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M E D I C I N E - A
PLASMAPHERESIS
INDICATIONS
• The basic premise of therapeutic plasma exchange
(TPE) is that removal of certain pathologic substances
from the plasma will reduce further damage and may
permit reversal of the pathologic process .
• The pathologic substance may be an autoantibody,
immune complex, cryoglobulin, myeloma light chains,
endotoxin, cholesterol-containing lipoprotein etc.
ASFA THERAPEUTIC CATEGORIES
• Category I – Category I includes disorders for which
apheresis is accepted as first-line therapy, either as
primary stand-alone treatment or in conjunction with
other modes of treatment. Examples include TPE in
Guillain-Barré syndrome or acquired autoimmune
thrombotic thrombocytopenic purpura (TTP), and
erythrocytapheresis in sickle cell diseases with certain
complications (eg, stroke)
• Category II – Category II includes disorders for which
apheresis is accepted as second-line therapy, either as
a stand-alone treatment or in conjunction with other
modes of treatment. Examples include TPE for life-
threatening hemolytic anemia for cold agglutinin disease
or Lambert-Eaton myasthenic syndrome
• Category III – Category III includes disorders for which
the optimum role of apheresis therapy is not
established. Decision making should be individualized.
Examples include TPE for hypertriglyceridemic
pancreatitis or extracorporeal photopheresis for
nephrogenic systemic fibrosis
• Category IV – Category IV includes disorders for which
published evidence demonstrates or suggests apheresis
to be ineffective or harmful.Examples include TPE for
active rheumatoid arthritis.
MODALITY: THERAPEUTIC PLASMA
EXCHANGE; CATEGORY: I
Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome)
•ANCA-associated rapidly progressive glomerulonephritis (granulomatosis with polyangiitis [Wegener's])
Dialysis dependence - or -
•Diffuse alveolar hemorrhage
•Anti-glomerular basement membrane disease (Goodpasture's syndrome)
Dialysis independence - or -
•Diffuse alveolar hemorrhage
Chronic inflammatory demyelinating polyradiculoneuropathy
•Cryoglobulinemia
Severe/symptomatic
•Focal segmental glomerulosclerosis
Recurrent in transplanted kidney
•Hemolytic-uremic syndrome
Atypical HUS due to autoantibody to factor H
•Hyperviscosity in monoclonal gammopathies
Prophylaxis for rituximab
•Treatment of symptoms
•Myasthenia gravis
Moderate-severe
•Pre-thymectomy
PANDAS (exacerbation)
•Paraproteinemic polyneuropathies
IgG/IgA
•IgM
•Renal transplantation, ABO compatible
Antibody-mediated rejection
•Desensitization, living donor, positive crossmatch due to donor-specific HLA antibody
•Renal transplantation, ABO incompatible
Desensitization, living donor
Sydenham's chorea
•Thrombotic microangiopathy, drug-associated
Ticlopidine
Thrombotic thrombocytopenic purpura
Wilson disease, fulminant
MODALITY: THERAPEUTIC PLASMA
EXCHANGE; CATEGORY: II
Acute disseminated encephalomyelitis
Antiphospholipid syndrome, catastrophic
•Autoimmune hemolytic anemia
Cold agglutinin disease (life-threatening)
•Familial hypercholesterolemia
Homozygotes with small blood volume
•Hematopoietic cell transplantation, major ABO incompatibility (recipient has anti-A or anti-B antibodies)
Bone marrow stem cells
•Peripheral blood stem cells
•Hemolytic-uremic syndrome
Atypical HUS due to complement factor gene mutations
Lambert-Eaton myasthenic syndrome
•Multiple sclerosis
Acute CNS inflammatory demyelinating disease
Myeloma cast nephropathy
Neuromyelitis optica (Devic's syndrome), acute
•Overdose, venoms, and poisoning
Mushroom poisoning
Phytanic acid storage disease (Refsum's disease)
•Renal transplantation, ABO incompatible
Humoral rejection
Systemic lupus erythematosus, severe (eg, cerebritis, diffuse alveolar hemorrhage)
Voltage gated potassium channel antibodies
MODALITY: THERAPEUTIC PLASMA
EXCHANGE; CATEGORY: III
•Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome)
Post-intravenous immune globulin
•ANCA-associated rapidly progressive glomerulonephritis (granulomatosis with polyangiitis [Wegener's])
Dialysis independence
•Anti-glomerular basement membrane disease (Goodpasture's syndrome)
Dialysis dependence and no diffuse alveolar hemorrhage
