heme Flashcards

(77 cards)

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
aplastic anemia
deficiency of hematopoetic stem cells resulting in pancytopenia and bone marrow aplasia with NO ABNORMAL CELLS
26
aplastic anemia s/s
pallor, purpura, petechiae recurrent infections
27
aplastic anemia diagnostic
2 of the following: -hgb <10 - plt <50 -ANC<1.5 smear - erythrocytes, high MCV, low/absent reticulocytes bone marrow biopsy
28
aplastic anemia treatment
supportive care severe - BMT, immunosuppression, abx/transfusions
29
sickle cell anemia characteristics
autosomal recessive single gene deficit in beta chain of hemoglobin A leading to chronic hemolytic anemia hemoglobin S is sickled, leading to hypoxia & pain
30
sickle cell s/s
acute pain - N/V, fever, swelling, tachypnea, HTN chronic - gallstones, jaundice, hepatomegaly, cardiomyopathy, systolic murmur, poor healing ulcers
31
sickle cell diagnostics
peripheral smear - sickled cells, reticulocytes, nucleated RBCs, howell-jolly bodies, target cells elevated WbC w reactive throbocytosis elevated indirect bili electrophoresis - hemoglobin S
32
sickle cell mgmt
avoid crisis & triggers abx prophylaxis or for chest syndrome oxygen - sx mgmt transfuse for vasoocclusive events folic acid supplemetation cure - SCT
33
autoimmune hemolytic anemia
acquired caused by IgG antibody rapid onset anemia, fatigue, dyspnea, jaundice, splenomegaly, chest pain, heart failure
34
autoimmune hemolytic anemia diagnostics
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
autoimmune hemolytic anemia tx
referral to heme removal of insult, treat underlying conditions corticosteroids!! prednisone 1mg/kg/day, then taper plasmapheresis splenectomy if steroids fail
36
myelodysplastic syndrome
ineffective hematopoiesis: significant dysplasias resulting in one or more cytopenias may evolve into AML
37
myelodysplastic syndrome risk factors
usually no cause age, exposure to chemo, SCT, other exposures
38
myelodysplastic syndrome diagnostics
cytopenias bone marrow biopsy - hypercellular marrow!!!
39
hereditary hemochromatosis
autosomal recessive caused by HFR (high iron) gene
40
hereditary hemochromatosis s/s
r/t iron accumulation! liver disease skin hyperpigementation weakness/lethargy arthropathy, arthralgias cardiac enlargement, EKG abnormalities impotence in males
41
diagnostic for hemochromatosis
liver biopsy
42
hemochromatosis treatment
therapeutic phlebotomy weekly iron chelation liver tx in severe cirrhosis
43
polycythemia vera
myeloproliferative disorder that causes overproduction of all three hematopoietic cell lines
44
RF for polycythemia vera
budd-chiari syndrome (abd pain, ascites, liver enlargement)
45
polycythemia vera s/s
fatigue, blurred vision, HA, dizziness pruritis redness o face, extremities thrombosis - most common complication plethora of engorged retinal veins splenomegaly
46
polycythemia vera diagnostics
RBC morphology normal high H&H, WBC, plt JAK2 mutation confirms diagnosis
47
polycythemia vera mgmt
therapeutic phlebotomy minimize risk factors cytoreductive agent - hydroxyurea
48
hemophilia A & B
X- linked hereditary recessive OR acquried reduction in coagulation factors A - factor VIII deficiency B-factor IX deficiency
49
hemophilia symptoms
muscle/joint bleeding complications - joint destruction, transmission of blood borne infection (2/2 transfusion)
50
most common inherited bleeeding disorder
von willenbrand disease
51
von willenbrand diseae
autosomal dominant types 1&3 (more common)- reduce quantity of circulating vWF type 2 - reduced quality of vWF
52
von willebrand s/s
bleeding involving skin and mucous membranes
53
von willebrand labs & dx & tx
ristocetin co-factor assay von willebrand factor antigen prolonged bleeding time, prolonged PTT give DDAVP, antifibrolytics, TXA, aminocaproic acid, factor VIII
54
ITP
autoimmune disorder causing platelet destruction most common cause of thrombocytopenia normal RBC, WBC
54
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55
ITP manifestations
bleeding into skin and mucosa petechiae/purpura
56
ITP diagnostics
platelets <100 maybe anemia excluse hep B, C, HIV bone marrow biopsy diagnosis of exclusion
57
ITP management
prednisone or dexamethasone IVIG platelet transfusion if bleeding
58
HIT
unfractionated heparin most likely offender super rare in LMWH IgG immune reaction activates platelet and promotes thrombosis in setting of thrombocytopenia
59
HIT RF
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
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61
HIT mgmt
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
HIT diagnostics
4T's probability scale HIT antigen assay coagulation studies Venous doppler US more imaging if concern for clot
63
DIC RF
OB infections/sepsis (gram -) neoplasm massive tissue injury - trauma, burns misc - snakebites, drugs, transfusion run, transplant rejection
64
DIC labs
platelet - normal, then low fibrinogen - normal, then low aPTT, PT - high D dimer - high elevated LFTs renal dysfunction anemia decreased clotting factors
65
DIC treatment
treat underlying disorder heme referral transfuse goals - platelets >20 - Fibrinogen >100 - give FFP, or cryo - hgb >8
66
transfusion reaction treatment
stop transfusion give NS try to maintain UOP <100cc/hr
67
acute hemolytic rxn
usually clerical error chills, fever, N/V,chest pain, back pain, anxiety, hemoglobinura
68
allergic transfusion rxn
hypersensitivity rxn to allergens in blood pruritus, flushing, dyspnea, urticarial, angioedema give epi, bronchodilator, antihistamines, steroids
69
febrile non-hemolytic transfusion reaction
multifactorial fever without other cause for fever give antipyretics
70
transfusion-related acute lung injury
granulocyte activation in pulm vasculature results in increased vascular permeability sudden onset dyspnea, tachypnea, fever, tachycardia, hypotension tx with supportive care, O2
71
delayed hemolytic transfusion reaction
fever and anemia days to weeks following transfusion may have jaundice, hemoglobinuria
72
delayed hemolytic transfusion reaction
fever and anemia days to weeks following transfusion may have jaundice, hemoglobinuria
73
blood transfusion reaction labs
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
anemic hgb
women <12 men <13
75
iron deficiency labs
serum ferritin <40 serum iron <60
76
normal serum B12
>300 normal