Heme/Onc Flashcards
In what case is prompt transfusion and isotonic fluid resuscitation NOT indicated in anemia?
In a chronically anemic patient
What are the 3 umbrella causes for anemia?
blood loss
increased RBC destruction
decreased RBC productio
What are the causes of macrocytic anemia?
Megaloblastic anemia - folic acid or vit B12 deficiency Liver disease Reticulocytosis Hypothyroidism Myelodysplastic syndrome Antiretrovirals (AZT)
What are the causes of microcytic anemia?
Iron deficiency Thalassemia Anemia of chronic disease Sideroblastic anemia Lead poisoning
What are the causes of normocytic anemia?
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
What are indicators on lab results of hemolysis?
decreased serum haptoglobin
elevated LDH
increased unconjugated bili
hematuria
What is the most common cause of atraumatic blood loss?
GI bleeding
What lab tests should always be ordered for patients with suspected blood loss?
CBC
retics
coags
type & screen
If etiology of blood loss is unclear, what studies should be considered to find the source?
stool guaiac
hemolysis labs
pregnancy test
urine
What are the initial steps for all patients who have known or suspected blood loss?
immediate vascular access
cardiorespiratory monitoring
administration of O2 regardless of O2 sat
True or false?: Hemoglobin changes typically lag behind acute blood loss
true
In what patients could use of tranexamic acid be consdered?
unstable patient with significant acute hemorrhage
By how much will the HGB rise with 10mL/kg of pRBCs?
2g/dL
How do you calculate the volume of pRBCs to transfuse in acute hemorrhage?
([desired HGB - current HGB] x pts blood volume) / HGB of packed RBCs
How do you calculate estimated blood volume of a patient in mL?
weight (kg) x 70mL/kg
What is the estimated HGB in a unit of packed RBCs?
19-25g/dL
What is the dose of platelets for transfusion?
one unit per 5-10kg
By how much should one unit of random donor platelets increase PLT count?
5000-10,000/microL
What is the dose of FFP?
10-20mL/kg/dose
What does FFP contain?
all plasma-clotting factors but with variable concentrations
What is the indication for FFP transfusion?
bleeding with abnormal coags
factor deficiency replacement
reversal of vitamin K antagonist
Massive transfusion requirement
What is the indication for cryoprecipitate?
Bleeding associated with deficiency of fibrinogen, Factor 8, Factor 13 or VWF
What is the dose of cryoprecipitate?
1-2 units per 10kg of body weight
What does cryoprecipitate contain?
Fibrinogen, Factor 8, Factor 13, VWF
What is the indication for DDAVP administration in hemorrhage?
Type 1 VWD or mild hemophilia A
What are the indications for tranexamic acid?
Mucocutaneous bleeding
Menorrhagia
M
M
What is the indication for aminocaproic acid?
mucocutaneous bleeding
What is the dose of aminocaproic acid?
50-100mg/kg loading dose
then 50mg/kg q6h max 12g/day
What are contraindications to aminocaproic acid?
DIC
upper urinary tract bleeding (hematuria)
What are contraindications to use of tranexamic acid?
upper urinary tract bleeding
Below what HGB level frequently requires transfusion?
50-60
What are the indications for transfusion in acute symptomatic anemia secondary to hemolysis?
< 50-60 in children
< 60-70 in adolescents
What are the reported mortality rates for pediatric patients with hemolytic anemia?
4-10%
What are the typical presenting features of hemolytic anemia specifically?
pallor
jaundice
dark urine
What are typical physical exam findings for patients with hemolytic anemia?
jaundice/icterus pallor tachycardia \+/- murmur secondary to high-output mild hepatosplenomegaly
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
Name the infectious causes of hemolytic anemia
Mycoplasma EBV CMV Parvovirus HHV-6 HIV
Name toxins that can cause hemolytic anemia
Naphthalene (mothballs) - if G6PD deficiency
Copper (wilson disease)
Lead
What medications can cause hemolytic anemia?
Penicillins Quinidine HCTZ Rifampin Sulfonamid Isoniazid Alpha-methyldopa Interferon-alpha IVIG
What autoimmune disorders can cause hemolytic anemia?
SLE
Evans syndrome
Thyroiditis
Rheumatic disease
What immunologic disorders can cause hemolytic anemia?
CVID
Autoimmune lymphoproliferative syndrome
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
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
What does a positive DAT indicate in hemolytic anemia?
presence of autoantibodies on erythrocytes
What is the differential diagnosis of DAT + hemolytic anemia?
Warm-reactive autoimmune hemolytic anemia
Paroxysmal cold hemoglobinuria
Cold Agglutinin Disease
What are the secondary causes of warm-reactive AIHA?
drug induced autoimmune disease malignancy immunodeficiency infection transfusion
What are the causes of paroxysmal cold hemoglobinuria?
viral infection
syphilis
What are the caues of cold agglutinin disease?
mycoplasma
EBV
parvovirus
CMV
What lab finding in DAT+ hemolytic anemia will lead to a diagnosis of warm-reactive AIHA?
thermal reactivity of IgG & C3
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
What lab finding in DAT+ hemolytic anemia will lead to a diagnosis of cold agglutinin disease?
C3 reactivity to cold and warm
IgM autoantibody
What is the differential diagnosis for presence of schistocytes on blood smear?
Microangiopathic hemolytic anemia TTP HUS DIC Mechanical destruction
What is the differential diagnosis of bite or blister cells on blood smear?
G6PD deficiency
Toxins
What features on history, physical & labs are concerning for TTP or HUS?
Diarrhea AKI Thrombocytopenia Fever Neurologic changes
What should be added to labs if a microangiopathic process is suspected?
coagulation panel
What hemoglobin level is considered stable post-transfusion?
60-80
What is the treatment for warm-reactive AIHA?
methylprednisolone 1-2mg/kg q6-8hrs
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
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
How should you treat asymptomatic cold-reactive hemolytic anemia?
oral supplementation of folic acid 1mg/day
What are the symptoms of an acute hemolytic transfusion reaction?
Fever, chills
abdominal or flank pain
chest tightness
hypotension
possible DIC
What is the reaction mechanism of an acute hemolytic transfusion reaction?
complement-fixing rBC antibody (ABO incompatibility)
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
What is the management of an acute hemolytic transfusion reaction?
stop transfusion
supportive care
What are the symptoms of a Transfusion-associated acute lung injury (TRALI)?
fever, chills, resp distress, hypoxia. pulmonary edema, hypotension
What is the mechanism of TRALI?
donor antibody to HLA or granulocyte speciic antigen
What are the symptoms of transfusion-associated circulatory overload (TACO)?
Respiratory distress, headache, hypertension. pulmonary edema,. congestive heart failure
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
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
what is the pattern of inheritance of vwf deficiency type 1?
autosomal dominant
what is the pattern of inheritance of vwf deficiency type 3?
autosomal recessive
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%
What are the lab findings in tumour lysis syndrome?
High K, P, UA and low ca
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
Infections transplant patients at most risk for in 1st 6 months post-transplant
EBV
CMV
Pneumocystis jirovecii pneumonia
candidiasis
Aspergillus
Herpes Zoster
Varicella
What is posttransplant lymphoproliferative disorer?
Spectrum of neoplastic diseases occurring after solid organ transplant
Often associated with EBV infection
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
