Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları

GÖKSÜGÜR, Sevil Bilir; Bekdas, Mervan; DEMİRCİOĞLU, Fatih

Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları

Mervan BEKDAŞ, (Abant İzzet Baysal Üniversitesi, Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Bolu, Türkiye)

Sevil Bilir GÖKSÜGÜR, (Abant İzzet Baysal Üniversitesi, Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Bolu, Türkiye)

Fatih DEMİRCİOĞLU (Abant İzzet Baysal Üniversitesi, Tıp Fakültesi, Çocuk Hematoloji Bilim Daklı, Bolu, Türkiye)

Harran Üniversitesi Tıp Fakültesi Dergisi

3 0

Yıl: 2015 Cilt: 12 Sayı: 1 Sayfa Aralığı: 154 - 164 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022

Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları

Öz:

Hemofagositik lenfohistiositoz, çok sayıda sistemi etkileyen, nadir görülen bir hastalıktır. Santral sinir sistemi tutulumu, prognozu etkilemesi ve ciddi sekel oluşturma riski nedeniyle çok önemlidir. Hemofagositik lenfohistiositozun santral sinir sistemi tutulumu ilişkili nörolojik bulgu ve komplikasyonların sıklıkları, özellikleri, morbidite ve mortaliteye etkisini irdeleyen yayın sayısı azdır. Bu yazıda, hemofagositik lenfohistiositozun santral sinir sistemi tutulumu ile ilişkili klinik tabloya dikkat çekmek istedik

Anahtar Kelime:

Konular: Genel ve Dahili Tıp

Central nervous system complications in hemophagocytic lymphohistiocytosis

Öz:

Hemophagocytic lymphohistiocytosis is a rare disease, affecting multiple systems. Central nervous system involvement is very important because of the effect on the prognosis. Arising neurological symptoms as a result of this involvement are included in some publications, but the number of publications related to their frequency, characteristics, and relations with morbidity and mortality is rare. In this article, we wanted to attract attention to the picture of the central nervous system involvement as a result of hemophagocytic lymphohistiocytosis

Anahtar Kelime:

