Adult onset hemophagocytic lymphohistiocytosis prognosis is affected by underlying disease and coexisting viral infection: analysis of a single institution series of 35 patients

2016 ◽  
Vol 35 (4) ◽  
pp. 828-834 ◽  
Author(s):  
Chiara Cattaneo ◽  
Margherita Oberti ◽  
Cristina Skert ◽  
Angela Passi ◽  
Mirko Farina ◽  
...  
Keyword(s):  
Viral Infection ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Underlying Disease ◽  
Adult Onset ◽  
Single Institution

scholarly journals Prognostic factors and outcomes of adult-onset hemophagocytic lymphohistiocytosis: a retrospective analysis of 34 cases

2015 ◽  
Vol 7 (2) ◽  
Author(s):  
Masafumi Oto ◽  
Kanako Yoshitsugu ◽  
Shima Uneda ◽  
Michiko Nagamine ◽  
Minoru Yoshida
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Age At Onset ◽  
Underlying Disease ◽  
Red Cross ◽  
Neoplastic Disease ◽  
Adult Onset ◽  
Multidrug Chemotherapy ◽  
Clinical Records ◽  
Underlying Diseases

Adult-onset hemophagocytic lymphohistiocytosis (HLH) has features that are distinct from that of HLH in pediatric patients. The clinical records at the Japanese Red Cross Kumamoto Hospital were reviewed. We retrospectively analyzed 34 patients who fulfilled the diagnostic criteria of HLH-2004. The median age of patients was 60.0 (range 15-86). Underlying diseases were diagnosed in 17 patients. They consisted of malignant lymphoma (n=3), other neoplastic disease (n=3), viral infection (n=4), collagen vascular disease (n=3), Kikuchi’s disease (n=3) and drug (n=1). Underlying diseases were not diagnosed in 17 patients despite examination. The treatments were steroids (n=18), dexamethasone + cyclosporine A (CSA) + etoposide (n=4), multidrug chemotherapy (n=2), steroids and CSA (n=3). Eleven patients died during observation. In a multivariate analysis, the significant predictor for death was age at onset (hazard ratio, 1.22; 95%CI, 1.02-1.44; P=0.027). Autopsy was performed in 4 cases, but the underlying disease remained unknown in 3 of those cases. Adult-onset HLH has high diversity and various outcomes. The mechanism of adult-onset HLH is not fully understood and further research is required.

Cytomegalovirus-Associated Hemophagocytic Syndrome

PEDIATRICS ◽  
1985 ◽  
Vol 75 (2) ◽  
pp. 280-283
Author(s):  
ELIZABETH H. DANISH ◽  
BEVERLY B. DAHMS ◽  
MARY L. KUMAR
Keyword(s):  
Viral Infection ◽  
Epstein Barr Virus ◽  
Hemophagocytic Syndrome ◽  
Clinical Course ◽  
Underlying Disease ◽  
Barr Virus ◽  
Pathologic Findings ◽  
Immunosuppressed Patients ◽  
Epstein Barr ◽  
Herpes Group

Virus-associated hemophagocytic syndrome, first described by Risdall and co-workers in 1979,1 is a rare histiocytic proliferative syndrome characterzed by fever, hepatosplenomegaly, pancytopenia, and erythrophagocytosis by histiocytes that appear benign by histologic criteria. The clinical course and pathologic findings may be identical with another histiocytic disorder, familial erythrophagocytic lymphohistiocytosis, which occurs predominantly in infants. Diagnosis of virus-associated hemophagocytic syndrome depends entirely on evidence of concurrent viral infection, usually of the herpes group. Epstein-Barr virus has been associated with this syndrome in the few cases reported in children without underlying disease, whereas cytomegalovirus (CMV) has been implicated in immunosuppressed patients. We report a case of fatal CMV-associated hemophagocytic syndrome which occurred in a previously healthy infant.

scholarly journals Hemophagocytic Lymphohistiocytosis Gene Mutations in Adult Patients Presenting With CLIPPERS-Like Syndrome

2021 ◽  
Vol 8 (3) ◽  
pp. e970
Author(s):  
Guillaume Taieb ◽  
Elsa Kaphan ◽  
Claire Duflos ◽  
Christine Lebrun-Frénay ◽  
Valérie Rigau ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Brain Mri ◽  
Gene Mutations ◽  
Adult Onset ◽  
Cytotoxic Lymphocyte ◽  
Familial Hemophagocytic Lymphohistiocytosis ◽  
Non Hodgkin Lymphoma ◽  
Treatable Condition ◽  
Perforin Expression

ObjectiveTo determine whether adult cases of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) may be related to familial hemophagocytic lymphohistiocytosis (HLH) causes, we have screened patients with adult-onset CLIPPERS for mutations in primary HLH-associated genes.MethodsIn our cohort of 36 patients fulfilling the criteria for probable or definite CLIPPERS according to the CLIPPERS-2017 criteria, we conducted a first study on 12 patients who consented to genetic testing. In these 12 patients, systemic HLH criteria were searched, and genetic analysis of 8 genes involved in primary HLH was performed.ResultsFour definite and 8 probable CLIPPERS were enrolled (n = 12). Mutations involved in HLH were identified in 2 definite and 2 probable CLIPPERS (4/12). Three of them had biallelic PRF1 mutations with reduced perforin expression in natural killer cells. The remaining patient had biallelic UNC13D mutations with cytotoxic lymphocyte impaired degranulation. None of the mutated patients reached the criteria for systemic HLH. During follow-up, 3 of them displayed atypical findings for CLIPPERS, including emergence of systemic non-Hodgkin lymphoma (1/3) and confluent gadolinium-enhancing lesions on brain MRI (3/3).ConclusionsIn our patients presenting with adult-onset CLIPPERS, one-third have HLH gene mutations. This genetic treatable condition should be searched in patients with CLIPPERS, especially in those presenting with atypical findings.

