Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature

BUZGAN, TURAN; DEMİRÖZ, Ali P.; UĞRAŞ, Serdar; IRMAK, HASAN; Akdeniz, Hayrettin

Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature

Hayrettin AKDENİZ, (Yüzüncü Yıl Üniversitesi, Tıp Fakültesi, İnfeksiyon Hastalıkları Anabilim Dalı, Van, Türkiye)

Hasan IRMAK, (Girilmedi)

Turan BUZGAN, (Girilmedi)

Serdar UĞRAŞ, (Girilmedi)

Ali P. DEMİRÖZ (Girilmedi)

Turkish Journal of Gastroenterology

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Yıl: 2000 Cilt: 11 Sayı: 2 Sayfa Aralığı: 166 - 169 Metin Dili: Türkçe İndeks Tarihi: 29-07-2022

Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature

Öz:

16 yaşında bir erkek çocuğu 20 gün önce gelişen ateş, iştahsızlık, sarılık, idrar koyulaşması ve son zamanlarda başlayan burun kanaması şikayetleriyle kliniğimize yatırıldı. Fizik muayenede skleralar ikterik, genel lenfadenopati, batında yaygın hassasiyet ve belirgin hepatosplenomegali saptandı. Laboratuvar incelemelerinde bisitopeni, koagülopati, hipofibrinojenemi, hipoproteinemi, ve yüksek eritrosit sedimentasyon hızı, C-reactive protein, serum bilirubini, transaminazlar, alkalen fosfataz, laktik dehidrojenaz, gama-glutamil transpeptidaz, ve trigliserid değerleri saptandı. Hepatitis B yüzey antijeni ve hepatitis B core IgM antikoru pozitif bulundu. Lenf düğümü biyopsisinde belirgin eritrofagositoz gösteren histiyositer seriden hücrelerin lenf nodunu infiltre ettiği görüldü. Hastaya hepatit B virus infeksiyonu ile ilişkili hemofagositik sendrom tanısı kondu. Klinik hızlı bir seyir takip etti ve hasta yatışının 12. günü ex oldu. Bu olgu dolayısıyla, hepatit B infeksiyonu olan hastalarda açıklanamayan ateş, trombositopeni sonucu lokal kanamalar ve hızlı klinik kötüleşme görüldüğünde tanı için erken kemik iliği veya lenf düğümü biyopsisi alınmasını ve tanıdan sonra yoğun etkin tedavinin yapılmasını öneriyoruz.

Anahtar Kelime: Erken tanı Trombositopeni Hepatit B yüzey antijenleri Hepatit B Hepatit B kor antijenleri Nedeni bilinmeyen ateş

Konular: Cerrahi

Hepatit B virüsü infeksiyonu ile ilişkili fatal hemofagositik sendrom: Olgu sunumu ve literatürün gözden geçirilmesi

Öz:

A 16 year old boy was admitted with a 20 day history of fever, loss of appetite, jaundice and dark urine, and recent epistaxis. Physical examination revealed icteric sclera, generalized lymphadenopathy, diffuse abdominal tenderness and marked hepatosplenomegaly. Laboratory findings showed bicytopenia, coagulopathy, hypofibrinogenemia and hypoproteinemia in addition to elevated ESR, C-reactive protein, serum bilirubin, transaminases, alkaline phosphates, lactic dehydrogenase, gamma-glutamyl transpeptidase, and triglyceride le-vels. Hepatitis-B surface antigen and hepatitis B core IgM antibody were positive, while lymph node biopsy demonstrated infiltration with histiocytic series displaying marked erythrophagocytosis. The patient was diagnosed with hepatitis B virus infection-associated hemophagocytic syndrome. The condition deteriorated rapidly and he died 12 days after admission. This report suggests that in patients with hepatitis B infection presenting unexplained fever, localized bleeding due to thrombocy topenia and showing a rapid clinical deterioration, early bone marrow or lymph node biopsy for diagnosis and intensive therapy should be performed.

