Haematology Flashcards
Acute Leukemia
Considerations
Acute Leukemia
Considerations
Impaired immunity with ↑ risk of infections
Chronic anemia
Thrombocytopenia with risk of hemorrhage
Hyperleukocytosis
Leukostasis, disseminated intravascular coagulopathy (DIC), tumor lysis syndrome (TLS)
Bleeding, thromboembolic events, neurologic & pulmonary complications
TLS: hyperuricemia, hyperkalemia, hyperphosphatemia, hypocalcemia, acute renal failure
Possible bone marrow transplantation with risk of graft vs host disease
Chemotherapy with end-organ dysfunction
Doxocubicin (cardiomyopathy)
Anemia
Background
Anemia
Background
Periop anemia a/w various complications & ↑ M&M
Common periop causes:
Nutritional deficiencies (incl Fe deficiency)
Inflammation
Chronic underlying disorders (ex cancer, CKD, menorrhagia)
Surgical blood loss
Classification – based on mechanism
↓ production
↑ destruction (hemolysis)
Blood loss
Classification – based on MCV
Microcytic (ex Fe-deficiency)
Normocytic
Blood loss
Hemolysis
Anemia of chronic dz
Marrow failure
Macrocytic
Vit B12/folate deficiency, MDS
Goals & Management
Patient Blood Management
Preop
Detection of anemia
History (bleeding hx, co-morbid dz)
Investigations - CBC, coagulation & iron studies where indicated
Management of preexisting anemia
If possible, delay surgery to optimize preop
Iron - po vs IV (IV if intolerance to po, poor GI uptake, or short timeline preop)
Vitamin B12/folate
Erythropoietin stimulating agents
Referral to hematology prn
Intraop
Optimize hemostasis
Hold antithrombotic medications preop where appropriate (including herbals that can affect hemostasis)
Correct coagulopathy
Tranexamic acid (antifibrinolytic)
Topical hemostatics
Maintain normothermia
Minimize blood loss
Meticulous hemostasis
Minimally invasive techniques
Tourniquet prn
Maintain blood volume & hemoglobin concentration
Replace losses w/ IV crystalloid or colloid until Hb drops below threshold
avoid large volume crystalloid infusion
Blood transfusion
Usual threshold ~70-80 g/L
Consider threshold of 90 g/L if:
significant ongoing bleeding
acute coronary syndrome
signs of myocardial/other organ ischemia
1 unit of pRBCs increased Hb by ~10 g/L
Use of interdisciplinary blood conservation modalities
Cell saver
Preop autologous donation (usually discouraged)
Acute normovolemic hemodilution
Patient-centered decision making
Postop
Prevention of new-onset (hospital-acquired) anemia (or exacerbation of existing anemia)
Monitor closely for postop bleeding
Limit vol & frequency of blood draws
Antiphospholipid Antibody Syndrome
Background
Considerations
Antiphospholipid Antibody Syndrome
Background
Antiphospholipid antibody syndrome (APS) = patients who experience thromboses or pregnancy complications and have laboratory evidence of antiphospholipid antibodies in their blood
Primary APS: the sole manifestation of an autoimmune process
Secondary APS: in association with another disease such as systemic lupus erythematosus
The deep veins of the lower extremities are the most common sites of venous thrombosis, and the cerebral vasculature (stroke and TIA) is the most common site for arterial thrombosis
Multiple body systems may be impacted, including lungs and heart
Considerations
Patients may be on chronic anticoagulant or antiplatelets agents that may impact perioperative management, including the need for bridging therapy
Patients are at increased risk of perioperative clotting events and need a DVT prophylaxis plan
Pulmonary involvement may include pulmonary thromboembolic disease and pulmonary hypertension
Cardiac involvement may include valvular thickening and valve nodules that may lead to valvular dysfunction
G6PD Deficiency
Background
Considerations
Goals
Common drugs to avoid
G6PD Deficiency
Background
X-linked disorder, the most common enzymatic disorder of RBCs
Hemolysis is the result of the inability of the RBC to protect itself from oxidative stress
