hematologic emergencies
DESCRIPTION
HEMATOLOGIC EMERGENCIES. Jason Mitchell Michael Szava-Kovats Joe Vipond May 17, 2012. HEMOSTASIS. HEMOSTASIS. HEMOSTASIS. HEMOSTASIS. HEMOSTASIS. PTT. PT/INR. CASE. 30 yo M Tooth extraction Consistent oozing Stable DDx? Investigations?. CASE. 63. 135. 140. 111. 130. 7. - PowerPoint PPT PresentationTRANSCRIPT
HEMATOLOGIC EMERGENCIES
Jason MitchellMichael Szava-Kovats
Joe VipondMay 17, 2012
HEMOSTASIS
HEMOSTASIS
HEMOSTASIS
HEMOSTASIS
HEMOSTASIS
PTT PT/INR
CASE
30 yo M Tooth extraction Consistent oozing Stable
DDx? Investigations?
CASE
135
7 130
140
111
4.0
24
63
PTT: 54
INR: 0.9
von WILLEBRAND DISEASE
von WILLEBRAND DISEASE
THREE TYPES
Type I: Partial quantitative deficiency vWF AD, Most common (75%)
Type II: Abnormally functioning vWF AD or AR, Subtypes: IIA, IIB, IIM, IIN
Type III: Total quantitative deficiency vWF AR, Leads to severe bleeding
von WILLEBRAND DISEASE
INVESTIGATIONS
Plasma vWF:Ag Decreased Plasma vWF Activity Decreased FVIII Levels Decreased
DISEASE PTT PT/INR PLATELET #
vWD N N
von WILLEBRAND DISEASE
MANAGEMENT OPTIONSStandard measures
DDAVP
vWF Replacement
von WILLEBRAND DISEASE
von WILLEBRAND DISEASE
DESMOPRESSIN (DDAVP/OCTOSTIM)
0.3 μg/kg IV (MAX 20 μg) SC/IV over 20-30 minutes
von WILLEBRAND DISEASE
vWF REPLACEMENT
Humate P 60 – 80 Units/kg IV
von WILLEBRAND DISEASE
?r-vWF
Some promising results in animal studies
Currently in phase III human trials.
HEMOSTASIS
CASE
51 yo M Fall down 3 stairs, Stable No LOC, no neuro deficits No neck pain C/O R knee pain/swelling Hemophiliac
HEMOPHILIA
Management priorities?
HEMOPHILIA
HEMOPHILIA A
Factor VIII Deficiency
X-linked recessive
1:5-10 000 males
HEMOPHILIA B
Factor IX Deficiency
X-linked recessive
1:25-30 000 males
HEMOPHILIA
Initial Investigations?
HEMOPHILIA
105
13
115
138
101
4.1
19
76
PTT: 76
INR: 0.9
HEMOPHILIA
INVESTIGATIONS
Factor VIII Level Decreased Factor IX Level Decreased
DISEASE PTT PT/INR PLATELET #
HEMOPHILIA N N
HEMOPHILIA
MANAGEMENT Depends on:
Clotting factor activity level
Location of bleed
HEMOPHILIA
SEVERITY % FACTOR ACTIVITY
Mild > 5
Moderate 1 - 5
Severe < 1
HEMOPHILIA
BLEEDING LOCATION
LIFE/LIMB THREATENING BLEED
MODERATE/MINOR BLEED
HEMOPHILIA
LIFE/LIMB THREATENING BLEED
HEMOPHILIA
MILD/MODERATE BLEED
HEMOPHILIA
MANAGEMENT OPTIONSCryoprecipitateFFPDDAVPrFVIII / rFIXAntifibrinolytics
HEMOPHILIA
CRYOPRECIPITATE
FVIII, vWF, Fibrinogen
2 bags / 10 kg
Not first line therapy – use if rFVIII not available
FFP
All coagulation factors
Not ideal
Need to double plasma volume level
HEMOPHILIA
DESMOPRESSIN (DDAVP/OCTOSTIM)
0.3 μg/kg IV (MAX 20 μg) SC/IV over 20-30 minutes
HEMOPHILIA rFVIII (KOGENATE)
MILD/MODERATE 30 U/kg
SEVERE 50 U/kg
rFIX (BeneFIX) MILD/MODERATE
50 U/kg > 15 70 U/kg < 15
SEVERE 120 U/kg > 15 160 U/kg < 15
HEMOPHILIA
WHEN IN DOUBT, TREAT AS SEVERE BLEED!
