71
Q

Chronic infection with —-can cause aplastic anemia

A

hep B/C

72
Q

Hodgkin lymphoma vs non-hodgekin lymphoma

which is widespread?

A
hodgkin = starts in neck then spread centrifugally
non-hodgkin = usually presents as widespread(& in late stages)
73
Q

dx of lymphoma

A

excisional lymph node bx

74
Q

tx of hodgekin lymphoma?

A

ABVD: adriamycin/doxorubicin, bleomycin, vinblastine, dacarbazine

75
Q

tx of nonhodgekin lymphoma?

A

CHOP: Cyclophosphamide, hydroxyadriamycin, oncovin/vincristine, prednisone

76
Q

Platelet bleeding vs factor bleeding

A

platelet: superficial/petechiae, nose, mucosal, gums
factor: deep/joints & muscles

77
Q

Von Willebrand’s Disease(VWD)

A
platelet dysfunction = superficial bleedings
\+/- elevated PTT due to VWF interfearing with F8
78
Q

Von Willebrand’s Disease(VWD)

dx?

A

Ristocetin cofactor assay

79
Q

Von Willebrand’s Disease(VWD)

tx?

A

desmopressin(DDAVP) & F8 replacement

80
Q

ITP

sx? dx? tx?

A

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
Q

tx of ITP

A

> 50k = no tx; <50k w/minor bleeding = prednisone; <10-20k = IVID or rhogam

82
Q

Heparin-Induced Thrombocytopenia(HIT)

tx? sx?

A

drop in platlets a few days after starting heparin

tx: stop heparin & start fondaparinux

83
Q

Antiphospholipid syndrome

sx? dx? tx?

A

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
Q

Protein C deficiency

sx?tx?

A

skin necrosis with the use of warfarin

tx: heparin then warfarin

85
Q

Factor V Leiden Mutation

sx? tx?

A

MCC of thrombophilia; venous thrombosis

tx: heparin then warfarin