Linfomatosis intravascular cerebral como causa de demencia rápidamente progresiva: reporte de un caso / Cerebral intravascular lymphomatosis as cause of subacute dementia: report of a case

Cerebral Intravascular Lymphomatosis is a type of non Hodgkin Lymphoma, generally composed with B cells, its ocurrence is infrequent, clinically progressive and has a fatal course. Vascular damage is related with tumoral infiltration and small caliber occlusion, with results in multi infarcts. Neurological symptoms and signs are frequently the first clinical manifestation, which include sub acute encephalopathy, cognitive impairment, delirium, aphasia, hemipharesis, visual disturbances, paraplegia, paresthesia and cranial nerves involvement. MRI shows images of vasculitis from CNS. Cerebral Intravascular Lymphomatosis must be considered in differential diagnosis of rapid and subacute dementias, and clinical cases with small vessel recurrent multi infarct of unusual etiology. We present the clinical case of a patient with rapid progressive dementia and systemic disease manifestation. The CNS involvement was characterized as a subacute encephalopathy, with confusion and agitation, seizures, motor disturbances, bilateral plantar extensor reflexes and cerebellar signs. The clinical symptomatic course was progressive, with weight loss and fluctuant fever. The patient had a fatal course after he was treated with Methylprednisolone. Postmortem pathologic examination revealed a diffuse non Hodgkin lymphoma of B cells, intravascular variant, with brain compromise, cerebellum, suprarenal glands, pancreas, myocardium, thyroid gland, lung kidney and the lever...

La linfomatosis intravascular cerebral es un tipo de Linfoma no Hodgkin generalmente de células B, de presentación infrecuente, curso clínico progresivo y fatal. El daño vascular es producto de oclusión de vasos de pequeño calibre por infiltración tumoral, con resultado de múltiples infartos. Las manifestaciones neurológicas suelen ser la forma de presentación clínica inicial, caracterizadas por un amplio espectro que incluye encefalopatía subaguda, deterioro cognitivo, delirio, afasia, hemiparesia, trastornos visuales, paraplejia, parestesias y compromiso de pares craneanos. Los estudios de RNM evidencian alteraciones indistinguibles de las vasculitis del SNC. La linfomatosis intravascular debe considerarse en el diagnóstico diferencial de las demencias rápidamente progresivas y en los cuadros de multi infarto cerebral de pequeño vaso de etiología inusual y recurrente. Se presenta el caso de un paciente con demencia rápidamente progresiva, con manifestaciones neurológicas de deterioro cognitivo y enfermedad sistémica. El compromiso del SNC se expresó como encefalopatía subaguda con confusión, agitación, y crisis convulsivas, trastornos motores con reflejo cutáneo plantar extensor bilateral y compromiso cerebeloso, de curso clínico sintomático progresivo, con baja de peso y fiebre fluctuante. El paciente fue sometido a tratamiento con metilprednisolona a pesar del cual falleció. El estudio necrópsico demostró alteraciones por Linfoma no Hodgkin difuso de células B, variante intra vascular, con compromiso del cerebro, cerebelo, glándulas suprarrenales, páncreas, miocardio, tiroides, pulmón riñón e hígado...

Intravascular Lymphomatosis of the Central Nervous System Presenting Progressive Paraparesis

No abstract available.

Intravascular Lymphomatosis Presenting as Demyelinating Disease: A Case Report

Intravascular lymphomatosis (IL) is known as a rare and fatal disease characterized by massive intravascular proliferations of B or T lymphoid cells and manifests in the nervous system and skin. However diagnosis is usually made at the time of autopsy because of the lack of characteristic clinical or laboratory findings. Therefore, IL may be confused with various forms of neurological diseases including demyelinating disease, encephalitis, vasculitis or stroke. We report a case of post-mortem confirmed IL presenting as demyelinating disease.

