Case Series
Volume 7 Issue 9 - 2018
Hemophagocytic Lymphohistiocytosis: A Case Series Over 4 Years in a Saudi Hospital
Ehab Hanafy1*, Al Shaman Duha2, Sultan Jameel2, Abdulrahman Aloufi2, Abdulaziz Dasaq2 and Faisal Nazim1
1Prince Sultan Oncology Center, King Salman Armed Forces Hospital, Tabuk, Kingdom of Saudi Arabia
2Pediatric Department, King Salman Armed Forces Hospital, Tabuk, Kingdom of Saudi Arabia
*Corresponding Author: Ehab Hanafy, Prince Sultan Oncology Center, King Salman Armed Forces Hospital, Tabuk, Kingdom of Saudi Arabia.
Received: July 28, 2018; Published: August 08, 2018
Citation: Ehab Hanafy., et al. “Hemophagocytic Lymphohistiocytosis: A Case Series Over 4 Years in a Saudi Hospital”. EC Paediatrics 7.9 (2018): 846-850.
Abstract
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare but rapidly fatal disorder. It is characterized by aggressive proliferation of activated macrophages and histiocytes which leads to the clinical manifestations of the disease. Although the mechanism is not entirely understood, a theory of inappropriate immune reaction is currently accepted. Presentation can be either primary (familial) which is an inherited form of HLH or secondary which is an acquired form that occurs in response to immunologic over-activation as seen in severe infections, immunodeficiency and malignancy.
Case Series: We retrospectively report a series of five cases who were diagnosed as secondary HLH or macrophage activation syndrome (MAS) between 2013 and 2017. These were Saudi Arabian patients fulfilled the criteria of diagnosis and were managed at our tertiary hospital that serves the northwestern region of Saudi Arabia. Three out of 5 patients have been managed successfully and currently alive, while the 2 other patients died of severe progressive disease. Effective early diagnosis and fast installation of treatment contributed to an instant clinical and laboratory improvement and success of management, whereas late diagnosis will inevitably lead to a progressive fatal outcome. Interestingly, tumor lysis syndrome was noticed in 3 patients after starting treatment, so it is advised to monitor this condition while treating this cohort of patients.
Conclusions: HLH is a rare and under-diagnosed disorder since it can mimic and overlap with other conditions. Prompt diagnosis and management is life saving and every effort should be done to differentiate it from other similar conditions that necessitate different pathways of management.
Keywords: Hemophagocytic lymphohistiocytosis; macrophage activation syndrome; tumor lysis syndrome; Saudi Arabia
Copyright: © 2018 Ehab Hanafy., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

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