Spontaneous Remission of a Massive CNS Inflammation with Eosinophilic Infiltrate

Tamaru, Yoshiko; Nakashita, Mayumi; Ito, Hidefumi; Okumura, Ryota; Matsumoto, Sadayuki; Imai, Terukuni

doi:10.2169/internalmedicine.42.424

Cited by 8 publications

(7 citation statements)

References 9 publications

(10 reference statements)

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“…Our case differed from classical HES because of the spontaneous resolution of eosinophilia and the absence of apparent organ dysfunction. Spontaneous remission of HES has been reported in a patient with brain involvement [6].Another peculiar feature of this case was that the hypereosinophilia was possibly caused by eosinophilopoietic cytokine IL-3. Previous studies have suggested that IL-5 is the principal cytokine inducing eosinophilia in HES [5]; however, the levels of IL-5 are not always increased in affected individuals [4].…”

mentioning

confidence: 60%

Interleukin-3-producing CD4+ T-cells support eosinophil survival in a patient with transient hypereosinophilia

Toyabe

Harada

Uchiyama

2003

European Journal of Pediatrics

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A boy with idiopathic hypereosinophilia showed spontaneous resolution after 20 months and presented with an excess production of interleukin-3 from a small population of clonal CD3+CD4+T-cell receptor ab+ T-cells.A 9-month-old boy was referred to our hospital for evaluation of hypereosinophilia. Physical examination revealed a small number of reddish papules on his trunk; no other organ involvement was noted. Cardiac complications were not found despite periodic echographic monitoring. Results of the haematological examination showed a total leukocyte count of 57.0·10 9 cells/l with 81% eosinophils (46.2·10 9 cells/l). Examination of smears of peripheral blood and bone marrow aspirate showed an abundance of mature eosinophils and no blast cells. The possibility of secondary eosinophilia caused by allergic, toxic, parasitic, neoplastic or autoimmune disorders was ruled out. Results of immunophenotypic examination of circulating lymphocytes revealed no increase in unusual cells. Among the levels of eosinophilopoietic cytokines measured, plasma interleukin (IL)-3 level was increased (63 pg/ml, normal <31 pg/ml), while levels of IL-5 and granulocyte/macrophage-colony stimulating factor (GM-CSF) were undetectable. Culture supernatants of peripheral blood lymphocytes of the patient contained high levels of IL-3, whereas the levels of IL-5 and GM-CSF were undetectable. IL-3 was mainly produced from CD4+T-cell receptor (TCR) ab+ T-cells (Table 1). We established a series of T-cell lines producing mainly IL-3 from peripheral blood of the patient. Results of inverse PCR [2] and subsequent sequencing analysis showed that these T-cell lines carried the same complementary determining region 3 (CDR3) sequence of TCRb, suggesting that IL-3-producing T-cells were clonal. Since there were no obvious signs of serious organ involvement, no treatment was started. The patientÕs total eosinophil count gradually decreased and had returned to normal (<1.5·10 5 cells/l) by 20 months after the onset of illness. Concomitantly, levels of IL-3 in plasma and culture supernatant returned to normal and the clonal T-cells producing IL-3 disappeared from peripheral blood. No recurrence was observed during a 40-month follow-up period.Eosinophilia of more than 1.5·10 5 cells/l is characteristic of idiopathic hypereosinophilic syndrome (HES) persisting for at least 6 months without an identifiable underlying cause and with multiple-organ dysfunction [3]. Our case differed from classical HES because of the spontaneous resolution of eosinophilia and the absence of apparent organ dysfunction. Spontaneous remission of HES has been reported in a patient with brain involvement [6].Another peculiar feature of this case was that the hypereosinophilia was possibly caused by eosinophilopoietic cytokine IL-3. Previous studies have suggested that IL-5 is the principal cytokine inducing eosinophilia in HES [5]; however, the levels of IL-5 are not always increased in affected individuals [4]. Our case raises the possibility that IL-3 is a candidate cytokine that ...

