Evidence-Based Guidelines on the Use of Intravenous Immune Globulin for Hematologic and Neurologic...

Evidence-Based Guidelines on the Use of IntravenousImmune Globulin for Hematologic and Neurologic Conditions

Paula Robinson, David Anderson, Melissa Brouwers, Thomas E. Feasby, and Heather Hume,

on behalf of the IVIG Hematology and Neurology Expert Panels

In Canada, intravenous immunoglobulin (IVIG) use

has increased by 115% over the past 7 to 8 years.

Given this increased usage, Canadian Blood Serv-

ices and the National Advisory Committee on

Blood and Blood Products for Canada identified

the need to develop and disseminate evidence-

based guidelines to facilitate appropriate IVIG use.

As a result, guidelines for IVIG use in hematologic

Transfusion Medicine Reviews, Vol 21, No 2, Suppl 1 (April), 2007:

and neurologic conditions have been developed

and are published in this supplement of Transfu-

sion Medicine Reviews. This commentary provides

a brief description of the process used to develop

these guidelines and includes a summary of the

recommendations for IVIG use in the various

conditions evaluated.

A 2007 Published by Elsevier Inc.

Address reprint requests to Heather Hume, MD, FRCPC,

Executive Medical Director, Transfusion Medicine, Canadian

Blood Services, 1800 Alta Vista Drive, Ottawa, ON CANADA

K1G 4J5. E-mail: [email protected]

0887-7963/07/$ - see front matter

n 2007 Published by Elsevier Inc.

doi:10.1016/j.tmrv.2007.01.004

C ANADA IS ONE of the world’s highest per

capita users of intravenous immune globulin

(IVIG), with its use increasing by 115% between

1997–1998 and 2005–2006. This expensive frac-

tionated blood product consists of concentrated

immunoglobulin, primarily IgG, derived from hu-

man plasma in pools of 3000 to more than 10000

donors. With a single infusion of 1 gm/kg for a

70-kg adult costing approximately $4000, IVIG

now accounts for more than one third of Canadian

Blood Services’ total budget for all fractionated and

recombinant products or approximately one sixth of

Canadian Blood Services’ entire budget.

Although Health Canada has licensed specific

IVIG preparations for the treatment of primary and

secondary immunodeficiencies, allogenic bone

marrow transplantation, chronic B-cell lymphocytic

leukemia, pediatric HIV infection, and idiopathic

thrombocytopenic purpura, IVIG is also being used

to treat a growing number of boff-labelQ indications.Much of the recent growth in IVIG use is believed to

be due to this increasing boff-labelQ usage.Given this escalating usage of IVIG, in 2004

Canadian Blood Services and the National Advisory

Committee on Blood and Blood Products (an

advisory group to the Canadian provincial and

territorial Ministries of Health and Canadian Blood

Services, composed of hospital-based transfusion

medicine experts) identified the need to develop and

disseminate evidence-based practice guidelines as a

way to facilitate appropriate use of IVIG.

Two separate panels of national experts were

convened to develop evidence-based practice

guidelines on the use of IVIG for 18 hematologic

conditions and for 22 neurologic conditions. The

panels’ mandates were to review the available

evidence regarding the use of IVIG and formulate

recommendations on IVIG use for each of the

identified conditions in each area of expertise. This

commentary provides a brief description of the

process used to develop the guidelines and a

summary of the recommendations for IVIG use

in each of the clinical conditions evaluated.

The primary sources used by the expert panels

were recent evidence-based reviews, both published

and unpublished. (For a complete set of references,

refer to each document.) Recommendations for use

of IVIG were developed based on interpretation of

the available evidence and, where evidence was

lacking, consensus of expert clinical opinion.

Interpretation of the evidence involved recognition

and discussion of factors influencing the decision-

making process. Both expert panels evaluated the

use of IVIG for a diverse range of conditions. The

level of evidence required to recommend IVIG

varied depending upon the condition. For rare

diseases that have no effective alternative treat-

ments, the threshold for recommendation of the use

of IVIG was lower than for common diseases with

established standard therapies.

The level of evidence available to inform the

recommendations for each condition was assessed

pp S3-S8 S3

Table 1. Summary of Recommendations for the Use of IVIG

in Patients With Hematologic Conditions

Hematologic conditions

Acquired hemophilia

IVIG is not recommended for routine use in the treatment of

acquired hemophilia.

Based on consensus by the expert panel, IVIG may be

considered one option among adjunctive therapies, such as

steroids, in urgent situations in this disorder.

