Hematology & Transfusion International Journal Case Report Open Access Hemophagocytic lymphohistiocytosis in a child with sickle cell disease Keywords: hemophagocytic lymphohistiocytosis, FHL, EBV, hemoglobin S, sickle cell disease, X-ray Volume 6 Issue 5 - 2018 Introduction Walaa Shoman,1 Yasmine El Chazli,2 Asmaa Elsharkawy,2 Neveen Mikhael,3 Akram Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening deghaidy,3 Abeer Al-Battashi,4 Yasser Wali2 hyper-inflammatory syndrome which represents the extreme end of 1Department of Pediatrics, Immunology/Rheumatology unit, a severe uncontrolled hyperinflammatory reaction that can occur in Alexandria University, Egypt many underlying conditions.1 HLH can be divided into primary and 2Department of Pediatrics, Hematology/Oncology unit, secondary HLH. Primary HLH is caused by gene mutation, either at Alexandria University, Egypt 3 one of the Familial HLH (FHL) loci or in a gene responsible for one Department of Clinical pathology, Alexandria University, Egypt 4Department of Child Health, Royal Hospital, Oman of several immunodeficiency syndromes.2 The most common form of secondary HLH is infection associated HLH. Infectious triggers Correspondence: Yasser Wali, Department of Pediatrics, include viruses (as EBV, cytomegalovirus, HHV8, HIV), bacteria (as Hematology/Oncology unit, Alexandria University, Egypt, mycobacteria, mycoplasma), parasites (as leishmania, plasmodium), Email
[email protected] 3,4 and fungi (as candida, cryptococcus). Sickle cell disease (SCD) Received: September 02, 2018 | Published: September 27, and its variants are genetic disorders resulting from the presence 2018 of a mutated form of hemoglobin, hemoglobin S (HbS), which can be detected by hemoglobin electrophoresis. SCD is suggested by a high-grade unremitting fever, grunting, marked pallor, jaundice, the typical clinical picture of chronic hemolytic anemia and vaso- and marked re-enlargement of both liver and spleen despite improved occlusive crisis.5,6 We present a case of HLH in an Egyptian boy who chest condition and radiogram.