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Ege Journal of Medicine
2018, Cilt 57, Sayı 1, Sayfa(lar) 014-018
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Cranial MRI findings in hemophagocytic lymphohistiocytosis
Cenk Eraslan1, Elçin Aydın2, Eltan Maharramova1, Akkız Şahin3, Hamiyet Hekimci Özdemir3, Deniz Yılmaz Karapınar3, Cem Çallı1, Ömer Kitiş1
1Ege Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, İzmir, Türkiye
2Başkent Üniversitesi Tıp Fakültesi, Radyoloji Anabilim Dalı, İzmir, Türkiye
3Ege Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Hematoloji Bilim Dalı, İzmir, Türkiye
Keywords: Hemophagocytic lymphohistiocytosis, magnetic resonance imaging, central nervous system, immune

Aim: Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome characterized by hyperinflammation causing uncontrolled immune response. It is classified as primary and secondary according to etiology. Fever, splenomegaly, hepatitis are among the clinical findings. The diagnosis is made with tissue sampling. The central nervous system (CNS) involvement of familial HLH patients is a factor that affects the prognosis and course of the disease. The findings of CNS involvement include progressive encephalopathy, irritability, attack, cranial nerve paralysis, ataxy, nistagmus, walking and visual impairment, psychomotor development deficiency. In this study, we aimed at presenting the radiologic imaging findings of 6 cases with CNS involvement.

Materials and Methods: Six patients between the ages of 1-11 years applied to our hospital with neurological findings such as irritability, attack, cranial nerve paralysis, ataxy, nistagmus, walking and visual impairment, psychomotor development deficiency. Following the neurological examination, the cranial magnetic resonance imaging (MRI) examination was conducted.

Results: HLH diagnosis was made through bone marrow biopsy after clinical and laboratory evaluations. Cranial MRI findings indicating CNS involvement were observed in all of our patients. Hyperintense foci in cerebral hemispheres and pathological contrast enhancement in postcontrast series were defined in T2 and FLAIR sequences in MRI examination.

Conclusion: The central nervous system involvement of HLH disease is observed with high mortality and morbidity. Cranial MRI plays a significant role in revealing CNS involvement.


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