Acquired hypogammaglobulinemia (secondary to malignancy)

Adult

IVIG is recommended for infection prophylaxis in adults with

malignant hematologic disorders associated with hypogam-

maglobulinemia or dysfunctional gammaglobulinemia and

either

(i) a recent episode of a life-threatening infection, which is

reasonably thought to be caused by low levels of polyclonal

immunoglobulins; or

(ii) recurrent episodes of clinically significant infections (eg,

pneumonia), which are reasonably thought to be caused by

low levels of polyclonal immunoglobulins.


considered one option for the treatment of acute life-

threatening infection in patients with malignant hematologic

disorders associated with hypogammaglobulinemia or, more

specifically, low levels of polyclonal immunoglobulins.

The panel also recommends that health care providers

consider the role of other contributing risks for infection

(eg, neutropenia or chemotherapy-related mucositis) in

determining the likely role of hypogammaglobulinemia in

the development of the infectious process.

Pediatric

IVIG is not recommended for routine use in children with

hematologic malignancies (with or without hypogammaglo-

bulinemia). The panel recognizes 2 possible exceptions to

this general recommendation:

(i) For children with hematologic malignancies with acquired

hypogammaglobulinemia and either a history of severe

invasive infection or recurrent sinopulmonary infections,

IVIG may be considered a treatment option according to

the above recommendations for adult patients. (ii) Some

multinational protocols for the treatment of hematologic

malignancies (and/or hematopoietic stem cell transplanta-

tion) in childhood recommend routine use of IVIG for

hypogammaglobulinemia, even in the absence of severe or

recurrent infections. These recommendations are protocol

specific and not necessarily consistent across protocols of

similar intensity. Consequently, the panel suggests that IVIG

may be administered to children registered on such clinical

trials to comply with protocol recommendations. However,

because the benefit of this approach has not been adequate-

ly studied and there is considerable uncertainty as to the

efficacy of such an approach, the panel suggests that IVIG

not be routinely used for nonregistered patients.

Acquired red cell aplasia or pure red cell aplasia (PRCA)

IVIG is not recommended as first line therapy for immunologic

PRCA.

Based on consensus by the expert panel, IVIG is a reasonable

option for patients with immunologic PRCA who have failed

other therapies (eg, prednisone or cyclosporin).

Table 1 (continued)


Acquired red cell aplasia or pure red cell aplasia (continued)

IVIG should be considered a first-line therapy for viral PRCA

associated with parvovirus B19 in immunocompromised

patients.

Acquired von Willebrand disease (AvWD)


AvWD.


considered one option among adjunctive therapies in the

treatment of AvWD in urgent situations (eg, active bleeding

or preoperatively).

Aplastic anemia

IVIG is not recommended for the treatment of aplastic anemia.

Autoimmune hemolytic anemia (AIHA)

IVIG is not recommended for routine use in either acute or

chronic treatment of AIHA.


considered among the options for treatment of severe life-

threatening AIHA.

Autoimmune neutropenia

IVIG is not recommended for routine treatment of autoim-

mune neutropenia.


considered among the treatment options in rare circum-

stances of autoimmune neutropenia when the standard of

care, G-CSF, fails.

Fetal or neonatal alloimmune thrombocytopenia (F/NAIT)

Treatment of F/NAIT before delivery

IVIG is recommended as the standard first-line antenatal

treatment of FAIT. Candidates for treatment include

(i) Pregnant women with a previously affected pregnancy.

Intravenous immunoglobulin should be initiated at a time in

the pregnancy that corresponds to a gestational age in the

present fetus that precedes by some weeks the time at

which bleeding was thought to occur in the first pregnancy.

(ii) Pregnant women with a familial history of F/NAIT or

those found on screening to have platelet alloantibodies.

Timing of IVIG treatment should be based on the severity of

fetal thrombocytopenia determined by cordocentesis. Expert

opinion suggests that treatment should be initiated around

20 wk and not later than 30 wk.

Given the complexity of this disorder and its management, it

is strongly recommended that treatment be under the

direction of a high-risk obstetrical center with specialized

expertise in the treatment of F/NAIT.

Treatment of a newborn with F/NAIT

In the opinion of the expert panel, the provision of antigen-

negative compatible platelets should be considered a first-

line therapy and IVIG adjunctive. Given the lack of evidence

to guide management in this rare disorder, it is strongly

recommended that treatment of newborns with F/NAIT be

under the direction of a center with specialized pediatric

expertise in the treatment of this condition.

