scholarly journals Hemophagocytic Lymphohistiocytosis Secondary to Adult-Onset Still’s Disease

Cureus ◽  
2021 ◽  
Author(s):  
Bhupen Barman ◽  
Md Jamil ◽  
Pranjal Kalita ◽  
Biswajit Dey
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

scholarly journals An Adult-Onset Still’s Disease During Pregnancy that Delivered a Neonate with Hemophagocytic Lymphohistiocytosis and Severe Liver Failure Requiring Liver Transplantation: A Case Report and Literature Review

2021 ◽  
Author(s):  
Eri Watanabe ◽  
Yohei Sugiyama ◽  
Hiroaki Sato ◽  
Toshiyuki Imanishi ◽  
Akinari Fukuda ◽  
...  
Keyword(s):  
Liver Transplantation ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Severe Liver ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Abstract Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology that is categorized as a non-hereditary disease. Neonatal hemophagocytic lymphohistiocytosis (HLH) is also a rare, but potentially fatal condition. Neonatal HLH is one of the causes of neonatal acute liver failure that often requires urgent liver transplantation. The relationship between AOSD during pregnancy and neonatal HLH currently remains unclear. We encountered a case of AOSD that developed during pregnancy, and an offspring was born with neonatal HLH resulting in severe liver failure. The mother with AOSD only presented with liver dysfunction during pregnancy; however, disease activity was exacerbated after delivery. The maternal clinical course was quite severe and refractory that she required biological therapy in addition to high-dose corticosteroids and immunosuppressants. Additionally, the severe condition of the neonate with HLH and acute liver failure required intensive care with the administration of steroids and intravenous immunoglobulin treatments, and ultimately liver transplantation. This is the first case that severe maternal AOSD associated with a neonatal HLH resulted in severe clinical courses. Physicians need to be aware of the risk of a mother with AOSD delivering an offspring with neonatal HLH with potentially acute liver failure.

scholarly journals Adult Onset Still’s Disease Associated withMycoplasma pneumoniaeInfection and Hemophagocytic Lymphohistiocytosis

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Abhishek Agnihotri ◽  
Allison Ruff ◽  
Lauren Gotterer ◽  
Addie Walker ◽  
Amy H. McKenney ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
The Body ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Elevated Liver Enzymes ◽  
Mycoplasma Pneumonia ◽  
Adult Onset Still's Disease

Adult Onset Still’s Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated byMycoplasma pneumoniainfection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated. She had hypoalbuminemia, elevated liver enzymes, a very high serum ferritin level, positive anti-Mycoplasma pneumoniaIgG and IgM antibodies, and normal rheumatoid factor and anti-nuclear antibodies. The chest X-ray showed diffuse bilateral infiltrates. Bone marrow biopsy revealed hemophagocytosis. The patient was treated with azithromycin, methylprednisolone, and anakinra and was discharged home on cyclosporine and prednisone. This case highlights that patients can develop features of both AOSD and HLH at the beginning of the disease and early diagnosis and treatment increase the likelihood of recovery.

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