heme Flashcards

1
Q

MCV values

A

normal - 80-100
microcytic <80
macrocytic >100

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2
Q

types of microcytic anemia

A

TICS

thalacemias
iron deficiency
chronic disease
sideroblastic

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3
Q

most common anemia worldwide

A

iron deficiency anemia

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4
Q

next step after dx of iron deficiency anemia

A

investigate cause

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5
Q

most common cause of iron deficiency anemia

A

chronic blood loss

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6
Q

iron deficiency anemia s/s

A

pica
Plummer-vinsonsyndrome (esophageal web)
atrophic glossitis
nail changes
angular stomatitis / cheilosis
blue sclera
RLS
allopecia

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7
Q

iron deficiency anemia labs

A

MCV<80
reticulocyte count low

TIBC high
serum ferritin low!!

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8
Q

iron deficiency anemia treatment

A

treat cause!!

iron supplementation
may take 6-8 weeks to correct anemia

325 mg PO TID - take with vitamin C

not all patients tolerate - N/C/D, metallic taste

can give parenteral if intolerant to AEs

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9
Q

anemia of chronic disease labs

A

MCV low or normal

  • TIBC low/normal***
  • ferritin high/normal***
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10
Q

thalassemia characteristics

A

autosomal recessive (need both parents)**

reduced alpha or beta chains that leads to decreased hgb synthesis, leads to hgb A displacement with abnormal types

Alpha - southeast asia/china
beta - mediterranean

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11
Q

thalassemia manifestations

A
  • stunted growth, boney deformities
  • splenomegaly, jaundice
  • secondary hemachromatosis
  • complications of iron overload

skull xray - crew cut appearance

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12
Q

thalassemia management

A

refer all to hematology and genetic counseling

SCT curative, transfuse as needed

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13
Q

sideroblastic anemia characteristics

A

congenital (X-linked) or acquired (ETOH, lead, B6 deficiency)

build up of iron in RBCs d/t inability to incorporate iron into hgb due to problem with heme synthesis

sx similar to hemochromatosis

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14
Q

sideroblastic anemia peripheral smear

A

pappenheimer bodies
ringed sideroblasts

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15
Q

sideroblastic anemia tx

A

pyroxidine, thiamine, folic acid
therapeutic phlebotomy
iron chelating agents
bone marrow or liver tx in sever cases

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16
Q

types of macrocytic anemias

A

B12 deficiency
folic acid deficiency

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17
Q

b12 deficiency anemia risk factors

A
  • nutritional (vegans)
  • malabsorption
  • pernicious anemia (autoimmune)
  • medications (PPI, H2RA, metformin)
  • age >65
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18
Q

pernicious anemia

A

destruction of parietal cells that produce intrinsic factor (d/t gastric atrophy)

autoimmune

type of b12 deficiency anemia

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19
Q

b12 deficiency anemia manifestation not present in folic acid deficiency

A

complex neurologic syndrome

paresthesias, difficulty with balance/proprioception, ataxia

decreased vibratory and position sense

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20
Q

b12 deficiency diagnostics

A
  • serum b12 low
  • elevated serum methylmalonic acid >1000
  • MCV elevated, low hct
  • peripheral smear -megaloblastic
  • decreased retic count
  • intrinsic factor antibody, antiparietal cell antibody
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21
Q

b12 deficiency treatment

A

cyanocobalamin 1000 mcg PO daily

response in 8 weeks

if no response, can do parenteral

simultaneous folic acid replacement

referral to neurology/hematology

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22
Q

folic acid deficiency characteristics

A

macrocytic

inadequate folic acid present for DNA synthesis and RBC maturation

RF: ETOH, age>65, dietary deficient, polypharmacy, malabsorption, increased demand (pregnancy/breast feeding)

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23
Q

folate acid deficiency treatment

A

rule out B12 deficiency
ID & treat cause
folate supplement - 1-5 mg PO for1-4 mos until recovery

repeat blood work in 4-6 weeks

folate rich diet

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24
Q

folate acid deficiency lab findings

A

serum folate low - screening
RBC folic acid low - diagnostic
MCV >100
retic count normal/decreased
B12 normal

