Hemophagocytic Lymphohistiocytosis: A Review

Siddaiahgari, Sirisha Rani; Agarwal, Shirali; Madukuri, Pallavi; Moodahadu, Latha Subramanyam

doi:10.4172/2155-9864.1000363

Cited by 8 publications

(9 citation statements)

References 41 publications

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“…In one study of 369 patients, the clinical findings included in HLH-2004 protocol were found to variably distributed in the patient population [8]. The prevalence of low or absent NK cell function was 71%.…”

Section: Discussionmentioning

confidence: 99%

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An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis

Quadri¹,

Jain²,

Brandon³

et al. 2020

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is an immune related clinical syndrome with protean manifestations, varying presentation, clinically complex, with diverse causes, and is an under-recognized entity which carries high morbidity and mortality. It is precipitated by an immunological trigger in a susceptible host resulting in immune activation and dysregulation leading to disruption of immune homeostasis, cytokine storm and multi-organ failure. We describe a case of Epstein-Barr virus (EBV) associated HLH with its typical diagnostic challenges and associated high mortality rate. Certain diagnostic criteria and online tools may help to arrive at an earlier presumptive diagnosis which, in turn, may expedite treatment and lead to better clinical outcomes.

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Section: Discussionmentioning

confidence: 99%

“…It is therefore to be noted that the characteristic finding of "Hemophagocytosis" in the liver, lymph nodes or bone marrow is not necessary for diagnosis, however, its presence can aid in diagnosis. Flow cytometry in a specialized laboratory can be very helpful with immunological markers [8].…”

Section: Discussionmentioning

confidence: 99%

An Intriguing Presentation of Epstein-Barr Virus-Associated Hemophagocytic Lymphohistiocytosis

Quadri¹,

Jain²,

Brandon³

et al. 2020

Cureus

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“…Hemophagocytosis may not be evident in very early stages of HLH, and thus its absence does not negate the diagnosis of HLH (21). The incidence of bone marrow involvement varies between 25% and 100% (22). This implies that the diagnosis of HLH requires a comprehensive clinical, biochemical and hematological approach.…”

Section: Discussionmentioning

confidence: 99%

Case series of hemophagocytic lymphohistiocytosis from a tertiary care centre: an underdiagnosed entity

Kumar

Sharma²,

Nigam³

2019

TJPATH

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Objective: Hemophagocytic Lymphohistiocytosis (HLH) is an uncommon, life-threatening hyperinflammatory syndrome, caused by severe hypercytokinemia, due to an overstimulated but ineffective immune process. The presenting features of HLH are non-specific, mimicking many other diseases, and hence its early recognition still remains a challenge. It requires a high index of suspicion and detailed analysis of clinical and laboratory findings to arrive at a conclusive diagnosis. The objective of this study is to present detailed clinical and laboratory features of a series of HLH cases. Material and Method: A retrospective study was conducted wherein all the bone marrow aspirate smears diagnosed as HLH between January 2015 to June 2018 were reviewed. Detailed correlation of clinical and laboratory criteria was done with the bone marrow findings. Results: A total of twelve cases were diagnosed as HLH from January 2015 to June 2018. Ten patients fulfilled 5 out of 8 clinical and lab diagnostic criteria of HLH (2004). After correlating clinical and laboratory criteria along with bone marrow findings the diagnosis of HLH was suggested. Conclusion: We present a series of twelve cases of Hemophagocytic Lymphohistiocytosis from a tertiary care hospital in New Delhi which will add not only to the understanding of this rare life threatening disease but also to the early diagnosis and intervention.

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“…There are five different subtypes of FHL, type 1 to type 5(FHL types 1-5). It is also found to be associated with certain primary immunodeficiency disorders like Chediak-Higashi syndrome (CHS-1), Griscelli syndrome type 2 (GS-II), Hermansky-Pudlak syndrome type 2 and X-linked proliferative syndrome (XLP-1) Journal Home: https://www.boffinaccess.com/journals/immunology-and-immunobiology/index.php 3/3 [8]and the overall prevalence of HLH in immunodeficiency syndrome is said to be 6%. Defective genes controlling the function of cytotoxic T-lymphocytes and NK -cells is said to be the cause of immune dysregulation in primary HLH.…”

Section: Casementioning

confidence: 99%