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mentioning

confidence: 60%

Interleukin-3-producing CD4+ T-cells support eosinophil survival in a patient with transient hypereosinophilia

Toyabe

Harada

Uchiyama

2003

European Journal of Pediatrics

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“…To date, cellular infiltration into the brain in HES has been verified in three cases (10,13,14). Among these cases, infiltrating cells of the case reported by Battineni et al comprised not only "scattered eosinophils" but also lympho-cytes, histiocytes and plasma cells (14).…”

Section: Discussionmentioning

confidence: 97%

“…However, HES patients with obvious tissue damage should not be observed for 6 months before diagnosis and subsequent treatment (2). In HES, nervous system involvement is sometimes seen and manifests as peripheral neuropathy, recurrent optic neuritis (3), transverse myelitis (4), subdural effusion (5), encephalopathy (6)(7)(8), eosinophilic meningitis (9, 10), benign encephalitis (11,12), mass lesion (10,13,14), brain infarction (6,(15)(16)(17)(18)(19), hemorrhage (6,19), and cerebral venous sinus thrombosis (20,21). The pathogeneses of neurologic dysfunction in HES include [1] direct infiltration of eosinophils into neural tissue, [2] direct secretion of eosinophil products onto neurons or secretion of intracytoplasmic contents into circulation, with subsequent damage to neural tissue, and [3] embolic brain infarction (22).…”

Section: Introductionmentioning

confidence: 99%

Fatal Encephalitis in a Case of Hypereosinophilic Syndrome: MRI and Autopsy Findings

et al. 2011

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A 34-year-old man developed fever and headache, followed by finger tremor and gait disturbance, and was admitted to our hospital about two months after onset. Blood tests showed a white blood cell count of 32,600/μL with an eosinophil count of 22,300/μL. There was no evidence of allergic drug reaction or parasitic infection. Cerebrospinal fluid examination demonstrated mononuclear pleocytosis without eosinophils or atypical cells. Brain MRI showed symmetric lesions bilaterally in the medial temporal lobe, frontobasal and insular regions and medulla oblongata. Herpes simplex virus-DNA was negative in the cerebrospinal fluid. The patient died about four months after onset. Histopathologically, there was infiltration of T cells, B cells and macrophages throughout the whole brain, but eosinophils or atypical cells were absent. Immunohistochemistry for herpes simplex virus type 1 and human herpesvirus 6 was negative. This case suggests that fatal encephalitis may develop in association with hypereosinophilic syndrome.

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“…No published case has previously showed CNS vasculitis. Indeed, we found only four published accounts of HES with any CNS disease that included histopathology [17][18][19][20]. Of these four, one included almost no pathological detail (being principally an MRI study, but mentioning ''reactive gliosis secondary to infarction with abundant intravascular eosinophils'', [19] without vasculitis), and one did not meet the diagnostic criteria for HES (but nonetheless showed no vasculitis) [20].…”

Section: Discussionmentioning

confidence: 99%

“…Various neurological complications have been reported [9][10][11][12][13][14][15], but there are extremely few accounts of CNS pathological changes (and none showing vasculitis) [16][17][18][19][20].…”

Section: Introductionmentioning

confidence: 99%

Idiopathic hypereosinophilic syndrome: a new cause of vasculitis of the central nervous system

et al. 2015

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Idiopathic hypereosinophilic syndrome (IHES) is a primary haematological condition characterised by persistent, otherwise unexplained hypereosinophilia sufficient to cause organ damage. Various neurological complications are reported, but very few have mentioned CNS pathology and none has included CNS vasculitis. Our objective here is to report IHES as a new cause of histopathologically confirmed CNS vasculitis. A 39-year-old man presented with a relapsing sub-acute encephalopathy, with severe headaches, confusion and drowsiness, myoclonus, ataxia and papilloedema. He had a history of nephrotic syndrome 18 years earlier, stable for the past 5 years on low-dose corticosteroids and low-dose tacrolimus (2 mg bd); lichen planus, and (15 years previously) aloplecia totalis. On admission, he had a marked peripheral eosinophilia (up to 9.1 × 10(9)/dL), which—it subsequently became clear—had been intermittently present for 16 years. After extensive investigation, biopsies of brain and bone marrow confirmed diagnoses of cerebral vasculitis, with lymphocytic and macrophage (but not eosinophilic) cellular infiltration of blood vessel walls, and IHES. CNS vasculitis can therefore now be added to the list of neurological complications of IHES. A dramatic and sustained neurological improvement, and likewise of the eosinophilia, following treatment with corticosteroids and cyclophosphamide, emphasises the tractability of this newly described form of CNS vasculitis.

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Spontaneous Remission of a Massive CNS Inflammation with Eosinophilic Infiltrate

Cited by 8 publications

References 9 publications

Interleukin-3-producing CD4+ T-cells support eosinophil survival in a patient with transient hypereosinophilia

Interleukin-3-producing CD4+ T-cells support eosinophil survival in a patient with transient hypereosinophilia

Fatal Encephalitis in a Case of Hypereosinophilic Syndrome: MRI and Autopsy Findings

Idiopathic hypereosinophilic syndrome: a new cause of vasculitis of the central nervous system

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