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.

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 Haemophagocytic Lymphohistiocytosis Associated with Dengue Fever - A case series

2020 ◽  
Vol 21 (2) ◽  
pp. 123-126
Author(s):  
Quazi Tarikul Islam ◽  
Hirinmoy Barman Sagor ◽  
Tasmina Chowdhury Tuli
Keyword(s):  
Intravenous Immunoglobulin ◽  
Dengue Fever ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Medical Condition ◽  
Case Series ◽  
Supportive Therapy ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Laboratory Findings ◽  
Good Outcome ◽  
Life Threatening

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening medical condition characterized by hyperphagocytosis secondary to an inappropriate over-activation of macrophages and lymphocytes that driven by excessive cytokines production which resulted in cellular destructions. Dengue induced hemophagocytic lymphohistiocytosis (HLH) is a serious condition and may prove fatal if not detected early and treated appropriately. Diagnosis of HLH is challenging and usually missed as clinical and laboratory findings are nonspecific. It should be suspected with prolonged fever beyond seven days associated with splenomegaly, hyperferritinemia, worsening cytopenias and development of multiorgan dysfunction. A proportion of patients recovered with supportive therapy, however most required interventions with corticosteroids, intravenous immunoglobulin or chemotherapy. We report 3 cases of dengue associated HLH . Among them 2 patients were treated with steroid with good outcome, and one died from MODS. J MEDICINE JUL 2020; 21 (2) : 123-126

Hemophagocytic Lymphohistiocytosis during the Postpartum Stage of Pregnancy: A Report of Eight Cases

2018 ◽  
Vol 141 (1) ◽  
pp. 55-60 ◽  
Author(s):  
Yue Song ◽  
Jing-Shi Wang ◽  
Yi-Ni Wang ◽  
Zhao Wang
Keyword(s):  
Clinical Features ◽  
Liver Dysfunction ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Standard Therapy ◽  
Case Series ◽  
Clinical Syndrome ◽  
Haemophagocytic Lymphohistiocytosis ◽  
First Case ◽  
Before And After ◽  
Almost All

Haemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome that can be classified as either primary or secondary. Secondary HLH can be triggered by a variety of diseases. Pregnancy-related HLH has already been observed clinically. However, most of these cases occur during pregnancy. Considering that the periods before and after delivery have different clinical features, we presented the first case series of HLH that presented during the postpartum stage of pregnancy. From these cases, we concluded that postpartum HLH is a common form of pregnancy-related HLH. Patients with postpartum HLH may suffer more complicated pathogenesis. Cytopenia was not as common as in other types of HLH, but liver dysfunction was present in almost all cases. The standard therapy of HLH-94/04 was very effective, and the outcomes of postpartum HLH were better.

scholarly journals Epstein-Barr Virus-Related Hemophagocytic Lymphohistiocytosis: Hematologic Emergency in the Critical Care Setting

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Neda Hashemi-Sadraei ◽  
Pimprapa Vejpongsa ◽  
Muhamed Baljevic ◽  
Lei Chen ◽  
Modupe Idowu
Keyword(s):  
Septic Shock ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Epstein Barr Virus ◽  
Care Setting ◽  
Clinical Syndrome ◽  
Barr Virus ◽  
Life Threatening ◽  
Triggering Conditions ◽  
Epstein Barr ◽  
Delayed Recognition

Hemophagocytic lymphohistiocytosis (HLH) is a rare and potential life-threatening clinical syndrome that results from uncontrolled activation of the immune system. Secondary HLH, more commonly observed in adult patients, is seen in the context of underlying triggering conditions. Epstein-Barr virus (EBV) has been recognized as the leading infectious cause and is associated with a poor outcome. As clinical and laboratory features of HLH could overlap with septic shock syndrome in most patients, the diagnosis of HLH, especially in adults, is the most challenging aspect of the disease that results in delayed recognition and treatment of rapidly progressive multiorgan system failure. We report a case of Hemophagocytic lymphohistiocytosis in a patient who presented with signs of septic shock syndrome and we review the literature on the topic.

Haemophagocytic Lymphohistiocytosis (HLH) in a case of Enteric Fever

2016 ◽  
Vol 5 (03) ◽  
pp. 4882
Author(s):  
Vineeta Pande ◽  
Agarkhedkar S. R. ◽  
Ayank Tandon* ◽  
Aditya Agarwal
Keyword(s):  
Inflammatory Reaction ◽  
Enteric Fever ◽  
Infectious Agent ◽  
Clinical Syndrome ◽  
Haemophagocytic Lymphohistiocytosis ◽  
Life Threatening ◽  
Stimulation Of

HLH is an uncommon, life threatening clinical syndrome cause by a severe hyper inflammatory reaction triggered by an infectious agent. The characteristic symptoms of HLH are due to the persistent stimulation of lymphocytes and histiocytes, leading to hyper-cytokinemia. We report a case of HLH in enteric fever in a13 year old female presenting with fever, lymphadenopathy and pancytopenia due an infection caused by Salmonella.

