scholarly journals A Case of Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Ivan Columbus-Morales ◽  
Lucas Maahs ◽  
Sanam Husain ◽  
Stuart C. Gordon ◽  
Kedar V. Inamdar ◽  
...  
Keyword(s):  
Liver Function ◽  
Autoimmune Diseases ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Immunocompromised Patient ◽  
Multiorgan Failure ◽  
Clinical Manifestations ◽  
Rare Condition ◽  
Immune Dysregulation ◽  
Multiorgan Dysfunction ◽  
Disseminated Histoplasmosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition characterized by a pathologic immune dysregulation resulting in extreme inflammation. Clinical manifestations are varied but can include severe multiorgan failure and death. HLH has been associated with malignancies, autoimmune diseases, and infections, such as histoplasmosis. Histoplasmosis commonly has subclinical manifestations but can also present in its disseminated form. We present the case of an immunocompromised patient with worsening liver function caused by hepatic histoplasmosis that later triggered HLH with severe multiorgan dysfunction.

scholarly journals Hemophagocytic Lymphohistiocytosis (HLH) in a Patient with Disseminated Histoplasmosis

2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Neeraja Swaminathan ◽  
Jorge M. Vinicius ◽  
Jesse Serrins
Keyword(s):  
Antifungal Therapy ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Histoplasma Capsulatum ◽  
Interleukin 1 ◽  
Clinical Manifestations ◽  
Rare Condition ◽  
Genetic Mutation ◽  
Rapid Progression ◽  
Disseminated Histoplasmosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare condition characterized by an overwhelming inflammatory cascade activation which is often associated with rapid progression and high mortality. It may be familial with an underlying genetic mutation or triggered by infection, malignancy, and autoimmune disease. Disseminated histoplasmosis caused by histoplasma capsulatum is a granulomatous fungal disease seen typically in immunocompromised patients with varied clinical manifestations and requires long-term antifungal therapy. We present the case of a 61-year-old immunocompromised female with significant travel history who came with fever, pancytopenia, and liver failure raising suspicion for HLH that prompted a bone marrow biopsy procedure. Hemophagocytic figures consistent with HLH and numerous encapsulated fungi resembling histoplasma were visualized. She was treated with intravenous (IV) liposomal amphotericin B. Etoposide chemotherapy and interleukin-1 (IL-1) antagonist anakinra were deferred in order to limit her immunosuppression, and treatment was focused on antifungal therapy.

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 Hemophagocytic Lymphohistiocytosis Secondary to Human Immunodeficiency Virus-Associated Histoplasmosis

2015 ◽  
Vol 2 (4) ◽  
Author(s):  
Anthony A. Castelli ◽  
David G. Rosenthal ◽  
Rachel Bender Ignacio ◽  
Helen Y. Chu
Keyword(s):  
Escherichia Coli ◽  
Human Immunodeficiency Virus ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Mycobacterium Avium Complex ◽  
Multiorgan Failure ◽  
Rapid Diagnosis ◽  
Disseminated Histoplasmosis ◽  
Immunodeficiency Virus ◽  
Underlying Disorder ◽  
Immunocompromised Hosts

Abstract Hemophagocytic lymphohistiocytosis (HLH) in immunocompromised hosts is a fulminant syndrome of immune activation with high rates of mortality that may be triggered by infections or immunodeficiency. Rapid diagnosis and treatment of the underlying disorder is necessary to prevent progression to multiorgan failure and death. We report a case of HLH in a patient with human immunodeficiency virus, disseminated histoplasmosis, Mycobacterium avium complex, and Escherichia coli bacteremia. We discuss management of acutely ill patients with HLH and treatment of the underlying infection versus initiation of HLH-specific chemotherapy.

scholarly journals Hemophagocytic lymphohistiocytosis and miliary tuberculosis in a previously healthy individual: a case report

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Linn Hereide Trovik ◽  
Miriam Sandnes ◽  
Bjørn Blomberg ◽  
Gunhild Holmaas ◽  
Aymen Bushra Ahmed ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Multiorgan Failure ◽  
Clinical Manifestations ◽  
Miliary Tuberculosis ◽  
Diagnostic Workup ◽  
Eastern Africa ◽  
Simultaneous Increase ◽  
Current Case ◽  
Timely Initiation

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a rare heterogenous genetic or acquired hyperinflammatory syndrome associated with a high degree of morbidity and mortality. HLH has clinical manifestations related to abnormal prolonged activation of T lymphocytes and macrophages with an excess of proinflammatory cytokines. The main causes of secondary HLH are malignancies and infectious diseases. Case presentation The patient was a 54-year-old man, originally from Eastern Africa, who had lived in Northern Europe for 30 years. Here we describe the clinical features, laboratory parameters, diagnostic workup, management and outcome data of a previously healthy 54-year-old man diagnosed with HLH secondary to tuberculosis. The patient was initially treated for a community-acquired pneumonia. He developed multiorgan failure with acute respiratory distress syndrome, hypertransaminasemia, and kidney and bone marrow dysfunction. The clinical course together with a simultaneous increase in serum ferritin raised the suspicion of HLH. The patient fulfilled seven out of eight diagnostic criteria for HLH. A thorough diagnostic workup with respect to HLH and a potential underlying disease was initiated. Cultivation of bronchoalveolar lavage fluid, stool and urine, and polymerase chain reaction of epithelioid cell granulomas in the bone marrow were all positive for Mycobacterium tuberculosis. He was treated for both HLH and tuberculosis, and he survived without any sequelae. Conclusions We present one of few published cases of a patient who survived HLH triggered by miliary tuberculosis. The current case illustrates the need for awareness of these two diagnoses, and the timely initiation of specific and supportive treatment to reduce mortality.

