AUTOIMMUNE HEMOLYTIC ANEMIA AND COMMON VARIABLE
IMMUNODEFICIENCY DIAGNOSED YEARS LATER
Simge Çınar Özel1, Ayşe Gonca Kaçar1, Burcu Kılınç1, Ayça Kıykım2, Süheyla Ocak1 and Tiraje Celkan1
(1)1. Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Depatment of Pediatric Hematology
and Oncology, Istanbul, Turkey
(2)1. Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Pediatric
Immunology, Istanbul, Turkey
I
ntroduction: Common variable immunodeficiency (CVID) is a heterogeneous group of
diseases characterized by antibody production defects and recurrent bacterial infections. The
disease is generally seen after 18 months; It peaks in two periods, 1-5 years old and 16-20
years old.
Its frequency is 1 / 10,000-100,000. Autoimmunity is in 20-25% of patients with CVID,
especially in pediatric patients, the first sign of the disease may be autoimmune cytopenia.
Autoimmune neutropenia, immune thrombocytopenia (ITP), autoimmune hemolytic anemia
(AIHA) or coexistence of these diseases can be seen. We describe the patient who was
followed up with a diagnosis of autoimmune hemolytic anemia and was diagnosed with CVID
years later.
Case: A twenty -year -old boy, who has been followed up with the diagnosis of AIHA for ten
years. He was treated with splenectomy and had decreased attacks after splenectomy. He had
frequent upper respiratory tract infection, otitis, pneumonia, and chronic cough for the last
year. Therefore, a decrease in immunoglobulins (IgG: 89 mg / dl, IgA: 0.5 mg / dl, IgM: 16.1
mg / dl, T. IgE: <2.0 IU / ml) was detected. CD4 / CD8 ratio: 1/2 (reverse distorted), B and T
cell counts were normal on flow cytometric immunophenotyping. On Thorax CT revealed
multiple lymphadenopathies with a subcarinal diameter of 2 cm and tubular bronchiectasis in
the lower lobe of the left lung. Thoracotomy was performed and biopsy was taken from the
lung and hilar lymph node. Biopsy revealed lymphoid hyperplasia in the hilar lymph node,
lymphocytic interstitial pneumonia in the lung and accompanying non-necrosing granuloma.
The patient was followed up with a diagnosis of CVID.
Conclusion: CVID may initially appear with AIHA. For this reason, it should be better that in
cases followed up with autoimmune hemolytic anemia or cytopenia, immunoglobulin may be
checked. Even if the values are found to be normal, patients should be followed closely for
CVID that may develop over the years.
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