Hemophagocytic lymphohistiocytosis: epidemiological, clinical and biological profile

Abstract

Background/aim: Hemophagocytic lymphohistiocytosis (HLH) is a clinical, biological, and pathological entity that is rare but has certain morbidity that may be life-threatening. This work aims to establish a focus on the hemophagocytic lymphohistiocytosis and analyze different aspects of diagnosis while emphasizing the biological data. Materials and methods: We report the results of a retrospective study conducted in the hematology department of Avicenna Hospital in Marrakesh. Thirty-one patients with hemophagocytic lymphohistiocytosis were enrolled. Results: The clinical presentation was dominated by fever and deterioration of the general state for almost all our patients. Splenomegaly was objectified in 90% of the patients. Hepatomegaly, lymphadenopathy, and hemorrhagic manifestations were observed in almost 50% of the patients. Biological assessments revealed bi-or pancytopenia in 96% of the patients, and coagulation disorders in 51% of the patients. On the other hand, hyperferritinemia was found in 84% of the patients, and hepatic cytolysis and hypertriglyceridemia in half of the patients. Hemophagocytosis was observed in all bone marrow samples taken from our patients. Concerning the evolution of patients, in 38.5% of the patients, the evolution was favorable with regression of clinical and biological signs. Twenty six percent of the patients had died, mainly from multiple organ failure and disseminated intravascular coagulation. Conclusion: HLH is a diverse condition with many causes and is likely to be under-recognized, which contributes to its high morbidity and mortality. Clinicians need to be able to recognize the signs and symptoms commonly seen in HLH and actively pursue this diagnosis in the cases of undiagnosed febrile illness with multiorgan dysfunction. Early recognition is crucial for any reasonable attempt at curative therapy to be made.

Hypofibrinogenemia is an independent predictor of hemophagocytic lymphohistiocytosis in children with sepsis

“…Notably, children with HLH exhibited significantly higher rates of prolonged fever exceeding 10 days (25% vs. 7.33%, P = 0.0017), hepatomegaly (30.56% vs. 5.08%, P < 0.0001), and splenomegaly (16.67% vs. 1.88%, P = 0.0002) than those without HLH. Past studies by Hanane Zahir and Feig JA reported that approximately 90% of patients with HLH presented with splenomegaly and approximately 61% with hepatomegaly 11 , 12 . This suggests that the presence of hepatosplenomegaly, a common feature in HLH but not sepsis, can aid in distinguishing HLH among patients with sepsis.…”

Accuracy of the Criteria for Hemophagocytic Lymphohistiocytosis

“…Based on the revised HLH-2004 diagnostic criteria, to diagnose HLH, a molecular diagnosis consistent with HLH or 5 of the 8 diagnostic criteria for HLH should be fulfilled ( . At the same time, the clinical appearance of these criteria is not always associated with the actual occurrences of HLH, which puts the physician in a difficult situation of making the diagnosis, subsequently leading to unnecessary or incorrect medical procedures and delays in treatment [7,17,18].…”

A Rare Case of Hemophagocytic Lymphohistiocytosis in an Adult

“…Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome characterized by excessive immune activation. Uncontrolled, activated macrophages and T lymphocytes secrete high amounts of cytokines leading to multiorgan failure [ 1 ]. Though usually a disease diagnosed in children, adult cases of HLH are under-recognized and, therefore, undertreated [ 2 ].…”