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Perth Haematology :: Dr Steven Ward

Blood Products :: Intravenous Immunoglobulin (IvIg)

 

 

Intravenous Immunoglobulin (IVIg) : Criteria for use

National Blood Authority December 2007

                                                                                                                                                                                                        ARCBS Request Forms:

There are three categories:   Established therapeutic role for ivIg - almost always approved                                      Haematological Indications

                                                    Emerging therapeutic role - may be approved                                                                   Immunological Indications

                                                    Exceptional circumstances only - usually not indicated                                                    Neurological Indications

 

Established therapeutic role for IVIg

 

Emerging therapeutic role for IVIg

Acquired hypogammaglobulinaemia 2y to Haem malignancy

NHL, CLL, MM, other B-malig with:

1. Recurrent or severe bactrtial infection and hypogammaglob OR

2. Hypogammaglob with IgG <4g/L with lack of IgG response to vaccine

Dose: 0.4g/kg monthly

 

Acute disseminated encephalomyelitis

1.        SDEM unresponsive to steroid (or C/I)

2.        Recurrent, multiphasic ADEM unresponsive to steroid or C/I

2g/kg in 2-5 divided doses

maintenace for mutliphasic 0.4-2g/Kg 4-6 weekly

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

1.        CIDP (neurologist Dx) AND

2.        Signif functional impairment of ADLs

2g/kg monthly for 3-6 months;

Miantenance 0.4-1g/kg 2-6 weekly

 

ANCA-positive necrotising vasculitis

MPO or PR3 ANCA +ve necrotising vasculitis with:

1.        Current (<6m) standard cytotoxic immunosuppresive Rx AND

2.        Persistent active disease

Guillain-Barre Syndrome

GBS with signif disability and dis progression

2g/kg in 2-5 divided doses. 2nd dose if relapse

 

Autoimmune haemolytic anaemia

1.        symtomatic or severe AIHA (Hb<60g/L) refractory to steroids OR

2.        as a termporising measure prior to splenectomy OR

3.        As initial and maintenance Rx in AIHA in pts unsuitable for splenectomy or simmunosupression

1.5g/kg.month for 3m ± maintenance

Inflammatory myopathies

PM, DM unresponsive to steroid and immunosupn

Inclusion Body myositis (IBM) with dysphagia

Rapidly progressive IBM

2g/kg in 2-5 divided dioses for 3-6m. Maint 0.4-1g/kg 4-6 weekly

Adult ITP

1.        Refractory ITP failed steroid; of splenectomy failed or C/I and where immunospun/daposen unsuccessful

2.        ITP with life-threaenting haemorrhage when steroids failed

3.        ITP in pregnancy <30; to keep plats >30 thru pregnancy every 3-4 weeks; impending delivery to achieve plats > 80

4.        Specific circumstances: planned surgery; Severe ITP; chronic refractory ITP

5.        HIC assoc ITP with faiolure of antiviral Rx or life threatening bleeding

6.        Initrial Rx: 1-2g/kg

Ongoing Rx: 1-2g/kg 4-6 weekly

 

Bullous Pemphigoid

Moderate to severe disease AND:

1.        C/I to steroid or immunosuppression OR

2.        Unresponsive to steroids and immunosupn OR

3.        Severe SE to Rx

2g/kg monthly

Kawasaki Disease

2g/kg single dose 10-12hr; single dose

Cicatricial Pemphigoid

As above

Lambert-Eaton Myasthenic Syndrome

Neurologist Ax and severely affected non-paraneoplastic LEMS where other Rx failed

(eg 3,4-daminopyridine)

2g/kg in 2-5 divided doses for 3-6m.

Maintenance 0.4-1g/kg 2-6 weekly

Evan’s Syndrome (AIHA and ITP)

1.        Refractory to steroids OR

2.        Steroids C/I

3.        Temporising measure prior to splenectomy

2g/kg in divided doses

Multifocal motor neuropathy

1st line Rx for persistent conduction block.

