Intravascular lymphomatosis and intracerebral haemorrhage

Passarin, Maria Grazia; Wen, Patrick Y.; Vattemi, Emanuela; Buffone, Ebba; Ghimenton, Claudio; Bontempini, L; Ottaviani, Sarah; Musso, Anna Maria; Pedersini, Rebecca

doi:10.1007/s10072-010-0284-7

Cited by 19 publications

(10 citation statements)

References 28 publications

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“…In those cases the spinal cord lesions showed eosinophilic inflammation on biopsy [8,9]. Recent publications have stressed the vascular features of hyperimmunoglobulin E syndrome, but perivascular abnormalities (as found in our cases) have not been described [10,13]. It seems premature to state that a clear causal relationship exists between the elevated IgE levels and the perivascular inflammation.…”

Section: Discussioncontrasting

confidence: 44%

“…Thus, the MRI appearance is unlike any of the features readily identified in CNS-involvement of other systemic diseases [2]. The primary clinical differential diagnoses were cerebral lymphoma, intravascular lymphomatosis, sarcoidosis or primary angiitis of the CNS [6,10]. Based on the radiological appearance a primary intracerebral B-cell lymphoma was considered unlikely, but despite the lack of typical intracerebral haemorrhage intravascular lymphomatosis was considered possible [10].…”

Section: Discussionmentioning

confidence: 99%

“…The primary clinical differential diagnoses were cerebral lymphoma, intravascular lymphomatosis, sarcoidosis or primary angiitis of the CNS [6,10]. Based on the radiological appearance a primary intracerebral B-cell lymphoma was considered unlikely, but despite the lack of typical intracerebral haemorrhage intravascular lymphomatosis was considered possible [10]. Systemic or primary central nervous system lymphoma was ruled out by haematological examination (including bone marrow biopsy), CT, repeated CSF analyses, histology, and molecular assays demonstrating the polyclonal origin of the lymphocytic infiltrates.…”

Section: Discussionmentioning

confidence: 99%

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Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study

et al. 2011

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The aim of the study was to further determine the pathophysiology, clinical course, MRI-features and response to therapy of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), which has recently been proposed as a rare chronic inflammatory central nervous system disorder responsive to immunosuppressive therapy. Three patients with this rare entity underwent serial clinical and bimonthly MRI follow-up over a period of up to 16 months. Extensive laboratory work-up and brain biopsy were performed. Intravenous methylprednisolone or oral dexamethasone was administered as treatment, additionally cyclophosphamide in one patient. Clinically, diplopia, nystagmus, ataxia and facial paresthesia were the cardinal symptoms. Magnetic resonance imaging (MRI) disclosed patchy spot-like gadolinium enhancement in a "salt-and-pepper like appearance" in the pons, midbrain and cerebellum, in two cases with thalamic and in the other with spinal involvement. Brain biopsies demonstrated a predominantly angiocentric but also diffuse infiltration pattern by small mature lymphocytes. Treatment with steroids led to rapid clinical improvement and marked resolution of MRI lesions. As discontinuation of steroids led to clinical relapse, one patient was treated with a further course of steroids and the other with steroids and cyclophosphamide as immunosuppressive therapy. This led to stable remission with only mild clinical residue and normalization of MRI. Extensive laboratory and radiological work-up could not identify any other cause of the disease. Of note, in two cases a marked elevation of IgE in serum was found initially and throughout the course. CLIPPERS seems to be a distinct inflammatory central nervous system disorder. It shows characteristic MRI core features. Extrapontine involvement seems to be frequent. Histologically it is characterised by predominantly angiocentric infiltration by small mature lymphocytes. A pathogenetic relationship between the elevated IgE levels and the perivascular infiltrates can be presumed. It is responsive to immunosuppressive therapy and can require prolonged or maintenance treatment.

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Section: Discussioncontrasting

confidence: 44%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study

et al. 2011

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“…[19,20] The utility of fluorodeoxyglucose positron emission tomography in its early detection remains controversial. [21,22] An organ biopsy is mandatory for the diagnosis of IVLBCL.…”

Section: Discussionmentioning

confidence: 99%

A 58-year-old lady with progressive neurological syndrome: Presence of an intravascular lymphoma

Jethwani¹,

Yadav²,

Chickabasaviah³

2015

Neurol India

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“…Some reports of PCNSL with intratumoral hemorrhage have suggested that a high level of vascular endothelial growth factor immunoreactivity in lymphoma cells is correlated with intratumoral hemorrhage [10, 15]. Additionally, some case reports of IVL with cerebral hemorrhage have described possible causes of cerebral hemorrhage, such as a complication of DIC [16], obstruction of the lumen of the vessel due to complete fullness of lymphoma cells [17], and an injury of the vessel wall due to direct interaction between lymphoma cells and endothelial cells of the cerebral vessel [12]. Streletz et al stated that when stroke and hemorrhage occur with a CNS lymphoma, it is usually IVL [10].…”

Section: Discussionmentioning

confidence: 99%

Secondary Central Nerve System Lymphoma With Intratumoral Hemorrhage Suggested as Intravascular Lymphoma by Autopsy: A Case Report

et al. 2017

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Intravascular large B-cell lymphoma (IVL) is a rare type of extranodal diffuse large B-cell lymphoma (DLBCL), which often infiltrates the central nervous system (CNS) during the clinical course. Cerebral hemorrhage in patients with CNS lymphoma at presentation is rare. Herein, we describe a case of secondary CNS lymphoma with intratumoral hemorrhage, which was suggested as IVL from autopsy findings. A 76-year-old Japanese man with a history of treatment for B-cell non-Hodgkin’s lymphoma was transferred to our hospital in an ambulance for generalized convulsions. Brain CT scan revealed a high-density tumor with edema and intratumoral hemorrhage in the left temporal lobes. He died in a rapid course, and autopsy revealed a focal hemorrhage with diffuse infiltration of lymphoma cells in the left temporal lobe and findings suggestive of IVL. Furthermore, the autopsy revealed a discrepancy in the CD20 immunostaining of lymphoma cells between the brain and other organs. Clinicians should not eliminate CNS lymphoma from the differential diagnosis of intracranial tumor with hemorrhage. Although many patients with IVL have rapidly progressive courses, it is very important to diagnose IVL at the initial onset, even in serious situations, to consider CNS prophylaxis.

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Intravascular lymphomatosis and intracerebral haemorrhage

Cited by 19 publications

References 28 publications

Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study

Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study

A 58-year-old lady with progressive neurological syndrome: Presence of an intravascular lymphoma

Secondary Central Nerve System Lymphoma With Intratumoral Hemorrhage Suggested as Intravascular Lymphoma by Autopsy: A Case Report

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