Bone marrow necrosis and fat embolism syndrome: a near fatal complication in previously undiagnosed sickle beta + thalassaemia

2021 ◽  
Vol 14 (1) ◽  
pp. e238317
Author(s):  
Nibash Budhathoki ◽  
Sunita Timilsina ◽  
Bebu Ram ◽  
Douglas Marks
Keyword(s):  
Bone Marrow ◽  
Multiorgan Failure ◽  
Rare Condition ◽  
Altered Mental Status ◽  
Fat Embolism ◽  
Bone Marrow Necrosis ◽  
Embolism Syndrome ◽  
Hb S ◽  
High Index Of Suspicion ◽  
Specific Symptoms

Prevalence of haemoglobin sickle-β+ thalassaemia (Hb S/β+thal) is variable with geography ranging from 0.2% to 10% among sickle cell patients. Clinical presentation of Hb S/β+thal patients depends on HbA level, with milder disease often going undiagnosed. However, rarely these patients can present with a fulminant vaso-occlusive crisis (VOC). Given VOC can present with non-specific symptoms, the diagnosis and treatment is often delayed. Here, we present a patient who initially developed altered mental status, pancytopenia and multiorgan failure due a critical VOC resulting in bone marrow necrosis and fat embolism. Subsequent workup confirmed that our patient had Sickle-β+ thalassaemia, which had gone undiagnosed, despite subclinical evidence of haemolysis on routine lab work for years. Following diagnosis and initiation of RBC exchange, he improved significantly and was discharged home. High index of suspicion and bone marrow biopsy is vital for early diagnosis and management of this rare condition.

scholarly journals Autoimmunity and Genetic Syndromes: A Focus on Down Syndrome

Genes ◽  
2021 ◽  
Vol 12 (2) ◽  
pp. 268
Author(s):  
Marta Ferrari ◽  
Stefano Stagi
Keyword(s):  
Down Syndrome ◽  
Autoimmune Diseases ◽  
Normal Population ◽  
Underlying Mechanism ◽  
Short Review ◽  
Immune Dysregulation ◽  
Infectious Complications ◽  
Genetic Syndromes ◽  
Genetic Syndrome

Within immune system-related diseases, autoimmunity has always represented a field of great interest, although many aspects remain poorly understood even today. Genetic syndromes associated with immunity disorders are common and represent an interesting model for a better understanding of the underlying mechanism of autoimmunity predisposition. Among these conditions, Down syndrome (DS) certainly deserves special attention as it represents the most common genetic syndrome associated with immune dysregulation, involving both innate and adaptive immunity. Autoimmunity represents a well-known complication of DS: it is estimated that people affected by this disease present a risk four to six times higher than the normal population to develop autoimmune diseases such as celiac disease, type 1 diabetes mellitus, and hypo- or hyperthyroidism. Several factors have been considered as possible etiology, including genetic and epigenetic modifications and immune dysregulation. In times in which the life expectancy of people with DS has been extremely prolonged, thanks to improvements in the diagnosis and treatment of congenital heart disease and infectious complications, knowledge of the mechanisms and proper management of autoimmune diseases within this syndrome has become essential. In this short review, we aim to report the current literature regarding the genetic, immune, and environmental factors that have been proposed as the possible underlying mechanism of autoimmunity in individuals with DS, with the intent to provide insight for a comprehensive understanding of these diseases in genetic syndromes.

scholarly journals Managing the combined consequences of COVID-19 infection and lock-down policies on athletes: narrative review and guidelines proposal for a safe return to sport

2020 ◽  
Vol 6 (1) ◽  
pp. e000849
Author(s):  
Jean-Bernard Fabre ◽  
Laurent Grelot ◽  
William Vanbiervielt ◽  
Julien Mazerie ◽  
Raphael Manca ◽  
...  
Keyword(s):  
Sports Medicine ◽  
Acute Myocarditis ◽  
Multiorgan Failure ◽  
Clinical Manifestations ◽  
Return To Sport ◽  
Disease Spread ◽  
Cardiac Complications ◽  
Professional Soccer ◽  
Physical Abilities ◽  
Fitness And Health

