2. Guillain Barre Syndrome
CIDP
Myasthenia Gravis
Demyelinating Neuropathy
Hemolytic Disease of fetus and New Born
Drug Poisonings Acute Hepatic Failure
Thrombotic Thrombocytopenic Purpura Possibility of
Good Pasture Syndrome Organ Transplant
Other RPGN Across ABO Barriers ?
Hypercholesterolemia Rheumatoid Arthritis Cryoglobulinemia
List is Long and evolving……..………….
Acute Crisis
Refractory Case
3. Evolution –Changing Roles
BLOOD BANKS
have evolved from Blood collection centres to full-fledged fully automated
departments of
Transfusion Medicine and Immunohematology
performing multiple specialised roles - including active patient management with
various cellular therapies, donor apheresis and patient therapeutic apheresis
including plasma exchange .
Glass bottles
Plastic Bags
Anticoagulant Additives
Whole Blood
Blood Components
Antigens-Antibodies-Immunohematology
4. APHERESIS
Word of Greek Derivation meaning
‘’SEPARATION Or TAKING AWAY”
An extracorporeal medical treatment
Blood of a donor/ patient is withdrawn from him , separated ex-vivo into some /all of its
components - Required component to be collected/removed is taken away and the
remainder is returned to the patients circulation
5. APHERESIS - 2 TYPES
1 DONOR APHERESIS :
Generates Apheresis components for the patients for
numerous indications
Includes
Platelets- Plateletpheresis – Specify Indications
Plasma- Plasmapheresis
Red Blood Cells(RBCs)-Erythrocytapheresis
Granulocytes - Granulocytapheresis
Hematopoietic Progenitor Cells(HPCs) - Stem cell apheresis
Multicomponent apheresis :
RBCs plus Plasma
2-units RBCs
Platelets & RBCs
Platelets & Plasma
Advantage
Reduced Donor Exposure
Standardized component
Good yield
Better dose
Safer for donor as well as
patients
6. 2.Therapeutic Apheresis
All types- treat diseases by removing substances from the blood causing severe
symptoms of disease.
What can be removed :
Injurious and Noxious
Large Molecular weight
substances :
antibodies-auto and
allo,
antigen-antibody
complexes,
toxins ,
protein bound drugs,
myeloma light chains,
endotoxins,
cryoglobulins ,
lipids such as
cholesterol & TGs & few
poisons in the plasma.
THERAPEUTIC PLASMA EXCHANGE (TPE ) : Removal of the liquid portion of blood
to remove harmful substances and replacement with a replacement solution.
Term plasmapheresis is popularly used for TPE, Plasmapheresis involves no
replacement.
THERAPEUTIC RED CELL EXCHANGE ---- Involves RBC exchange
LDL APHERESIS - Removal of low density lipoprotein in patients with familial
hypercholesterolemia.
THERAPEUTIC CYTAPHERESIS :
Photopheresis - Collection of circulating Mononuclear cells ,exposing them to
7. Photo activating 8-Methoxypsoralen,and then
exposure to Ultraviolet A light. (To treat graft-
versus-host disease, cutaneous T-cell lymphoma
and rejection in heart transplantation.)
Leukocytapheresis - Removal of malignant white
blood cells (in patients with leukemia )esp when
very high white blood cell counts are causing
symptoms.
Thrombocytapheresis - Removal of platelets in
cases with symptoms from extreme elevation in
platelets (as in cases with myeloproliferative
disorders, essential
thrombocythemia or polycythemia vera)
IMMUNOADSORPTION(IAS):
A blood purification
technique that enables the
selective removal of
Immunoglobulins from separated
plasma through high-affinity
adsorbers.
8. IMMUNOADSORPTION(IAS)Conti.
esp. effective in Autoimmune diseases were TPE failed to be effective.
Lack of any Controlled Trials for Immunoadsorption.
Devices available in market :
Non selective adsorbers : Selesorb: Dextran sulphate column
Semi-selective: Prosorba ,Immunosorba:with Staphylococcal protein A-agarose column
Selective:Therasorb :with antihuman Ig Adsober column
Indian Experience:
Evaflux Columns 2A: Pretransplant and Post Transplant
Evaflux column 5A: LDL Apheresis.
9. Patient Parameters to be
monitored :
1. Metabolic Functions
2.Acid-Base Balance
3.Respiratory Function(esp
intubated patients with FFP
exchange)
4.Cardiocirculatory
function(removal of drugs during
the procedure)Heart rate,Rhythmn
,BP monitored
5. signs of Transfusion Reactions
6. signs of circulatory overload
Reference source: To evaluate the appropriateness of
therapeutic apheresis:
Guidelines of the American Society for Apheresis(ASFA):
Drawn up in 2007 and revised and updated from time to
time(every 3 years)
Using the criteria of Evidence based medicine .
Clinical Trials continue to clarify the appropriate use of
Therapeutic Plasmapheresis(TPE) in various diseases.
Especially Important is Evaluation of scientific literature to the
clinical use of therapeutic apheresis to the pathologies in
category III
which in particular clinical situations can have a strong grade of
recommendation for TPE as provided by series of clinical case
results as well as summary fact sheets.
10. Rational for Use of TPE(Few Examples)
GULLIAN BARRE SYNDROME Evidence suggests : Improvement in
Clincal Grade
Shortening of Recovery Time
Documented Imp.in mildly affected
pat.as well
Circulating Myelin Antibodies
Chronic Inflammatory Demyelinating
Polyneuropathy (CIDP)
Evidence suggests:
TPE especially useful in patients not
responding to or not tolerating
steroids.
