Clinical immunology Thrombocytopenia in common variable immunodeficiency patients – clinical course, management, and effect of immunoglobulins

Abstract

Common variable immunodeficiency (CVID) is a primary immunodeficiency of humoral immunity with heterogeneous clinical features. Diagnosis of CVID is based on hypogammaglobulinaemia, low production of specific antibodies, and disorders of cellular immunity. The standard therapy includes replacement of specific antibodies with human immunoglobulin, prophylaxis, and symptomatic therapy of infections. High prevalence of autoimmunity is characteristic for CVID, most commonly: thrombocytopaenia and neutropaenia, celiac disease, and systemic autoimmune diseases.The study included seven children diagnosed with CVID and treated with immunoglobulin substitution from 2 to 12 years. Thrombocytopenia was diagnosed prior to CVID in four children, developed during immunoglobulin substitution in three children. In one boy with CVID and thrombocytopaenia, haemolytic anaemia occurred, so a diagnosis of Evans syndrome was established. Therapy of thrombocytopaenia previous to CVID included steroids and/or immunoglobulins in high dose, and azathioprine. In children with CVID on regular immunoglobulin substitution, episodes of acute thrombocytopaenia were associated with infections and were treated with high doses of immunoglobulins and steroids. In two patients only chronic thrombocytopaenia was noted. Splenectomy was necessary in one patient because of severe course of thrombocytopaenia.The results of the study indicated a supportive role of regular immunoglobulin substitution in patients with CVID and chronic thrombocytopaenia. However, regular substitution of immunoglobulins in CVID patients did not prevent the occurrence of autoimmune thrombocytopaenia episodes or exacerbations of chronic form. In episodes of acute thrombocytopaenia or exacerbations of chronic thrombocytopaenia, infusions of immunoglobulins in high dose are effective, despite previous regular substitution in the replacing dose.

Drug‐induced aseptic meningitis: a mini‐review

“…Some IVIG brands are more likely to cause aseptic meningitis for unknown reasons, probably related to the IgA concentration in each brand. Indeed, in patients with serum anti-IgA, administration of IVIG containing IgA may lead to dramatic clinical reactions [40]. Another reason could be the use of each brand in various labeled conditions, thus, with various dosage regimen and route of administration.…”

Common variable immunodeficiency with granulomatous-lymphocytic interstitial lung disease and preceding neurological involvement: a case-report

“…CVID accounts for 90% of symptomatic antibody deficiencies and is known to present at any age [ 2 ]. The disorder is further associated with autoimmune conditions such as autoimmune haemolytic anaemia (AIHA), idiopathic thrombocytopenic purpura (ITP) and autoimmune neutropenia [ 2 , 3 ].…”

Dengue hemorrhagic fever as a rare cause of chronic immune thrombocytopenic purpura—a pediatric case report

“…Immune thrombocytopenic purpura (ITP) is a rare autoimmune disorder which can be either primary or secondary due to a number of medical disorders [ 1 ]. Secondary ITP is known to occur in association with systemic lupus erythematosus [ 2 ], anti-phospholipid antibody syndrome [ 3 ], immunodeficiency disorders [ 4 ], lymphoproliferative disorders [ 5 ], viral infections [ 6 ], and medications such as quinine and heparin [ 7 ]. Immune thrombocytopenia can be either acute or chronic.…”