Type and location of bronchiectasis in a cohort of adults with common variable immunodeficiency / Tipo y localización de bronquiectasias en una cohorte de adultos con inmunodeficiencia común variable

Background: Bronchiectasis are permanent dilatations of the bronchi. Its prevalence in patients with variable common immunodeficiency (CVID) is high, however there is little information regarding the type and location of the same; therefore the objective of this study is to know the type and location of bronchiectasis in a cohort of adult patients with CVID. Methods: It has been made a transversal, observational and descriptive study that included 32 adult patients with diagnosis of CVID according to the criteria of the European Society of Immunodeficiencies (ESID). All patients underwent high resolution pulmonary computed tomography (HRCT), which were interpreted by an expert radiologist. The frequency, type and location of bronchiectasis were reported using descriptive statistics. Results: Thirty-two adult patients, ten men (31.25%) and 22 women (68.7%), were included. 40.6% had bronchiectasis. 23% had a lobe involvement, 15.3% two lobes, 46.1% 3 lobes and 15.3% complete involvement of the parenchyma. The types of bronchiectasis were distributed as follows: tubular 38.4%, varicose 23% and cystic and tubular combinations 15.3%, cystic and varicose 15.3% and cystic, tubular and varicose 7.6%. Conclusions: Our results show that 40% of adult patients with CVID have BQs, usually affecting three pulmonary lobes, located mainly in the right and middle lower lobe; The tubular type, is the most common. Their timely diagnosis and treatment can improve survival and reduce costs for patients and health care.

Introducción: Las bronquiectasias (BQs) son dilataciones permanentes de los bronquios. Su prevalencia en pacientes con inmunodeficiencia común variable (IDCV) es alta, sin embargo existe escasa información respecto al tipo y localización de las mismas. El objetivo de este estudio es conocer el tipo y localización de las bronquiectasias en una cohorte de pacientes adultos portadores de IDCV. Métodos: Estudio transversal, observacional y descriptivo que incluyó a 32 pacientes adultos con diagnóstico de IDCV de acuerdo con los criterios de la Sociedad Europea de Inmunodeficiencias. A todos se les realizó tomografía de alta resolución pulmonar, las cuales fueron interpretadas por un médico radiólogo experto. Se reportó la frecuencia, tipo y localización de las bronquiectasias mediante estadística descriptiva.Resultados: se incluyeron 32 pacientes adultos, diez hombres y 22 mujeres. El 40.6% presentaron bronquiectasias. El 23% tenía afección en un lóbulo, el 15.3% dos lóbulos, 46.1% 3 lóbulos y el 15.3% afectación completa del parénquima; distribuidos de la siguiente manera: tubulares 38.4%, varicosas 23% y las combinaciones quísticas y tubulares 15.3%, quísticas y varicosas 15.3% y quísticas, tubulares y varicosas 7.6%. Conclusión: Nuestros resultados muestran que el 40% de los pacientes adultos con IDCV tienen BQs, suelen afectar tres lóbulos pulmonares, el tipo más común fue el tubular. Su diagnóstico y tratamiento oportuno puede mejorar la supervivencia y reducir los costos para el paciente y las instituciones de salud.

[Chronic myeloid leukemia in an adult with common variable immunodeficiency]. / Leucemia mieloide crónica en un adulto con inmunodeficiencia común variable.

BACKGROUND: Common variable immunodeficiency is a primary immunodeficiency, in which from 70 to 80 % of patients have tumors and 25 % of cases are associated with autoimmune diseases. Common variable immunodeficiency patients have a higher incidence of neoplasms, with a risk 12-18 times higher than the general population. There are few cases of common variable immunodeficiency patients with leukemia. CLINICAL CASE: Female of 36 years old, with left upper quadrant pain, early satiety, weight loss of 8 kg in three months and splenomegaly. The complete blood count showed: leukocytosis 206 000/mL, with 8 % blasts, platelets 530 000/mL and hemoglobin 8 mg/dL. Abdominal ultrasound: 19??12 cm splenomegaly. Karyotype BCR/ABL IS 64.20 %, 100 % Philadelphia chromosome. The diagnosis was of chronic myeloid leukemia. Given the presence of recurrent respiratory tract infection, frequent diarrheas and reduced concentrations of IgG, IgM and IgA, common variable immunodeficiency was diagnosed and human immunoglobulin was used successfully. CONCLUSIONS: The association between chronic myeloid leukemia and common variable immunodefficiency is unusual. Given the high frequency of hematological neoplasm in common variable immunodeficiency patients, we suggest that hematological patients with repeated infections and decreased concentrations of immunoglobulin be referred to an immunological evaluation.

