ADULT SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS – A CASE SERIES

2021 ◽  
pp. 64-66
Author(s):  
Athira P A ◽  
Indumathi K DCP ◽  
Theranirajan Theranirajan ◽  
Priyadharshini Swaminathan
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Macrophage Activation ◽  
Case Series ◽  
Treatment Phase ◽  
Marrow Transplant ◽  
Allogenic Bone ◽  
Familial Form ◽  
Life Threatening ◽  
Antigen Removal ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis is a hyper-inammatory condition that is either Familial (Primary) or Secondary to autoimmune diseases , infection, malignancy or other triggers.It is a cytokine storm syndrome where there inefcient antigen removal that leads to sustained cytokine release.It is a rare phenomenon occuring in adults that has got a specic trigger which is less documented and have a good response to steroids where as Familial form is a childhood disease due to genetic defects, both of which are life threatening and may need Allogenic bone marrow transplant. Macrophage activation syndrome is also a subtype of this entity that occurs in the treatment phase of SLE and Still's disease.We describe here 8 cases of secondary HLH, their primary triggers and treatment response.

scholarly journals Macrophage Activation Syndrome in Adults: A Retrospective Case Series

2021 ◽  
Vol 9 ◽  
pp. 232470962110264
Author(s):  
Taylor Warmoth ◽  
Malvika Ramesh ◽  
Kenneth Iwuji ◽  
John S. Pixley
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Pediatric Patients ◽  
Inflammatory Diseases ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Case Series ◽  
Adult Patients ◽  
Retrospective Case ◽  
Life Threatening ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a form of hemophagocytic lymphohistocytosis that occurs in patients with a variety of inflammatory rheumatologic conditions. Traditionally, it is noted in pediatric patients with systemic juvenile idiopathic arthritis and systemic lupus erythematous. It is a rapidly progressive and life-threatening syndrome of excess immune activation with an estimated mortality rate of 40% in children. It has become clear recently that MAS occurs in adult patients with underlying rheumatic inflammatory diseases. In this article, we describe 6 adult patients with likely underlying MAS. This case series will outline factors related to diagnosis, pathophysiology, and review present therapeutic strategies.

scholarly journals Death of unknown cause? Post-mortem diagnosis of fulminant course of an EBV-associated secondary hemophagocytic lymphohistiocytosis

2021 ◽  
Author(s):  
Josia Fauser ◽  
Stefan Köck ◽  
Eberhard Gunsilius ◽  
Andreas Chott ◽  
Andreas Peer ◽  
...  
Keyword(s):  
Immune Response ◽  
Case Report ◽  
Macrophage Activation ◽  
Post Mortem ◽  
Life Threatening ◽  
Appropriate Therapy ◽  
Diagnostic Work ◽  
Work Up ◽  
Diagnostic Work Up ◽  
Secondary Hemophagocytic Lymphohistiocytosis

SummaryHLH is a life-threatening disease, which is characterized by a dysregulated immune response with uncontrolled T cell and macrophage activation. The often fulminant course of the disease needs a fast diagnostic work-up to initiate as soon as possible the appropriate therapy. We present herein the case of a 71-year-old patient with rapidly progressive hyperinflammatory syndrome, which post mortem resulted in the diagnosis of EBV-associated HLH. With this case report, we intend to highlight the relevance of the HScore in the diagnosis of HLH, to create a greater awareness for EBV as a trigger of HLH, and to demonstrate the importance of treating EBV-associated HLH as early as possible.

scholarly journals Hemophagocytic lymphohistiocytosis: a rare and life-threatening diagnosis