Spectrum of disease: chronic hemolysis, intermittent hemolysis, hemolysis only with triggers, no hemolysis
Acute insults that either precipitate or aggravate hemolysis include infection, certain drugs, & fava beans
Considerations
Chronic hemolysis:
Chronic pRBC transfusions
Difficult crossmatch
Anemia
Avoidance of oxidative stress as it precipitates acute hemolysis:
Oxidative drugs (see list below)
Infection, hypoxia, hypothermia, stress
Metabolic abnormalities (e.g., diabetic ketoacidosis)
Foods (e.g., fava beans)
Unable to reduce methemoglobin:
Methemoglobinemia: oxidative stress
Methylene blue: oxidative stress
Consider hematology consultation
Goals
Identify patients at risk
Avoid precipitants of oxidative stress
Avoid precipitants of methemoglobinemia
Manage hemolysis by removing trigger, pRBC transfusion as needed, supportive management
Common Drugs to Avoid
Acetaminophen
Acetylsalicylic acid
Ciprofloxacin
Dapsone
Methylene blue
Nitrofurantoin
Phenytoin
Streptomycin
Sulpha drugs
For a more complete list, visit:
https://www.g6pd.org/en/G6PDDeficiency/SafeUnsafe.aspx
Disseminated Intravascular Coagulation (DIC)
Considerations
Goals
Disseminated Intravascular Coagulation (DIC)
Considerations
Emergency: mobilize resources & delegate tasks
Search for & correct precipitant:
Obstetrical hemorrhage, placental abruption, amniotic fluid embolism, pregnancy-induced hypertension
Trauma/burns
Transfusion
Sepsis
Aggressive treatment: potential for massive transfusion:
Hypothermia
Hypocalcemia
Factor dilution
Thrombocytopenia
Acidosis
Volume overload (acute respiratory distress syndrome, acute lung injury)
Sequelae of bleeding & thromboembolism:
Bleeding: intracranial, thorax, abdomen, cardiac tamponade
Thrombus: pulmonary embolism, acute renal failure, liver failure, stroke
Goals
Platelets: maintain above 50 000 (1 unit/10 kg; 0.1-0.2 units/kg)
Fresh frozen plasma: 10ml/kg to maintain INR < 1.5 – 2
Cryoprecipitate to maintain fibrinogen > 1.5 (2 units / 10 kg) (maintain > 2.0 in pregnant patients)
pRBC’s to maintain organ perfusion
Follow coagulation profile, fibrinogen, hemoglobin & platelets at least hourly
Heparin not recommended (except possibly in DIC secondary to malignancies causing thrombosis)
Antifibrinolytics not recommended
Factor V Leiden
Background
Considerations
Factor V Leiden
Background
The factor V Leiden mutation results in resistance to activated protein C thus causing thrombosis.
Factor V Leiden is the The most common inherited thrombophilia in individuals with venous thromboembolism
Other major hereditary disorders linked to hypercoagulability include antithrombin deficiency, protein C and S deficiencies, and prothrombin 20210A
Considerations
Patients may be on chronic anticoagulant or antiplatelets agents that may impact perioperative management, including the need for bridging therapy
Patients are at increased risk of perioperative clotting events and need a DVT prophylaxis plan
Hemophilia A&B
Background
Considerations
Goals
Optimisation and Treatment
Hemophilia A&B
Background
X-linked recessive disorder characterized by a deficiency of Factor 8 (A) or 9 (B) resulting in spontaneous hemorrhage or uncontrolled bleeding with trauma or surgery
Classification by factor levels:
Mild: 5-25%
Moderate: 1-5%
Severe: <1%
↑ PTT, normal INR
Considerations
High risk for perioperative bleeding
Sequelae of bleeding into enclosed spaces (joints, intracranium, pericardium, thorax)
Potential contraindication to neuraxial anesthesia
Factor optimization (replacement/supplementation) & identification of factor antibodies (e.g., inhibitors)
Coexisting viral infections secondary to transfusions: HIV, hepatitis (less now with recombinant products)
Consider preoperative hematology consultation
Goals
Optimize factor activity & coagulation profile in perioperative period
Minimize perioperative blood loss; consider blood conservation strategies
Optimization & Treatment
Hemophilia A
DDAVP for mild disease
