| Abstract |
Introduction: Hemophagocytic syndrome or hemophagocytic lymphohistiocytosis (HLH) is not only a rare and potentially fatal disease of children and adults, but is also a clinical laboratory syndrome characterized by cytokine dysfunction and uncontrolled activation of T-lymphocytes and NK cells against some cause (infections, neoplasia). Despite the intense effort of the immune system mechanism to cope with the pathogenic cause, the immune response is reduced and this leads to clinical manifestations, hyper-inflammatory conditions and katastrophic end-organ damage. Diagnosis is difficult in the initial phase as HLH shares a common phenotype with many other conditions and diseases. With the HLH -1994 and HLH-2004 protocols as well as recording the global incidence, symptoms and cause of HLH have helped in it’s early diagnosis. The set of diagnostic criteria can help the clinician to recognize the condition early preventing it’ s rapid progression resulting in the death of the patient. Purpose: To make a retrospective record of the children diagnosed with hemophagocytic syndrome at the Children’s Hematology – Oncology Clinic of the University General Hospital of Heraklion and then to compare the epidemiological and clinical characteristics, the diagnostic approach, the therapeutic treatment as well as the outcome between the patients and with international bibliography.
Methodogy: The study includes all patients diagnosed with hemophagocytic syndrome between May 1999 and referred to the Children’s Hematology – Oncology Clinic of the University General Hospital of Heraklion. Patient dempographics, epidiomiological and prognostic factors, clinical data (symptoms, laboratory results, treatment protocols) aw well as their health outcome were recorded. Based on the laboratory and genetic findings, the division into Primary HLH and Seconadary HLH was given.
Results: Our study included seven patients (four girls and three boys) aged 2,5 months to 15,5 years. Six patients were diagnosed with secondary HLH and one with primary familial HLH. In more detail regarding Secondary HLH:
Two patients were diagnosed with Leishmania
One with Systemic Juvenile Arthritis
One with a respiratory infection (Rhinovirus – Enterovirus were positive
One had relapsed ALCL
One had Epstain Bar Virus (EBV) infection.
Regarding patient outcome, six are doing well to date, while one patient with secondary HLH, who had received the HLH -1994 tratment protocol, died in the transplant unit. The epidemiological – laboratory findings and the therapeutic protocols of the study agree with the international literature.
Conclusion: The study patients were diagnosed and treated according to the respective treatment protocols. Several of the study’ s conclusions agree with the international literature. A respective is the continuation of the registration in depth of several years, perhaps with the participation of patients from other centers and other groups and the addition of more information regarding family history. Some of the patients who participated in the study were second trimester children from a twin pregnancy. Perhaps in the future a study could be done on whether the pregnancy was carried out by an in vitro method, the age of the parents at the time of conception, how many attempts were made to give birth.
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