Aplastic anemia
•Autoimmune hemolytic anemia
Warm autoimmune hemolytic anemia
Burn shock resuscitation
•Cardiac transplantation
Desensitization, positive crossmatch due to donor-specific HLA antibody
•Treatment of antibody-mediated rejection
Chronic focal encephalites (Rasmussen's encephalites)
Coagulation factor inhibitor, autoantibody
•Dilated cardiomyopathy, idiopathic
New York Heart Association functional class II-IV
•Hemolytic-uremic syndrome
S. pneumoniae-associated
•Henoch-Schönlein purpura
Crescentic - or -
•Severe extrarenal disease
•Heparin-induced thrombocytopenia
Pre-cardiopulmonary bypass - or -
•With thrombosis
Hypertriglyceridemic pancreatitis
Immune complex rapidly progressive glomerulonephritis
•Immunoglobin A nephropathy
Crescentic
•Chronic progressive
Liver failure, acute
•Liver transplantation, ABO incompatible
Desensitization, deceased donor
•Humoral rejection
•Lung transplantation
Antibody-mediated rejection
•Multiple sclerosis
Chronic progressive
Nephrogenic systemic fibrosis
Neuromyelitis optica (Devic's syndrome), maintenance
•Overdose, venoms, and poisoning
Envenomation
•Natalizumab-associated progressive multifocal leukoencephalopathy
Paraneoplastic neurologic syndromes
•Paraproteinemic polyneuropathies
Multiple myeloma
Pemphigus vulgaris, severe
Post-transfusion purpura
Pure red cell aplasia
Red cell alloimmunization in pregnancy, prior to intrauterine transfusion availability
•Renal transplantation, ABO compatible
Desensitization, high panel reactive antibody, deceased donor
Scleroderma (progressive systemic sclerosis)
Sensorineural hearing loss, sudden
Sepsis with multiorgan failure
Stiff-person syndrome
•Thrombotic microangiopathy, drug-associated
Clopidogrel
•Cyclosporine/tacrolimus
Thrombotic microangiopathy, hematopoietic stem cell transplant-associated, refractory
Thyroid storm
Toxic epidermal necrolysis, refractory
MODALITY: THERAPEUTIC PLASMA
EXCHANGE; CATEGORY IV
Amyloidosis, systemic
Amyotrophic lateral sclerosis
Coagulation factor inhibitors, alloantibody
Dermatomyositis, polymyositis
•Hemolytic-uremic syndrome
Atypical HUS due to membrane cofactor protein (CD64) mutations
•Shiga toxin-associated
Immune thrombocytopenia, refractory
Inclusion body myositis
POEMS syndrome
Psoriasis
•Renal transplantation, ABO incompatible
Group A2/A2B into B, deceased donor
Schizophrenia
•Systemic lupus erythematosus
Nephritis
•Thrombotic microangiography, drug associated
Gemcitabine
•Quinine
THANK YOU

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Plasmapheresis

  • 1. M E D I C I N E - A PLASMAPHERESIS
  • 2. INDICATIONS • The basic premise of therapeutic plasma exchange (TPE) is that removal of certain pathologic substances from the plasma will reduce further damage and may permit reversal of the pathologic process . • The pathologic substance may be an autoantibody, immune complex, cryoglobulin, myeloma light chains, endotoxin, cholesterol-containing lipoprotein etc.
  • 3.
  • 4. ASFA THERAPEUTIC CATEGORIES • Category I – Category I includes disorders for which apheresis is accepted as first-line therapy, either as primary stand-alone treatment or in conjunction with other modes of treatment. Examples include TPE in Guillain-Barré syndrome or acquired autoimmune thrombotic thrombocytopenic purpura (TTP), and erythrocytapheresis in sickle cell diseases with certain complications (eg, stroke)
  • 5. • Category II – Category II includes disorders for which apheresis is accepted as second-line therapy, either as a stand-alone treatment or in conjunction with other modes of treatment. Examples include TPE for life- threatening hemolytic anemia for cold agglutinin disease or Lambert-Eaton myasthenic syndrome
  • 6. • Category III – Category III includes disorders for which the optimum role of apheresis therapy is not established. Decision making should be individualized. Examples include TPE for hypertriglyceridemic pancreatitis or extracorporeal photopheresis for nephrogenic systemic fibrosis
  • 7. • Category IV – Category IV includes disorders for which published evidence demonstrates or suggests apheresis to be ineffective or harmful.Examples include TPE for active rheumatoid arthritis.