Intravascular Lymphomatosis Presenting as Fever of Unknown Origin with Peripheral Polyneuropathy / 감염과화학요법

Fever of unknown origin (FUO) is caused by infections (30-40%), neoplasms (20-30%), collagen vascular diseases (10-20%), and numerous miscellaneous diseases (15-20%). The literatures also reveal that 5-15% of FUO cases defy diagnosis, despite extensive studies. Diagnostic advances continuously modify the spectrum of FUO-causing diseases. Modern imaging techniques (e.g., ultrasound, CT scan, MRI) enable early detection of abscesses and solid tumors that used to be difficult to diagnose. But the correct diagnosis can be delayed because the tumor, such as rare hematologic malignancy, is difficult to be detected by various imaging studies. Intravascular lymphomatosis (IVL) is a rare form of non-Hodgkin's lymphoma and generally fatal disease characterized by extensive proliferation of neoplastic mononuclear cells within the lumina of blood vessels. The prognosis is usually extremely poor, with rapid death despite chemotherapy. The diagnosis is most frequently made after biopsy of skin or brain but is often established post mortem. Most patients with IVL present with FUO and nonspecific cutaneous and neurologic manifestations. Peripheral nerve system or pulmonary involvements are not common as initial menifestations. We report a case of intravascular lymphomatosis, presenting as FUO with peripheral polyneuropathy. We made early diagnosis of IVL by video-assisted thoracoscopic lung biopsy. She is improving with good performance after the 4th cycle of chemotherapy till now.

Intravascular Lymphomatosis Presenting as Fever of Unknown Origin with Peripheral Polyneuropathy / 감염과화학요법

Fever of unknown origin (FUO) is caused by infections (30-40%), neoplasms (20-30%), collagen vascular diseases (10-20%), and numerous miscellaneous diseases (15-20%). The literatures also reveal that 5-15% of FUO cases defy diagnosis, despite extensive studies. Diagnostic advances continuously modify the spectrum of FUO-causing diseases. Modern imaging techniques (e.g., ultrasound, CT scan, MRI) enable early detection of abscesses and solid tumors that used to be difficult to diagnose. But the correct diagnosis can be delayed because the tumor, such as rare hematologic malignancy, is difficult to be detected by various imaging studies. Intravascular lymphomatosis (IVL) is a rare form of non-Hodgkin's lymphoma and generally fatal disease characterized by extensive proliferation of neoplastic mononuclear cells within the lumina of blood vessels. The prognosis is usually extremely poor, with rapid death despite chemotherapy. The diagnosis is most frequently made after biopsy of skin or brain but is often established post mortem. Most patients with IVL present with FUO and nonspecific cutaneous and neurologic manifestations. Peripheral nerve system or pulmonary involvements are not common as initial menifestations. We report a case of intravascular lymphomatosis, presenting as FUO with peripheral polyneuropathy. We made early diagnosis of IVL by video-assisted thoracoscopic lung biopsy. She is improving with good performance after the 4th cycle of chemotherapy till now.

Two Cases of Intravascular Lymphomatosis / 대한혈액학회지

Intravascular lymphomatosis is a rare lymphoma characterized by neoplastic proliferation of malignant cells within the lumen of small blood vessels, usually presenting in the central nervous system or on the skin. Intravascular lymphomatosis is manifested clinically by fever, dementia, cutaneous nodules or plaques, and occasionally, dyspnea. The diagnosis of intravascular lymphomatosis is difficult because of misleading clinical features mimiking vasculitis, infection, stroke, or other neoplasm. We report two cases of intravascular lymphomatosis pesented as fever and skin rash. Those are confirmed by involved tissue biopsy. All cases were treated by combination chemotherapy, but the response was not good. Infectious problems were complicated and disease were progressed.

Intravascular Lymphomatosis in Central Nervous System

Intravascular lymphomatosis (IL) is a variant of non-Hodgkin's lymphoma with an predilection for the CNS. Most cases are not diagnosed until postmortem. IL is characterized by neoplastic proliferation of lymphoid cells within the lumen of small veins and arteries with minimal involvement of the parenchyma. We experienced a 62-year-old woman who presented with seizure and fever associated with anemia, elevated LDH and beta 2-microglobulin without systemic involvement. This report illustrates the diagnostic challenge of this rare disorder with a grave prognosis. (J Korean Neurol Assoc 19(4):413~416, 2001)

A Case of Pulmonary Intravascular Lymphomatosis / 결핵

Intravascular lymphomatosis(IVL) which was first described by pfleger and Tappeiner in 1959 is rare malignancy characterized by neoplastic proliferation of lymphoid cell lineage within the vascular lumen with little or no adjacent parenchymal involvement. Its usual sites of involvement are central nervous system and skin or infrequently heart, lungs, pancreas, liver, spleen, kidney, adrenal glands, genitourinary tract, and bone marrow. Pulmonary involvement of IVL is not common. Symptoms of pulmonary involvement include dyspnea, cough and fever. Radiologicially, the disease is manifested with diffuse interstitial infiltrates. We report a recently experienced case of pulmonary intravascular lymphomatosis which was manifested with fever and chest pain.