Recombinant factor VIII (Humate P)
Factor VIII concentrates (fresh frozen plasma contains minimal factor VIII)
Recombinant factor VIIa for inhibitors (approved indication)
Cryoprecipitate if nothing else available (this is the only standard fractionated blood product containing meaningful amounts of factor VIII)
Cryoprecipitate contains factor VIII, fibrinogen, von Willebrand factor, fibronectin, factor XIII
Hemophilia B
Recombinant factor IX
HIT (Heparin-Induced Thrombocytopenia)
Background
Considerations
Prevention
Management
HIT (Heparin-Induced Thrombocytopenia)
Background
Severe immune-mediated disease from exposure to heparin causing thrombosis (venous & arterial) & thrombocytopenia
d/t formation of IgG antibodies against heparin-platelet factor 4 complexes resulting in plt activation & consumption, & activation of multiple prothrombotic pathways
Typically see ↓ plts by >30%, 5-10 days after heparin started
Plts rarely drop <20
Risk UFH > LMWH
4 Ts scoring system (pre-test probability):
Thrombocytopenia/↓ plts (Nadir ≥20, 50% drop)
Timing of plts ↓ (5-10 days)
Thrombosis
OTher causes ruled out (periop: sepsis, shock +/- mechanical circulatory support, DIC)
Dx based on detection of PF4 antibodies & functional HIT assay
Note: can take several days to get assay results; if strong suspicion, tx as HIT until proven otherwise
Mortality ~10-20%
Considerations
Acute HIT:
Intraop anticoagulation during CVS surgery in patients w/ active HIT
Bivalirudin - requires modification of perfusion technique during CPB
May have excess bleeding (esp if impaired renal fxn/complex cardiac surgery)
Heparin + reversible antiplatelet
Periop plasmapheresis to reduce HIT antibody burden
High-dose IVIG + ultra short acting plt inhibitor (ex cangrelor) during CPB to facilitate heparin use
Prior HIT:
Delay non-urgent surgery >1 month after dx where possible
May require bridging anticoagulation (ex argatroban) if on warfarin/DOAC
Short term heparin tx may be possible if remote hx of HIT
Neuraxial techniques contraindicated w/ argatroban, bivalirudin
Prevention
Avoid heparin where possible
Use LMWH instead of UFH where possible
Monitor plt counts daily in high risk patients
Management
Stop all heparin exposure
Treated w/ direct thrombin inhibitor (DTI) (ex argatroban, bivalirudin)
Fondaparinux another option
May transition to warfarin or DOAC for several months d/t ↑ risk of thrombosis
If refractory to DTIs, can consider IVIG or plasmapheresis
Idiopathic Thrombocytopenic Purpura - ITP
Background
Considerations
Idiopathic Thrombocytopenic Purpura - ITP
Background
ITP is an acquired thrombocytopenia caused by autoantibodies against platelet antigens
ITT is a diagnosis of exclusion: one must exclude all other causes of nonimmune and immune platelet destruction, in a patient with isolated thrombocytopenia.
The goal of treatment in a non-surgical setting is not to normalize platelets, but rather to prevent significant bleeding
Critical bleeding events are treated with platelets transfusions, and glucocorticoids (typically, pulse dexamethasone) plus intravenous immune globulin (IVIG)
Severe bleeding events are treated with glucocorticoids
Therapies for refractor cases: Rituximab, TPO receptor agonist, and Splenectomy
Considerations
↑ Risk of surgical bleeding and the need for a specific platelet count for surgery:
Hematology involvement is recommended
Platelet threshold discussion with surgeon, but generally keep platelets >50,000/microL for major surgery and >100,000/microL for neurosurgery
Platelets transfusions may be needed in emergency surgery
Steroids, IVIG, or a TPO receptor agonist like romiplostim can be used to help raise the platelet count
Neuraxial anesthesia considerations:
The exact platelet level for performing spinal and epidural techniques is controversial and varies by clinician
Generally, ITP patients may be safe to receive neuraxial anesthesia if the platelet count is >70,000/microL.