2g/kg in 2-5 divided doses; maintenance 0.4-2g/kg 2-6 weekly use for 3-6 months

 

Feto-Maternal/Neonatal Alloimmune Thrombocytopenia (NAIT)

Clinical suspicion of NAIT based on clina dnlab features:

1.        thrombocytopenia or spont haem in fetus OR

2.        thrombocytopenia in neonate

3.        unexplained fetal death and specific maternal plat-specific alloAbs (eg HPA1a or 5b)

maternal dose: 1g/kg weekly thru pregnancy

Neonate: 1g/kg  single dose

Myasthenia Gravis

Dx and Ax by Neurologist AND

1.        as alternative to plasma exchange in acute exacerbation or prior to surgery OR

as maintenance Rx for mod-severe MG where other Rx ineffective

Neonatal Haemochromatosis

Indic: women with prev perg affected; 1g/kg weekly from wk 18 to delivery

 

Haemophagocytic Syndrome

BM or tissue Dx;

2g/kg

Primary Immunodeficiency Diseases

Eg CVID; SCID; Transient hypogammaglob of infancy; Wiskott-Aldrich; X-Linked agammaglob with Ab production failure

Maintenance 0.4g/kg every month

 

High-risk Allogeneic BMT

Precvention of GVHD

0.4g/kg weekly to d100

Stiff Person Syndrome

2g/kg; maint 1-2g/kg 4-6 weekly

 

IgM paraproteinaemic neuropathy

Dx by neurologyist with:

1.        functional impariemnt of ADLs AND
other therapy has failed or C/I

2g/kg inj 2-5 divided doses for 3-6 months

 

 

ITP in Children

ITP with plat <30 and signif bleeding

Acute ITP up to 2g/kg as 2 doses; others 0.5g/kg single dose

 

 

Kidney transplant

Pre-Transplant: Ab prevents transplant (eg HLA or blood group Abs)

Post-transplant: biopsy proven cellular rejection unresponsive to steroid with graft dysfunction OR acute Ab-mediated rejection OR treatment/prophylaxis for rejection where immunospn C/I

IVIg with plasma exchange: 0.1 – 0.5g/kg post-exchange

IVIG alone: 2g/kg 2-4 weekly

 

 

Multiple Sclerosis

Short term Rx with relapsing-remiting MS with

1.        pregnancy and immediate port-partum pd

2.        young pts with severe relapsing-reminting dis with failed other Rx

3.        severe relpase with no response to HD methylpred

4.        1-2g/kg in 2-5 divided doses; maint: 0.4-1g/kg 4-6 weekly

 

 

Opsoclonal Myoclonus Ataxia

Dx OMA by neurologist

1.        In children OR

2.        As 2nd line Rx following ACTH or steroids

1-2g/kg  in 2-5 divided doses; Main 0.4-1g/kg 4-6 weekly

 

 

Phemphigus Foliaceus

As other pemphigus

 

 

Phemphigus Vulgaris

As other pemphigus

 

 

Post-Transfusion Purpura

PTP with thrombocytopenia and life-threatenign bleeding

1g/kg

 

 

Secondary Hypogammaglobulinaemia

Hypogammaglob 2y to underlying dis or Rx with all of:

1.        IgG < LLN on 2 occasions AND

2.        Underlying cause cannot be reversed

3.        At least 1 of; invasive or life threatening bacterial infection in last yr; clinically active bronchiectasis

Maint: 0.4g/kg every month

 

 

Specific Ab deficiency

“Functional” Ab deficiency.

Approval by Clin Immuno AND

Freq bact infections despite 3m of Ab AND

Documented failure of serum Ab response to pneumoocacal or protein vaccine Ag

Maint: 0.4g/kg every month

 

 

Toxic epidermal necrolysis / Steven Johnson Syndrome

Ten with all of:

1.        Dx by dermatologist AND

2.        >-10% surface affected AND

3.        rapid evoluition

2g/kg single dose

 

 

Toxic Shock Syndrome

Dx of strep or staph TSS AND failure to achieve rapid improvement with resus

2g/kg single dose

 

Exceptional circumstances only

Acute leukaemia in children

HIV in children

Autoimmune congential heart block

PANDAS (paediatric AI neuropyschiatric disorder)

Autoimmune diabetic neuropathy

Paraneoplastic Syndromes

Autoimmune Neutropenia

Potassium chanbel Ab-Associated encephalopathy

Autoimmune Uveitis

Pure Red Cell Aplasia

Catastrophic Antiphospholipid Syndrome (CAPS)

Pure White Cell Aplasia

Coagulation Factor Inhibitors

Scleromyxedema

Devic Disease (neuromyelitis optica)

Sepsis - neonatal

Epidermolysis Bullosa Acquisita

Sjogren’s Syndrome

Epilespy

Solid Organ Transplant

Graves Ophthalmopathy

Susac Syndrome – micro-angiopathic disorder

HDN