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Extreme Hyperferritinemia

Yi-Zhen Liu, Lan-Qing Bi, Gui-Lin Chang, Ye Guo, Si Sun
Background: Extranodal natural killer (NK) / T cell lymphoma is a subtype of non-Hodgkin's lymphoma (NHL) that usually has an aggressive clinical course. It is the predominant trigger of lymphoma-associated hemophagocytic syndrome (LAHS), which is highly lethal and with extremely poor prognosis. This study is aiming to characterize the associated clinical features and prognostic factors of the disease. Patients and methods: Twenty-eight patients with extranodal NK/T cell lymphoma associated hemophagocytic lymphohistiocytosis (HLH) were retrospectively analyzed...
2019: Cancer Management and Research
Bixia Gao, Xiaoyu Jia, Jicheng Lv, Jie Dong
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is characterized by excessive activation of the immune system due to infection, autoimmune diseases, or malignancy. As an aggressive and life-threatening clinical syndrome, HLH secondary to peritoneal dialysis associated peritonitis (PDAP) has never been reported. CASE PRESENTATION: A 34-year-old female peritoneal dialysis (PD) patient was hospitalized for fever, progressively multi-organ damage (including cytopenias, abnormalities of coagulation and liver enzyme) after an episode of organism-specific peritonitis...
January 29, 2019: BMC Nephrology
Shuhei Kurosawa, Noritaka Sekiya, Kazuaki Fukushima, Kazuhiko Ikeuchi, Akito Fukuda, Hideyuki Takahashi, Fangyi Chen, Hideki Hasegawa, Harutaka Katano, Tsunekazu Hishima, Keigo Setoguchi
BACKGROUND: Herpes simplex virus (HSV) has various presentations, depending on the patient's immune status, age, and the route of transmission. In adults, HSV type 1 is found predominantly in the oral area, and HSV type 2 (HSV-2) is commonly found in the genital area. HSV-2 infection without genital lesions is uncommon. Herein we report a unique case of pharyngotonsillitis as an initial manifestation of disseminated HSV-2 infection without genital involvement. CASE PRESENTATION: A 46-year-old male was admitted to our hospital with a 1-week history of fever and sore throat...
January 17, 2019: BMC Infectious Diseases
Kate F Kernan, Lina Ghaloul-Gonzalez, Bita Shakoory, John A Kellum, Derek C Angus, Joseph A Carcillo
Post-hoc subgroup analysis of the negative trial of interleukin-1β receptor antagonist (IL1RA) for septic shock suggested that patients with features of macrophage activation syndrome (MAS) experienced a 50% relative risk reduction for mortality with treatment. Here we seek a genetic basis for this differential response. From 1341 patients enrolled in the ProCESS trial of early goal directed therapy for septic shock, we selected 6 patients with MAS features and the highest ferritin, for whole exome sequencing (mean 24,030...
July 6, 2018: Genes and Immunity
Robert Szulawski, Peter J Kourlas, Marc Antonchak
BACKGROUND Systemic lupus erythematosus (SLE) has myriad manifestations that can affect any organ system in the body. Macrophage activation syndrome (MAS) is a disease of uncontrolled lymphocyte and macrophage proliferation and activation, which has various triggers, including autoimmune disorder, viral infection, and malignancy. We report here on MAS as a complication of adult SLE, a rare association in the literature, in a patient with an unknown past medical history. CASE REPORT A 38-year-old male patient presented with severe muscle weakness, diffuse abdominal cramps with vomiting and incontinence of stool, confusion, cough, and sweating increasing in severity for about 1 week...
June 22, 2018: American Journal of Case Reports
Na Li, Li Zhang, Jie Liu, Jing Zhang, Hua-Wei Weng, Hong-Yu Zhuo, Li-Qun Zou
Nasal type, extranodal nature killer (NK)/T cell lymphoma-associated hemophagocytic syndrome (NK/T-LAHS) is a rare and fatal disorder with extremely poor prognosis. To investigate its clinical characteristics and risk factors, we retrospectively analyzed 295 patients with nasal type, extranodal nature killer/T cell lymphoma, of which 21 were diagnosed with hemophagocytic syndrome, with a cumulative incidence of 7.1%. The most frequently clinical characteristics were fever, lymphadenopathy, hepatosplenomegaly, pancytopenia, hyperferritinemia, liver dysfunction, hypertriglyceridemia, hypofibrinogenemia and evaluated lactate dehydrogenase (LDH) level...