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25
Q

aplastic anemia

A

deficiency of hematopoetic stem cells resulting in pancytopenia and bone marrow aplasia with NO ABNORMAL CELLS

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26
Q

aplastic anemia s/s

A

pallor, purpura, petechiae
recurrent infections

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27
Q

aplastic anemia diagnostic

A

2 of the following:
-hgb <10
- plt <50
-ANC<1.5

smear - erythrocytes, high MCV, low/absent reticulocytes

bone marrow biopsy

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28
Q

aplastic anemia treatment

A

supportive care

severe - BMT, immunosuppression, abx/transfusions

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29
Q

sickle cell anemia characteristics

A

autosomal recessive single gene deficit in beta chain of hemoglobin A leading to chronic hemolytic anemia

hemoglobin S is sickled, leading to hypoxia & pain

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30
Q

sickle cell s/s

A

acute pain - N/V, fever, swelling, tachypnea, HTN

chronic - gallstones, jaundice, hepatomegaly, cardiomyopathy, systolic murmur, poor healing ulcers

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31
Q

sickle cell diagnostics

A

peripheral smear - sickled cells, reticulocytes, nucleated RBCs, howell-jolly bodies, target cells

elevated WbC w reactive throbocytosis

elevated indirect bili

electrophoresis - hemoglobin S

32
Q

sickle cell mgmt

A

avoid crisis & triggers

abx prophylaxis or for chest syndrome

oxygen - sx mgmt

transfuse for vasoocclusive events

folic acid supplemetation

cure - SCT

33
Q

autoimmune hemolytic anemia

A

acquired

caused by IgG antibody

rapid onset anemia, fatigue, dyspnea, jaundice, splenomegaly, chest pain, heart failure

34
Q

autoimmune hemolytic anemia diagnostics

A

hgb low
retic count increased

peripheral smear - schistocytes, spherocytes, elliptocytes, spur cells, blister cells, bite cells, tear drops

indirect bili elevated
LDH elevated
haptoglobin low

direct coombs test - positive

35
Q

autoimmune hemolytic anemia tx

A

referral to heme

removal of insult, treat underlying conditions

corticosteroids!! prednisone 1mg/kg/day, then taper

plasmapheresis

splenectomy if steroids fail

36
Q

myelodysplastic syndrome

A

ineffective hematopoiesis: significant dysplasias resulting in one or more cytopenias

may evolve into AML

37
Q

myelodysplastic syndrome risk factors

A

usually no cause

age, exposure to chemo, SCT, other exposures

38
Q

myelodysplastic syndrome diagnostics

A

cytopenias

bone marrow biopsy - hypercellular marrow!!!

39
Q

hereditary hemochromatosis

A

autosomal recessive caused by HFR (high iron) gene

40
Q

hereditary hemochromatosis s/s

A

r/t iron accumulation!

liver disease
skin hyperpigementation
weakness/lethargy
arthropathy, arthralgias
cardiac enlargement, EKG abnormalities
impotence in males

41
Q

diagnostic for hemochromatosis

A

liver biopsy

42
Q

hemochromatosis treatment

A

therapeutic phlebotomy weekly

iron chelation

liver tx in severe cirrhosis

43
Q

polycythemia vera

A

myeloproliferative disorder that causes overproduction of all three hematopoietic cell lines

44
Q

RF for polycythemia vera

A

budd-chiari syndrome (abd pain, ascites, liver enlargement)

45
Q

polycythemia vera s/s

A

fatigue, blurred vision, HA, dizziness

pruritis

redness o face, extremities

thrombosis - most common complication

plethora of engorged retinal veins
splenomegaly

46
Q

polycythemia vera diagnostics

A

RBC morphology normal

high H&H, WBC, plt

JAK2 mutation confirms diagnosis

47
Q

polycythemia vera mgmt

A

therapeutic phlebotomy

minimize risk factors

cytoreductive agent - hydroxyurea

48
Q

hemophilia A & B

A

X- linked hereditary recessive OR acquried

reduction in coagulation factors

A - factor VIII deficiency
B-factor IX deficiency

49
Q

hemophilia symptoms

A

muscle/joint bleeding

complications - joint destruction, transmission of blood borne infection (2/2 transfusion)

