Prevalence of inhibitors and clinical characteristics in patients with haemophilia in a middle-income Latin American country.

INTRODUCTION: Development of inhibitors is the most serious complication in patients with haemophilia (PWH). The prevalence of inhibitors in patients with severe haemophilia A (HA) is approximately 25%-30%. Inhibitor prevalence differs among populations. Some studies report a prevalence of almost twice in Hispanic as compared to Caucasian patients. Most data available, on the prevalence of inhibitors and their predisposing factors, originate from centres in developed countries. AIM: Establish the prevalence of inhibitors of FVIII and FIX in Mexico. METHODS: This was an observational, cross-sectional and descriptive study. The records of all patients diagnosed with haemophilia A (HA) or B (HB), with and without inhibitors, were included. Clinical and demographical characteristics of patients with inhibitors were assessed. Statistical analysis was performed using IBM SPSS version 22. The Ethics Committees of the various participating institutions approved this study. RESULTS: A total of 1455 patients from the 20 participating centres were recruited, from which 1208 (83.02%) had HA and 247 (16.97%) were diagnosed with HB. The presence of inhibitors in severe HA was reported in 93/777(11.96%), and 10/162 (6.17%) in severe HB. Of them, 91.7% exhibited high titres in HA and 100% in HB. CONCLUSION: In Mexico, the general prevalence of inhibitors varies considerably among centres. This study established a basis of comparison for future development and advances in the treatment and follow-up of patients. These findings also augment our understanding of risk factors related to inhibitor development.

[Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis]. / Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans.

Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.

Neumotórax bilateral espontáneo como forma de presentación de histiocitosis de células de Langerhans / Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis

La histiocitosis de células de Langerhans pulmonar es una patología intersticial en la que existe un acumulo de células histiocíticas específicas a nivel pulmonar. El neumotórax espontáneo es una complicación reconocida de histiocitosis de células de Langerhans pulmonar y es secundario a la destrucción del parénquima pulmonar con cambios quísticos asociados. Reportamos el caso de un niño de 2 años con neumotórax espontáneo bilateral recurrente, con una tomografía axial computada de tórax con infiltrado intersticial, fibrosis, lesiones quísticas e imágenes bullosas. El diagnóstico fue establecido por examen histológico e inmunohistoquímica de tejido de biopsia pulmonar con anticuerpos CD1 y S100 positivos. El niño recibió tratamiento con prednisona y etopósido, con buena respuesta clínica y tomográfica.

Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.