  • 8. MODALITY: THERAPEUTIC PLASMA EXCHANGE; CATEGORY: I Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome) •ANCA-associated rapidly progressive glomerulonephritis (granulomatosis with polyangiitis [Wegener's]) Dialysis dependence - or - •Diffuse alveolar hemorrhage •Anti-glomerular basement membrane disease (Goodpasture's syndrome) Dialysis independence - or - •Diffuse alveolar hemorrhage Chronic inflammatory demyelinating polyradiculoneuropathy
  • 9. •Cryoglobulinemia Severe/symptomatic •Focal segmental glomerulosclerosis Recurrent in transplanted kidney •Hemolytic-uremic syndrome Atypical HUS due to autoantibody to factor H •Hyperviscosity in monoclonal gammopathies Prophylaxis for rituximab •Treatment of symptoms
  • 10. •Myasthenia gravis Moderate-severe •Pre-thymectomy PANDAS (exacerbation) •Paraproteinemic polyneuropathies IgG/IgA •IgM •Renal transplantation, ABO compatible Antibody-mediated rejection •Desensitization, living donor, positive crossmatch due to donor-specific HLA antibody
  • 11. •Renal transplantation, ABO incompatible Desensitization, living donor Sydenham's chorea •Thrombotic microangiopathy, drug-associated Ticlopidine Thrombotic thrombocytopenic purpura Wilson disease, fulminant
  • 12. MODALITY: THERAPEUTIC PLASMA EXCHANGE; CATEGORY: II Acute disseminated encephalomyelitis Antiphospholipid syndrome, catastrophic •Autoimmune hemolytic anemia Cold agglutinin disease (life-threatening) •Familial hypercholesterolemia Homozygotes with small blood volume •Hematopoietic cell transplantation, major ABO incompatibility (recipient has anti-A or anti-B antibodies) Bone marrow stem cells •Peripheral blood stem cells
  • 13. •Hemolytic-uremic syndrome Atypical HUS due to complement factor gene mutations Lambert-Eaton myasthenic syndrome •Multiple sclerosis Acute CNS inflammatory demyelinating disease Myeloma cast nephropathy Neuromyelitis optica (Devic's syndrome), acute
  • 14. •Overdose, venoms, and poisoning Mushroom poisoning Phytanic acid storage disease (Refsum's disease) •Renal transplantation, ABO incompatible Humoral rejection Systemic lupus erythematosus, severe (eg, cerebritis, diffuse alveolar hemorrhage) Voltage gated potassium channel antibodies
  • 15. MODALITY: THERAPEUTIC PLASMA EXCHANGE; CATEGORY: III •Acute inflammatory demyelinating polyneuropathy (Guillain-Barré syndrome) Post-intravenous immune globulin •ANCA-associated rapidly progressive glomerulonephritis (granulomatosis with polyangiitis [Wegener's]) Dialysis independence •Anti-glomerular basement membrane disease (Goodpasture's syndrome) Dialysis dependence and no diffuse alveolar hemorrhage Aplastic anemia •Autoimmune hemolytic anemia Warm autoimmune hemolytic anemia
  • 16. Burn shock resuscitation •Cardiac transplantation Desensitization, positive crossmatch due to donor-specific HLA antibody •Treatment of antibody-mediated rejection Chronic focal encephalites (Rasmussen's encephalites) Coagulation factor inhibitor, autoantibody •Dilated cardiomyopathy, idiopathic New York Heart Association functional class II-IV •Hemolytic-uremic syndrome S. pneumoniae-associated
  • 17. •Henoch-Schönlein purpura Crescentic - or - •Severe extrarenal disease •Heparin-induced thrombocytopenia Pre-cardiopulmonary bypass - or - •With thrombosis Hypertriglyceridemic pancreatitis Immune complex rapidly progressive glomerulonephritis •Immunoglobin A nephropathy Crescentic •Chronic progressive Liver failure, acute
  • 18. •Liver transplantation, ABO incompatible Desensitization, deceased donor •Humoral rejection •Lung transplantation Antibody-mediated rejection •Multiple sclerosis Chronic progressive Nephrogenic systemic fibrosis Neuromyelitis optica (Devic's syndrome), maintenance
  • 19. •Overdose, venoms, and poisoning Envenomation •Natalizumab-associated progressive multifocal leukoencephalopathy Paraneoplastic neurologic syndromes •Paraproteinemic polyneuropathies Multiple myeloma Pemphigus vulgaris, severe Post-transfusion purpura Pure red cell aplasia Red cell alloimmunization in pregnancy, prior to intrauterine transfusion availability
  • 20. •Renal transplantation, ABO compatible Desensitization, high panel reactive antibody, deceased donor Scleroderma (progressive systemic sclerosis) Sensorineural hearing loss, sudden Sepsis with multiorgan failure Stiff-person syndrome •Thrombotic microangiopathy, drug-associated Clopidogrel •Cyclosporine/tacrolimus
  • 21. Thrombotic microangiopathy, hematopoietic stem cell transplant-associated, refractory Thyroid storm Toxic epidermal necrolysis, refractory
  • 22. MODALITY: THERAPEUTIC PLASMA EXCHANGE; CATEGORY IV Amyloidosis, systemic Amyotrophic lateral sclerosis Coagulation factor inhibitors, alloantibody Dermatomyositis, polymyositis •Hemolytic-uremic syndrome Atypical HUS due to membrane cofactor protein (CD64) mutations •Shiga toxin-associated Immune thrombocytopenia, refractory
  • 23. Inclusion body myositis POEMS syndrome Psoriasis •Renal transplantation, ABO incompatible Group A2/A2B into B, deceased donor Schizophrenia •Systemic lupus erythematosus Nephritis •Thrombotic microangiography, drug associated Gemcitabine •Quinine