Hematopoietic stem cell transplantation

IVIG is not recommended for use after hematopoietic stem cell

transplantation. The panel recognizes as a possible exception

to this recommendation that some multinational protocols for

(continued on next page)

ROBINSON ET ALS4

Table 1 (continued)


Hematopoietic stem cell transplantation (continued)

the treatment of hematologic malignancies and/or hemato-

poietic stem cell transplantation in childhood recommend

routine use of IVIG for hypogammaglobulinemia. These

recommendations are protocol specific and not necessarily

consistent across protocols of similar intensity.

Consequently, the panel suggests that IVIG may be admin-

istered to children registered on such clinical trials to comply

with protocol recommendations. However, because the

benefit of this approach has not been adequately studied

and there is considerable uncertainty as to the efficacy of

such an approach, the panel suggests that IVIG not be

routinely used for nonregistered patients.

Hemolytic disease of the newborn (HDN)

IVIG is not recommended for use in the management of HDN

without established hyperbilirubinemia.

Thepanel recommends that IVIG beoffered to patientswithHDN

as treatment for severe hyperbilirubinemia and endorses the

recommendations outlined in the American Academy of Pediat-

rics (AAP) guideline on the management of hyperbilirubinemia.

AAP recommendations (July 2004) state:

In isoimmune hemolytic disease, administration of IVIG (0.5-

1.0 g/kg over 2 h) is recommended if the total serum bilirubin

(TSB) is rising despite intensive phototherapy or the TSB

level is within 2-3 mg/dL (34-51 lmol/L) of the exchange

level. If necessary, this dose can be repeated in 12 h.

Although the AAP recommendation states that a dose of IVIG

may be given over 2 h, this may represent an infusion

administration faster than that recommended by the manu-

facturer; this needs to be taken into consideration in

treatment decisions.

Hemolytic transfusion reaction

IVIG is not recommended for either the prophylaxis or routine

treatment of hemolytic transfusion reactions.


considered as an option among supportive therapies for

urgent situations in this disorder.

Hemolytic transfusion reaction in sickle cell disease

IVIG is not recommended for the routine treatment of non–

life-threatening delayed hemolytic transfusion reactions in

patients with sickle cell disease.


considered among the options for treatment of serious, life-

threatening, delayed hemolytic transfusion reactions in

patients with sickle cell disease.

Hemolytic uremic syndrome (HUS) and thrombotic throm-

bocytopenic purpura (TTP)

IVIG is not recommended as first-line therapy for HUS or TTP

in either the pediatric or adult population.


considered one option among adjunctive therapies when

first-line therapy has failed.

Heparin-induced thrombocytopenia

IVIG is contraindicated for treatment of heparin-induced

thrombocytopenia.

HIV-associated thrombocytopenia

IVIG is recommended as a treatment option for HIV-associ-

ated thrombocytopenia when there is active bleeding or

when platelet counts are less than 10 � 109/L.

Table 1 (continued)


Idiopathic thrombocytopenic purpura (ITP) in children

Pediatric ITP

IVIG is recommended as one option for first-line therapy of

acute ITP in children with platelet counts less than 20 �109/L.

IVIG is recommended as part of a multimodality approach (ie,

in conjunction with platelet transfusions and bolus intrave-

nous methylprednisolone) for treatment of children with ITP

who have life-threatening bleeding.


considered as one option for treatment of children with

chronic ITP. Another related option (ie, a plasma derived

immunoglobulin product) for Rh-positive nonsplenectomized

children is anti-D, provided that there are no contraindica-

tions to the use of this product.

Neonates of mothers with ITP

The expert panel endorses the American Society of

Hematology (ASH) guideline recommendations, which are

bIn newborns without evidence of intracranial hemorrhage,

treatment with IVIG is appropriate if the infant’s platelet

count is b20000/lL (20 � 109/L). Newborns with platelet

counts of 20000-50000/lL (20 � 109/L to 50 � 109/L) do

not necessarily require IVIG treatment. Newborns with

counts N50000/lL (50 � 109/L) should not be treated with

IVIG or glucocorticoids.Q

Newborns with imaging evidence of intracranial hemorrhage

should be treated with combined glucocorticoid and IVIG

therapy if the platelet count is b20000/lL (20 � 109/L). In

this setting it is prudent to use glucocorticoids in combina-

tion with other treatment.

