scholarly journals Hemophagocytic Lymphohistiocytosis Secondary to Disseminated Histoplasmosis in Rheumatologic Disease

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Yael Kusne ◽  
Michael Christiansen ◽  
Christopher Conley ◽  
Juan Gea-Banacloche ◽  
Ayan Sen
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Histoplasma Capsulatum ◽  
Hematologic Malignancies ◽  
Rapid Diagnosis ◽  
Rheumatologic Disease ◽  
Disseminated Histoplasmosis ◽  
Serious Infections ◽  
Time To Diagnosis ◽  
Prompt Diagnosis

Background. Hemophagocytic lymphohistiocytosis (HLH) was originally described in pediatric patients presenting with fever, hepatosplenomegaly, and blood cell abnormalities. Later, HLH was recognized to occur in adults, often associated with hematologic malignancies or serious infections. Conclusion. Patients presenting with HLH are critically ill, and rapid diagnosis is key. In adults, the search for the trigger must begin promptly as time to diagnosis effects survival. The underlying trigger in our patients was Histoplasma capsulatum infection, which is rare in the southwestern United States. Prompt diagnosis led to recovery in one patient, while the other did not survive.

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 (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 as Initial Presentation of Malignancy in Pediatric Patients: Rare but Not to Be Ignored

Children ◽  
2021 ◽  
Vol 8 (12) ◽  
pp. 1083
Author(s):  
Hye-ji Han ◽  
Kyung Taek Hong ◽  
Hyun Jin Park ◽  
Bo Kyung Kim ◽  
Hong Yul An ◽  
...  
Keyword(s):  
T Cell ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Large Cell ◽  
Hematologic Malignancies ◽  
Initial Presentation ◽  
Rapid Screening ◽  
Response To Treatment ◽  
Response Monitoring ◽  
Complete Control

It is complicated to establish a consensus on the management and diagnosis of malignancy-triggered hemophagocytic lymphohistiocytosis (M-HLH) in children, as an initial presentation of malignancy is complicated. In this paper, we analyze the clinical characteristics and outcomes of eight pediatric patients in which M-HLH was the initial presentation of malignancy. All patients had hematologic malignancies: three subcutaneous panniculitis-like T-cell lymphomas, two acute lymphoblastic leukemias, two anaplastic large cell lymphomas, and a systemic EBV + T-cell lymphoma of childhood. The incidence rate of M-HLH among leukemia and malignant lymphoma patients in our institution was 1.9%. From the initial diagnosis of HLH, the median time taken to be diagnosed as a malignancy was about 1.3 months. The majority of patients received HLH-targeted immunosuppression and/or etoposide at first. The patients’ clinical response to treatment for HLH and malignancies were varied. Five out of the eight patients died, one of whom died due to HLH-related cerebral edema after the initiation of chemotherapy. The median overall survival was 1.6 years. In order to improve the survival rate, the early detection of M-HLH, rapid screening for malignancy, and complete control of M-HLH with HLH-directed therapy followed by a thorough response monitoring are required.

Extracorporeal membrane oxygenation outcomes in children with hemophagocytic lymphohistiocytosis

Perfusion ◽  
2016 ◽  
Vol 32 (2) ◽  
pp. 151-156 ◽  
Author(s):  
Katherine Cashen ◽  
Roland L Chu ◽  
Justin Klein ◽  
Peter T Rycus ◽  
John M Costello
Keyword(s):  
Risk Factors ◽  
Extracorporeal Membrane Oxygenation ◽  
Cardiac Failure ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Pediatric Patients ◽  
Life Support ◽  
Extracorporeal Life Support ◽  
Hemorrhagic Complication ◽  
Membrane Oxygenation ◽  
Va Ecmo

Introduction: Pediatric patients with hemophagocytic lymphohistiocytosis (HLH) may develop refractory respiratory or cardiac failure that warrants consideration for extracorporeal membrane oxygenation (ECMO) support. The purposes of this study were to describe the use and outcomes of ECMO in pediatric HLH patients, to identify risk factors for hospital mortality and to compare their ECMO use and outcomes to the ECMO population as a whole. Methods: Pediatric patients (⩽ 18 years) with a diagnosis of HLH in the Extracorporeal Life Support Organization (ELSO) Registry were included. Results: Between 1983 and 2014, data for 30 children with HLH were available in the ELSO registry and all were included in this study. All cases occurred in the last decade. Of the 30 HLH patients, 24 (80%) had a respiratory indication for ECMO and six (20%) had a cardiac indication (of which 4 were E-CPR and 2 cardiac failure). Of the 24 respiratory ECMO patients, 63% were placed on VA ECMO. Compared with all pediatric patients in the ELSO registry during the study period (n=17,007), HLH patients had worse hospital survival (non-HLH 59% vs HLH 30%, p=0.001). In pediatric HLH patients, no pre-ECMO risk factors for mortality were identified. The development of a hemorrhagic complication on ECMO was associated with decreased mortality (p=0.01). Comparing HLH patients with respiratory failure to patients with other immune compromised conditions, the overall survival rate is similar (HLH 38% vs. non-HLH immune compromised 31%, p=0.64). Conclusions: HLH is an uncommon indication for ECMO and these patients have increased mortality compared to the overall pediatric ECMO population. These data should be factored into decision-making when considering ECMO for pediatric HLH patients.

