Pediatric Hemophagocytic Lymphohistiocytosis — A Single Center Study

Paul, Tanusree; Kalra, Manas; Danewa, Arun; Sachdeva, Pallavi; Thatikonda, Kasi Bharathi; Sachdeva, Divij; Anupam, Sachdeva

doi:10.1007/s13312-022-2491-8

Cited by 3 publications

(10 citation statements)

References 16 publications

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“…FHLH was observed in only 5.5% of cases as all patients were not screened keeping in view the limitation of resources and huge financial burden of genetic testing. This number is quite higher in previous literature with Alsohime et al reporting 43% of cases of FHLH and 23.5% cases reported by Paul et al[3,17].Seventeen children (15.7%) were diagnosed cases of Griscelli syndrome, which is a known association observed in previous literature. Sasan et al observed Griscelli syndrome in 12% of cases[14].…”

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confidence: 67%

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Outcome and Prognostic Factors of Hemophagocytic Lymphohistiocytosis in Children: Experience From a Low- and Middle-Income Country

Hira,

Siddique,

Ahmed

et al. 2024

Cureus

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Objective Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition especially in low- and middle-income countries (LMICs). This study was done to evaluate the outcome and prognostic factors of HLH in patients presenting to our center. Methods The study was carried out at the Paediatric Oncology Department of Combined Military Hospital (CMH) in Rawalpindi, Pakistan. All cases of HLH, from one month to 15 years of age enrolled between January 1, 2013 to June 30, 2023, were included. IBM SPSS Statistics for Windows, version 25.0 (released 2017, IBM Corp., Armonk, NY) was used for statistical analysis, and t-test and chi-square tests were used for comparison between continuous and categorical variables. Frequencies and percentages were calculated for categorical variables. Results Out of 115 patients, seven (6%) abandoned the treatment. The data of 108 cases, including 58 males (53.7%), were analyzed. The mean age at diagnosis was 31.5 ± 39.03 months. The mean time to reach a pediatric oncologist was 30.20 ± 22.15 days. Fever and pallor were common symptoms occurring in 107 (99.1%) and 98 (90.7%) cases, respectively. Jaundice was present in 44 (40.7%), visceromegaly in 64 (59.3%), and bruising/bleeding in 16 cases (14.8%). Twenty-six (24.1%) patients underwent hematopoietic stem cell transplant (HSCT), out of which 17 (65.4%) children were cured. Overall survival at two years, five years, and 10 years was 38%, 37%, and 36.1%, respectively. Disease-free survival at two years, five years, and 10 years was 33.3%, 32.4%, and 31.5%, respectively. Conclusion HLH leads to high mortality due to delayed or misdiagnosis in LMICs. Early diagnosis and early referral to a pediatric oncologist is the detrimental factor in survival for HLH. HSCT is the treatment of choice for primary, refractory, or relapse cases.

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confidence: 67%

“…Hemophagocytic lymphohistiocytosis (HLH) is caused by immune cell dysfunction of T lymphocytes, macrophages, and natural killer cells leading to hyperinflammation [1][2][3]. In children, mostly inherited immune deficiencies lead to this syndrome [4].…”

Section: Introductionmentioning

confidence: 99%

Outcome and Prognostic Factors of Hemophagocytic Lymphohistiocytosis in Children: Experience From a Low- and Middle-Income Country

Hira,

Siddique,

Ahmed

et al. 2024

Cureus

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“…Lymphomas, though relatively uncommon at pediatric age, are frequently reported in association with HLH ( 138 ). Among the cases we reviewed in the present study, HLH has been associated with Hodgkin lymphoma ( 73 , 121 , 140 , 144 ), anaplastic large cell lymphoma ( 73 , 145 , 146 ), peripheral T-cell lymphoma ( 145 , 147 ), post-transplant lymphoproliferative disorder-lymphoma ( 148 ), subcutaneous panniculitis-like T-cell lymphoma ( 71 ) extranodal NK/T cell lymphoma, hepatosplenic T-cell lymphoma, systemic EBV-positive T-cell lymphoma of childhood ( 145 ). In addition, HLH and lymphoma can be alternative diagnoses as well ( 149 ), and extreme caution is required in differentiating the two conditions before starting steroid therapy.…”

Section: Classificationmentioning

confidence: 99%

“…EBV has been frequently reported from all across the world, and especially from the Asian Countries (37,(65)(66)(67)(68)(69)(70)(71)(72)(73). The reason for this geographical heterogeneity may involve a higher virulence in EBV viral strains circulating in Asia (74), or a different immune predisposition in the Asian patients, but this issue has not been clarified yet.…”

Section: Classificationmentioning

confidence: 99%

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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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