Idiopathic thrombocytopenic purpura (ITP) in adults

Adult acute ITP with bleeding

Based on consensus by the expert panel, IVIG is strongly

recommended as part of multimodality therapy for adults

with acute ITP and major or life-threatening bleeding compli-

cations and/or clinically important mucocutaneous bleeding.

Adult acute ITP with severe thrombocytopenia but no

bleeding

Based on consensus by the expert panel, IVIG is not

recommended as first-line therapy alone for acute ITP with

severe thrombocytopenia but no major bleeding or wet

purpura, except for patientswith contraindications to steroids.

Adult ITP with no or slow response to adequate dose

steroids


considered as a possible adjunctive therapy for patients not

responding or slowly responding to steroids.

Adult chronic ITP postsplenectomy


considered as a possible adjunctive therapy as a steroid-

sparing measure.

Pregnancy-associated ITP

When pregnancy-associated ITP requires treatment, as out-

lined in the ASH guidelines, IVIG is recommended as one

option for first-line therapy, as are steroids. Clinical judgment

should inform the decision.

The expert panel endorses the treatment parameters outlined

in the ASH recommendations.


IVIG CLINICAL GUIDELINES S5

Table 1 (continued)


Summary of ASH recommendations:

Platelet counts greater than 50� 109/L do not routinely need

treatment and should not receive steroids or IVIG. Platelet

counts between 30 and 50 � 109/L in first or second

trimester also should not receive treatment. Treatment is

required if platelet count is b10 � 109/L at any time in the

pregnancy or between 10 and 30 � 109/L in second or third

trimester or if there is bleeding. Pregnant women who fail

steroids and IVIG should be considered for splenectomy in the

second trimester if platelet count is less than 10 � 109/L and

there is bleeding. A platelet count of 50 � 109/L is sufficient

for vaginal delivery or cesarean section. Intravenous immu-

noglobulin may be useful if very rapid elevation of platelet

count is needed before delivery. These recommendations are

based on clinical experience and expert consensus.

Posttransfusion purpura (PTP)

IVIG is recommended as the standard first-line therapy for

PTP.

Viral-associated hemophagocytic syndrome (VAHS)


VAHS.


considered among the options for treatment of severe life-

threatening VAHS.

Table 2. Summary of Recommendations for the Use of IVIG

in Patients With Neurologic Conditions

Neurologic conditions

Acute disseminated encephalomyelitis (ADEM)

IVIG is recommended as an option for treatment of mono-

phasic ADEM when first-line therapy with high-dose cor-

ticosteroids fails or when there are contraindications to

steroid use.


considered as an option for treatment of relapsing ADEM to

eliminate steroid dependency or for those patients who fail to

respond, or have contraindications, to steroids.

Adrenoleukodystrophy

IVIG is not recommended for the treatment of adrenoleuko-

dystrophy.

Amyotrophic lateral sclerosis

IVIG is not recommended for the treatment of amyotrophic

lateral sclerosis.

Autism

IVIG is not recommended for the treatment of autism.

Chronic inflammatory demyelinating polyneuropathy

IVIG is recommended as an option for the short-term

management of new-onset chronic inflammatory demyelin-

ating polyneuropathy (CIDP) or CIDP relapses.


considered as an option in combination with other immuno-

suppressive therapy for the long-term management of CIDP.

If IVIG is to be used in the long-term management of CIDP,

the patient should be under the care of a qualified expert with

specialized knowledge of CIDP, and a systematic approach

should be taken to determine the minimal effective dose.

Critical illness polyneuropathy

IVIG is not recommended for the treatment of critical illness

polyneuropathy.

Dermatomyositis

Based on consensus by the expert panel, pathologic confir-

mation by means of a skeletal muscle biopsy is required for

the diagnosis of dermatomyositis. It is critical that the

muscle specimen be procured, processed, and interpreted

in a laboratory familiar with the correct handling of muscle

biopsy specimens and that the final interpretation be made

by an expert in neuromuscular pathology.

Based on consensus by the expert panel, use of IVIG for

the treatment of patients with dermatomyositis should be

made in consultation with an expert in neuromuscular

disease.

IVIG is not recommended asmonotherapy for dermatomyositis.

IVIG is recommended as an option, in combination with other

agents, for patients with dermatomyositis who have not

adequately responded to other immunosuppressive therapies.

IVIG is recommended, in combination with other agents, as a

steroid-sparing option for patients with dermatomyositis.

