ABSTRACT
OBJECTIVE: Introduction: Haemophagocytic lymphohistiocytosis (HLH) is an extremely rare, life-threatening disease, caused by uncontrolled activation of lymphocytes T and macrophages. This situation leads to cytokine storm, infiltration and internal organs failure. HLH can be categorised into either primary (familiar) or secondary which may be associated with infections, immunodeficiency syndromes, autoimmune diseases and malignancy. The secondary HLH is difficult to diagnose due to nonspecific symptoms and complicated differential diagnostics. The aim: To conduct a comparative analysis of pregnant and puerperal patients diagnosed with HLH. PATIENTS AND METHODS: Material and methods: Review of available literature on haemophagocytic lymphohistiocytosis during pregnancy and the puerperium. RESULTS: Results: Review of the latest literature shows that HLH can occur at any time during pregnancy and in the puerperium. Symptoms of the disease are non-specific: fever not responding to antibiotic therapy, sometimes hectic, hepatosplenomegaly, swelling, lymphadenopathy, disseminated intravascular coagulation, multi-organ failure and death. In laboratory tests, worsening bicytopenia or pancytopenia, increasing indicators of organ damage, hypertriglyceridemia, hypofibrinogenemia and abnormally high serumferritin levels are observed. CONCLUSION: Conclusions: HLH, due to non-specific symptoms and rarity, is often overlooked in the diagnostic process. Due to the high mortality and morbidity rates of HLH during pregnancy for mother and foetus, timely diagnosis and the inclusion of specialist treatment are particularly important. An interdisciplinary approach to the patient is necessary to make an accurate diagnosis. The assessment of serum ferritin concentrations facilitates diagnosis. The bone marrow is essential to diagnosis and should be performed as early as possible.