April 3, 2017: Cancer Biology & Therapy
Ersin Gumus, Osman Abbasoglu, Cahit Tanyel, Fatma Gumruk, Hasan Ozen, Aysel Yuce
The use of extended criteria donors who might have previously been deemed unsuitable is an option to increase the organ supply for transplantation. This report presents a pediatric case of a successful liver transplantation from a donor with β-thalassemia intermedia. A patient, 6-year-old female, with a diagnosis of cryptogenic liver cirrhosis underwent deceased donor liver transplantation from a thalassemic donor. Extreme hyperferritinemia was detected shortly after transplantation. The most probable cause of hyperferritinemia was iron overload secondary to transplantation of a hemosiderotic liver...
May 2017: Pediatric Transplantation
Neelima Verma, Jyoti Chakraverty, Pankaj Baweja, Alka Girotra, Leena Chatterjee, Manish Chugh
Hyperferritinemia (>10,000 ng/ml) is an important hallmark used as an indicator of infection triggered macrophage activation syndrome leading to hemophagocytic lympho histiocytosis (HLH). Measurement of serum ferritin can be used in diagnosis as well as disease monitoring indicator and prognosis related to HLH, cAPS, sepsis, neoplasm and inflammatory conditions. It is a major contributor to manage critically ill patients as predicting and monitoring indicator. It can be used as acute phase response in conditions of MAS, AOSD, cAPS etc...
March 2017: Indian Journal of Clinical Biochemistry: IJCB
Simone Schüller, Andishe Attarbaschi, Angelika Berger, Caroline Hutter, Katrin Klebermass-Schrehof, Manuel Steiner
OBJECTIVE: To present the diagnostic workup in an extremely low birth weight infant patient with signs of both sepsis and hemophagocytosis. PARTICIPANTS: A preterm infant presented with clinical and laboratory signs of early-onset sepsis including hepatosplenomegaly, thrombocytopenia, direct hyperbilirubinemia, and elevated liver enzymes. METHODS: Despite extensive septic workup, no underlying infection was detected. Additional hyperferritinemia and other elevated inflammatory parameters raised the suspicion of a primary or secondary hemophagocytic lymphohistiocytosis (HLH)...
October 2016: Pediatric Hematology and Oncology
Dídac Casas-Alba, Antonio Martínez-Monseny, Laura Monfort, Carmen Muñoz-Almagro, María Cabrerizo, Àngela Deyà, Cristian Launes
Human parechovirus-3 has been associated with severe clinical manifestations in infants, such as sepsis-like illness and meningoencephalitis. Nevertheless, the vast majority of patients have a favorable outcome. We report the occurrence of this infection in dizygotic infants with extreme hyperferritinemia and a transient impairment of natural killer cell cytotoxicity.
December 2016: Pediatric Infectious Disease Journal
Damien Denimal, Louise Ménégaut, Cédric Rossi, Laurence Duvillard, David Masson
INTRODUCTION: Major hyperferritinemia is a rare feature in clinical laboratories associated with a wide variety of disorders, including hemophagocytic lymphohistiocytosis (HLH). The diagnosis of HLH is based on clinical and biological criteria, such as those proposed by the Histiocyte Society. However, several of these criteria are not relevant in the specific setting of hematologic malignancies. MATERIALS AND METHODS: A 69-year-old male was treated for an acute myeloid leukaemia...
2016: Biochemia Medica: časopis Hrvatskoga Društva Medicinskih Biokemičara
Martine Regenboog, André B P van Kuilenburg, Joanne Verheij, Dorine W Swinkels, Carla E M Hollak
Gaucher disease (GD) is characterized by large amounts of lipid-storing macrophages and is associated with accumulation of iron. High levels of ferritin are a hallmark of the disease. The precise mechanism underlying the changes in iron metabolism has not been elucidated. A systematic search was conducted to summarize available evidence from the literature on iron metabolism in GD and its potential pathophysiological implications. We conclude that in GD, a chronic low grade inflammation state can lead to high ferritin levels and increased hepcidin transcription with subsequent trapping of ferritin in macrophages...
November 2016: Blood Reviews
Katie Sackett, Maros Cunderlik, Nishant Sahni, Anthony A Killeen, Andrew P J Olson
OBJECTIVES: Hyperferritinemia can be a result of inflammation, infection, chronic iron overload, or other uncommon pathologies including hemophagocytic lymphohistiocytosis (HLH). There is a historical association between extreme hyperferritinemia and HLH, but in reality HLH is associated with a minority of hyperferritinemic states. METHODS: We identified conditions most associated with hyperferritinemia by identifying 65,536 serum ferritin levels at the University of Minnesota Hospital over a five-year period, with 86 values higher than 10,000 ng/mL...