2021 ◽  
Vol 9 (12) ◽  
pp. 3705
Author(s):  
Anu Yarky ◽  
Vipan Kumar ◽  
Nidhi Chauhan ◽  
Neha Verma
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Presentation ◽  
Laboratory Findings ◽  
Delay In Diagnosis ◽  
Life Threatening ◽  
Sporadic Cases ◽  
Prompt Diagnosis ◽  
Excessive Activation ◽  
Variable Clinical Presentation ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of excessive activation of immune system. It frequently affects infants from birth to 18 months of age, but is also observed in children and adults of all ages. HLH can occur as a familial or sporadic disorder, and it is triggered by a variety of events, Infection being the most common trigger both in familial and in sporadic cases. Prompt treatment is very critical in cases of HLH, but the greatest barrier is often delay in diagnosis due to the rarity of this syndrome, variable clinical presentation, and lack of specificity of the clinical and laboratory findings. The key clinical features of HLH are high persistent fever, hepatosplenomegaly, blood cytopenia, elevated aminotransferase and ferritin levels, and coagulopathy. A diagnosis of HLH is mostly under-recognized, and is associated with high mortality, especially in adults; thus, prompt diagnosis and treatment are essential. We here present a rare case of HLH in an adult which was non-familial and infection being the trigger causing secondary hemophagocytic lymphohistiocytosis.

scholarly journals P022 Anakinra for use in non- juvenile idiopathic arthritis (JIA) related haemophagocytic lymphohistiocytosis (HLH): evidence base and funding

2019 ◽  
Vol 104 (7) ◽  
pp. e2.26-e2
Author(s):  
Octavio Aragon Cuevas
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Case Reports ◽  
Case Series ◽  
Evidence Base ◽  
British Society ◽  
Paediatric Patients ◽  
Activation Syndrome

BackgroundNon JIA related HLH is a life-threatening complication that is increasingly recognised in paediatric patients, particularly in those who are unwell in the paediatric intensive care unit (PICU). Untreated or insufficiently treated HLH has a significant mortality rate (up to 53%).1AimTo review the evidence base for the use of anakinra in paediatric patients with non-JIA HLH refractory to systemic corticosteroids in patients who are not fit for treatment as per HLH 2004 protocol.MethodsA PubMed search with words ‘anakinra’ and ‘hemophagocytic lymphohistiocytosis’ was carried out on July 2018 to find out the evidence base with regards to the use of anakinra in non-JIA related HLH. Any published peer reviewed clinical studies or trials (including but not limited to retrospective or prospective controlled trials, comparative studies and observational/cohort studies) were considered. Case reports and series were considered if better evidence studies were not available. A recent case study from a tertiary paediatric centre will be used to illustrate the pathway followed to diagnose non-JIA related HLH and funding options.ResultsAlthough a protocol exists for primary HLH treatment (HLH 2004), including chemotherapy and stem cell transplantation,2 there is no consensus on how to treat secondary HLH. The literature mainly showed case reports and small case series,3 describing the use of anakinra collectively for 35 patients (median age 14 to 48 years) who met the HLH 2004 diagnostic criteria with an overall survival rate of up to 88% at time of discharge from the PICU3. Anakinra was used at standard doses always in combination with corticosteroids. Some patients also received intravenous immunoglobulin (IVIG) and ciclosporin at the discretion of the medical teams.ConclusionThe evidence for use of anakinra in non JIA secondary HLH is limited to retrospective observational studies and mostly restricted to adult populations. Despite this caveat, these studies have demonstrated that anakinra therapy alongside other non-etoposide immunomodulatory therapies is associated with an improvement in short term survival. In patients with multi-organ dysfunction, who are too unstable to receive the existing etoposide based HLH-2004 treatment regimen due to concerns regarding significant treatment toxicity, personalised non-etoposide therapies including dexamethasone, IVIG, ciclosporin and anakinra may be better tolerated and provide a bridge to future more standardised treatment. Evidence to date shows that relapse of secondary HLH is possible with ciclosporin therapy. In JIA related HLH, anakinra was considered better than ciclosporin at inducing remission and having a lower incidence of adverse effects,4 and NHS England granted funding for the treatment based on these findings. The available evidence did not show any serious adverse events related to anakinra.RecommendationsThis tertiary centre approved the use of anakinra for this patient and future patients with this indication despite lack of reimbursement from NHS England for the drug. An urgent interim policy review will be put together by a team of the British Society of Paediatric and Adolescent Rheumatology (BSPAR) and presented to the NHS England commissioners to seek funding for anakinra for paediatric patients with this indicationReferencesMiettunen, et al. Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients. Rheumatology (Oxford) 2011;50:417–9Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124–31.Kumar B, Aleem S, Saleh H, Petts J, Ballas ZK. A Personalized Diagnostic and Treatment Approach for Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Adults. J Clin Immunol 2017;37:638–643Boom V, et al. Evidence-based diagnosis and treatment of macrophage activation syndrome in systemic juvenile idiopathic arthritis. Pediatr Rheumatol Online J 2015;13:55.

