Nezelof syndrome; combined immunodeficiency with normal immunoglobulins

Introduction

Described by Nezelof & associates in 1964.

Epidemiology

• infants

Pathology

• combined immunodeficiency with normal immunoglobulins

Clinical manifestations

• recurrent infections
• failure to thrive
• diminished lymphoid tissue
• abnormal structure or agenesis of the thymus
• impaired antibody synthesis

Laboratory

• complete blood count
  • lymphopenia
• serum immunoblobulins
  • normal or increased levels of one or more of the major immunoglobulin classes

Complications

• malignancy
• death

More general terms

• syndrome
• combined immune deficiency