Diabetic neuropathy

IVIG is not recommended for treatment of diabetic polyneur-

opathy, mononeuropathy, or proximal lower limb neuropathy.

Based on consensus by the expert panel, IVIG use for patients

with diabetes who have evidence of a CIDP phenotype

should follow the recommendations outlined in the CIDP

section of this guideline.


ROBINSON ET ALS6

using the system developed by Bob Phillips et al

from the National Health Service (UK) Centre for

Evidence-Based Medicine. (A detailed description

of the classification system is given in the full

guidelines document.)

Using membership lists for appropriate profes-

sional organizations, we circulated drafts of the

practice guidelines to hematologists and neurologists

across Canada, and feedback was obtained via a

questionnaire using a web-based survey tool. The

results from this consultation process were then

reviewed by the expert panels, and subsequent

modifications to the guidelines were made where

appropriate.

The guidelines produced through this process

include a brief clinical description plus a summary

of the available evidence and the expert panel’s

interpretation and recommendations regarding use

of IVIG for each of the conditions reviewed.

HEMATOLOGIC CONDITIONS

For most of the hematologic conditions

reviewed by the hematology expert panel, routine

use of IVIG was not recommended. Recommen-

dations for routine use of IVIG (ie, clinical settings

in which IVIG could be considered an appropriate

therapeutic option) were made for the following

Table 2 (continued)


Guillain-Barre syndrome (GBS)

IVIG is recommended as a treatment option for GBS within

2 wk of symptom onset for

(i) Patients with symptoms of grade 3 severity (able to walk

with aid) or greater; or

(ii) Patients with symptoms less than grade 3 severity

whose symptoms are progressing.

Basedonconsensusby the expert panel, IVIGmaybeconsidered

as a treatment option for patients who initially responded

to IVIG and who are experiencing a relapse of symptoms.

Based on consensus by the expert panel, the recommenda-

tions for use of IVIG for GBS also apply to patients with Miller-

Fisher and other variants of GBS. Diagnosis of GBS variants

should be made by a specialist with expertise in this area.

Inclusion body myositis



the diagnosis of inclusion body myositis. It is critical that the

muscle specimen be procured, processed, and interpreted in

a laboratory familiar with the correct handling of muscle

biopsy specimens and that the final interpretation be made

by an expert in neuromuscular pathology.

IVIG is not recommended for the treatment of inclusion body

myositis.

Intractable childhood epilepsy

IVIG is not recommended for the treatment of intractable

childhood epilepsy.

Lambert-Eaton myasthenic syndrome

IVIG is recommended as an option for treatment of Lambert-

Eaton myasthenic syndrome. Objective evidence of clinical

improvement is needed for sustained use of IVIG.

Multifocal motor neuropathy

IVIG is recommended as first-line treatment for multifocal

motor neuropathy.

Based on consensus by the expert panel, diagnosis of

multifocal motor neuropathy should be made by a neuro-

muscular specialist because the diagnosis requires very

specific electrodiagnostic expertise.

Multiple sclerosis (MS)

IVIG is recommended as anoption for treatment of patientswith

relapsing-remittingMSwho fail, decline, or are not able to take

standard immunomodulatorydrug therapies. For thosepatients

with rapidly advancing relapsing-remitting MS, consideration

should first be given to immunosuppression therapy.

IVIG is not recommended for the treatment of primary or

secondary progressive MS.

Based on consensus by the expert panel, IVIG is not recom-

mended for treatment of acute exacerbations of MS, except in

patientswith severe, refractory, optic neuritiswhohave hadno

recoveryof vision after 3months of standard steroid therapyor

patients for whom corticosteroid therapy is contraindicated.


considered as a treatment option for patients with relaps-

ing-remitting MS who are pregnant or breast-feeding or in the

immediate postpartum period for women whose exacerba-

tion rate was high before pregnancy and who were on

disease-modifying agents before pregnancy with plans to

recommence therapy following birth or breast-feeding.

Table 2 (continued)


Multiple sclerosis (MS) (continued)


considered as a treatment option for patients with Marburg

disease, given the life-threatening nature of this disease.

Myasthenia gravis

Adult and juvenile myasthenia gravis

IVIG is recommended as a treatment option for patients with

severe exacerbations of myasthenia gravis or myasthenic

crises.


considered as an option to stabilize patients with myasthenia

gravis before surgery.

IVIG is not recommended as maintenance therapy for

patients with chronic myasthenia gravis.

