Subcutaneous immunoglobulin replacement therapy for primary immunodeficiency in children and young adults. Radana Zachová Department of Immunology University.

Subcutaneous immunoglobulin

replacement therapy for primary

immunodeficiency in children and young

adults.

Radana Zachová

Department of Immunology University hospital Motol, Charles University

Prague, Czech Republic

ESID Prague Spring Meeting 2009

Immunoglobulin replacement therapy in primary immunodeficiencies

Life –long therapy for patients with PID

Possible administration ways – intravenous, subcutaneous

Historically - intramuscular

Disorders:

CVID

X- linked agammaglobulinemia

Specific antibody deficiencies


Subcutaneous immunoglobulin replacement therapy

Patient Diagnosis Age (years)

Subcutaneous therapy started

Age at beginnig of

therapy

Results

ZP CVID 29 2007 27 Home therapy

MK CVID 16 2007 14 Home therapy

JJ CVID 15 2007 13 Home therapy

SM CVID 16 2009 16 Home therapy

MK XLA 2 2009 2 Home therapy

FV CVID 52 2008 51 therapy in hospital

SV XLP 19 2007 17 refused s.c.therapy

Small infusion pump for scig administration

NOT refunded from insurance !

WE lend this pump from company

Subcutaneous immunoglobulin replacement therapy in 1,5 year old boy.

Case report:

MK *2007

History: boy, 1. pregnancy, unrelative parents, born in term, 3100g,

anorectal atresia, surgical treatment after delivery ,

3 month - pyelonephritis

11 month - bilateral bronchopneumonia

Immunoglobulin levels IgG, IgA, IgM – very low

CD 19-1,1% CD 20-0,5%

BTK mutation –pR28C missence mutation

Family history: grandmother´s brother ( mother´s side) died at 18 years ( cerebral absces, recurrent bronchopneumonias)


MK *2007

Therapy:

Immunolgobulin substitution therapy was indicated

Weight : 10 kg

after first application of IVIG mother refused intravenous application of immunoglobulins

Intramuscular administration was started 1x in 14 days - painfull, uncomfortable - mother refused I.M. application too

↓

We started subcutaneous application of immunoglobulins


Thinks taken into consideration

parents reffused IV and IM immunoglobulin substitution completelly

subcutaneous therapy ?

Disadvantages for SCIG

low BMI – small subcutaneous tissue - big local swelling???

scar on abdominal wall after surgical treatment - only one side of abdominal wall can be used for replacement therapy

2 adult persons have to apply the therapy ( 1 has to fix the child)

Actually:

subcutaneous replacement therapy in home administration


MK *2007


MK *2007

Our experience with SCIG therapy

SCIG replacement therapy is not limited by age

Patients pretreatet with IVIG may refuse SCIG

SCIG is suitable for patients with previous adverse reactions to IVIG

Thank you for your attention!


Subcutaneous immunoglobulin replacement therapy for primary immunodeficiency in children and young adults. Radana Zachová Department of Immunology University.

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