Konular: Genel ve Dahili Tıp

Belge Türü: Makale Makale Türü: Derleme Erişim Türü: Erişime Açık

)Imashuku S, Teramura T, Morimoto A, et al. Recent developments in the management of haemophagocytic lymphohistiocytosis. Expert Opin Pharmacother ;2:1437-48
)Imashuku S, Hibi S, Sako M, et al. Soluble interleukin- receptor: a useful prognostic factor for patients with hemophagocytic lymphohistiocytosis. Blood 1995, :4706-7 1)
)Ravelli A: Macrophage activation syndrome. Curr Opin Rheumatol 2002;14:548-52
)Osugi Y, Hara J, Tagawa S, et al. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood 1997; : 4100-3
)Teruya-Feldstein J, Setsuda J, Yao X, et al. MIP-1 expression in tissues from patients with h e m o p h a g o c y t i c s y n d r o m e . L a b I n v e s t ;79:1583-90
)Schneider EM, Lorenz I, Müller-Rosenberger M, et al. Hemophagocytic lymphohistiocytosis is associated with deficiencies of cellular cytolysis but normal expression of transcripts relevant to killer-cell- inducedapoptosis. Blood 2002;100:2891-8
)Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 2004;124:4-14
)Janka GE: Hemophagocytic syndromes. Blood Rev ;21:245-53
)Henter JI, Elinder G. Incidence in Sweden and clinical f e a t u r e s o f f a m i l i a l h e m o p h a g o c y t i c lymphohistiocytosis. Acta Paediatr Scand 1991;80:428
)Gurgey A, Gogus S, Ozyurek E et al. Primary hemophagocytic lymphohistiocytosis in Turkish children. Pediatr Hematol Oncol 2003;20:367-71
)Behrens EM, Canna SW, Slade K, et al: Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice. J Clin Invest ;121:2264-771)
)De Saint Basile G, Fischer A. Defective cytotoxic granule-mediated cell death pathway impairs T lymphocyte homeostasis. Curr Opin Rheumatol ;15:436-45
)Zhang K, Jordan MB, Marsh RA, et al: Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial hemophagocytic lymphohistiocytosis. Blood ;118:5794-98
)Clementi R, Emmi L, Maccario R, et al. Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Blood 2002;100:2266-67
)Ansuini V, Rigante D, Esposito S. Debate around infection- dependent hemophagocytic syndrome in paediatrics. BMC Infectious Diseases 2013;13:15-23
)Henter JI, Ehrnst A, Andersson J, Elinder G. Familial hemophagocytic lymphohistiocytosis and viral infections. Acta Paediatr 1993;82:369- 72
)Rajagopala S, Singh N. Diagnosing and treating hemophagocytic lymphohistiocytosis in the tropics: Systematic review from the indian subcontinent. Acta Medica Academica 2012;41:161-174
)De Saint Basile G, Menasche G, Fischer A. Molecular mechanisms of biogenesis and exocytosis of cytotoxic granules. Nat Rev Immunol 2010;10:568-79
)Katano H, Cohen JI. Perforin and lymphohistiocytic proliferative disorders. Br J Haematol 2005;128:739-50 )
)Stepp SE, Dufourcq-Lagelouse R, Le Deist F, et al. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science 1999; 286:1957-9
)Menasche G, Feldmann J, Fischer A, et al. Primary hemophagocytic syndromes point to a direct link between lymphocyte cytotoxicity and homeostasis. Immunol Rev 2005;203:165-79
)Zur Stadt U, Schmidt S, Kasper B, et al. Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11. Hum Mol Genet 2005;14:827
)Brochetta C, Vita F, Tiwari N, et al. Involvement of Munc18 isoforms in the regulation of granule exocytosis in neutrophils. Biochim. Biophys. Acta. ;1783:1781-91
)Zur Stadt U, Rohr J, Seifert W, et al. Familial hemophagocytic lymphohistiocytosis type 5 (FHL-5) is caused by mutations in Munc18-2 and impaired binding to syntaxin 11. Am J Hum Genet 2009;85:482- Meeths M,
)Entesarian M, Al-Herz W, et al. Spectrum of clinical presentations in familial hemophagocytic lymphohistiocytosis type 5 patients with mutations in STXBP2. Blood. 2010;116:2635-43
)Janka G: Hemophagocytic lymphohistiocytosis: when the immune system runs amok. Klin Pediatr ;221:278-285
)Machaczka M, Vaktnas J, Klimkowska M, et al. M a l i g n a n c y - a s s o c i a t e d h e m o p h a g o c y t i c lymphohistiocytosis in adults: a retrospective population- based analysis from a single center. Leuk Lymphoma 2011;52:613-191)
)Bryceson YT, Pende D, Maul-Pavicic A, et al. A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes. Blood 2012;119:2754-63
)Lartigue A, Courville P, Auquit I, et al. Role of TLR9 in anti- nucleosome and anti-DNA antibody production in lpr mutation-induced murine lupus. J Immunol 2006;177:1349-54
)Fisman DN. Hemophagocytic syndromes and infection. Emerg Infect Dis 2000;6:601-8
)Rigante D, De Rosa G, Bertoni B, et al. Large pericardial effusion requiring pericardiocentesis as cardinal sign of macrophage activation syndrome in systemic onset juvenile idiopathic arthritis. Rheumatol Int 2007;27:767-70
)Janka G, Imashuku S, Elinder G, et al. Infection and malignancy associated hemophagocytic s y n d r o m e s . S e c o n d a r y h e m o p h a g o c y t i c lymphohistiocytosis. Hematol Oncol Clin North Am ;12:435-44
)Wong K, Chan JK. Reactive hemophagocytic syndrome-a clinicopathologic study of 40 patients in an oriental population. Am J Med 1992;93:177-80
)Henter JI, Nennesmo I. Neuropathologic findings and neurologic symptoms in twenty-three children with hemophagocytic lymphohistiocytosis. J Pediatr ;130:358-65
)Haddad E, Sulis ML, Jabado N, et al. Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. Blood ;89:794-8001) 6 ) G u r g e y A , Ay t a c S , B a l t a G , e t a l .
Lymphohistiocytosis Central Nervous System Involvement in Turkish Children With Primary Hemophagocytic. J Child Neurol 2008;23:1293-99
)Janabi N, Chabrier S, Tardieu M. Endogenous nitric oxide activates prostaglandin F2a production in human microglial cells but not in astrocytes. J Immunol 1996;157:2129
)Ören H. Hemofagositik Sendrom. Türk Çocuk Hematoloji Dergisi 2007;1:7-13
)Horne A, Trottestam H, Aricò M, et al. Frequency and spectrum of central nervous system involvement in 9 3 c h i l d r e n w i t h h a e m o p h a g o c y t i c lymphohistiocytosis. Br J Haematol 2008;140:327-35