COVID-19 pandemic is a global health matter. The disease spread rapidly across the globe and brought the world of sports to an unprecedented stoppage. Usual symptoms of the disease are fever, cough, myalgia, fatigue, slight dyspnoea, sore throat and headache. In more severe cases, dyspnoea, hypoxaemia, respiratory failure, shock and multiorgan failure occur. This appears to be a self-limiting phenomenon related to individuals with coexisting medical conditions, such as hypertension, diabetes and cardiovascular disorders. Nevertheless, cases have been reported in professional soccer players in extremely good fitness condition, demonstrating that athletes are not spared by the disease. Despite COVID-19 clinical manifestations are mainly respiratory, major cardiac complications are being reported, leading to acute myocarditis. One difficulty is that symptoms of COVID-19 vary among individuals, with athletes being affected with no apparent sign of the disease. This could be a real danger for amateur or professional athletes when returning to their usual training and thus to play. Another threat is that the lock-down policies did not allow most athletes to follow their usual training routines. There is thus a need for a careful approach by the sports medicine community to ensure safety of all athletes before they return to sport. Here, we propose evaluation guidelines of fitness and health of athletes to (1) reduce any lethal risk of practice, especially myocarditis and sudden cardiac death; (2) evaluate the combined consequences of the disease and detraining on the physical abilities and biological profile of athletes; and (3) monitor postinfection fatigue symptoms.

Varied Clinical Manifestations of LRBA Deficiency (Immune Dysregulation Disorder)

2021 ◽  
Vol 58 (3) ◽  
pp. 285-286
Author(s):  
Deenadayalan Munirathnam ◽  
Vimal Kumar ◽  
Meena Sivasankaran ◽  
Silky Agrawal
Keyword(s):  
Clinical Manifestations ◽  
Immune Dysregulation ◽  
Lrba Deficiency

scholarly journals Pathobiology of A/Chicken/Hong Kong/220/97 (H5N1) Avian Influenza Virus in Seven Gallinaceous Species

2001 ◽  
Vol 38 (2) ◽  
pp. 149-164 ◽  
Author(s):  
L. E. L. Perkins ◽  
D. E. Swayne
Keyword(s):  
Hong Kong ◽  
Avian Influenza ◽  
Pulmonary Edema ◽  
Multiorgan Failure ◽  
Clinical Manifestations ◽  
Highly Pathogenic ◽  
H5n1 Avian Influenza Virus ◽  
Multiple Organs ◽  
H5n1 Avian Influenza ◽  
Virus Localization

Direct bird-to-human transmission, with the production of severe respiratory disease and human mortality, is unique to the Hong Kong-origin H5N1 highly pathogenic avian influenza (HPAI) virus, which was originally isolated from a disease outbreak in chickens. The pathobiology of the A/chicken/Hong Kong/ 220/97 (H5N1) (HK/220) HPAI virus was investigated in chickens, turkeys, Japanese and Bobwhite quail, guinea fowl, pheasants, and partridges, where it produced 75-100% mortality within 10 days. Depression, mucoid diarrhea, and neurologic dysfunction were common clinical manifestations of disease. Grossly, the most severe and consistent lesions included splenomegaly, pulmonary edema and congestion, and hemorrhages in enteric lymphoid areas, on serosal surfaces, and in skeletal muscle. Histologic lesions were observed in multiple organs and were characterized by exudation, hemorrhage, necrosis, inflammation, or a combination of these features. The lung, heart, brain, spleen, and adrenal glands were the most consistently affected, and viral antigen was most often detected by immunohistochemistry in the parenchyma of these organs. The pathogenesis of infection with the HK/220 HPAI virus in these species was twofold. Early mortality occurring at 1-2 days postinoculation (DPI) corresponded to severe pulmonary edema and congestion and virus localization within the vascular endothelium. Mortality occurring after 2 DPI was related to systemic biochemical imbalance, multiorgan failure, or a combination of these factors. The pathobiologic features were analogous to those experimentally induced with other HPAI viruses in domestic poultry.

Enoxaparin-induced Wunderlich syndrome in a young patient with anti-GAD 65-associated opsoclonus and limbic encephalitis: a rare complication in a rare disease

2021 ◽  
Vol 14 (10) ◽  
pp. e244916
Author(s):  
Saranya B Gomathy ◽  
Animesh Das ◽  
Awadh Kishor Pandit ◽  
Achal Kumar Srivastava
Keyword(s):  
Rare Disease ◽  
Young Patient ◽  
Clinical Condition ◽  
Limbic Encephalitis ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Autoimmune Encephalitis ◽  
Rare Condition ◽  
Gad 65 ◽  
Good Improvement

Wunderlich syndrome is a rare condition characterised by acute spontaneous non-traumatic renal haemorrhage into the subcapsular and perirenal spaces. Our case of anti-GAD65-associated autoimmune encephalitis (AE), aged 30 years, developed this complication following use of enoxaparin and was managed by selective glue embolisation of subsegmental branches of right renal cortical arteries. Our case had opsoclonus as one of the clinical manifestations, which has till now been described in only two patients of this AE. This patient received all forms of induction therapies (steroids, plasmapheresis, intravenous immunoglobulin and rituximab) following which she had good improvement in her clinical condition. The good response to immunotherapy is also a point of discussion as this has been rarely associated with anti-GAD65 AE.

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