Heme/Onc Flashcards

1
Q

In what case is prompt transfusion and isotonic fluid resuscitation NOT indicated in anemia?

A

In a chronically anemic patient

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2
Q

What are the 3 umbrella causes for anemia?

A

blood loss
increased RBC destruction
decreased RBC productio

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3
Q

What are the causes of macrocytic anemia?

A
Megaloblastic anemia - folic acid or vit B12 deficiency
Liver disease
Reticulocytosis
Hypothyroidism
Myelodysplastic syndrome
Antiretrovirals (AZT)
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4
Q

What are the causes of microcytic anemia?

A
Iron deficiency
Thalassemia
Anemia of chronic disease
Sideroblastic anemia
Lead poisoning
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5
Q

What are the causes of normocytic anemia?

A
Low retics
Iron deficiency (early)
Anemia of chronic disease
Chronic renal disease
Hypothyroidism
Adrenal insufficiency
Hypopituitarism
Primary bone marrow disorder
Aplastic anemia
Malignancy
Myelodysplastic syndromes
Infection - Parvo B19
Blood loss
Hemolysis
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6
Q

What are indicators on lab results of hemolysis?

A

decreased serum haptoglobin
elevated LDH
increased unconjugated bili
hematuria

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7
Q

What is the most common cause of atraumatic blood loss?

A

GI bleeding

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8
Q

What lab tests should always be ordered for patients with suspected blood loss?

A

CBC
retics
coags
type & screen

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9
Q

If etiology of blood loss is unclear, what studies should be considered to find the source?

A

stool guaiac
hemolysis labs
pregnancy test
urine

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10
Q

What are the initial steps for all patients who have known or suspected blood loss?

A

immediate vascular access
cardiorespiratory monitoring
administration of O2 regardless of O2 sat

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11
Q

True or false?: Hemoglobin changes typically lag behind acute blood loss

A

true

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12
Q

In what patients could use of tranexamic acid be consdered?

A

unstable patient with significant acute hemorrhage

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13
Q

By how much will the HGB rise with 10mL/kg of pRBCs?

A

2g/dL

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14
Q

How do you calculate the volume of pRBCs to transfuse in acute hemorrhage?

A

([desired HGB - current HGB] x pts blood volume) / HGB of packed RBCs

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15
Q

How do you calculate estimated blood volume of a patient in mL?

A

weight (kg) x 70mL/kg

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16
Q

What is the estimated HGB in a unit of packed RBCs?

A

19-25g/dL

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17
Q

What is the dose of platelets for transfusion?

A

one unit per 5-10kg

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18
Q

By how much should one unit of random donor platelets increase PLT count?

A

5000-10,000/microL

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19
Q

What is the dose of FFP?

A

10-20mL/kg/dose

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20
Q

What does FFP contain?

A

all plasma-clotting factors but with variable concentrations

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21
Q

What is the indication for FFP transfusion?

A

bleeding with abnormal coags
factor deficiency replacement
reversal of vitamin K antagonist
Massive transfusion requirement

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22
Q

What is the indication for cryoprecipitate?

A

Bleeding associated with deficiency of fibrinogen, Factor 8, Factor 13 or VWF

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23
Q

What is the dose of cryoprecipitate?

A

1-2 units per 10kg of body weight

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24
Q

What does cryoprecipitate contain?

A

Fibrinogen, Factor 8, Factor 13, VWF

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25
Q

What is the indication for DDAVP administration in hemorrhage?

A

Type 1 VWD or mild hemophilia A

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26
Q

What are the indications for tranexamic acid?

A

Mucocutaneous bleeding

Menorrhagia

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27
Q

M

A

M

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28
Q

What is the indication for aminocaproic acid?

A

mucocutaneous bleeding

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29
Q

What is the dose of aminocaproic acid?

A

50-100mg/kg loading dose

then 50mg/kg q6h max 12g/day

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30
Q

What are contraindications to aminocaproic acid?

A

DIC

upper urinary tract bleeding (hematuria)

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31
Q

What are contraindications to use of tranexamic acid?

