Evaluación imaginológica de un caso de histiocitosis durante tres años / Imaging evaluation of a case of histiocytosis for three years

La histiocitosis de células de Langerhans es un trastorno celular dendrítico, resultado de la proliferación clonal de este tipo de células, morfológicamente redondeadas e inmaduras inmunofenotípica y funcionalmente; asociadas con eosinófilos, macrófagos, linfocitos y células multinucleadas gigantes, en ocasiones. Puede presentarse de forma monosistémica, cuando afecta a un solo sitio u órgan; o multisistémica cuando se presenta en múltiples órganos o sistemas corporales. La literatura médica y las publicaciones en las que se asocia la imaginología al estudio y caracterización de las enfermedades hematológicas son escasas, aun en la era tecnológica. En este trabajo se presenta un caso de histiocitosis de células de Langerhans que fue seguido imaginológicamente durante 3 años con el consiguiente apoyo al diagnostico, tratamiento, seguimiento y valoración; lo que demuestra la utilidad de la Imaginología como herramienta para lograr un mejor manejo del paciente y como principio de la atención medica multidisciplinaria(AU)

Langerhans cells histiocytosis is a dendritic cell disorder, result of clonal proliferation of this type of cells, morphologically rounded and immature immunophenotypically and functionally; associated with eosinophils, macrophages, lymphocytes and giant multinucleated cells at times. It can present monosystemic, affecting a single site or organ; or multisystemic disease when present in multiple organs or body systems. Medical literature and publications in which imaging is associated with the study and characterization of hematological diseases are scarce, even in the technological era. This paper presents a case of Langerhans cells histiocytosis that was followed imaging during 3 years with the subsequent support to diagnosis, treatment, monitoring and evaluation; demonstrating the usefulness of imaging as tool to achieve better patient management and as a principle of multidisciplinary medical care(AU)

Histiocitosis de células de Langerhans con compromiso esternal aislado: Caso clínico / Langerhans cell histiocytosis with isolated sternum involvement: A clinical case

La histiocitosis de células de Langerhans es una entidad poco frecuente, que se caracteriza por la proliferación clonal de células dendríticas con amplia variabilidad clínica. En algunos casos, la enfermedad tiene afectación multisistémica con compromiso de vida y, en otros casos, de compromiso exclusivamente óseo, en los que la sobrevida es del 100%. Se presenta a una niña de 8 años que consultó al Servicio de Urgencias por dolor esternal de 4 días de evolución. Mediante estudios de imágenes, se observó una imagen lítica en el esternón. Se decidió la internación para el manejo del dolor y estudio. Se realizó una punción-aspiración con aguja fina, cuya inmunohistoquímica fue positiva para el marcador CD1a, que confirmó el diagnóstico de histiocitosis. La paciente recibió 40 mg/día de metilprednisolona, con buena evolución. Se decide exponer este caso por ser una forma de presentación atípica de una enfermedad poco prevalente, pero cuyo motivo de consulta amerita la sospecha diagnóstica.

The Langerhans Cell Histiocytosis (LCH) is a rare condition, characterized by the proliferation of dendritic cells. Its clinical presentation is variable and ranges from an isolated skin or bone disease, mainly the skull, to a life-threatening multisystemic disease. This case is about a healthy 8-year-old girl with a history of four days of severe sternum pain and no other symptomatology. At the initial evaluation at the emergency department a chest X-ray (Figure 1) and thoracic computed tomography scan with 3-D reconstruction were performed and they showed the sternal osteolytic lesion (Figure 2). She was admitted to the hospital for further evaluation. The immunohistochemistry evaluation of the fine needle aspiration sample was positive for CD1a, confirming the diagnosis of histiocytosis. The patient received methylprednisolone 40 mg/day with clinical improvement.

Langerhans' cell histiocytosis with neurological injuries diagnosed from a single cutaneous lesion

Abstract: Histiocytoses are rare diseases caused by the proliferation of histiocytes. The pathogenesis remains unknown and the highest incidence occurs in pediatric patients. The clinical presentations can be varied, in multiple organs and systems, and the skin lesions are not always present. Evolution is unpredictable and treatment depends on the extent and severity of the disease. It is described the case of a patient with various neurological symptoms, extensively investigated, who had its was diagnosed with histiocytosis from a single skin lesion. This report highlights the importance of Dermatology in assisting the investigation of difficult cases in medical practice.

Fluid-fluid level in Langerhans cell histiocytosis of the skull

We present a case of solitary eosinophilic granuloma in the skull of a 6-year-old Saudi boy. This osteolytic lesion has fluid-fluid level on CT and MRI. We are presenting a rare radiological finding of eosinophilic granuloma

Recurrent bilateral spontaneous pneumothorax in early infancy: a case of langerhans cell histiocytosis

Langerhans cell histiocytosis [LCH] is a rare disorder characterized by infiltration of either single or multiple organs by S100 and CD1a positive cells. Patients with pulmonary LCH are predisposed to pneumothorax due to destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH who presented at 2 months of age with simultaneous bilateral spontaneous pneumothorax

Langerhans' cell histiocytosis [Histiocytosis X]

The terms Langerhans' cell histiocytosis, histiocytosis x, Langerhans' cell granulomatosis and eosinophilic granuloma are used for a specific but variable clinico-pathology entity that are presented by proliferation of Langerhans' cell accompanied by a variable number of eosinophils, giant cells and neutrophils. Langerhans' cells usually have characteristic nuclei. The nuclei are often elongated, lobulated with prominent grooves and folds. The Cytoplasm is acidophilic and abundant. Most Langerhans' cells are mononuclear but occasionally they have several nuclei. On electron microscopic examination the Langerhans' cell shows specific intracytoplasmic organelles [Bubeck's granules]. For years this method has been used for identifying the Langerhans' cell. Today immunohistochemistry stain is a reliable method for definite diagnosis. According to Histiocyte Society even a typical clinical and light microscopic picture must be confirmed by a positive S100 or CD a immunohistochemistry stains

Honey comb lung

Honey comb lung is a radiological term indicating the presence of fairly thick walled cysts, usually 0.5-2 cm in diameter, which don,t fill with contrast material at bronchography. These changes may be diffuse or localized. The cysts probably represent dilated and thickened terminal and respiratory bronchioles. The main causes of diffuse honey combing are idiopathic pulmonary fibrosis; rheumatoid lung; histiocytosis X; tuberous sclerosis and neurofibromatosis