Bronquiolitis folicular asociada a inmunodeficiencia común variable

Common variable immunodeficiency is one of the most frequent immunity alterations. The most common clinical presentation occurs with recurrent respiratory infections, from pneumonia to otitis, and may be associated with other diseases such as bronchiectasis or interstitial lung diseases. We report the case of a 28-year-old patient with frequent respiratory infections and nodular pulmonary infiltrates, who was diagnosed with common variable immunodeficiency and follicular bronchiolitis. In some cases, follicular bronchiolitis is associated with immunodeficiencies and should be included in a differential diagnosis with lymphoid nodular hyperplasia, lymphocytic interstitial pneumonia and low-grade BALT lymphoma.     

Treatment of COVID-19 with convalescent plasma in patients with humoral immunodeficiency – Three consecutive cases and review of the literature

Patients lacking humoral response have been suggested to develop a less severe COVID-19, but there are some reports with a prolonged, relapsing or deadly course. From April 2020, there is growing evidence on the benefits of COVID-19 convalescent plasma (CCP) for patients with humoral immunodeficiency. Most of them had a congenital primary immunodeficiency or were on treatment with anti CD20 antibodies. We report on three patients treated in our hospital and review thirty-one more cases described in the literature. All patients but three resolved clinical picture with CCP. A dose from 200 to 800 ml was enough in most cases. Antibody levels after transfusion were negative or low, suggesting consumption of them in SARS-CoV-2 neutralization. These patients have a protracted clinical course shortened after CCP. CCP could be helpful for patients with humoral immunodeficiency. It avoid relapses and chronification. CCP should be transfused as early as possible in patients with COVID-19 and humoral immunodeficiency.     

Utilización de inmunoglobulina subcutánea durante el embarazo en déficit humoral

The use of the subcutaneous route of administration of immunoglobulin G replacement therapy is described. Publications in the English-language literature, with small case series, demonstrate the safety and efficacy of this route of administration. Given the lack of experience in Spain, the first case of the use of immunoglobulin replacement in a pregnant woman with humoral deficiency (common variable immunodeficiency) is described.     

El descenso del número de linfocitos B de memoria en pacientes con inmunodeficiencia variable común está asociado a una apoptosis aumentada de esta población celular. Desarrollo de un método nuevo para la detección de la apoptosis en muestras de sangre completa

Common Variable Immunodeficiency (CVID) is a heterogeneous disease characterised by low memory B cell counts in peripheral blood (PB). Although previous reports have attributed memory CD27+ B cell decrease to a possible defective germinal centre development, the cause of this defect remains basically unknown. On the other hand, increased apoptosis has been implicated in the pathogenesis of several diseases, and could be another factor that contributes to explain memory B cell reduction. However, a negative balance between apoptosis/survival of memory B cells in CVID patients has still not been documented. Therefore, we asked whether increased apoptosis was relevant in CVID. To test this concept, total and memory B cells from CVID patients and healthy controls were studied by flow cytometry to determine spontaneous and induced apoptosis. Apoptosis was measured by Annexin-V expression after a 24 hour culture with and without anti-CD95 proapoptotic monoclonal antibody. We also developed a new and sensitive method that uses 7-amino-actinomycin D (7-AAD) to measure very low numbers of apoptotic cells in whole PB. Our results clearly indicate diminished absolute counts and higher apoptosis in memory B cells from CVID patients than in healthy controls (P < .01). Moreover, we also demonstrate that CD95 is not implicated in regulating memory B cell apoptosis.     

Diagnóstico genético preimplantacional en un caso de una paciente portadora de una inmunodeficiencia combinada severa. Revisión crítica de este procedimiento en ciclos de FIV-ICSI

Objective

To perform a critical analysis from a clinical case of preimplantation genetic diagnosis (PGD).

Patients and methods

A patient is described who has a disease of genetic origin (severe combined immunodeficiency) in which PGD was of vital importance for her treatment.

Conclusions

PGD is a very important tool in assisted reproduction but its indications, except in monogenic diseases and those linked to sex, are still under debate.