INTRODUCCIÓN: la inmunodeficiencia común variable es la inmunodeficiencia primaria más sintomática, 70 a 80 % de los pacientes presentan neoplasias. Existen escasos informes de pacientes portadores de leucemia mieloide crónica e inmunodeficiencia común variable. CASO CLÍNICO: mujer de 36 años, quien inició su padecimiento con dolor en hipocondrio izquierdo, pérdida de peso de 8 kg en tres meses y esplenomegalia. Los resultados de la biometría hemática indicaron cuenta leucocitaria de 206 000/mL, 8 % de blastos, cuenta plaquetaria de 530 000/mL y hemoglobina de 8 g/dL. Con la ultrasonografía abdominal se identificó esplenomegalia de 19 ´ 12 cm. El cariotipo mostró el gen BCR/ABL 64.20 % IS y cromosoma Filadelfia 100 %. Se diagnosticó leucemia mieloide crónica. Por infecciones gastrointestinales y respiratorias frecuentes, así como por concentraciones reducidas de IgG, IgM e IgA, la paciente fue valorada por el servicio de alergia e inmunología clínica, donde se diagnosticó inmunodeficiencia común variable. CONCLUSIONES: la relación de la leucemia mieloide crónica con la inmunodeficiencia común variable es poco frecuente. Es importante que los pacientes portadores de leucemia mieloide crónica que cursen con infecciones de repetición, se realicen cuantificación de inmunoglobulinas séricas y, en caso de disminución de las mismas, sean referidos para su valoración y tratamiento.

[Latex Allergy in Patients Who Underwent Surgical Treatment. Case series]. / Alergia a látex en pacientes operados. Serie de casos.

Latex allergy is an underdiagnosed condition, and it is the second cause of perioperative anaphylaxis. This condition represents a clinical challenge due to the severity of reactions caused by the exposure to latex in allergic individuals. It is necessary to know the risk factors in order to suspect the diagnosis of latex allergy, and to apply the preventive measures in the medical care of patients allergic to latex. Other safety measures and pre-medication, as avoiding objects and medical equipment made of latex, should be taken since they can cause anaphylaxis. We present six cases of patients with latex allergy, who underwent surgical procedures. In five of them with positive skin test with latex, we used preventive measures and pre-medication 72 hours before and 48 hours after surgery. Only one patient, a sixty year old woman, with urticaria related to kiwi, avocado and potatoes, who was surgically intervened without pre-medication, presented grade IV anaphylaxis.

[Autoimmune hematologic diseases in adult patients with common variable immunodeficiency]. / Enfermedades hematológicas autoinmunes en adultos con inmunodeficiencia com˙n variable.

BACKGROUND: Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by defective immunoglobulin production. Twenty percent of patients may also have associated autoimmune diseases, especially hematologic, whose prevalence is estimated at 5 to 8%. OBJECTIVE: To report the occurrence of autoimmune haematological disorders in CVID adults. METHODS: We included 18 patients with CVID, who underwent hematologic evaluation, which included: CBC, serum folate or vitamin B complex and bone marrow aspirate. RESULTS: Of a total of 18 patients, 11 women (61%), mean age 38 ± 13.5, six patients (33.3%) had autoimmune diseases, five of them had blood diseases (4 with PTI and one with Evans syndrome). Three patients had more than one autoimmune disease and one presented three autoimmune diseases (Evans syndrome, hypoparathyroidism and celiac disease with a history of autoimmune hemolytic anemia). CONCLUSIONS: The prevalence of autoimmune diseases in this group of patients with CVID was higher than the 20% reported in the literature. Hematological diseases should be suspected in CVID patients, since half of our cases presented them.