Dysgammaglobulinemia

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Dysgammaglobulinemia
Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Recurrent infections, autoimmune disorders
Complications	Increased risk of infection, potential for autoimmune disease
Onset	Variable, often in childhood
Duration	Chronic
Types	N/A
Causes	Genetic mutations, B cell dysfunction
Risks	Family history of immunodeficiency
Diagnosis	Serum protein electrophoresis, immunoglobulin level testing
Differential diagnosis	Common variable immunodeficiency, X-linked agammaglobulinemia
Prevention	N/A
Treatment	Immunoglobulin replacement therapy, antibiotics for infections
Medication	N/A
Prognosis	Variable, depends on severity and management
Frequency	Rare
Deaths	N/A

Dysgammaglobulinemia is a type of immunodeficiency disorder characterized by an abnormality in the production of immunoglobulins, which are also known as antibodies. This condition results in an imbalance in the levels of different classes of immunoglobulins, leading to increased susceptibility to infections.

Introduction

Immunoglobulins are crucial components of the immune system, produced by B cells and plasma cells. They play a vital role in identifying and neutralizing pathogens such as bacteria and viruses. In dysgammaglobulinemia, the production of these immunoglobulins is impaired, resulting in either a deficiency or an excess of certain types.

Types of Dysgammaglobulinemia

Dysgammaglobulinemia can be classified based on the specific immunoglobulin classes affected:

• IgA dysgammaglobulinemia: Characterized by abnormal levels of IgA.
• IgG dysgammaglobulinemia: Involves abnormal levels of IgG.
• IgM dysgammaglobulinemia: Involves abnormal levels of IgM.

Clinical Manifestations

Patients with dysgammaglobulinemia may present with recurrent infections, particularly of the respiratory tract, gastrointestinal tract, and skin. The severity and frequency of infections depend on the specific immunoglobulin deficiency and the degree of immune system impairment.

Diagnosis

Diagnosis of dysgammaglobulinemia involves measuring serum immunoglobulin levels through blood tests. A detailed immunological profile can help identify specific deficiencies or imbalances in immunoglobulin classes.

Treatment

Treatment strategies for dysgammaglobulinemia focus on managing infections and correcting immunoglobulin deficiencies. This may include:

• Immunoglobulin replacement therapy: Administering intravenous or subcutaneous immunoglobulin to supplement deficient antibodies.
• Antibiotic prophylaxis: Preventive use of antibiotics to reduce the risk of infections.
• Vaccinations: Ensuring patients receive appropriate vaccinations to prevent infections.

See Also

• Immunodeficiency
• Immunoglobulin
• B cell
• Plasma cell