scholarly journals Proliferation through activation: hemophagocytic lymphohistiocytosis in hematologic malignancy

2017 ◽  
Vol 1 (12) ◽  
pp. 779-791 ◽  
Author(s):  
Eric J. Vick ◽  
Kruti Patel ◽  
Philippe Prouet ◽  
Mike G. Martin
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Hematologic Malignancy ◽  
Lymphocytic Infiltration ◽  
Hematologic Malignancies ◽  
Interferon Γ ◽  
Host Immune System ◽  
T Helper ◽  
T Helper 1 ◽  
Factor Α ◽  
Necrosis Factor

AbstractHemophagocytic lymphohistiocytosis (HLH) is a syndrome of cytokine-driven immune activation. Cardinal features include fever, hemophagocytosis, hepatosplenomegaly, lymphocytic infiltration, and hypercytokinemia that result in multisystem organ dysfunction and failure. Familial HLH is genetically driven, whereas secondary HLH (SHL) is caused by drugs, autoimmune disease, infection, or cancer. SHL is associated with worse outcomes, with a median overall survival typically of less than 1 year. This reflects difficulty in both diagnostic accuracy and in establishing reliable treatments, especially in cases of malignancy-induced SHL, which have significantly worse outcomes. Malignancy-induced HLH is seen almost exclusively with hematologic malignancies, constituting 97% of cases in the literature over the past 2 years. In these situations, the native immune response driven by CD8 T cells produces an overabundance of T helper 1 cytokines, notably interferon-γ, tumor necrosis factor-α, and interleukin-6, which establish a positive feedback loop of inflammation, enhancing replication of hematologic malignancies while leaving the host immune system in disarray. In this paper, we present 2 case studies of secondary HLH driven by HM, followed by a review of the literature discussing the cytokines driving HLH, diagnostic criteria, and current treatments used or undergoing investigation.

scholarly journals Oropharyngeal histoplasmosis: report of eleven cases and review of the literature

2011 ◽  
Vol 44 (1) ◽  
pp. 26-29 ◽  
Author(s):  
Vicente Sperb Antonello ◽  
Vanice Ferrazza Zaltron ◽  
Marcela Vial ◽  
Flávio Mattos de Oliveira ◽  
Luiz Carlos Severo
Keyword(s):  
Medical Records ◽  
Histoplasma Capsulatum ◽  
Systemic Mycosis ◽  
Database Analysis ◽  
Laboratory Findings ◽  
Active Pulmonary Tuberculosis ◽  
Disseminated Histoplasmosis ◽  
Amphotericin B Deoxycholate ◽  
One Year ◽  
Hiv Seropositive

INTRODUCTION: Histoplasmosis is a systemic mycosis endemic in Brazil, especially in the State of Rio Grande do Sul, where Histoplasma capsulatum was isolated from the soil. H. capsulatum may compromise unusual areas, including the oropharynx, particularly in patients presenting disseminated histoplasmosis; which is associated with a state of immunosuppression, such as AIDS. METHODS: During database analysis of a total of 265 cases of histoplasmosis, the medical records of 11 patients with histological or microbiological diagnoses of oral histoplasmosis (OH) between 1987 and 2008 were retrospectively reviewed. RESULTS: This work reports 11 cases of OH, the majority presenting histopathological or microbiological evidence of disseminated histoplasmosis (DH). In the patients with DH, OH was the first manifestation of histoplasmosis. Five of the 11 patients discussed were HIV-seropositive with clinical and laboratory findings of AIDS. Four patients presented active pulmonary tuberculosis concomitant with histoplasmosis. Treatment was based on the use of itraconazole and amphotericin B deoxycholate. Eight patients responded successfully to therapy after one year, two did not come back for reevaluation and one died despite adequate therapy. CONCLUSIONS: Oral histoplasmosis is closely associated with immunosuppression status, especially in patients presenting AIDS; moreover, in many cases, OH is the first sign of disseminated histoplasmosis.

scholarly journals Bats, Fever and Adenopathy – What Is the Link?

2013 ◽  
Vol 24 (1) ◽  
pp. 35-37 ◽  
Author(s):  
Joshua J Manolakos ◽  
Mohan Cooray ◽  
Ameen Patel ◽  
Shariq Haider
Keyword(s):  
Costa Rica ◽  
Environmental Exposure ◽  
Salvage Therapy ◽  
Histoplasma Capsulatum ◽  
Treatment Options ◽  
Diagnostic Approach ◽  
Disseminated Histoplasmosis ◽  
Progressive Disseminated Histoplasmosis

A case of travel-related, subacute, progressive disseminated histoplasmosis in a nonimmunocompromised individual is described. The present case highlights the environmental exposure toHistoplasma capsulatumin Costa Rica, the diagnostic approach and treatment options, as well as new alternatives for salvage therapy for histoplasmosis infection.

Sign in / Sign up

Export Citation Format

Share Document