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
What is the indication for DDAVP administration in hemorrhage?
Type 1 VWD or mild hemophilia A
26
What are the indications for tranexamic acid?
Mucocutaneous bleeding | Menorrhagia
27
M
M
28
What is the indication for aminocaproic acid?
mucocutaneous bleeding
29
What is the dose of aminocaproic acid?
50-100mg/kg loading dose | then 50mg/kg q6h max 12g/day
30
What are contraindications to aminocaproic acid?
DIC | upper urinary tract bleeding (hematuria)
31
What are contraindications to use of tranexamic acid?
upper urinary tract bleeding
32
Below what HGB level frequently requires transfusion?
50-60
33
What are the indications for transfusion in acute symptomatic anemia secondary to hemolysis?
< 50-60 in children | < 60-70 in adolescents
34
What are the reported mortality rates for pediatric patients with hemolytic anemia?
4-10%
35
What are the typical presenting features of hemolytic anemia specifically?
pallor jaundice dark urine
36
What are typical physical exam findings for patients with hemolytic anemia?
``` jaundice/icterus pallor tachycardia +/- murmur secondary to high-output mild hepatosplenomegaly ```
37
What are some indicators on physical exam of imminent cardiovascular collapse in hemolytic anemia?
``` JV distension significant hepatosplenomegaly gallop rhythm resp distress poor perfusion hypotension ```
38
Name the infectious causes of hemolytic anemia
``` Mycoplasma EBV CMV Parvovirus HHV-6 HIV ```
39
Name toxins that can cause hemolytic anemia
Naphthalene (mothballs) - if G6PD deficiency Copper (wilson disease) Lead
40
What medications can cause hemolytic anemia?
``` Penicillins Quinidine HCTZ Rifampin Sulfonamid Isoniazid Alpha-methyldopa Interferon-alpha IVIG ```
41
What autoimmune disorders can cause hemolytic anemia?
SLE Evans syndrome Thyroiditis Rheumatic disease
42
What immunologic disorders can cause hemolytic anemia?
CVID | Autoimmune lymphoproliferative syndrome
43
What can cause mechanical fragmentation leading to hemolytic anemia?
``` Heart valve prostheses/homografts Uncorrected valvular disease HUS TTP Collagen vascular disease Bone marrow transplantation - associated microangiopathy ```
44
What baseline labs MUST be ordered if there is suspicion of hemolytic anemia?
``` CBC with diff retics DAT Blood smear Type & screen Indirect & direct bili Lytes BUN/CR ```
45
What does a positive DAT indicate in hemolytic anemia?
presence of autoantibodies on erythrocytes
46
What is the differential diagnosis of DAT + hemolytic anemia?
Warm-reactive autoimmune hemolytic anemia Paroxysmal cold hemoglobinuria Cold Agglutinin Disease
47
What are the secondary causes of warm-reactive AIHA?
``` drug induced autoimmune disease malignancy immunodeficiency infection transfusion ```
48
What are the causes of paroxysmal cold hemoglobinuria?
viral infection | syphilis
49
What are the caues of cold agglutinin disease?
mycoplasma EBV parvovirus CMV
50
What lab finding in DAT+ hemolytic anemia will lead to a diagnosis of warm-reactive AIHA?
thermal reactivity of IgG & C3
51
What lab finding in DAT+ hemolytic anemia will lead to a diagnosis of Paroxysmal Cold Hemoglobinuria?
Reactivity of C3 to warm and cold | Positive Donath-Landsteiner test IgG
52
What lab finding in DAT+ hemolytic anemia will lead to a diagnosis of cold agglutinin disease?
C3 reactivity to cold and warm | IgM autoantibody
53
What is the differential diagnosis for presence of schistocytes on blood smear?
``` Microangiopathic hemolytic anemia TTP HUS DIC Mechanical destruction ```
54
What is the differential diagnosis of bite or blister cells on blood smear?
G6PD deficiency | Toxins
55
What features on history, physical & labs are concerning for TTP or HUS?
``` Diarrhea AKI Thrombocytopenia Fever Neurologic changes ```
56
What should be added to labs if a microangiopathic process is suspected?
coagulation panel
57
What hemoglobin level is considered stable post-transfusion?
60-80
58
What is the treatment for warm-reactive AIHA?
methylprednisolone 1-2mg/kg q6-8hrs
59
How should you proceed with RBC transfusion in symptomatic or unstable patients with warm reactive AIHA?
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
How should you proceed with transfusion of RBC for a patient with symptomatic cold-reactive hemolytic anemia?
transfuse with warmed blood or keep patients warm during transfusion
61
How should you treat asymptomatic cold-reactive hemolytic anemia?
oral supplementation of folic acid 1mg/day
62
What are the symptoms of an acute hemolytic transfusion reaction?
Fever, chills abdominal or flank pain chest tightness hypotension possible DIC
63
What is the reaction mechanism of an acute hemolytic transfusion reaction?
complement-fixing rBC antibody (ABO incompatibility)
64
What labs need to be sent to look for acute hemolytic transfusion reaction?
DAT + coombs hemoglobin bili LDH Urinalysis renal function coagulation blood samples - patient and transfused unit to double check testing
65
What is the management of an acute hemolytic transfusion reaction?
stop transfusion supportive care
66
What are the symptoms of a Transfusion-associated acute lung injury (TRALI)?
fever, chills, resp distress, hypoxia. pulmonary edema, hypotension
67
What is the mechanism of TRALI?
donor antibody to HLA or granulocyte speciic antigen
68
What are the symptoms of transfusion-associated circulatory overload (TACO)?
Respiratory distress, headache, hypertension. pulmonary edema,. congestive heart failure
69
How does vWF work?
VWF plays a key role in coagulation by facilitating platelet aggregation and adhesion and serving as a carrier molecule for factor VIII
70
What are the 3 types of vWF deficiency?
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
what is the pattern of inheritance of vwf deficiency type 1?
autosomal dominant
72
what is the pattern of inheritance of vwf deficiency type 3?
autosomal recessive
73
What tests are needed to make a diagnosis of vWF deficiency?
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
What are the lab findings in tumour lysis syndrome?
High K, P, UA and low ca
75
How is tumour lysis syndrome treated?
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
Infections transplant patients at most risk for in 1st 6 months post-transplant
EBV CMV Pneumocystis jirovecii pneumonia candidiasis Aspergillus Herpes Zoster Varicella
77
What is posttransplant lymphoproliferative disorer?
Spectrum of neoplastic diseases occurring after solid organ transplant Often associated with EBV infection
78
Clinical signs and symptoms of posttransplant lymphoproliferative disorder
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