Neuroradiologic findings and follow‐up with magnetic resonance imaging of the genetic forms of haemophagocytic lymphohistiocytosis with CNS involvement

Rego, Inês; Severino, Mariasavina; Micalizzi, Concetta; Faraci, Maura; Pende, Daniela; Dufour, Carlo; Aricò, Maurizio; Rossi, Andrea

doi:10.1002/pbc.23405

Cited by 31 publications

(36 citation statements)

References 18 publications

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“…Neurologic symptoms are inconstant, and seizures, cranial nerve abnormalities, ataxia, motor symptoms, irritability, and coma have been reported [6,1]. While not unusual, the typical presentation of HLH is not neurologic; but, when seen, is a poor prognostic indicator [5].…”

Section: Discussionmentioning

confidence: 98%

“…In addition, correlation with bone marrow, spleen, lymph node, or other solid organ biopsy showing hemophagocytosis can aid in diagnosis. Previous case reports have described imaging findings of CNS involvement; however, these cases have been predominantly of children [4,5]. We present a rare case of an adult patient with a clinical and radiological diagnosis of HLH with CNS involvement.…”

Section: Introductionmentioning

confidence: 85%

“…7 preferentially supratentorial involvement [5]. In pediatric patients, the histopathologic findings of CNS involvement have been classified by the microscopic stages of disease.…”

Section: Accepted Manuscriptmentioning

confidence: 99%

“…In pediatric patients, the histopathologic findings of CNS involvement have been classified by the microscopic stages of disease. The stages are delineated by increasing severity: stage I involves leptomeningeal lymphocyte and histiocyte infiltration, stage II progresses to adjacent parenchymal involvement with perivascular infiltration, and stage III represents massive tissue infiltration, blood vessel destruction, gliosis, and tissue necrosis [5,7].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

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Central nervous system imaging findings of hemophagocytic syndrome

2015

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Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 85%

“…7 preferentially supratentorial involvement [5]. In pediatric patients, the histopathologic findings of CNS involvement have been classified by the microscopic stages of disease.…”

Section: Accepted Manuscriptmentioning

confidence: 99%

Section: Accepted Manuscriptmentioning

confidence: 99%

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Central nervous system imaging findings of hemophagocytic syndrome

2015

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“…Görüntülemede odaksal veya yaygın parankim tutulumu ve damar çevrelerin-de spesifik olmayan infiltrasyonlar görülebilir. Kemoterapi ve/veya KHN tedavisi sonrası bu bulgularda düzelme saptanabiliyor olsa da kalıcı nörolojik sekeller olduğu da gösterilmiştir (17,18) . Literatürdeki olgu sayısı çok bir çalışmada, en sık bildirilmiş SSS bulguları nöbet ve meningismustur (17) .…”

Section: Discussionunclassified

Hemophagocytic Lymphohistiocytosis Single Center Experience

Töret¹,

Ay²,

Karapınar³

et al. 2018

BUCH

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ÖZAmaç: Hemofagositk Lenfohistiyositoz (HLH) sitokin fırtınası sonucu gelişen kontrolsüz hemofagositozun görüldüğü nadir bir hastalıktır. Ateş, splenomegali, hiperferritinemi ve sitopeni sendromun en iyi bilinen bulgularıdır. Ailesel (primer) tetikleyen altta yatan bir hastalığın olduğu akkiz (sekonder) HLH olmak üzere sınıflandırılır. Akkiz nedenler arasında infeksiyonlar, maliniteler, otoimmün hastalıklar ve bazı metabolik hastalıklar sayılabilir. Tedavinin temelini immünsüpresyon ve apopitotik kemoterapi oluşturmaktadır. Ailesel HLH veya ailesel olmayan persistan HLH'de küratif tedavi seçeneği kök hücre naklidir. Yöntem: Kliniğimizde tanı alan ve tedavi edilen 46 HLH olgusunu tanı kriterleri ve tedavi planları açısından retrospektif olarak irdeledik. Bulgular: Tanı kriteri olarak ateşin yanı sıra hiperferritinemi ve hemofagositozun en sık kaydedilen tanı kriterleri olduklarını belirledik. Olguların %46'sı dekzametazon, siklosporin A (CsA) ve etoposidden oluşan HLH-2004 tedavi kılavuzundaki üçlü tedaviye gereksinim duyduğunu saptadık. Sonuç: Sonuç olarak, hektik-persistan ateşle başvuran çocuklarda HLH aramanın erken tanı ve tedavi için önemli olduğunu düşünüyoruz.Anahtar kelimeler: Ateş, çocukluk çağı, hemofagositik lenfohistiyositoz, tedavi ABSTRACT Objective: Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder which develops as a result of cytokine storm syndrome due to uncontrolled hemophagocytosis. Fever, splenomegaly, hyperferritinemia and cytopenias are well-known clinical manifestations of the disease. Hemophagocytic lymphohistiocytosis is classified as familial (primary/genetic) and secondary (acquired) where an underlying disease is present. Among acquired causes infections, malignancies, autoimmune disorders and some metabolic disorders may be enumerated. The basis of treatment for HLH is immunosuppression and apoptotic chemotherapy. The curative treatment alternative for familial or non-familial persistent HLH is stem cell transplantation. Methods: We retrospectively analyzed 46 HLH cases diagnosed and treated in our clinic in terms of diagnostic criteria and treatment plans. Results: We identified the most frequently recorded diagnostic criteria as fever, hemophagocytosis and hyperferritinemia. We found that 46% of the cases needed treatment consisting of dexamethasone, cyclosporine (CsA) and etoposide according to HLH-2004 treatment guideline. Conclusion: As a result, we think that search for HLH in children who presented with hectic-persistent fever is important for the early diagnosis and treatment of HLH.

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A Diagnostic Dilemma: Similarity of Neuroradiological Findings in Central Nervous System Hemophagocytic Lymphohistiocytosis and Aspergillosis

Foley

Borders

Kurt

2016

Pediatric Blood & Cancer

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Central nervous system (CNS) involvement in the context of hemophagocytic lymphohistiocytosis (HLH) is not uncommon. Given the immunosuppressive nature of HLH therapy, infectious complications are also seen. We describe a 9-year-old male who developed acute neurological decline secondary to aspergillosis while undergoing HLH therapy. The significant overlap observed in CNS neuroimaging of HLH and aspergillosis and the subtleties that may help differentiate the two are discussed. The importance of obtaining tissue for definitive diagnosis is underscored.

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Neuroradiologic findings and follow‐up with magnetic resonance imaging of the genetic forms of haemophagocytic lymphohistiocytosis with CNS involvement

Cited by 31 publications

References 18 publications

Central nervous system imaging findings of hemophagocytic syndrome

Central nervous system imaging findings of hemophagocytic syndrome

Hemophagocytic Lymphohistiocytosis Single Center Experience

A Diagnostic Dilemma: Similarity of Neuroradiological Findings in Central Nervous System Hemophagocytic Lymphohistiocytosis and Aspergillosis

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