Secondary Haemophagocytic Lymphohistiocytosis – The Differential Diagnosis Dilemma In Paediatrics

Abstract

ABSTRACTSecondary haemophagocytic lymphohistiocytosis (SHFLH) is a rare, potentially fatal disorder, most commonly caused by the Epstein–Barr virus. It is characterized by neoplastic proliferation of cells that belong to the monocyte–macrophage system and by varied clinical expression.A girl aged 3 years and 7 months was hospitalized due to continuing high febricity, yellow skin colouring, hepatosplenomegaly and cytopenia in a complete blood count (CBC). Four weeks before hospitalization, she had a lacunar angina and lymphadenopathy.A low number of erythrocytes, leukocytes and thrombocytes were noted in CBC, with anaemia and the presence of virocytes in a peripheral blood smear. Biochemical blood analyses indicated hyperbilirubinaemia, increased values of transaminases, lactic dehydrogenase, ferritin, triglycerides, D-dimer, acceleration of the activated partial thromboplastin time and decreased values of fibrinogen, with increased values of C-reactive protein and procalcitonin. Using an ultrasound examination of the abdomen, hepatosplenomegaly was perceived; using echocardiographic examination, pericardium layering was noticed; and using a roentgen graphic picture of the lungs, the presence of pleural effusion was detected. In a bone marrow biopsy, the percentage of blasts did not exceed 25%, and rare chemophagocytes were noticed. Using serologic tests, positivity to Epstein-Barr virus in IgM class was demonstrated.According to the criteria by Histiocyte Society, there were sufficient criteria to establish a diagnosis of SHFLH. With the exception of symptomatic therapies, according to the protocol for SHFLH treatment, a double antibiotic therapy and IV immunoglobulins were given, to which the patient responded with a clinical and laboratory recovery. Therefore, there was no demand for a treatment protocol with cytostatics or bone marrow transplantation.To resolve a differential diagnosis dilemma in solving cases of uncertain febrile neutropenia.