Neuraxial anesthesia may be acceptable in patients with platelet counts between 50,000-70,000/microL where the risk:benefit analysis favors a neuraxial approach
Neuraxial anesthesia is contraindicated in those with platelet counts <50,000/microL
Considerations of medications a patient with ITP may be receiving
Jehovah’s Witness Patients
Background
Considerations
Conflicts
Management
In Pregnancy
Jehovah’s Witness Patients
Background
Traditionally, orthodox Jehovah’s Witnesses won’t accept homologous or autologous whole blood, pRBCs, plasma, platelets & WBCs, even when necessary to prevent morbidity/mortality
Refusal is based on religious beliefs deriving from strict literal interpretation of passages in the Bible forbidding the “eating” of blood
Belief that eternal life may be forfeited if they do not exactly follow biblical commands
Usually refused
Whole blood
RBCs
Platelets
FFP
Cryoprecipitated antihemophilic factor
Granulocytes
Fibrin glue/sealant
Predeposited autologous blood/components
Usually accepted
Normovolemic hemodilution*
Intraoperative RBC salvage*
Erythropoietin (albumin-free)
Hemodialysis**
Heart-lung equipment**
*Usually accepted if patient remains in continuous contact with blood
**If non-blood prime used
Individual Decision (according to the individual’s preference)
Albumin
Immune globulins
Factor concentrates
Organ & tissue transplants
Considerations
Need for preoperative hemoglobin optimization & perioperative blood conservation
Legal issues
A patient’s legal right to refuse or consent to treatment is based on common law & is therefore is constantly evolving as new cases are decided
Clinicians should not administer emergency treatment without consent if they have reason to believe that the patient would refuse such treatment if he or she were capable
In certain pediatric cases, the child may be made a ward of the court in order to administer clinically necessary blood transfusions
Informed consent
Must outline risks & benefits of receiving or refusing transfusions to the individual patient in their clinical situation
Discuss alternatives to transfusion (may include transferring patient to another facility with more experience)
Determine specifically which blood products/procedures the patient will accept & refuse
If necessary, follow appropriate procedures to obtain court intervention (ex pediatric patients, patients with dementia, comatose, etc)
Conflicts
Conflict between beneficence & autonomy, where autonomy is generally given precedence over beneficence
Physicians are ordinarily taught to preserve life, yet they must also respect a competent adult patient’s right to refuse treatment
Management
Optimize preop hemoglobin
Enhance RBC production
Iron supplementation if deficient
Oral in divided doses
IV if short time before surgery, intolerance to po Fe, or GI absorption problems
Folate and/or vitamin B12 supplementation if deficient
Erythropoietin
If anemia of renal/chronic disease
Minimize iatrogenic blood loss
Avoid unnecessary testing
Minimize test sample volume (ex pediatric tubes)
Minimize intraop blood loss
Meticulous surgical technique
Regional anesthesia
Maintain normothermia, physiologic pH
Intraoperative cell salvage
Normovolemic hemodilution
Ensure hemostasis
Antifibrinolytic agents (tranexamic acid, aprotinin, etc)
Fibrinogen concentrate (if acceptable to patient)
Desmopressin
Prothrombin complex concentrates (where appropriate)
Recombinant Factor VIIa (controversial)
Maintain circulating blood volume
Crystalloid
Synthetic colloid
In Pregnancy
Epidural blood patch may be acceptable if blood remains in constant connection to patient (i.e. injecting syringe is connected to vein via tube)
Sickle Cell Disease
Considerations
Goals
Optimisations
Acute Chest Syndrome
Acute Pain Crisis
Pregnancy Considerations
Sickle Cell Disease
Considerations
Multisystem disease with end-organ dysfunction:
CNS: stroke
Cardiovascular: LV hypertrophy, high-output cardiac failure (anemia), myocardial infarction without coronary artery disease
Respiratory: acute chest syndrome (ACS), restrictive lung disease (pulmonary fibrosis), pulmonary hypertension, RV hypertrophy, cor-pulmonale
Renal: renal failure, renal infarction
Spleen: sequestration, infarcts; if splenectomy → ↓ immunity
Potential for perioperative exacerbations of vaso-occlusive crises:
Acute pain crisis
Acute chest syndrome
Chronic hemolytic anemia:
Chronic RBC transfusion & its complications (alloimmunization, iron overload, viral transmission)
Chronic pain & potential opioid tolerance
Preoperative optimization: consider preoperative transfusion for goal hematocrit: 30%, hemoglobin: 100 (see discussion in optimization)