scholarly journals Hemophagocytic lymphohistiocytosis in a patient with glioblastoma: a case report

CNS Oncology ◽  
2019 ◽  
Vol 8 (4) ◽  
pp. CNS45 ◽  
Author(s):  
Vaibhav Kumar ◽  
Patrick J Eulitt ◽  
Ana Bermudez ◽  
Simon Khagi
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Hematologic Malignancy ◽  
Multiorgan Failure ◽  
Rare Condition ◽  
Solid Organ ◽  
Adult Onset ◽  
Barr Virus ◽  
Epstein Barr ◽  
High Index Of Suspicion

Adult onset hemophagocytic lymphohistiocytosis (HLH) is a rare condition, usually secondary to either a precipitating infective or hematologic malignancy. We present a case of Epstein–Barr virus associated HLH in a 55-year-old female receiving treatment for a glioblastoma (GBM). It is possible that HLH is under recognized, as patients with GBM often have features of a nonspecific systemic inflammatory response syndrome, multiorgan failure and cognitive decline. A high index of suspicion and increased awareness can help improve timeliness of diagnosis. Therapeutically, Epstein–Barr virus associated HLH in patients with solid organ malignancy poses significant challenges. An individualized, multidisciplinary approach is essential when managing adult-onset HLH and providers will need to be mindful of the high mortality rate despite treatment.

scholarly journals Contribution of clonal hematopoiesis to adult-onset hemophagocytic lymphohistiocytosis

Blood ◽  
2020 ◽  
Vol 136 (26) ◽  
pp. 3051-3055
Author(s):  
Peter G. Miller ◽  
Adam S. Sperling ◽  
Christopher J. Gibson ◽  
Kaushik Viswanathan ◽  
Cecilia Castellano ◽  
...  
Keyword(s):  
Older Adults ◽  
Mouse Model ◽  
Genetic Variants ◽  
Blood Cells ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Somatic Mutations ◽  
Adult Onset ◽  
Clonal Hematopoiesis ◽  
Inflammatory State ◽  
Life Threatening

Abstract Adult-onset hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease of immune hyperactivation. Unlike pediatric HLH, adult HLH is rarely driven by germline genetic variants. Although numerous precipitating etiologies have been identified, the reason that HLH occurs in only a subset of individuals and how other factors contribute to the disease remains unknown. We hypothesized that clonal hematopoiesis (CH), a state in which somatic mutations in blood cells cause an expanded population of mutant hematopoietic cells and drive an aberrant inflammatory state, could contribute to adult-onset HLH. In a highly annotated cohort of older adults with HLH we found that CH was more prevalent than in control cohorts. Using the adult-onset HLH mouse model in which repeated treatments of the TLR9 agonist, ODN1826, was delivered to the mouse, we observed that macrophages carrying mutations in Tet2, one of the most commonly mutated genes in CH, have an enhanced inflammatory response to TLR9 agonism. Finally, mice carrying Tet2 mutations in the hematopoietic compartment (a common model for CH) displayed an exaggerated response to TLR9 agonism, including worse splenomegaly and anemia. Our data suggest that CH is more common in individuals with adult-onset HLH and can contribute to the pathophysiology of this disease.

scholarly journals Miliary Tuberculosis-Related Acute Respiratory Distress Syndrome Complicated with Hemophagocytic Lymphohistiocytosis Syndrome

2019 ◽  
Vol 2019 ◽  
pp. 1-4
Author(s):  
Sz-Jiun Shiu ◽  
Ting-Ting Li ◽  
Bor-Jen Lee ◽  
Pin-Kuei Fu ◽  
Chen-Yu Wang ◽  
...  
Keyword(s):  
Acute Respiratory Distress Syndrome ◽  
Respiratory Distress Syndrome ◽  
Respiratory Distress ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Distress Syndrome ◽  
Case Reports ◽  
Multiorgan Failure ◽  
Miliary Tuberculosis ◽  
Bone Marrow Aspiration ◽  
Underlying Disease

Acute respiratory distress syndrome (ARDS) and hemophagocytic lymphohistiocytosis (HLH) are accompanied with poor outcome and high mortality when miliary tuberculosis is a causative pathogen for both of them. A patient complicated with ARDS and HLH is unusual in critical care, and few case reports are present in PudMed. Besides, the relationship between HLH and ARDS is still unknown and has not been reviewed in the literature. In this report, we present the case of a 74-year-old Taiwanese woman suffering from pulmonary tuberculosis and miliary tuberculosis, and she developed ARDS and HLH on the 3rd day after admission. We arranged serial laboratory examination, various serum markers, bone marrow aspiration, and bronchoscopy with alveolar lavage for survey; we prescribed empirical antibiotics and antituberculosis medication soon after alveolar lavage showing positive acid-fast stain. She was extubated on hospital day 31 and discharged on hospital day 73. In conclusion, early diagnosis and intervention for underlying disease and intensive bundle care for multiorgan failure are crucial for both ARDS and HLH.

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