scholarly journals Successful Modified Therapy in a Patient with Probable Infection-Associated Hemophagocytic Lymphohistiocytosis

2019 ◽  
Vol 2019 ◽  
pp. 1-6
Author(s):  
Carl L. Kay ◽  
Matthew J. Rendo ◽  
Paul Gonzales ◽  
Sead G. Beganovic ◽  
Magdalena Czader
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Course ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Good Patient ◽  
Good Patient Outcome ◽  
Distributive Shock ◽  
Probable Infection ◽  
Clinical Signs And Symptoms ◽  
Baseline Renal Function

Hemophagocytic lymphohistiocytosis (HLH) is a rare, hyperinflammatory syndrome characterized by clinical signs and symptoms of extreme inflammation. In adults, HLH is typically a complication of infections, autoimmune diseases, and malignancies. While the disease is often fatal, classic management of HLH revolves around early diagnosis and initiation of protocolized therapy. We present a case of a previously healthy 56-year-old female who developed distributive shock requiring intubation, vasopressors, and continuous venovenous hemofiltration. In the setting of multiple infectious syndromes, severe cytopenias, and rising direct hyperbilirubinemia, her diagnosis of HLH was confirmed. Therapy was initiated with dexamethasone and two doses of reduced-intensity etoposide based on the patient’s clinical course. Over the next few weeks, she continued to improve on dexamethasone monotherapy and has maintained remission up to the present with complete resolution of her cytopenias and return of baseline renal function. Our case highlights the variability in the management of probable infection-associated HLH (IHLH) with a good patient outcome. We demonstrate the potential to treat IHLH with partial protocols and minimal chemotherapeutics.

scholarly journals Specific clinical signs and symptoms are predictive of clinical course in sporadic Creutzfeldt−Jakob disease

2016 ◽  
Vol 23 (9) ◽  
pp. 1455-1462 ◽  
Author(s):  
E. Nakatani ◽  
Y. Kanatani ◽  
H. Kaneda ◽  
Y. Nagai ◽  
S. Teramukai ◽  
...  
Keyword(s):  
Clinical Course ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Jakob Disease ◽  
Clinical Signs And Symptoms

scholarly journals Birdshot retinochoroiditis in Brazil: a multicenter review of 40 patients

2022 ◽  
Vol 8 (1) ◽  
Author(s):  
Maria Luisa Gois da Fonsêca ◽  
Raul N. G. Vianna ◽  
Anna C. H. Rocha ◽  
Antonio M. B. Casella ◽  
Arnaldo Cialdini ◽  
...  
Keyword(s):  
Retrospective Cohort ◽  
Clinical Course ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Clinical Findings ◽  
Birdshot Retinochoroiditis ◽  
Hispanic Patients ◽  
The Mean ◽  
Clinical Signs And Symptoms ◽  
Few Data

Abstract Background Birdshot retinochoroiditis (BRC) is a rare and chronic bilateral uveitis mostly found in Caucasians. As few data are available about the clinical course of BRC in Hispanic patients, we aimed to report the clinical findings and the evolution of BRC in Brazilian patients. Methods This retrospective cohort multicenter nationwide study was performed by analyzing the records of patients with BRC diagnoses from Brazilian ophthalmological centers from April 1995 to May 2020. Results Forty patients (80 eyes) with a diagnosis of BRC were evaluated. The mean age was 53 years, and there was no sex predominance. All tested patients (34/40) were positive for HLA-A29. The diagnosis of BRC was made following the Levinson et al. criteria, and all ancillary tests were performed to exclude differential diagnoses. Clinical signs and symptoms, such as complications and treatment, were described. Conclusions BRC evolution in Brazilian patients seems to have some peculiarities that diverge from the published literature available about Caucasians, as AS inflammation is higher in this population.

Laboratory Examination in Hemophagocytic Lymphohistiocytosis

2021 ◽  
Vol 28 (1) ◽  
pp. 84
Author(s):  
Wulyansari Wulyansari ◽  
Yetti Hernaningsih
Keyword(s):  
Bone Marrow ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Bone Marrow Cells ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Laboratory Findings ◽  
Central Nervous System Dysfunction ◽  
Life Threatening ◽  
Clinical Signs And Symptoms

