Expression of Hemophagocytic Lymphohistiocytosis with Hepatic Dysfunction in a 50 days Old Infant

Borjian, L.; Nazmieh, H.

Volume 6, Issue 3 (12-2007) JRUMS 2007, 6(3): 213-216 | Back to browse issues page

‎ 20.1001.1.17353165.1386.6.3.5.1

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Borjian L, Nazmieh H. Expression of Hemophagocytic Lymphohistiocytosis with Hepatic Dysfunction in a 50 days Old Infant. JRUMS 2007; 6 (3) :213-216
URL: http://journal.rums.ac.ir/article-1-348-en.html

Expression of Hemophagocytic Lymphohistiocytosis with Hepatic Dysfunction in a 50 days Old Infant

L. Borjian

, H. Nazmieh

Abstract: (15146 Views)

Expression of Hemophagocytic Lymphohistiocytosis with Hepatic Dysfunction in a 50 days Old Infant L. Borjian MD , H. Nazmieh MSc Received: 27/03/07 Sent for Revision: 13/06/07 Received Revised Manuscript: 08/07/07 Accepted: 26/07/07 Background and Objective: The Hemophagocytic Lymphohistiocytosis (HLH) is a rare disease with the pathologic hallmark of having aggressive proliferation of activated macrophages and histiocytes in the tissues and organs, which phagocyte other cells. The Incidence of HLH was estimated to be 1 case per 50000 – 300000. Case report: A 50 days infant was referred to Shahid Sadoughi Hospital with hepatic manifestations such as ascites, hepatosplenomegaly, markedly elevated liver function tests, herniated umbelical cord, abnormal coagulation profiles, and the bone marrow revealed HLH. Unfortunately the parents did not permit the appropriate treatment to be done and the infant died at age of 90 days old. Conclusion: since the HLH have nonspecific clinical signs, it should be considered as differential diagnosis of neonatal liver disease, especially when it is accompanied by cytopenias. Key words: Hepatic manifestation, Hemophagocytic lymphohistiocytosis, Familial hemophagocytic lymphohistiocy_ tosis

Keywords: Hepatic manifestation, Hemophagocytic lymphohistiocytosis, Familial hemophagocytic lymphohistiocy_ tosis

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Type of Study: Research | Subject: Physiology
Received: 2008/02/10 | Published: 2007/12/15