50
Q

most common inherited bleeeding disorder

A

von willenbrand disease

51
Q

von willenbrand diseae

A

autosomal dominant

types 1&3 (more common)- reduce quantity of circulating vWF

type 2 - reduced quality of vWF

52
Q

von willebrand s/s

A

bleeding involving skin and mucous membranes

53
Q

von willebrand labs & dx & tx

A

ristocetin co-factor assay
von willebrand factor antigen
prolonged bleeding time, prolonged PTT

give DDAVP, antifibrolytics, TXA, aminocaproic acid, factor VIII

54
Q

ITP

A

autoimmune disorder causing platelet destruction

most common cause of thrombocytopenia

normal RBC, WBC

54
Q

fasdfad

A

dfasdfads

55
Q

ITP manifestations

A

bleeding into skin and mucosa

petechiae/purpura

56
Q

ITP diagnostics

A

platelets <100
maybe anemia
excluse hep B, C, HIV
bone marrow biopsy
diagnosis of exclusion

57
Q

ITP management

A

prednisone or dexamethasone

IVIG

platelet transfusion if bleeding

58
Q

HIT

A

unfractionated heparin most likely offender
super rare in LMWH

IgG immune reaction activates platelet and promotes thrombosis in setting of thrombocytopenia

59
Q

HIT RF

A

4 T’s

thrombocytopenia (2 pts - >50% fall, 1 pt 30-50% fall)

timing compatible w HIT (2 pts - 5-10d after heparin exposure or <1d if previously exposed, 1 pt >10 d)

thrombosis (2 - yes, 1 pt - possible)

oTher dx (2 pts - no, 1 pt- possible)

high probability: 6-8, moderate (start tx): 4-5, low 0-3

60
Q

dfsadsfa

A

dsafsdadfa

61
Q

HIT mgmt

A

review meds
d/c heparin
initiate DTI, then transition to Coumadin
avoid platelet transfusions
duplex doppler of lower extremities

argatroban (no liver disease)
bivalrudin (no renal disease)
fondaparinux

62
Q

HIT diagnostics

A

4T’s probability scale
HIT antigen assay
coagulation studies
Venous doppler US

more imaging if concern for clot

63
Q

DIC RF

A

OB
infections/sepsis (gram -)
neoplasm
massive tissue injury - trauma, burns
misc - snakebites, drugs, transfusion run, transplant rejection

64
Q

DIC labs

A

platelet - normal, then low
fibrinogen - normal, then low
aPTT, PT - high
D dimer - high
elevated LFTs
renal dysfunction
anemia
decreased clotting factors

65
Q

DIC treatment

A

treat underlying disorder
heme referral
transfuse goals
- platelets >20
- Fibrinogen >100 - give FFP, or cryo
- hgb >8

66
Q

transfusion reaction treatment

A

stop transfusion
give NS
try to maintain UOP <100cc/hr

67
Q

acute hemolytic rxn

A

usually clerical error

chills, fever, N/V,chest pain, back pain, anxiety, hemoglobinura

68
Q

allergic transfusion rxn

A

hypersensitivity rxn to allergens in blood

pruritus, flushing, dyspnea, urticarial, angioedema

give epi, bronchodilator, antihistamines, steroids

69
Q

febrile non-hemolytic transfusion reaction

A

multifactorial
fever without other cause for fever

give antipyretics

70
Q

transfusion-related acute lung injury

A

granulocyte activation in pulm vasculature results in increased vascular permeability

sudden onset dyspnea, tachypnea, fever, tachycardia, hypotension

tx with supportive care, O2

71
Q

delayed hemolytic transfusion reaction

A

fever and anemia days to weeks following transfusion

may have jaundice, hemoglobinuria

72
Q

delayed hemolytic transfusion reaction

A

fever and anemia days to weeks following transfusion

may have jaundice, hemoglobinuria

73
Q

blood transfusion reaction labs

A

direct antiglobulin test - Coombs test: positive in hemolytic rxn

repeat ABO testing on blood sample

UA - will show free hgb in acute hemolytic

LDH/bilirubin - may be elvated
haptoglobin - may be low

74
Q

anemic hgb

A

women <12
men <13

75
Q

iron deficiency labs

A

serum ferritin <40
serum iron <60

76
Q

normal serum B12

A

> 300 normal