Neonatal myasthenia gravis


considered among the treatment options for neonates

severely affected with myasthenia gravis.

Opsoclonus-myoclonus


as an option for treatment of opsoclonus-myoclonus.

Paraproteinemic neuropathy (IgM variant)

IVIG is not recommended for the treatment of IgM para-

proteinemic neuropathy.

Pediatric autoimmune neuropsychiatric disorders associat-

ed with streptococcal infections (PANDAS)

IVIG is recommended as an option for treatment of patients

with PANDAS.

Based on consensus by the expert panel, diagnosis of

PANDAS requires expert consultation.

POEMS syndrome

IVIG is not recommended for the treatment of POEMS

syndrome.

Polymyositis



the diagnosis of polymyositis. It is critical that the muscle

specimen be procured, processed, and interpreted in a

laboratory familiar with the correct handling of muscle biopsy

specimens and that the final interpretation be made by an

expert in neuromuscular pathology.


among the treatment options for patients with polymyositis

who fail to respond to first-line therapies (eg, steroids).

Rasmussen encephalitis

IVIG may be an option as a short-term temporizing measure

for patients with Rasmussen encephalitis.

IVIG is not recommended for long-term therapy for Rasmus-

sen encephalitis because surgical treatment is the current

standard of care.

Stiff person syndrome

IVIG is recommended as an option for treatment of stiff person

syndrome, if GABAergic medications fail or for patients who

have contraindications to GABAergic medications.

Abbreviations: G-CSF, granulocyte colony-stimulating factor;

ANDAS, pediatric autoimmune neuropsychiatric disorders

ssociated with streptococcal infections; POEMS, polyneurop-

thy, organomegaly, endocrinopathy, M protein, skin changes.

IVIG CLINICAL GUIDELINES S7

P

a

a

ROBINSON ET ALS8

7 hematologic conditions: acquired red cell aplasia,

acquired hypogammaglobulinemia (secondary to

malignancy), fetal-neonatal alloimmune thrombo-

cytopenia, hemolytic disease of the newborn, HIV-

associated thrombocytopenia, idiopathic thrombo-

cytopenic purpura, posttransfusion purpura.

Intravenous immunoglobulin was not recommen-

ded for use, except under certain urgent or life-

threatening circumstances, for 8 conditions, namely,

acquired hemophilia, acquired von Willebrand

disease, autoimmune hemolytic anemia, autoim-

mune neutropenia, hemolytic transfusion reaction,

hemolytic transfusion reaction associated with

sickle cell disease, hemolytic uremic syndrome/

thrombotic thrombocytopenic purpura, and viral-

associated hemophagocytic syndrome. Intravenous

immunoglobulin was not recommended for aplastic

anemia and hematopoietic stem cell transplantation.

Intravenous immunoglobulin was considered to

be contraindicated for heparin-induced thrombocy-

topenia.

Table 1 provides a summary of the recommen-

dations for the clinical use of IVIG for hematologic

conditions. For a complete discussion of the

panel’s recommendations, including recommended

dose and duration of IVIG therapy, please refer to

the specific condition of interest in the accompa-

nying guideline article in this issue of Transfusion

Medicine Reviews.

NEUROLOGIC CONDITIONS

Of the 22 neurologic conditions reviewed by the

neurology expert panel, specific recommendations

for use of IVIG were made for 14 conditions,

including acute disseminated encephalomyelitis,

chronic inflammatory demyelinating polyneurop-

athy, dermatomyositis, diabetic neuropathy, Guil-

lain-Barre syndrome, Lambert-Eaton myasthenic

syndrome, multifocal motor neuropathy, multiple

sclerosis, myasthenia gravis, opsoclonus-myoclo-

nus, pediatric autoimmune neuropsychiatric disor-

ders associated with streptococcal infections

(PANDAS), polymyositis, Rasmussen encephalitis,

and stiff person syndrome.

Intravenous immunoglobulin was not recom-

mended for the following 8 neurologic conditions:

adrenoleukodystrophy, amyotrophic lateral sclero-

sis, autism, critical illness polyneuropathy, inclu-

sion body myositis, intractable childhood epilepsy,

paraproteinemic neuropathy (IgM variant), and

POEMS syndrome.

Table 2 provides a summary of the recom-

mendations for the clinical use of IVIG for

neurologic conditions. Please consult the com-

plete guideline for a complete discussion of the

panel’s recommendations, including information

on recommended dose and duration of IVIG

therapy in this issue of Transfusion Medicine

Reviews.

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