)Yang S, Zhang L, Jia C, et al. Frequency and development of CNS involvement in Chinese children with hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2010;54:408-15 1 ) J a n k a G E . F a m i l i a l h a e m o p h a g o c y t i c lymphohistiocytosis. Eur tion of graft failure by a monoclonal antibody (anti-LFA-1-CD11a). J Pediatr ;140:221 Tardieu M,
)Lacroix C, Neven B, et al. Progressive neurologic dysfunctions 20 years after allogeneic bone marrow transplantation for Chediak- Higashi syndrome. Blood ;106:40-42
)Rostasy K, Kolb R, Pohl D, et al. CNS disease as the m a i n m a n i f e s t a t i o n o f h e m o p h a g o c y t i c lymphohistiocytosis in two children. Neuropediatrics ;35:45-91) )Deiva K, Mahlaoui N, Beaudonnet F, et al. CNS involvement at the onset of primary hemophagocytic lymphohistiocytosis. Neurology 2012;78:1150-56 Kim MM
)Yum MS, Choi HW, et al. Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis. Hematol 2012;47:273-80
)Hirst WJ, Layton DM, Singh S, et al. Haemophagocytic lymphohistiocytosis: experience at two U.K. centres. Br J Haematol 1994;88:731-39 Ramachandran B,
)Balasubramanian S, Abhishek N, et al. Profile of hemophagocytic lymphohistiocytosis in children in a tertiary care hospital in India. Indian Pediatr ;48:31-5
)Kieslich M, Vecchi M, Driever PH, et al. Acute encephalopathy as a primary manifestation of hemophagocytic lymphohistiocytosis. Dev Med Child Neurol 2001;43:555-58
)Thompson PA, Allen CE, Horton T, et al. Severe neurologic sideeffects in patients being treated for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2009;52:621-5 0 ) F i l i p o v i c h A H . H e m o p h a g o c y t i c lymphohistiocytosis (HLH) and related disorders.
Hematology Am Soc Hematol Educ Program. :127-31
)Palazzi DL, McClain KL, Kaplan SL. Hemophagocytic syndrome in children: an important diagnostic consideration in fever of unknown origin. Clin Infect Dis 2003;36:306-121)
)Arico 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;10:197-203
)Niece JA, Rogers ZR, Ahmad N, et al. Hemophagocytic lymphohistiocytosis in Texas: observations on ethnicity and race. Pediatr Blood Cancer ;54:424-28
)Ost A, Nilsson-Ardnor S, Henter JI. Autopsy findings i n 2 7 c h i l d r e n w i t h h a e m o p h a g o c y t i c lymphohistiocytosis. Histopathology 1998;32:310-16
)Stapp J, Wilkerson S, Stewart D, et al. Fulminant neonatal liver failure in siblings: probable congenital hemophagocytic lymphohistiocytosis. Pediatr Dev Pathol ;9:239-44
)Ouachee-Chardin M, Elie C, De Saint BG, et al. H e m a t o p o i e t i c s t e m c e l l t r a n s p l a n t a t i o n i n hemophagocytic lymphohistiocytosis: a single-center report of 48 patients. Pediatrics. 2006;117:e743-e750
)Fitzgerald N, McClain KL. Imaging characteristics of hemophagocytic lymphohistiocytosis. Pediatr Radiol ;33:392-401 8 ) P a l a z z i D L , M c C l a i n K L , K a p l a n S L . Hemophagocytic syndrome in children: an important diagnostic consideration in fever of unknown origin. Clin Infect Dis 2003;36:306-12
)Feldmann J, Menasche G, Callebaut I, et al. Severe and progressive encephalitis as a presenting manifestation of a novel missense perforin mutation and impaired cytolytic activity. Blood 2005;105:2658- )
)Gholam C, Grigoriadou S, Gilmour KC, et al. Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management. Clin Exper Immunol 2011;163: 271-83
)Gupta A, Weitzman S, Abdelhaleem M. The role of hemophagocytosis in bone marrow aspirates in the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2008;50:192-94
)Kuwata K, Yamada S, Kinuwaki E, et al. Peripheral hemophagocytosis: an early indicator of advanced s y s t e m i c i n f l a m m a t o r y r e s p o n s e s y n d r o m e / hemophagocytic syndrome. Shock 2006;25:344-50
)Gauvin F, Toledano B, Champagne J, et al. Reactive hemophagocytic syndrome presenting as a component of multiple organ dysfunction syndrome. Crit Care Med ;28:3341-45
)Turtzo LC, Lin DDM, Hartung H, et al. A Neurologic P r e s e n t a t i o n o f F a m i l i a l H e m o p h a g o c y t i c Lymphohistiocytosis Which Mimicked Septic Emboli to the Brain. J Child Neurol 2007;22:863-64
)Schaer DJ, Schleiffenbaum B, Kurrer M, et al. Soluble hemoglobin- haptoglobin scavenger receptor CD163 as a lineagespecific marker in the reactive hemophagocytic syndrome. Eur J Haematol 2005;74:6-10
) Bleesing J, Prada A, Siegel DM, et al. The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor a-chain in macrophage activation syndrome and unrelated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum 2007;56:965-711)
)Rooms L, Fitzgerald N, McClain KL. Hemophagocytic lymphohistiocytosis masquerading as child abuse: presentation of three cases and review of central nervous system findings in hemophagocytic lymphohistiocytosis. Pediatrics 2003;111:636-40
)Chung TW. CNS Involvement in hemophagocytic lymphohistiocytosis: CT and MR findings. Korean J Radiol 2007;8:78-81 9 ) G o o H W, We o n Y C . A s p e c t r u m o f neuroradiological findings in children with haemophagocytic lymphohistiocytosis. Pediatr Radiol 2007;37:1110-17
)Tang YM, Xu XJ. Advances in Hemophagocytic Ly m p h o h i s t i o c y t o s i s : P a t h o g e n e s i s , E a r l y Diagnosis/Differential Diagnosis, and Treatment. The Scientific World J 2011;11:697-708
)Munoz Ruano MM, Castillo M. Brain CT and MR i m a g i n g i n f a m i l i a l h e m o p h a g o c y t i c lymphohistiocytosis. AJR Am J Roentgenol ;170:802-5
)Jordan MB, Allen CE, Weitzman S, et al. How I treat hemophagocytic lymphohistiocytosis. Blood ;118:4041-52
)Bör Ö. Çocukluk Çağında Hemofagositik Sendromlar. Güncel Pediatri 2006;4:100-1
)Bode SFN, Lehmberg K, Maul-Pavicic A, et al. Recent advances in the diagnosis and treatment of hemophagocytic lymphohistiocytosis. Arthritis Research&Therapy 2012;14:213-16
)Gupta S, Weitzman S. Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy. Expert Rev Clin Immunol 2010;6:137 541)
)Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr 2007;166:95-109
)Trottestam H, Berglof E, Horne A, et al. Risk factors for early death in children with haemophagocytic lymphohistiocytosis. Acta Paediatr 2011;101:313- 18
)Sung L, King SM, Carcao M, et al. Adverse outcomes in primary hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol 2002;24:550-54