A

upper urinary tract bleeding

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32
Q

Below what HGB level frequently requires transfusion?

A

50-60

33
Q

What are the indications for transfusion in acute symptomatic anemia secondary to hemolysis?

A

< 50-60 in children

< 60-70 in adolescents

34
Q

What are the reported mortality rates for pediatric patients with hemolytic anemia?

A

4-10%

35
Q

What are the typical presenting features of hemolytic anemia specifically?

A

pallor
jaundice
dark urine

36
Q

What are typical physical exam findings for patients with hemolytic anemia?

A
jaundice/icterus
pallor
tachycardia
\+/- murmur secondary to high-output
mild hepatosplenomegaly
37
Q

What are some indicators on physical exam of imminent cardiovascular collapse in hemolytic anemia?

A
JV distension
significant hepatosplenomegaly
gallop rhythm
resp distress
poor perfusion
hypotension
38
Q

Name the infectious causes of hemolytic anemia

A
Mycoplasma
EBV
CMV
Parvovirus
HHV-6
HIV
39
Q

Name toxins that can cause hemolytic anemia

A

Naphthalene (mothballs) - if G6PD deficiency
Copper (wilson disease)
Lead

40
Q

What medications can cause hemolytic anemia?

A
Penicillins
Quinidine
HCTZ
Rifampin
Sulfonamid
Isoniazid
Alpha-methyldopa
Interferon-alpha
IVIG
41
Q

What autoimmune disorders can cause hemolytic anemia?

A

SLE
Evans syndrome
Thyroiditis
Rheumatic disease

42
Q

What immunologic disorders can cause hemolytic anemia?

A

CVID

Autoimmune lymphoproliferative syndrome

43
Q

What can cause mechanical fragmentation leading to hemolytic anemia?

A
Heart valve prostheses/homografts
Uncorrected valvular disease
HUS
TTP
Collagen vascular disease
Bone marrow transplantation - associated microangiopathy
44
Q

What baseline labs MUST be ordered if there is suspicion of hemolytic anemia?

A
CBC with diff
retics
DAT
Blood smear
Type & screen
Indirect & direct bili
Lytes
BUN/CR
45
Q

What does a positive DAT indicate in hemolytic anemia?

A

presence of autoantibodies on erythrocytes

46
Q

What is the differential diagnosis of DAT + hemolytic anemia?

A

Warm-reactive autoimmune hemolytic anemia
Paroxysmal cold hemoglobinuria
Cold Agglutinin Disease

47
Q

What are the secondary causes of warm-reactive AIHA?

A
drug induced
autoimmune disease
malignancy
immunodeficiency
infection
transfusion
48
Q

What are the causes of paroxysmal cold hemoglobinuria?

A

viral infection

syphilis

49
Q

What are the caues of cold agglutinin disease?

A

mycoplasma
EBV
parvovirus
CMV

50
Q

What lab finding in DAT+ hemolytic anemia will lead to a diagnosis of warm-reactive AIHA?

A

thermal reactivity of IgG & C3

51
Q

What lab finding in DAT+ hemolytic anemia will lead to a diagnosis of Paroxysmal Cold Hemoglobinuria?

A

Reactivity of C3 to warm and cold

Positive Donath-Landsteiner test IgG

52
Q

What lab finding in DAT+ hemolytic anemia will lead to a diagnosis of cold agglutinin disease?

A

C3 reactivity to cold and warm

IgM autoantibody

53
Q

What is the differential diagnosis for presence of schistocytes on blood smear?

A
Microangiopathic hemolytic anemia
TTP
HUS
DIC
Mechanical destruction
54
Q

What is the differential diagnosis of bite or blister cells on blood smear?

A

G6PD deficiency

Toxins

55
Q

What features on history, physical & labs are concerning for TTP or HUS?

A
Diarrhea
AKI
Thrombocytopenia
Fever
Neurologic changes
56
Q

What should be added to labs if a microangiopathic process is suspected?