Comparable to IVIG
Removal of antibodies to myelin
protein antigen
Peripheral Neuropathy & Monoclonal
Gammopathy
Commonly done in patients with IgM
paraproteins.
Myelin associated monoclonal
glycoprotein
11. Myasthenia Gravis Esp useful for rapid symptomatic
improvementesp those with
breathing, swallowing ,walking
compromise.
Useful for those intolerant or
unresponsive to other therapies.
Useful for in patients who are
negative for antibody .
Useful to optimize muscle
function before thymectomy or
other surgery.
Comparison with IVIG: Median
response time significantly
shorter with TPE(p=0.14)
Antibody to Acetylcholine
receptor ,or antibody to muscle
specific kinase on the motor end
plate of muscle cells
Hyperviscosity Syndrome 1)Serum Viscosity > 3 centipoise
greater than water esp with
neurological signs
2) Cellular causes of
Hyperviscosity(Hyperleucocytosis;
Thrombocytosis)
IgM paraproteins(WM) ; Multiple
Myeloma
Single session reduces plasma
viscosity by 20-30%
Rapid recovery of Renal Functions
12. Hemolytic Disease of Foetus and New
Born
1.To slow foetal hemolysis
2.Esp useful when IUT is not feasible
before 18 weeks of gestation
Sensitized D Neg mothers carrying D
positive foetus.
Allogenic Solid Organ Transplant
across ABO barrier
Pretransplant TPE of recipient-To
avoid delayed engraftment
Post Transplant – To correct
engraftment and prevent rejection
removes Isoagglutinins
Allogenic Hematopoietic Stem Cell Tx
across major ABO barrier
PreTransplantTPE in Recipient-
Hemolytic Transfusion Reaction can
be avoided
Post TransplantT PE in recipient- For
Cell Engraftment delay correction
Thrombotic Thrombocytopenic
Purpura
Urgent daily TPE improves survival
drastically
Deficiency of metalloproteinase
ADAMTS13 or Presence of
autoantibody inhibitor of the enzyme
ADAMTS13
Acute Fulminating Hepatitis Rapid removal of aromatic
aminoacids,ammoniac
endotoxins,mercaptans,activated
coagulation factors,FDPs,TPA,phenols.
13. Idiopathic Thrombocytopenic
Purpura(ITP)
An option for chronic ITP refractory to
more standard therapies
Protein A silica column
Immunoadsorption
Pure Red Cell Aplasia & Aplastic
Anemia
Not a primary therapy-Can be offered
to patients who failed to improve
from conventional tt ,esp those found
to have serum inhibitory factors
HELLP Syndrome Patients unresponsive to
steroids,supportive therapy like anti
hypertensives,fluids,blood
&Components,antibiotics
Early initiation is life saving
Antiphospholipid Syndrome Urgently used to remove pathogenic
autoantibodies and procoagulant
factors
Antiphospholipid antibodies,like
Anticardiolipin Antibody,Anti
Beta2group1antibody,Lupus
anticoagulant
Poisoning by Heavy Metals,Amanita
Phalloides,herbal products
Drug should bind strongly to plasma
proteins
Removal of drug
14. Plasma Exchange-ASFA
Urgent Plasma Exchange – Emergency
• Thrombotic Thrombocytopenic
Purpura
• Catastrophic Antiphospholipid
Syndrome
• Acute pancreatitis c Triglyceridemia
• Intoxication by drugs or poisons
• Hyperviscosity syndromes
• Acute fulminating hepatitis
• Acute Inflammatory Demyelinating
Polyneuropathy
• Myasthenia Gravis
ASFA Indication Categories
Category I: Standard first line
Therapy
Category II: Second -line therapy
Category III: Uncertainty of effects of
tt due to inadequate data
CategoryIV: Negative data from
controlled trails or ancedotal reports
Available Machines:
Amicus ; ALYX;COM-TEC;Spectra;Optia;MCS+ etc..
Intensity of Treatment
Aggressive A: Daily treatment : 3 to Indefinite
Routinue R: 3times a week:5-7tts
Prolonged P: 1-2 times/week: 3-8 weeks
Chronic C: Every 1-4weeks: Indefinite
15. Comparing TPE and IVIG :
1.Slow onset of action
2.Malaise associated with high-dose IVIG
3.Risk of IVIG associated aseptic meningitis
4.Risk of acute kidney injury
5.Risk of anaphylaxis and stroke
Trends:
In UK and Canada: IVIG is being conserved for
indications were there is no alternate
treatment option available ,eg
Immunodeficiency state.
In USA,use of IVIG discouraged for economic
reasons when TPE can be an equally good
alternative
Our Experience at SCH:
Guillain Barre Syndrome
Wegners Granulomatosis(cANCA pos)
Myasthenia Gravis
Anti GBM (24 procedures) disease
CIDP
SLE
Adverse Effects:
Continuous Observation ,Proper Monitoring of patients Required
Throughout
Rarely serious complications in ICU patients (2.16% procedures)
Ref: Complications;Anaesthiology Intensive Therapy 2013,vol45,no1,7-13.
Common AE:
Arterial BP fall
Arrythmias
Anxiety/Agitation
Sensation of cold/paresthesias
Allergic Reactions
Lowe Limb pain
Fever
Abdominal pain
