A Patient Admitted With Diabetic Ketoacidosis and Developing Secondary Hemophagocytic Syndrome
Hemophagocytic lymphohistiocytosis is characterized by high body temperature, splenomegaly, bicytopenia,hypertriglyceridemia, hyperferritinemia, hypofibrinogenemia, decreased natural killer cell activity, increasedsoluble CD25 activity and the presence of hemophagocytosis in organs such as the bone marrow, the lymphglands, spleen and liver. We described a patient who was admitted to the intensive care unit with a diagnosisof diabetic ketoacidosis and transferred to the ward when her general condition improved, but who was alsodiagnosed with hemophagocytic lymphohistiocytosis by bone marrow investigation performed due to a resistantfever, splenomegaly, bicytopenia and hyperferritinemia.
DIABETIK KETOASIDOZ TANISIYLA YATIRILAN VE SEKONDER HEMOFAGOSITIK SENDROM GELIŞEN BIR OLGU
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.
___
- 1. Oren H. Hemophagocytic syndrome. Turk J Hematol 2007; 1:7-13.
- 2. Filipovich AH. Hemophagocytic lymphohistiocytosis and related
disorders. Curr Opin Allergy Clin İmmunol 2006;6:410-5. [CrossRef]
- 3. 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]
- 4. Maakaroun NR, Moanna A, Jacob JT, Albrecht H.Viral infections
associated with haemophagocytic syndrome. Rev Med Virol 2010;20:
93-105. [CrossRef]
- 5. Janka GE. Familial and acquired hemophagocytic
lymphohistiocytosis. Eur J Pediatr 2007; 166: 95-109. [CrossRef]
- 6. Wada Y, Sato M, Saito A, Gejyo T. Infection-associated
hemophagocytic syndrome in a diabetic patient undergoing
chronic hemodialysis. Clin Exp Nephrol 2003; 7:163-6. [CrossRef]
- 7. 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.
- 8. 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.
- 9. Lu XX, Gao J. Research advances in the pathogenesis, diagnosis
and treatment of hemophagocytic lymphohistiocytosis in children.
Zhongguo Shi Yan Xue Ye Xue Za Zhi. 2014;22:1162-6. [CrossRef]
- 10. Minoia F, Davi S, Home A, Demirkaya E, Bovis F, Li C. Clinical features
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]