Öz
Hemophagocytic lymphohistiocytosis is characterized by high body temperature, splenomegaly, bicytopenia, hypertriglyceridemia, hyperferritinemia, hypofibrinogenemia, decreased natural killer cell activity, increased soluble CD25 activity and the presence of hemophagocytosis in organs such as the bone marrow, the lymph glands, spleen and liver. We described a patient who was admitted to the intensive care unit with a diagnosis of diabetic ketoacidosis and transferred to the ward when her general condition improved, but who was also diagnosed with hemophagocytic lymphohistiocytosis by bone marrow investigation performed due to a resistant fever, splenomegaly, bicytopenia and hyperferritinemia
Anahtar Kelimeler
Kaynakça
- Oren H. Hemophagocytic syndrome. Turk J Hematol 2007; 1:7-13.
- Filipovich AH. Hemophagocytic lymphohistiocytosis and related disorders. Curr Opin Allergy Clin İmmunol 2006;6:410-5. [CrossRef]
- Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C. Infections associated with haemophagocytic syndrome. Lancet Infect Dis 2007; 7:814-22. [CrossRef]
- Maakaroun NR, Moanna A, Jacob JT, Albrecht H.Viral infections associated with haemophagocytic syndrome. Rev Med Virol 2010;20: 93-105. [CrossRef]
- Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr 2007; 166: 95-109. [CrossRef]
- Minoia F, Davi S, Home A, Demirkaya E, Bovis F, Li C. Clinical features
- Wada Y, Sato M, Saito A, Gejyo T. Infection-associated Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2014;22:1162-6. [CrossRef] hemophagocytic syndrome in a diabetic patient undergoing chronic hemodialysis. Clin Exp Nephrol 2003; 7:163-6. [CrossRef]
- Makino C, Yanagi T, Toyama J, Yamada H, Seta K, Kitamura S. Case of diabetes mellitus succumbing to multiple organ failure due to hemophagocytic syndrome complicating mucormycosis. Nihon Naika Gakkai Zasshi 1999;88:133-4.
- Oren H, Gulen H, Uçar C, Duman M, Irken G. Successful Treatment of Infection-Associated Hemophagocytic Syndrome with Intravenous Immunoglobulin. Turk J Haematol 2003; 20: 95-9.
- Lu XX, Gao J. Research advances in the pathogenesis, diagnosis treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational multicenter study of 362 patients. Arthritis Rheumatol 2014;66:3160- 9. [CrossRef]
Öz
Hemofagositik lenfohistiositoz, yüksek ateş, splenomegali, bisitopeni, hipertrigliseridemi, hiperferritinemi, hipofibrinojenemi, doğal öldürücü hücre aktivitesinde azalma, çözünür solüble CD25 aktivitesinde artma, kemikiliği, lenfbezi, dalak, karaciğer gibi organlarda hemofagositoz varlığı ile karakterizedir. Burada diabetik ketoasidoz tanısı ile yoğun bakım ünitesine yatırılan,genel durumunun düzelmesi üzerine serviste takibine devam edilen ancak dirençli ateş, splenomegali, bisitopeni, hiperferritinemi nedeniyle yapılan kemikiliği incelemesi ile hemofagositik lenfohistiositoz tanısı alan hasta sunulmuştur
Anahtar Kelimeler
Kaynakça
- Oren H. Hemophagocytic syndrome. Turk J Hematol 2007; 1:7-13.
- Filipovich AH. Hemophagocytic lymphohistiocytosis and related disorders. Curr Opin Allergy Clin İmmunol 2006;6:410-5. [CrossRef]
- Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C. Infections associated with haemophagocytic syndrome. Lancet Infect Dis 2007; 7:814-22. [CrossRef]
- Maakaroun NR, Moanna A, Jacob JT, Albrecht H.Viral infections associated with haemophagocytic syndrome. Rev Med Virol 2010;20: 93-105. [CrossRef]
- Janka GE. Familial and acquired hemophagocytic lymphohistiocytosis. Eur J Pediatr 2007; 166: 95-109. [CrossRef]
- Minoia F, Davi S, Home A, Demirkaya E, Bovis F, Li C. Clinical features
- Wada Y, Sato M, Saito A, Gejyo T. Infection-associated Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2014;22:1162-6. [CrossRef] hemophagocytic syndrome in a diabetic patient undergoing chronic hemodialysis. Clin Exp Nephrol 2003; 7:163-6. [CrossRef]
- Makino C, Yanagi T, Toyama J, Yamada H, Seta K, Kitamura S. Case of diabetes mellitus succumbing to multiple organ failure due to hemophagocytic syndrome complicating mucormycosis. Nihon Naika Gakkai Zasshi 1999;88:133-4.
- Oren H, Gulen H, Uçar C, Duman M, Irken G. Successful Treatment of Infection-Associated Hemophagocytic Syndrome with Intravenous Immunoglobulin. Turk J Haematol 2003; 20: 95-9.
- Lu XX, Gao J. Research advances in the pathogenesis, diagnosis treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational multicenter study of 362 patients. Arthritis Rheumatol 2014;66:3160- 9. [CrossRef]
Ayrıntılar
| Birincil Dil | Türkçe |
|---|---|
| Bölüm | Case Report |
| Yazarlar | |
| Yayımlanma Tarihi | 1 Haziran 2019 |
| Yayımlandığı Sayı | Yıl 2019Sayı: 2 |