Anahtar Kelime: Hepatitis B Hepatitis B Core Antigens Fever of Unknown Origin Early diagnosis Thrombocytopenia Hepatitis B Surface Antigens

Konular: Cerrahi

Belge Türü: Makale Makale Türü: Diğer Erişim Türü: Erişime Açık

1) Shimazaki C, Inaba T, Shimura K, et al. B-cell lymphoma associated with haemophagocytic syndrome: a clinical, immunological and cytogenetic study. Br J Haematol 1999; 104: 672-9.
2) Hess CE. Hairy cell leukemia, malignant histiocytosis, and related disorders. In: Lee GR, Bithell TC, Foerster J, et al, (Editors). Wintrobe’s Clinical Hematology, Vol 2, 9th ed, Philadelphia, Lea and Febiger 1993; 2171-201.
3) Wong KF, Chan JKC. Reactive hemophagocytic syndrome- a clinicopathologic study of 40 patients in an oriental population. Am J Med 1992; 93: 177-80.
4) Risdall RJ, McKenna RW, Nesbit ME, et al. Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer 1979; 44: 993-1002.
5) Yang CW, Lee JH, Kim YG, et al. Tuberculosis-associated hemophagocytic syndrome in a hemodialysis patient: case report and review of the literature. Nephron 1996; 72: 690-2.
6) Favara BE. Hemophagocytic lymphohistiocytosis: a hemophagocytic syndrome. Semin Diagn Pathol 1992; 9: 63-74.
7) Jaffe ES, Costa J, Fauci AS, et al. Malignant lymphoma and erythrophagocytosis simulating malignant histiocytosis. Am J Med 1983; 75: 741-9.
8) Okano M, Gross TG. Epstein-Barr virus-associated hemophagocytic syndrome and fatal infectious mononucleosis. Am J Hematol 1996; 53: 111-5.
9) Akashi K, Hayashi S, Gondo H, et al. Involvement of interferon-gamma and macrophage colony-stimulating factor in pathogenesis of haemophagocytic lymphohistiocytosis in adults. Br J Haematol 1994; 67: 243-7.
10) Ohga S, Matsuzaki A, Nishizaki M, et al. Inflammatory cytokines in virus-associated hemophagocytic syndrome. Interferon-gamma as a sensitive indicator of disease acti-vity. Am J Pediatr Hematol Oncol 1993; 15: 291-8.

APA	Akdeniz H, IRMAK H, BUZGAN T, UĞRAŞ S, DEMİRÖZ A (2000). Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. , 166 - 169.
Chicago	Akdeniz Hayrettin,IRMAK HASAN,BUZGAN TURAN,UĞRAŞ Serdar,DEMİRÖZ Ali P. Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. (2000): 166 - 169.
MLA	Akdeniz Hayrettin,IRMAK HASAN,BUZGAN TURAN,UĞRAŞ Serdar,DEMİRÖZ Ali P. Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. , 2000, ss.166 - 169.
AMA	Akdeniz H,IRMAK H,BUZGAN T,UĞRAŞ S,DEMİRÖZ A Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. . 2000; 166 - 169.
Vancouver	Akdeniz H,IRMAK H,BUZGAN T,UĞRAŞ S,DEMİRÖZ A Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. . 2000; 166 - 169.
IEEE	Akdeniz H,IRMAK H,BUZGAN T,UĞRAŞ S,DEMİRÖZ A "Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature." , ss.166 - 169, 2000.
ISNAD	Akdeniz, Hayrettin vd. "Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature". (2000), 166-169.

APA	Akdeniz H, IRMAK H, BUZGAN T, UĞRAŞ S, DEMİRÖZ A (2000). Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. Turkish Journal of Gastroenterology, 11(2), 166 - 169.
Chicago	Akdeniz Hayrettin,IRMAK HASAN,BUZGAN TURAN,UĞRAŞ Serdar,DEMİRÖZ Ali P. Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. Turkish Journal of Gastroenterology 11, no.2 (2000): 166 - 169.
MLA	Akdeniz Hayrettin,IRMAK HASAN,BUZGAN TURAN,UĞRAŞ Serdar,DEMİRÖZ Ali P. Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. Turkish Journal of Gastroenterology, vol.11, no.2, 2000, ss.166 - 169.
AMA	Akdeniz H,IRMAK H,BUZGAN T,UĞRAŞ S,DEMİRÖZ A Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. Turkish Journal of Gastroenterology. 2000; 11(2): 166 - 169.
Vancouver	Akdeniz H,IRMAK H,BUZGAN T,UĞRAŞ S,DEMİRÖZ A Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature. Turkish Journal of Gastroenterology. 2000; 11(2): 166 - 169.
IEEE	Akdeniz H,IRMAK H,BUZGAN T,UĞRAŞ S,DEMİRÖZ A "Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature." Turkish Journal of Gastroenterology, 11, ss.166 - 169, 2000.
ISNAD	Akdeniz, Hayrettin vd. "Fatal hemophagocytic syndrome associated with Hepatitis B virus infection: Case report and review of the literature". Turkish Journal of Gastroenterology 11/2 (2000), 166-169.