scholarly journals IMPORTANCE OF HYPERBILURUBINEMIA IN DIFFERENTIATION OF PRIMARY AND SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN PEDIATRIC CASES

2014 ◽  
Vol 6 (1) ◽  
pp. e2014067 ◽  
Author(s):  
Seval Ozen ◽  
Alper Dai ◽  
Enes Coskun ◽  
Serdar Oztuzcu ◽  
Sercan Ergun ◽  
...  
Keyword(s):  
Inflammatory Disease ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Findings ◽  
Initial Diagnosis ◽  
Direct Bilirubin ◽  
Pediatric Hematology ◽  
Life Threatening ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Background and objective: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyper inflammatory disease. It is difficult to differentiate between primary and secondary HLH based on clinical findings at the onset of disease. We aimed to find parameters that can help to differentiate primary and secondary HLH at initial diagnosis especially for physicians working in developing countries.Patient and Method: We retrospectively analyzed data of 38 HLH patients who were admitted to the Pediatric Hematology Department of Gaziantep University between January 2009 and December 2013.Results: Of 38 patients, 20 were defined as primary and 18 were secondary HLH. The average age of primary and secondary HLH patients was 31±9 and 81±14 months, respectively (p=0.03). We found consanguinity rates significantly higher in primary HLH patients compared to secondary HLH patients (p=0.03). We found that total and direct bilirubin levels significantly increased in primary HLH patients compared to secondary HLH patients (p=0.006, p=0.044). Also, CRP levels were found markedly increased in secondary HLH patients compared to primary ones (p=0.017).Conclusion: We showed that cholestasis and hyperbilurubinemia findings of HLH patients at the initial diagnosis should be considered in favor of primary HLH and increased level of CRP should be considered in favor of secondary HLH.

Cytokine adsorption is a promising tool in the therapy of hemophagocytic lymphohistiocytosis

2019 ◽  
Vol 42 (11) ◽  
pp. 658-664 ◽  
Author(s):  
Silvius Frimmel ◽  
Michael Hinz ◽  
Jan Schipper ◽  
Simon Bogdanow ◽  
Steffen Mitzner ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Animal Studies ◽  
Marked Decrease ◽  
Case Series ◽  
Clinical Syndrome ◽  
Porous Polymer ◽  
Vasopressor Therapy ◽  
Promising Tool ◽  
Cytokine Levels ◽  
Life Threatening

Hemophagocytic lymphohistiocytosis is a life-threatening clinical syndrome caused by severe hypercytokinemia brought on by a highly stimulated but ineffective immune response. Animal studies and case series have demonstrated that a reduction in blood cytokine levels achieved with an extracorporeal adsorption cartridge that contains blood-compatible porous polymer beads (CytoSorb®) can effectively attenuate the inflammatory response during sepsis and possibly improve outcomes. We report a case series of two patients in which three episodes of severe hemophagocytic lymphohistiocytosis triggered by infections with herpesviridae were treated successfully with cytokine adsorption. A marked decrease in interleukin-6 plasma levels and a stable or decreasing need of vasopressor therapy were the most significant results of this treatment. Importantly, treatment was safe and well-tolerated, without any adverse events.

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