A Challenging Case of Haemophagocytic Lymphohistiocytosis (HLH) Secondary to CMV Infection in an Adolescent with B-acute Lymphoblastic Leukemia

Lohana, Chandumal; Jhatial, Mussadique Ali; Bokhari, Syed W

doi:10.29271/jcpsp.2021.08.1011

Cited by 2 publications

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“…Cytomegalovirus (CMV) is typical of newborns and immunocompromised patients ( 72 , 73 , 77-81 ) and so is the varicella zoster virus ( 82 ). Dengue virus plays a significant role in tropical countries ( 73 , 83-90 ).…”

Section: Classificationmentioning

confidence: 99%

Secondary hemophagocytic lymphohistiocytosis in children (Review)

et al. 2023

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Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening condition characterized by hyperinflammation in an uncontrolled and ineffective immune response. Despite great improvement in diagnosis and treatment, it still represents a challenge in clinical management, with poor prognosis in the absence of an aggressive therapeutic approach. The present literature review focuses on secondary HLH at pediatric age, which represents a heterogeneous group in terms of etiology and therapeutic approach. It summarizes the most recent evidence on epidemiology, pathophysiology, diagnosis, treatment and prognosis, and provides a detailed description and comparison of the major subtypes of secondary HLH. Finally, it addresses the open questions with a focus on diagnosis and new treatment insights.

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Section: Classificationmentioning

confidence: 99%

Secondary hemophagocytic lymphohistiocytosis in children (Review)

et al. 2023

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Concomitant Hemophagocytic Lymphohistiocytosis and Cytomegalovirus Disease: A Case Based Systemic Review

et al. 2022

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BackgroundHemophagocytic lymphohistiocytosis (HLH) is an immune mediated life-threatening condition. It is driven by an overactivation of the immune system and causes inflammatory tissue damage potentially leading to organ failure and death. Primary HLH is caused by genetic mutations, while secondary HLH is triggered by external factors. Viral infections are a well-known cause of secondary HLH. Cytomegalovirus (CMV) is a virus in the herpes family known to cause HLH in rare cases.MethodsWe report a recent case of CMV-induced HLH, followed by a systematic review of described cases of this rare disease entity, through a structured search in the medical database PubMed. All articles were assessed on a predetermined set of inclusion criteria.ResultsA total of 74 patients (age > 18 years) with CMV-related HLH were identified, 29 men, 42 women, and three patients with unspecified gender. Median age was 37.5 years (range 18–80). Sixty-six patients (88%) had one or more comorbid conditions and 22 patients (30%) had inflammatory bowel disease (IBD), the most frequent comorbidity. Forty patients (54%) received some form of immunomodulating treatment prior to HLH development. The general treatment approach was in general dual, consisting of antiviral treatment and specific immunomodulating HLH treatment approaches. Treatment outcome was at 77% survival, while 23% had fatal outcome.ConclusionThe findings highlight the importance of early diagnostic work up and treatment intervention. Ability to recognize the characteristic clinical traits and perform specific HLH diagnostic workup are key factors to ensure targeted diagnostic work and treatment intervention for this patient group.

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A Challenging Case of Haemophagocytic Lymphohistiocytosis (HLH) Secondary to CMV Infection in an Adolescent with B-acute Lymphoblastic Leukemia

Cited by 2 publications

References 0 publications

Secondary hemophagocytic lymphohistiocytosis in children (Review)

Secondary hemophagocytic lymphohistiocytosis in children (Review)

Concomitant Hemophagocytic Lymphohistiocytosis and Cytomegalovirus Disease: A Case Based Systemic Review

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