Medications: immunosuppresants, antineoplastics
Goals
Avoid precipitants of sickle cell crisis:
Hypoxia
Vascular stasis
Hypothermia
Hypovolemia/hypotension
Acidosis
Optimize perioperative pain control
Monitor for:
Vaso-occlusive crisis
Acute chest syndrome
Aplastic crisis
Splenic sequestration syndrome
Right upper quadrant syndrome
Optimization (in consultation with hematology)
Risk factors for acute pain crises:
Age, frequency of hospitalizations &/or transfusions for episodes of crisis, evidence of organ damage (e.g., low baseline oxygen saturation, elevated creatinine, cardiac dysfunction), history of central nervous system events, concurrent infection
Procedural risk for complications:
Low: minor surgery (e.g., inguinal hernia & extremity surgery)
Intermediate: intra-abdominal operations (e.g., cholecystectomy)
High: intracranial & intrathoracic procedures, hip surgery
Hematology consult, optimize treatment:
Hydroxyurea to ↑ fetal hemoglobin production
Cancel non-emergent cases if patient experiencing a crisis
Only low risk patients should be considered for outpatient surgery
IV fluid to avoid dehydration while NPO
Preoperative transfusion therapy:
Controversial without good evidence
Purpose is to correct pre-existing anemia, ↓ hemoglobin S concentration & ↑ adult hemoglobin
Consider target hemoglobin 100 or hematocrit 30% for intermediate & high risk surgeries & always have blood available for any surgery
Exchange transfusions are not routinely recommended
Acute chest syndrome (ACS)
Background:
Second most common reason for hospitalization after vaso-occlusive crisis
Mortality 2-12%; accounts for 25% of deaths in sickle cell patients
Characterized by acute respiratory symptoms concurrent with new infiltrate on CXR
Spectrum of pathology:
Infection
Infarction (especially ribs)
Pulmonary sequestration
Fat embolism
Pulmonary vaso-occlusion due to sequestration of sickled cells in small pulmonary vessels
Infection or fat emboli may lead to vaso-occlusion & sequestration
Clinical picture: fever, tachypnea, pleuritic pain & cough
CXR: normal to complete opacification but usually demonstrates a new lobar infiltrate:
Children: upper lobe disease common
Adults: multilobe & lower lobe disease more common
Strong relationship between ACS & stroke
Treatment:
Admit to monitored setting, may need ICU
Hydration to euvolemia
Oxygen, noninvasive PPV if necessary
Bronchodilators (even if not wheezing)
Broad spectrum antibiotics: infection is one of the most common causes of ACS
Transfusion: both simple & exchange transfusion:
Simple transfusion: goal is to ↑ hematocrit to > 30%:
Indications:
To improve oxygenation, particularly in patients with oxygen saturation below 92% on room air & to prevent progression to respiratory failure
For accentuated anemia, defined as a hematocrit that is 10% to 20% below the patient’s usual hematocrit, or with a dropping hematocrit during hospitalization
Clinical or radiological progression of disease but not impending respiratory failure
For patients in whom exchange transfusion will be delayed; simple transfusions may be used to temporize the clinical situation until the exchange transfusion can be performed
Exchange transfusion: goal is to ↓ the level of hemoglobin S to < 30%:
Indications:
Progression of ACS despite simple transfusion
Severe hypoxemia
Multi-lobar disease
Previous history of severe ACS or cardiopulmonary disease
Analgesia: adequate analgesia of spine, thoracic, & abdominal pain is important to prevent hypoventilation & atelectasis
Possibly inhaled nitric oxide for severe cases
Acute pain crisis (Vaso-occlusive crisis)
Anesthesia may be requested to assist with analgesia (e.g., patient-controlled analgesia)
Typically occurs in long bones, ribs, spine, or abdomen
Precipitants: infection, dehydration, hypothermia, hypoxia, stress, alcohol intake, menstruation
Bone pain from ischemia & infarction of marrow or cortex
Abdominal pain from bowel ischemia, organ infarction, or referred from the ribs
Severity of pain can range from annoying to disabling
Treatment: rest, warmth, reassurance, analgesia, fluid replacement:
Oral analgesics may be sufficient for minor attacks
Opioids (IM, SC, IV, PO):
PCA opioids with baseline analgesia provided by background infusion or fentanyl patch
Acetaminophen & NSAIDs:
NSAIDS particularly good for bone pain
Ketamine as adjunct
Regional blocks as appropriate, epidural use has been reported
Pregnancy Considerations
Interaction with pregnancy:
Pregnancy typically exacerbates the complications of sickle cell anemia