scholarly journals Case Series of Three Patients with Rifampicin-Induced Thrombocytopenia

2020 ◽  
Author(s):  
Chandramouli M.T
Keyword(s):  
Pulmonary Tuberculosis ◽  
Adverse Reactions ◽  
Rare Complication ◽  
Case Series ◽  
Tuberculosis Treatment ◽  
Effective Drug ◽  
Antitubercular Drugs ◽  
Short Course ◽  
Life Threatening

AbstractLife-threatening adverse reactions of antitubercular drugs are uncommon; however, thrombocytopenia is one such rare complication encountered with rifampicin, isoniazid, ethambutol, and pyrazinamide. Rifampicin is the most effective drug and its use in the tuberculosis treatment led to the emergence of modern and effective short-course regimens. I am reporting case series of three patients with pulmonary tuberculosis presented with rifampicin-induced thrombocytopenia.

scholarly journals Treatment and therapeutic strategies for pituitary apoplexy in pregnancy: a case series

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
Yuya Kato ◽  
Yoshikazu Ogawa ◽  
Teiji Tominaga
Keyword(s):  
Pituitary Apoplexy ◽  
Clinical Symptoms ◽  
Case Series ◽  
Optimal Timing ◽  
Presenting Symptoms ◽  
Pathological Evaluation ◽  
Mother And Child ◽  
Life Threatening ◽  
In Pregnancy

Abstract Background Pregnancy is a known risk factor for pituitary apoplexy, which is life threatening for both mother and child. However, very few clinical interventions have been proposed for managing pituitary apoplexy in pregnancy. Case presentation We describe the management of three cases of pituitary apoplexy during pregnancy and review available literature. Presenting symptoms in our case series were headache and/or visual disturbances, and the etiology in all cases was hemorrhage. Conservative therapy was followed until 34 weeks of gestation, after which babies were delivered by cesarean section with prophylactic bolus hydrocortisone supplementation. Tumor removal was only electively performed after delivery using the transsphenoidal approach. All three patients and their babies had a good clinical course, and postoperative pathological evaluation revealed that all tumors were functional and that they secreted prolactin. Conclusions Although the mechanism of pituitary apoplexy occurrence remains unknown, the most important treatment strategy for pituitary apoplexy in pregnancy remains adequate hydrocortisone supplementation and frequent hormonal investigation. Radiological follow-up should be performed only if clinical symptoms deteriorate, and optimal timing for surgical resection should be discussed by a multidisciplinary team that includes obstetricians and neonatologists.

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 Autoimmune Hemolytic Anemia as a Complication of Congenital Anemias. A Case Series and Review of the Literature

2021 ◽  
Vol 10 (15) ◽  
pp. 3439
Author(s):  
Irene Motta ◽  
Juri Giannotta ◽  
Marta Ferraresi ◽  
Kordelia Barbullushi ◽  
Nicoletta Revelli ◽  
...  
Keyword(s):  
Hemolytic Anemia ◽  
Autoimmune Hemolytic Anemia ◽  
Case Series ◽  
Intravenous Immunoglobulins ◽  
Point Of View ◽  
First Line ◽  
Human Erythropoietin ◽  
Immune Mediated ◽  
Hemolytic Crisis ◽  
Life Threatening

Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.

scholarly journals Risk stratification in cardiogenic shock: a focus on the available evidence

2021 ◽  
Author(s):  
C. Sciaccaluga ◽  
G. E. Mandoli ◽  
N. Ghionzoli ◽  
F. Anselmi ◽  
C. Sorini Dini ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Cardiogenic Shock ◽  
Clinical Syndrome ◽  
Diagnostic Assessment ◽  
Prognostic Scores ◽  
Current Evidence ◽  
Efficient Resource ◽  
Threatening Condition ◽  
Life Threatening ◽  
Future Direction

AbstractCardiogenic shock is a clinical syndrome which is defined as the presence of primary cardiac disorder that results in hypotension together with signs of organ hypoperfusion in the state of normovolaemia or hypervolaemia. It represents a complex life-threatening condition, characterized by a high mortality rate, that requires urgent diagnostic assessment as well as treatment; therefore, it is of paramount important to advocate for a thorough risk stratification. In fact, the early identification of patients that could benefit the most from more aggressive and invasive approaches could facilitate a more efficient resource allocation. This review attempts to critically analyse the current evidence on prognosis in cardiogenic shock, focusing in particular on clinical, laboratoristic and echocardiographic prognostic parameters. Furthermore, it focuses also on the available prognostic scores, highlighting the strengths and the possible pitfalls. Finally, it provides insights into future direction that could be followed in order to ameliorate risk stratification in this delicate subset of patients.

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