ABSTRACT
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Subject(s)
Male , Aged , Humans , Glucocorticoids/therapeutic use , Immunocompromised Host , Opportunistic Infections/diagnosis , Pneumonia, Bacterial/diagnosis , Pneumonia, Staphylococcal/diagnosis , Pseudomonas Infections/diagnosis , Pulmonary Disease, Chronic Obstructive/drug therapyABSTRACT
Intravascular lymphomatosis (IVL) is a rare malignancy characterized by a proliferation of atypical lymphoid cells occluding small blood vessels (venules, capillaries and small arteries). The symptoms are caused by embolisms due to massive proliferation. Nervous system and skin are the most common sites of involvement but all organs may be involved, although it is typified by the absence of malignant cells in lymphoid tissues. We describe three cases of IVL: first patient was a 57 years old man with rapidly progressive dementia and neurological involvements and second case of a 69 years old man hospitalised with predominant symptoms in the lung (cough, dyspnea and fever), and a woman presenting as fever of unknown origin (FUO) with systemic inflammatory response syndrome. In all cases that we reported have been diagnosed post-mortem because of rapid progression of a multisystem disease and the absence of pathognomonic clinical manifestations. Diagnosis can be made using biopsy of one of the involved organs. In conclusion, we propose that IVL should be included in the differential diagnosis of acute confusional state, dementia or other unexplained neurological manifestations, fever of unknown origin, vasculitis, occult neoplasia or infections with signs of a systemic disease and marked elevation of serum lactate dehydrogenase (LDH).
Subject(s)
Lymphoma/pathology , Vascular Neoplasms/pathology , Aged , Autopsy , Diagnosis, Differential , Female , Humans , Male , Middle AgedABSTRACT
La linfomatosis intravascular (LIV) es una neoplasia poco frecuente caracterizada por la proliferación de células linfoides atípicas en la luz de pequeños vasos (vénulas, capilares y arteriolas). La sintomatología está causada por fénomenos trombóticos debido a la oclusión de los vasos por células tumorales. Los órganos más afectados son el sistema nervioso y la piel, aunque puede estar involucrado cualquier otro sistema, sin afectación de los tejidos linfoides. Presentamos tres casos de LIV: el primero se trata de un varón de 57 años que debuta con demencia y síntomas neurológicos; un segundo caso en un paciente de 69 años con manifestaciones predominantemente pulmonares (tos, disnea y fiebre) y una mujer con fiebre de origen desconocido (FOD) y síndrome inflamatorio sistémico. Dada la ausencia de signos específicos de esta entidad multiorgánica con evolución rápidamente progresiva, su diagnóstico es postmorten en todos nuestros casos. Para su confirmación se precisó de biopsia de uno de los órganos afectados. En conclusión, consideramos la necesidad de plantear el diagnóstico diferencial con el síndrome confusional agudo, demencia o síndromes neurológicos de etiología no filiada, vasculitis, neoplasias ocultas, fiebre de origen desconocido o infecciones con afectación multiorgánica y valores muy elevados de LDH
Intravascular lymphomatosis (IVL) is a rare malignancy characterized by a proliferation of atypical lymphoid cells occluding small blood vessels (venules, capillaries and small arteries). The symptoms are caused by embolims due to massive proliferation. Nervous system and skinare the most common sites of involvement but all organs may be involved, although it is typified by the absence of malignant cells in lymphoid tissues. We describe three cases of IVL: first patient was a 57 years old man with rapidly progressive dementia and neurological involvementsand second case of a 69 years old man hospitalised with predominant symptoms in the lung (cough, dyspnea and fever), and a woman presenting as fever of unknown origin (FUO) with systemic inflammatory response syndrome. In all cases that we reported have been diagnosed postmortenbecause of rapid progression of a multisystem disease and the absence of pathognomonic clinical manifestations. Diagnosis can be made using biopsy of one of the involved organs. In conclusion, we propose that IVL should be inclucled in the differential diagnosis of acute confusional state, dementia or other unexplained neurological manifestations, fever of unknown origin, vasculitis, occult neoplasia or infections with signs of a systemic disease and marked elevation of serum lactate dehydrogenase (LDH)