Maternal mortality is as high as 1%
Pulmonary embolism & infection are the leading causes of death
Management:
Hemoglobin goals:
> 80 for vaginal delivery
> 100 for cesarean delivery
Epidural strongly recommended in labor to reduce stress/pain
Safe to use either GA or neuraxial for cesarean section
Goals: avoid hypovolemia, hypoxemia, hypercarbia, hypothermia, pain, stress
Thalassemia
Background
Considerations (4 types)
Goals
Thalassemia
Background
↓ synthesis of alpha or beta chains of hemoglobin → precipitation of unpaired chains & premature RBC destruction
Considerations
Chronic hemolytic anemia:
Compensation: ↑ cardiac output, ↑ 2,3-DPG, ↑ plasma volume
Cholilithiasis, splenomegaly
Sequelae of multiple transfusions/iron overload:
Cardiomyopathy, dysrhythmias
Pulmonary hypertension
Hepatic fibrosis, diabetes
Alloimmunization
4 types:
Beta thalassemia major:
Potential difficult airway due to maxillary overgrowth (from bone marrow stimulation)
Hemochromatosis (deposition of hemosiderin into cardiac muscle → dilated cardiomyopathy, heart failure, conduction delays)
Jaundice secondary to hemolysis
Hemolytic anemia
Thinning of cortical bone → potentially difficult regional secondary to vertebral destruction
Beta thalassemia minor:
Mild hemolytic anemia & iron deficiency
Alpha thalassemia major: incompatible with life
Alpha thalassemia minor: mild anemia
Goals
Ensure not anemic for surgery (typical goal hemoglobin > 100)
Anticipate difficult airway in beta-thalassemia major
Tumor Lysis Syndrome
Considerations
Treatment
Tumor Lysis Syndrome
Considerations
Severe acute life threatening condition with potential for multi-system failure:
Hyperkalemia & secondary arrhythmias
Hyperuricemia & acute renal failure
Hypocalcemia & risk of seizures, tetany
Hyperphosphatemia & prolonged QT interval
Underlying hematologic malignancy:
4M’s (mass effects, metastases, metabolic abnormalities, medications)
Chemotherapy &/or radiation with end-organ dysfunction
Treatment
Need for admission to high acuity setting
Rapidly diagnose & treat metabolic derangements
Prevent & support renal failure:
Volume loading (20 mL/kg) & 1.5-2 times maintenance
If volume overloaded → diuretics, potentially dialysis
Acute treatment of hyperkalemia & life threatening arrhythmias
Rasburicase/allopurinol for hyperuricemia
Von Willebrand’s Disease (vWD)
Considerations
High risk for perioperative bleeding
Sequelae of bleeding in enclosed spaces (joints, intracranial, pericardium, thorax)
Potential contraindication to neuraxial anesthesia & analgesia
Consultation with hematology for factor optimization (replacement/supplementation)
Goals
Optimize factor activity & coagulation profile in perioperative period
Techniques for minimizing perioperative blood loss
Optimization
Consultation with hematologist for appropriate factor management
Schedule OR early in the week & early in the day (ensure all lab/blood bank/consultant resources available)
Assay factor levels 48h prior to OR & restore levels to 40% of normal prior to surgery, as dictated by the surgical procedure (see tables below). Key trough factor VIII levels:
Obstetric > 50%
Minor surgery > 30%
Major surgery > 50%
Repeat factor assay after initial administration to confirm factor activity (within 2 hours of expected OR start)
Continue to monitor factor activity level intra-operatively as dictated by clinical situation
Avoid all anti-platelet medications
Consider antifibrinolytics during perioperative period (up to 3-5 days)
Management
DDAVP 0.3 mcg/kg (provides 3-5 fold increase in activity); only if known responder:
Maximum effect 30 min post dose but levels remain elevated for 6-8 hours
DDAVP works by stimulating the release of vWF from endothelial cells
Factor VIII-vWF concentrates (Humate P)
Platelet concentrates (contains vWF)
Recombinant factor VIII
Recombinant factor VIIa
Emergency: cryoprecipitate (contains vWF, FVIII, FXIII, fibronectin, fibrinogen)
Pregnancy
Establish baseline factor VIII, vWF, ristocetin cofactor early in pregnancy & at 34 weeks
During pregnancy, prophylactic treatment if factor VIII level < 25% (typically levels increase with pregnancy)
Know the patient’s type & factor VIII level:
Responder: at labour onset, DDAVP 0.3mcg/kg IV q12h
Non-responder: Humate P or cryoprecipitate
Labour & delivery/cesarean section: maintain levels > 50% of normal
Post-partum: follow levels & treat if < 25% or significant hemorrhage
Regional: epidural safe if factor VIII & vWF > 50%
Treatment Choices Based on Sub-types
Recommended Dosages of Factor VIII for Patients with Severely Reduced (<10%) Factor Levels
