Advances in the diagnosis and management of Haemophagocytic Lymphohistiocytosis: A review of literature &#x0D;

Alvi, Saima; Naqvi, Ahmed

doi:10.14785/lymphosign-2017-0010

Cited by 3 publications

(2 citation statements)

References 85 publications

(109 reference statements)

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“…Hemophagocytosis was found in bone marrow (BM) in 57 % of patients which is in accordance with literature reports depending on the time of the BM aspirate, this finding is reported between 40 and 80 %. [ 2 , 24 ] In our population hemophagocytosis was not sought in tissues other than BM since this practice is not protocolized in our institution.…”

Section: Discussionmentioning

confidence: 99%

Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country

et al. 2021

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Background Hemophagocytic lymphohistiocytosis (HLH) is an exaggerated inflammatory reaction secondary to a host’s inadequate immune response causing a self-perpetuating loop of altered regulation. Signs and symptoms of HLH are compatible with other common diseases and are nonspecific. Underdiagnosis makes it difficult to estimate the real incidence of HLH, especially in developing countries. Methods Retrospective, descriptive study of pediatric patients admitted to a high-complexity institution in Cali, Colombia between 2012 and 2019 with HLH diagnosis. Medical history review to complete an electronic database and a secondary, descriptive analysis was carried out. The study was approved by the Institutional Ethics Committee. Results Twenty-one patients were included. 52.4 % of the population was male with a median age of 9.3 years [IQR (3.0-13.7 years)]. More than half of patients (66.6 %) had viral disease at diagnosis, the most frequent being Epstein-Barr Virus (EBV) (52.3 %) and dengue (14.3 %). Three patients had confirmed gene mutations (G6PC3, XIAP, and UNC13D). 95 % of the patients were treated with the HLH 2004 protocol, half of them received incomplete protocol with intravenous immunoglobulin (IVIG) and/or systemic steroids, while the other half received the complete protocol including etoposide and cyclosporine. More than three-fourths (76.2 %) required admission to an ICU with a median stay of 14 days [IQR (11–37 days)] and a median hospital stay of 30 days [IQR (18–93 days)]. 14.3 % (n = 3) of patients died. Conclusions HLH is a complex disease that requires multidisciplinary management with secondary HLH due to EBV infection being a common cause. There is increasing awareness of HLH diagnosis in developing countries such as Colombia which can offer earlier treatment options and better outcomes.

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Section: Discussionmentioning

confidence: 99%

Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country

et al. 2021

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“…20,21 Hemophagocytes were found in bone marrow (BM) in 57% of patients which is in accordance with literature reports depending on the time of the BM aspirate, this finding is reported between 40% and 80%. 2,22 In our population hemophagocytes were not sought in tissues other than BM since this practice is not protocolized in our institution.…”

Section: Discussionmentioning

confidence: 99%

Pediatric Hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country

Cleves

Lotero

Medina

et al. 2020

Preprint

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Introduction: Hemophagocytic lymphohistiocytosis (HLH) is an exaggerated inflammatory reaction secondary to a host's inadequate immune response causing a self-perpetuating loop of altered regulation. Signs and symptoms of HLH are compatible with other common diseases and are nonspecific. Underdiagnosis makes it difficult to estimate the real incidence of HLH, especially in developing countries. Materials and Methods: Retrospective, descriptive study of hospitalized pediatric patients admitted to a high-complexity institution in Cali, Colombia between 2012 and 2019 with HLH diagnosis. Medical history review to complete a virtual database. A secondary descriptive analysis was carried out. The study was approved by the Institutional Ethics Committee. Results: Twenty-one patients were included. 52.4% of the population was male with a median age of 9.3 years [IQR (3.0-13.7years)]. More than half of patients (66.6%) had viral isolation at diagnosis, the most frequent being Epstein-Barr Virus (EBV) (52.3%) and dengue (14.3%). Three patients had gene mutations (LYST, XIAP, and UNC13D). Ninety-five percent of the patients were treated with the HLH 2004 protocol, half of them received incomplete protocol with IgIV at high doses and/or systemic steroids, while the other half received the complete protocol including etoposide and cyclosporine. More than three-fourths (76.2%) required admission to an ICU with a median stay of 14 days [IQR (11-37 days)] and a median hospital stay of 30 days [IQR (18-93 days)]. 14.3% (n = 3) of patients died. Conclusions: HLH is an underdiagnosed pathology that requires greater sensitization in developing countries in order to make early diagnoses and obtain better outcomes.

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Difficult Diagnosis: A Case of Hemophagocytic Lymphohistocytosis in an Infant

Пшениснов

Aleksandrovich

Udaltcov

et al. 2022

Vestn. anesteziol. reanimatol.

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Hemophagocytic lymphohistocytosis is an extremely rare disease requiring early diagnosis and specific treatment.The objective: to demonstrate the clinical case of hemophagocytic lymphohistocytosis in an infant. The course of the disease in a 6-month-old child requiring treatment in the intensive care unit was retrospectively analyzed. The article presents the procedure of differential diagnosis of sepsis and hemophagocytic lymphohistocytosis in children, special attention is paid to the diagnostic criteria, and the clinical and laboratory differences of these diseases are described.Conclusion. The presence of leukopenia, neutropenia, thrombocytopenia, hypoalbuminemia and hypocoagulation in an infant with persistent fever is the basis for a thorough differential diagnosis in order to eliminate severe systemic diseases that manifest as childhood infections and sepsis.

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Advances in the diagnosis and management of Haemophagocytic Lymphohistiocytosis: A review of literature

Cited by 3 publications

References 85 publications

Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country

Pediatric hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country

Pediatric Hemophagocytic lymphohistiocytosis: A rarely diagnosed entity in a developing country

Difficult Diagnosis: A Case of Hemophagocytic Lymphohistocytosis in an Infant

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