HEMOPHILIA
ANTIFIBRINOLYTICS
Tranexamic Acid
Epsilon Aminocaproic Acid
HEMOPHILIA
PLASMINOGEN
HEMOPHILIA
ANTIFIBRINOLYTICS
25 mg/kg PO TID 1-7 days
HEMOPHILIA
THERAPEUTIC ENDPOINTS
Bleeding cessation
Symptom resolution
Correction of PTT
Raised factor activity level
BACK TO CASE
51 yo M Fell down three stairs Stable R knee hemarthrosis
HEMOPHILIA A
HEMOPHILIA
What type of bleed?
HEMOPHILIA A
HEMOPHILIA A
Management?
HEMOPHILIA
HEMOPHILIA
Why is the patient not responding?
HEMOPHILIA
INHIBITORS
Formation of anti-factor antibodies (IgG)
1/3 severe Hemophilia A; 1/50 mild/moderate Hemophelia A
1/100 Hemophelia B
Can be transient
HEMOPHILIA
INHIBITOR MANAGEMENT aPCC (FEIBA)
rFVII (NIASTASE)
90 μg/kg q2h x 3 or until bleeding stops
HEMOPHILIA A
CASE
46 yo M, Altered LOC T:38.7 RR:26 BP:76/41 O2: 92% Oozing from IV sites Hematuria from Foley LOOKS SICK
?Investigations
CASE
76
16
40
135
105
6.3
16
131
PTT: 68INR: 2.4
Schistocytes present
LIVER PANEL: Transaminitis and Hyperbilirubinemia
CASE
WORKING DIAGNOSIS?
DIC
DIC
DIC
DIC
SEPSIS ACIDOSIS
TRAUMA HYPOTHERMIA
OBSTETRICAL COMPLICATIONS BURNS
HEPATIC FAILURE AAA
TISSUE DESTRUCTION AMPHETAMINES
MALIGNANCY ENVENOMATIONS
ABO INCOMPATIBILITY HEAT STROKE
DIC
DIC
FDP Elevated Fibrinogen Decreased Factor Levels Decreased D-Dimer Elevated
DISEASE PTT PT/INR PLATELET #
DIC
DIC
MANAGEMENT
Treat cause
Supportive measures
DIC
PLATELET TRANSFUSION
No evidence if not bleeding
Consider if ongoing bleeding
If platelet count < 50 1-2 U / 10 kg / day
FFP/CRYO
No evidence
Usage similar to platelet transfusion considerations
If INR elevated and/or fibrinogen low
DIC
HEPARIN ATIII
DIC
?HEPARIN No controlled trials
Consider use if DIC predominately thrombogenic
May increase bleeding
Start with 500 U/hour, target PTT 45 sec
DIC
?ATIII
Controversial
May have benefit if heparin not also given
DIC
PROTEIN C
DIC
?PROTEIN C
REVIEW
DISEASE PTT PT/INR PLATELET #
vWD N N
HEMOPHILIA N N
DIC
CASE
81 yo M from Africa.
C/O general malaise, fever, cough x 3 days
BIBA from care facility for confusion
Immunization status unknown
O/E
T 38.7 P 130 BP 105/60 RR 20 O2 94% CNS: Confused, agitated. GCS 13 CV: N Resp: N Abdo: Soft. Multiple surgical scars. No
mass.
CASE120
17 124
138
101
4.1
18
140
U/A 6-10 WBC/hpf; 0-2 RBC/hpf.
ECG – Sinus Tach
Howell-Jolly Bodies Present
FEVER AND ASPLENIA
Splenic Function
Active phagocytosis
IgM and Complement production
T lymphocyte reservoir
Scavenger of abnormal cells (eg. spherocytes, inclusion bodies)
FEVER AND ASPLENIA
Asplenia Congenital Surgical removal Autosplenectomy
Increased risk for fulminant bacterial infection Postsplenectomy sepsis (PSS)
FEVER AND ASPLENIA
Organisms are typically encapsulated:
Streptococus pneumoniae
H. influenza
Neisseria meningitidis
FEVER AND ASPLENIA
EVALUATION: Fever must be viewed as PSS
P/E: Toxic and acutely ill
Marked tachycardia and hypotension
Altered LOC
?Primary focus of infection
FEVER AND ASPLENIA
LABS
Left shift +/- bandemia
Thrombocytopenia
DIC
Liver function abnormalities
ABG: Hypoxemia/Hypocarbia
FEVER AND ASPLENIA
MANAGEMENT Regimen:
2 g Ceftriaxone daily 1 g vanco q12h (if multiple drug resistance)
Obtain BC and UC +/- CSF
Low threshold for volume, vasopressor, ventilatory support