May 2016: American Journal of Clinical Pathology
M Raley
A 35-year-old woman presented to our emergency department complaining of arthralgia, rash, fevers, and muscle weakness for the past year. These symptoms initially began as unilateral upper lip swelling, which then progressed to a facial rash and orbital swelling over two weeks with associated fevers, which eventually subsided. The patient then began having fluctuating arthralgia and muscle weakness with associated rash and subjective fevers. She had rheumatologic evaluation several months prior to our encounter at another facility, which failed to reveal an explanation for her symptoms...
May 2015: Journal of the Louisiana State Medical Society: Official Organ of the Louisiana State Medical Society
Akiko Masuda, Masahiro Jona, Akira Honda, Yumiko Satoh, Mineo Kurokawa, Yutaka Yatomi
A 64-year-old man was referred to our hospital because of mild anemia, erythroblasts in peripheral blood, and hyperferritinemia. In our hospital, no evidence of cytopenia was found. The peripheral blood smear showed pseudo-Pelger-Huët anomaly and giant platelets. The serum ferritin level was extremely high (1,405 ng/ml). A bone marrow examination was performed for further evaluation and revealed trilineage dysplasia. Since the patient did not fulfill the diagnostic criteria for cytopenias associated with myelodysplastic syndromes, he was diagnosed as having idiopathic dysplasia of undetermined/uncertain significance (IDUS)...
December 2015: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Isha Sood, Gaurav M Kasundra, Prathibha Shankaranarayana Supriya, Aradhana Gupta, Bharat Bhushan
A 50-year-old female presented with fever, symmetrical arthralgias, rash, painful oral ulcerations and alopecia since 8 weeks. Examination showed mild hepatospleenomegaly. Investigations revealed leucocytosis, neutrophilia, elevated sedimentation rate and raised ferritin levels (3850 ng/ml). Computerized tomography (CT) abdomen showed hepatospleenomegaly, mild ascitis and mild bilateral pleural-effusion. After ruling out occult infections, tuberculosis, malignancies and autoimmune diseases by appropriate investigations, and due to raised ferritin levels, adult onset stills disease (AOSD) was diagnosed...
July 2015: Journal of Mid-life Health
Diego A Adrianzen Herrera, Matthew Scott, Mala Varma
Myelomatous involvement of pleural effusions developing in patients with multiple myeloma is extremely rare and only a few cases have been reported so far. It is thought to represent an aggressive clinical progression of disease and is usually associated with severe complications, poor prognosis and high mortality. Ferritin is a marker of inflammatory pathways that plays a significant role in plasma cell malignancies and has been studied as a prognostic factor for multiple myeloma. In severe inflammatory states such as septic shock or hemophagocytic lymphohistiocytosis, extreme levels of ferritin are thought to precipitate a cytokine storm associated with poor clinical outcomes...
May 2015: Case Reports in Oncology
Ethan S Sen, Sarah L N Clarke, Athimalaipet V Ramanan
Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic diseases such as systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus. It is often considered a type of secondary hemophagocytic lymphohistiocytosis (HLH) and results from over-activation of T lymphocytes and macrophages leading to a "cytokine storm". Characteristic features are persistent fever, lymphadenopathy, hepatosplenomegaly, cytopenias (anemia, leucopenia, thrombocytopenia), raised C-reactive protein, falling erythrocyte sedimentation rate, hypofibrinogenemia, transaminitis, hypertriglyceridemia and extreme hyperferritinemia often associated with multi-organ impairment...
March 2016: Indian Journal of Pediatrics
N Issa, O Guisset, G Mourissoux, C Gabinski, F Camou
INTRODUCTION: Leptospirosis is a worldwide zoonosis caused by the spirochete Leptospira interrogans. The spectrum of symptoms reported in leptospirosis is extremely broad. Thrombocytopenia is common during the acute phase of leptospirosis but its pathophysiological mechanism remains not well defined. CASE REPORT: We report a 56-year-old man hospitalized for severe sepsis with acute kidney injury and liver failure. Because of the recent flood of his house, we suspected leptospirosis...
August 2015: La Revue de Médecine Interne
Grant S Schulert, Alexei A Grom
Macrophage activation syndrome (MAS) is an acute episode of overwhelming inflammation characterized by activation and expansion of T lymphocytes and hemophagocytic macrophages. In rheumatology, it occurs most frequently in patients with systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus. The main clinical manifestations include cytopenias, liver dysfunction, coagulopathy resembling disseminated intravascular coagulation, and extreme hyperferritinemia. Clinically and pathologically, MAS bears strong similarity to hemophagocytic lymphohistiocytosis (HLH), and some authors prefer the term secondary HLH to describe it...
2015: Annual Review of Medicine
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