Hemophagocytic Lymphohistiocytosis Complicating Systemic Sarcoidosis

Abughanimeh, Omar; Qasrawi, Ayman; Ghanimeh, Mouhanna Abu

doi:10.7759/cureus.2838

Cited by 8 publications

(22 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In the present study, prognosis was very poor in patients with HLH caused by autoimmune diseases and immunodeficiency. HLH that results from rheumatic or other systemic diseases, including Still's disease and sarcoidosis, is termed MAS [18][19][20]. In our cohort, 13 patients had HLH caused by autoimmune disease, including systemic lupus erythematosus, juvenile rheumatoid arthritis, dermatomyositis, Kawasaki disease, and anaphylactoid purpura, and the survival rate was 65%.…”

Section: Discussionmentioning

confidence: 96%

Treatment outcomes and prognostic factors for non- malignancy associated secondary hemophagocytic lymphohistiocytosis in children

et al. 2020

View full text Add to dashboard Cite

Background: Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that requires prompt diagnosis and appropriate treatment. A risk-stratification model that could be used to identify high-risk pediatric patients with HLH who should be considered for second-line therapies, including salvage regimens and allogeneic hematopoietic cell transplantation (HCT), was developed. Methods: The medical records of 88 pediatric patients (median age 1.4 years, range 0.2-15 years) with nonmalignancy associated secondary HLH were retrospectively reviewed. Treatment strategies included dexamethasone, etoposide, and cyclosporine. Results: Survival analysis showed HLH patients with infections other than Epstein-Barr virus (EBV) and unknown causes experienced better 5-year overall survival (OS) than patients with HLH due to autoimmune disease, EBV or immunodeficiency (76% vs. 65, 33.3, 11%, p < 0.001). On multivariate analysis, among all patients, non-response at 8 weeks was the most powerful predictor of poor OS. When treatment response was excluded, hemoglobin < 60 g/L and albumin < 25 g/L at diagnosis were associated with poor OS. In patients with EBV-HLH, hemoglobin < 60 g/L at diagnosis was associated with poor OS. A prognostic risk score was established and weighted based on hazard ratios calculated for three parameters measured at diagnosis: hemoglobin < 60 g/L (2 points), platelets < 30 × 10 9 /L (1 point), albumin < 25 g/L (2 points). Five-year OS of low-risk (score 0-1), intermediate-risk (score 2), and poor-risk (score ≥ 3) patients were 88, 38, and 22%, respectively (p < 0.001). Conclusions: These findings indicate that clinicians should be aware of predictive factors at diagnosis and consider 8-week treatment response to identify patients with high-risk of disease progression and the need for second-line therapy and allogeneic HCT.

show abstract

Section: Discussionmentioning

confidence: 96%

Treatment outcomes and prognostic factors for non- malignancy associated secondary hemophagocytic lymphohistiocytosis in children

et al. 2020

View full text Add to dashboard Cite

show abstract

“…A national survey conducted in France over two years found 26 patients with MAS, only one of which was related to sarcoidosis [9]. Other isolated case reports implicate sarcoidosis as a cause of MAS [10]. These studies and reports show that there is a rare albeit established association between MAS and sarcoidosis, and therefore it should be considered as a possible underlying etiology when working up a patient with suspected HLH/MAS.…”

Section: Discussionmentioning

confidence: 99%

Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis

Kasparian

Anand

Burns

et al. 2019

Cureus

View full text Add to dashboard Cite

Hemophagocytic lymphohistiocytosis (HLH) due to an underlying rheumatologic condition is known as macrophage activation syndrome (MAS), a rare and serious complication that often has a delayed diagnosis. MAS can complicate any rheumatologic disease, although it is most prevalent in systemic juvenile idiopathic arthritis. MAS occurring as a sequela of sarcoidosis is seldom reported. Herein, we present an uncommon case of MAS occurring secondary to suspected extrapulmonary sarcoidosis and the associated diagnostic challenges. A 53-year-old White female presented with a 20-month history of constitutional symptoms of an unclear etiology. Her extensive workup included equivocal bone marrow and liver biopsies, suggestive of occasional hemophagocytosis. On admission, she met criteria for HLH based on the HLH-94 diagnostic guidelines. A repeat liver biopsy was performed revealing non-necrotizing granulomas in the parenchyma. Given the concern for an extrapulmonary sarcoidosis, she was started on pulse-dose steroids with subsequent symptomatic resolution. Two years later, she remains in complete remission. As a systemic disease, sarcoidosis can manifest in any organ and present in a variety of ways. While HLH and MAS have numerous etiologies, sarcoidosis should be considered as a potential underlying diagnosis, and prompt treatment initiation with steroids may reduce morbidity and mortality.

show abstract

“…Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal inflammatory syndrome characterized by histiocyte proliferation and hemophagocytosis (Abughanimeh et al, 2018;Bartholo et al, 2012;Atteritano et al, 2012;Fukaya et al, 2008;Dhote et al, 2003). It often presents with fever, hepatosplenomegaly and pancytopenia (Abughanimeh et al, 2018;Bartholo et al, 2012;Atteritano et al, 2012;Fukaya et al, 2008;Dhote et al, 2003;Henter et al, 2007). HLH is classified as primary or secondary (Abughanimeh et al, 2018;Bartholo et al, 2012;Atteritano et al, 2012;Fukaya et al, 2008).…”

Section: Introductionmentioning

confidence: 99%

“…It often presents with fever, hepatosplenomegaly and pancytopenia (Abughanimeh et al, 2018;Bartholo et al, 2012;Atteritano et al, 2012;Fukaya et al, 2008;Dhote et al, 2003;Henter et al, 2007). HLH is classified as primary or secondary (Abughanimeh et al, 2018;Bartholo et al, 2012;Atteritano et al, 2012;Fukaya et al, 2008). Primary HLH, which is more common in the pediatric population is caused by genetic defects of immune system regulation while secondary HLH is often triggered by infections, malignancies or rheumatic diseases in susceptible individuals (Abughanimeh et al, 2018;Bartholo et al, 2012;Atteritano et al, 2012;Fukaya et al, 2008;Dhote et al, 2003).…”

Section: Introductionmentioning

confidence: 99%

Hemophagocytic Lymphohistiocytosis Associated with Sarcoidosis: A Case Report and Review of the Literature

Sackey¹,

Angelini²

2020

FMCR

View full text Add to dashboard Cite

Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal inflammatory syndrome characterized by histiocyte proliferation and hemophagocytosis. Primary HLH primarily occurs in children and is caused by genetic mutations. Secondary HLH, more often seen in adults, occurs as a result of infections, malignancies or rarely rheumatic diseases. HLH secondary to sarcoidosis is rare. We present a case of HLH associated with recently diagnosed sarcoidosis in a 61-year-old female.

show abstract

Hemophagocytic Lymphohistiocytosis Complicating Systemic Sarcoidosis

Cited by 8 publications

References 15 publications

Treatment outcomes and prognostic factors for non- malignancy associated secondary hemophagocytic lymphohistiocytosis in children

Treatment outcomes and prognostic factors for non- malignancy associated secondary hemophagocytic lymphohistiocytosis in children

Macrophage Activation Syndrome Secondary to Underlying Sarcoidosis

Hemophagocytic Lymphohistiocytosis Associated with Sarcoidosis: A Case Report and Review of the Literature

Contact Info

Product

Resources

About