APA	Bekdas M, GÖKSÜGÜR S, DEMİRCİOĞLU F (2015). Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. , 154 - 164.
Chicago	Bekdas Mervan,GÖKSÜGÜR Sevil Bilir,DEMİRCİOĞLU Fatih Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. (2015): 154 - 164.
MLA	Bekdas Mervan,GÖKSÜGÜR Sevil Bilir,DEMİRCİOĞLU Fatih Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. , 2015, ss.154 - 164.
AMA	Bekdas M,GÖKSÜGÜR S,DEMİRCİOĞLU F Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. . 2015; 154 - 164.
Vancouver	Bekdas M,GÖKSÜGÜR S,DEMİRCİOĞLU F Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. . 2015; 154 - 164.
IEEE	Bekdas M,GÖKSÜGÜR S,DEMİRCİOĞLU F "Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları." , ss.154 - 164, 2015.
ISNAD	Bekdas, Mervan vd. "Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları". (2015), 154-164.

APA	Bekdas M, GÖKSÜGÜR S, DEMİRCİOĞLU F (2015). Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. Harran Üniversitesi Tıp Fakültesi Dergisi, 12(1), 154 - 164.
Chicago	Bekdas Mervan,GÖKSÜGÜR Sevil Bilir,DEMİRCİOĞLU Fatih Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. Harran Üniversitesi Tıp Fakültesi Dergisi 12, no.1 (2015): 154 - 164.
MLA	Bekdas Mervan,GÖKSÜGÜR Sevil Bilir,DEMİRCİOĞLU Fatih Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. Harran Üniversitesi Tıp Fakültesi Dergisi, vol.12, no.1, 2015, ss.154 - 164.
AMA	Bekdas M,GÖKSÜGÜR S,DEMİRCİOĞLU F Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. Harran Üniversitesi Tıp Fakültesi Dergisi. 2015; 12(1): 154 - 164.
Vancouver	Bekdas M,GÖKSÜGÜR S,DEMİRCİOĞLU F Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları. Harran Üniversitesi Tıp Fakültesi Dergisi. 2015; 12(1): 154 - 164.
IEEE	Bekdas M,GÖKSÜGÜR S,DEMİRCİOĞLU F "Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları." Harran Üniversitesi Tıp Fakültesi Dergisi, 12, ss.154 - 164, 2015.
ISNAD	Bekdas, Mervan vd. "Hemofagositik lenfohistiositozda santral sinir sistemi komplikasyonları". Harran Üniversitesi Tıp Fakültesi Dergisi 12/1 (2015), 154-164.