Abstract
Gereç ve Yöntem: Bir-onbir yaş aralığında 6 hasta irritabilite, nöbet, ataksi, nistagmus, yürüme ve görme bozukluğu, psikomotor gelişim geriliği gibi nörolojik bulgularla hastanemize baş vurdu ve nörolojik muayene sonrasında kranyal manyetik rezonans görüntüleme (MRG) incelemesi gerçekleştirildi.
Bulgular: Hastalarımıza klinik ve laboratuvar değerlendirmeleri sonrasında yapılan kemik iliği biyopsisi ile HLH tanısı konuldu. Hastalarımızın tamamında SSS tutulumunu gösteren kraniyal MRG bulguları saptandı. MRG incelemesinde T2 ağırlıklı ve FLAIR sekanslarında serebral hemisferlerde hiperintens odaklar ve postkontrast serilerde patolojik kontrastlanma ortak bulgular olarak belirlendi.
Sonuç: HLH hastalığının santral sinir sistemi tutulumu yüksek mortalite ve morbidite ile seyretmektedir. Kraniyal MRG SSS tutulumunu göstermede önemli rol oynamaktadır.
References
- Ozgen B, Karli-Oguz K, Tavil B, Gurgey A. Diffusion-weighted cranial MR. Imaging findings in a patient with hemophagocytic syndrome AJNR 2006; 27(6):1312-14.
- Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis: The FHL Study Group of the Histiocyte Society. Semin Oncol 1991;18(1):29-33.
- Henter JI, Nennesmo I. Neuropathologic findings and neurologic symptomsin twenty-three children with hemophagocytic lymphohistiocytosis. J Pediatr 1997;130(3):358-65.
- Balcı YI, Özgürler Akpınar F, Polat A, et al. Hemophagocytic lymphohistiocytosis case with newly defined UNC13D (c.175G>C; p.Ala59Pro) mutation and a rare complication. Turk J Haematol 2015;32(4):355-8.
- Yang S, Zhang L, Jia C, Ma H, Henter J-I, Shen K. Frequency and development of CNS involvement in Chinese children with hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2010; 54(3):408-15.
- Haddad E, Sulis ML, Jabado N. Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. Blood 1997;89(3):794-800.
- Goo HW, Weon YC. A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis. Pediatr Radiol 2007; 37(11):110-7.
- Ouachée-Chardin M, Elie C, de Saint Basile G, et al. Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: A single center report of 48 patients. Pediatrics 2006;117(4):743-50.
- Özdemir MA, Torun YA, Yıkılmaz A, Karakükcü M, Çoban D. Hemofagositik lenfohistiositozda kranial MR ve proton MR spektroskopi bulguları. Çocuk Sağlığı ve Hastalıkları Dergisi 2006;49(4): 307-11.
- Beken B. Hacettepe Üniveristesi Tıp Fakültesi, Çocuk Hematoloji Ünitesinde izlenen familyal hamofagositik lenfohistiyositoz hastalarının değerlendirilmesi. Uzmanlık Tezi 2013.
- Blanche S, Caniglia M, Girault D, et al. Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: A single center study of 22 cases. Blood 1991;78(1):51-4.
- Nespoli L, Locatelli F, Bonetti F, et al. Familial hemophagocytic lymphohistiocytosis treated with allogenic bone marrow transplantation. Bone marrow transplant 1991;7(Suppl 3):139-42.
- Aricò M, Janka G, Fischer A, et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia 1996;10(2):197-201.
Abstract
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.
Keywords
Hemophagocytic lymphohistiocytosis magnetic resonance imaging central nervous system immune
References
- Ozgen B, Karli-Oguz K, Tavil B, Gurgey A. Diffusion-weighted cranial MR. Imaging findings in a patient with hemophagocytic syndrome AJNR 2006; 27(6):1312-14.
- Henter JI, Elinder G, Ost A. Diagnostic guidelines for hemophagocytic lymphohistiocytosis: The FHL Study Group of the Histiocyte Society. Semin Oncol 1991;18(1):29-33.
- Henter JI, Nennesmo I. Neuropathologic findings and neurologic symptomsin twenty-three children with hemophagocytic lymphohistiocytosis. J Pediatr 1997;130(3):358-65.
- Balcı YI, Özgürler Akpınar F, Polat A, et al. Hemophagocytic lymphohistiocytosis case with newly defined UNC13D (c.175G>C; p.Ala59Pro) mutation and a rare complication. Turk J Haematol 2015;32(4):355-8.
- Yang S, Zhang L, Jia C, Ma H, Henter J-I, Shen K. Frequency and development of CNS involvement in Chinese children with hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2010; 54(3):408-15.
- Haddad E, Sulis ML, Jabado N. Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. Blood 1997;89(3):794-800.
- Goo HW, Weon YC. A spectrum of neuroradiological findings in children with haemophagocytic lymphohistiocytosis. Pediatr Radiol 2007; 37(11):110-7.
- Ouachée-Chardin M, Elie C, de Saint Basile G, et al. Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: A single center report of 48 patients. Pediatrics 2006;117(4):743-50.
- Özdemir MA, Torun YA, Yıkılmaz A, Karakükcü M, Çoban D. Hemofagositik lenfohistiositozda kranial MR ve proton MR spektroskopi bulguları. Çocuk Sağlığı ve Hastalıkları Dergisi 2006;49(4): 307-11.
- Beken B. Hacettepe Üniveristesi Tıp Fakültesi, Çocuk Hematoloji Ünitesinde izlenen familyal hamofagositik lenfohistiyositoz hastalarının değerlendirilmesi. Uzmanlık Tezi 2013.
- Blanche S, Caniglia M, Girault D, et al. Treatment of hemophagocytic lymphohistiocytosis with chemotherapy and bone marrow transplantation: A single center study of 22 cases. Blood 1991;78(1):51-4.
- Nespoli L, Locatelli F, Bonetti F, et al. Familial hemophagocytic lymphohistiocytosis treated with allogenic bone marrow transplantation. Bone marrow transplant 1991;7(Suppl 3):139-42.
- Aricò M, Janka G, Fischer A, et al. Hemophagocytic lymphohistiocytosis. Report of 122 children from the International Registry. FHL Study Group of the Histiocyte Society. Leukemia 1996;10(2):197-201.
Details
| Primary Language | Turkish |
|---|---|
| Subjects | Health Care Administration |
| Journal Section | Research Articles |
| Authors | |
| Publication Date | March 1, 2018 |
| Submission Date | September 4, 2016 |
| Published in Issue | Year 2018 |
Cited By
YİRMİALTI HAFTALIK GEBEDE PARVOVİRUS B19 KAYNAKLI HEMOFAGOSİTİK LENFOHİSTİYOSİTOZ
Haliç Üniversitesi Sağlık Bilimleri Dergisi
Sevil ALKAN
https://doi.org/10.48124/husagbilder.901799