whentosuspectimmunodeficiency

The Hallmark of primary Immunodeficiency is

• • Infections

  • Autoimmunity

  • Malignancies

Infections:

• • Recurrent

  • Fulminant & Overwhelming infection

  • infection with atypical Organism e.g Atypical Mycobacteria

  • infection difficult to clear

Autoimmunity:

• • AITP

  • AIHA

  • Autoimmune Neutropenia

  • Autoimmune Hepatitis

  • Autoimmune colitiis

very often seen in CVID

Malignancies:

• • Lymphoma e.g in WAS; Ataxia Telangiectasia

  • Leukaemia e.g Severe Congenital Neutropenia

  • Solid Tumors e.g Ataxia Telangiectasia

The important consideration in suspecting primary immunodeficiency is High Index of Suspicion.

• • Even in developed countries only 1/10 primary immunodeficiency cases are diagnosed.

  • Do Keep a High Index of Suspicion & Think of PID (Primary Immunodeficiency) in any child with recurrent infections, autoimmunity and malignancies.

Immunologists feel that every ICU child is possibly an immunodeficienct Child whose immunodeficiency is not yet identified.