A

coagulation panel

57
Q

What hemoglobin level is considered stable post-transfusion?

A

60-80

58
Q

What is the treatment for warm-reactive AIHA?

A

methylprednisolone 1-2mg/kg q6-8hrs

59
Q

How should you proceed with RBC transfusion in symptomatic or unstable patients with warm reactive AIHA?

A

Transfuse “least incompatible” unit of RBC at 5ml/kg
Infuse first 5mL over 10-15 min and observe for transfusion reaction
if no reaction, transfuse remainder over 2-4hrs
If reaction - select different donor

60
Q

How should you proceed with transfusion of RBC for a patient with symptomatic cold-reactive hemolytic anemia?

A

transfuse with warmed blood or keep patients warm during transfusion

61
Q

How should you treat asymptomatic cold-reactive hemolytic anemia?

A

oral supplementation of folic acid 1mg/day

62
Q

What are the symptoms of an acute hemolytic transfusion reaction?

A

Fever, chills
abdominal or flank pain
chest tightness
hypotension
possible DIC

63
Q

What is the reaction mechanism of an acute hemolytic transfusion reaction?

A

complement-fixing rBC antibody (ABO incompatibility)

64
Q

What labs need to be sent to look for acute hemolytic transfusion reaction?

A

DAT + coombs
hemoglobin
bili
LDH
Urinalysis
renal function
coagulation
blood samples - patient and transfused unit to double check testing

65
Q

What is the management of an acute hemolytic transfusion reaction?

A

stop transfusion
supportive care

66
Q

What are the symptoms of a Transfusion-associated acute lung injury (TRALI)?

A

fever, chills, resp distress, hypoxia. pulmonary edema, hypotension

67
Q

What is the mechanism of TRALI?

A

donor antibody to HLA or granulocyte speciic antigen

68
Q

What are the symptoms of transfusion-associated circulatory overload (TACO)?

A

Respiratory distress, headache, hypertension. pulmonary edema,. congestive heart failure

69
Q

How does vWF work?

A

VWF plays a key role in coagulation by facilitating platelet aggregation and adhesion and serving as a carrier molecule for factor VIII

70
Q

What are the 3 types of vWF deficiency?

A

Type 1 is a deficiency of VWF
type 2 subtypes are functional defects associated with VWF
and type 3 is an absence of VWF

The vast majority of patients with VWD have type 1 - variable penetrance

71
Q

what is the pattern of inheritance of vwf deficiency type 1?

A

autosomal dominant

72
Q

what is the pattern of inheritance of vwf deficiency type 3?

A

autosomal recessive

73
Q

What tests are needed to make a diagnosis of vWF deficiency?

A

VWF antigen level (VWF:Ag), its function, often ristocetin-induced platelet agglutination (VWF:RCo), and factor VIII activity (FVIII:C), the protein that it carries in plasma. The normal level for each of these components is greater than 50%

74
Q

What are the lab findings in tumour lysis syndrome?

A

High K, P, UA and low ca

75
Q

How is tumour lysis syndrome treated?

A

fluids at 1.5-2x maintenance with sodium bicarb 40mEq
allopurinol (preventative+tx)
rasburicase
Replace PO4 if < 1
Furosemide/insulin+glucose/kayexalate for hyperkalemia
only replace Ca if symptomatic

76
Q

Infections transplant patients at most risk for in 1st 6 months post-transplant

A

EBV
CMV
Pneumocystis jirovecii pneumonia
candidiasis
Aspergillus
Herpes Zoster
Varicella

77
Q

What is posttransplant lymphoproliferative disorer?

A

Spectrum of neoplastic diseases occurring after solid organ transplant
Often associated with EBV infection

78
Q

Clinical signs and symptoms of posttransplant lymphoproliferative disorder

A

Prior EBV infection with increasing viral load measured by pCR
Progressive and unremitting GI findings (diarrhea, bloody stool, abdo pain. anorexia, weight loss), pulmonary symptoms or vague systemic malaise

79
Q
A