Epidemiology

The incidence and prevalence rates of hypogammaglobulinemia are not clearly defined because the condition results from an extensive variety of primary and secondary defects. Primary hypogammaglobulinemia is less common than secondary hypogammaglobulinemia.[3]

The estimates for prevalence of primary immunodeficiency (PID) and hypogammaglobulinemia vary widely depending on the population studied. In 2007, reported estimates derived from US administrative healthcare databases ranged from 41.1 per 100,000 and 50.5 per 100,000 (publicly insured vs. privately insured persons, respectively).[4] Among hospitalized children in the US, the national prevalence of all PID diseases per 100,000 was 126.8 in 2012.[5] However, not all of the defined PID diseases (>150) result in hypogammaglobulinemia.

Predominantly antibody deficiencies are the most common form of PID, accounting for 77% of a PID registry survey.[6][7] Selective IgA deficiency is the most prevalent of these deficiencies (between 1/300 and 1/700), although the condition is often asymptomatic. About 30 out of 100,000 people have common variable immunodeficiency in the US and it is the most clinically relevant antibody deficiency requiring immunoglobulin replacement therapy.[7][8][9]

Newborn screening in 11 programs in the US identified severe combined immunodeficiency (SCID) in 1 in 58,000 infants.[10]

Hypogammaglobulinemia can present at any age depending on the underlying cause. SCID presents early in infancy, whereas transient hypogammaglobulinemia of infancy and X-linked agammaglobulinemia (XLA) may present in early childhood when maternal IgG levels fall, or later in infancy. Common variable immunodeficiency (CVID) can have early or late onset but usually manifests in adolescence or early adulthood, although it may not be evident until patients are middle-aged or older.[11][12] Reports suggest that most children with hypogammaglobulinemia and PID have transient hypogammaglobulinemia of infancy or CVID, while approximately 7% have SCID.[3]

Most diseases that cause hypogammaglobulinemia affect both sexes equally, although there are some rarer X-linked conditions (e.g., XLA). Secondary hypogammaglobulinemia related to myeloma and chronic lymphocytic leukemia tends to present in older adults, ages >50 years.

Use of this content is subject to our disclaimer