Hemophagocytic Lymphohistiocytosis (HLH) is derived from the word hemophagocytosis, in which macrophagesinfiltrate tissue extensively, and unspecifically phagocyte blood and bone marrow cells. The deviant activation of cytotoxicCD8+ T-cells causing the release of inflammatory cytokines is the core pathogenesis of HLH. Hemophagocyticlymphohistiocytosis is a regulatory disorder of the immune system, with clinical signs and symptoms of extremeinflammation and cytopenia, hepatitis, and severe and life-threatening central nervous system dysfunction. The name of theHLH disorder was recently proposed to be "Hyperinflammatory Lymphohistiocytosis" (also known as HLH). Enforcement ofHLH diagnosis by the Histiocyte Society based on HLH 2004 updated diagnostic criteria consists of five of the following eightdiagnostic criteria: fever, splenomegaly, cytopenia (two or more of three lineages in peripheral blood), hypertriglyceridemiaor hypofibrinogenemia, hyperferritinemia, hemophagocytes in the bone marrow/lien/lymph, the low or non-existentactivity of Natural Killer (NK) cells, increased sCD25. H-score, MH-score, and systemic Juvenile Idiopathic Arthritis(sJIA)/Macrophage Activated Syndrome (MAS) classification criteria are also used to enforce HLH diagnoses.Hemophagocytic lymphohistiocytosis is challenging to recognize and has a high mortality rate, especially in adults, rangingfrom 42 to 88%. Therefore, immediate diagnosis and therapy are essential. The introduction of HLH triggers is criticalbecause treatment is based on the underlying trigger. Cytokine storms due to Coronavirus Disease 19 (COVID-19) infectionhave significant similarities to the clinical and laboratory findings of HLH. Secondary HLH (sHLH) is suspected in severeCOVID-19 patients, so early diagnosis is potentially made based on the H-score.

scholarly journals 741. Ehrlichia chaffeensis Induced Hemophgocytic lymphohistiocytosis: A Descriptive Case Series

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S468-S469
Author(s):  
jeffrey lin ◽  
Hanine El Haddad ◽  
Ayman Qasrawi ◽  
Gerhard Hildebrandt
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Nk Cell ◽  
Spotted Fever ◽  
Immunosuppressive Treatment ◽  
Laboratory Data ◽  
Cell Activity ◽  
Clinical Signs ◽  
Case Series ◽  
Signs And Symptoms ◽  
Clinical Signs And Symptoms

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) secondary to tick borne illnesses is rarely reported. Clinical signs and symptoms of tick borne illnesses and HLH might overlap with fever, cytopenias and increased liver enzymes being common. We describe findings from case series of ehrlichiosis induced HLH. Methods We reviewed patients with ICD-10 codes corresponding to a diagnosis of HLH or macrophage activation syndrome (MAS) at University of University of Kentucky Medical Center between January 2008 and April 2020. Inpatients who were >18 years of age without known immune compromise were included. 4 cases with confirmed underlying ehrlichiosis were identified at our institution. We searched PubMed for English-language articles containing the terms “ Hemophagocytic lymphohistiocytosis “ and “infection” or “tick borne” or “Ehrlichia”. Data on patient demographics, clinical signs and symptoms, laboratory data such as ferritin, platelet count, Il-2, NK cell activity, and outcomes were collected. Results We identified 16 cases of ehrlichiosis (1 had a coinfection with Rocky Mountain Spotted fever). Eleven out of 6 (68%) were male, median age was 58. All patients were febrile and thrombocytopenic on presentation and 8/14 (57%) were neutropenic. All had elevated ferritin (mean 36187 ng/mL, range 860 – more than 100000). CNS involvement was reported in 4 patients with a positive CSF Ehrlichia chaffensis PCR. All patients met at least 5 2004-HLH defining criteria and 10/14 (71%) patients had evidence of hemophagocytosis on bone marrow biopsy (table 1). Fourteen out of 15 (93%) patients received doxycycline and 9/15 (60%) received steroids +/- etoposide. Mortality for Ehrlichia induced HLH was 12.5%, significantly lower than that reported for all secondary HLH mortality (45%). Conclusion This review highlights the importance of considering Ehrilichiosis as a cause of HLH in endemic areas particularly as clinical signs and symptoms of the 2 entities overlap. While overall mortality rate due to HLH is elevated, Ehrlichia induced HLH seems to have a much favorable prognosis with prompt institution antimicrobial treatment. Additional prognostic factors that correlate with a more severe course dictate need for immunosuppressive treatment need to be further elucidated. Disclosures Gerhard Hildebrandt, MD, Bayer, Scotts-Miracle, Charlottes Webb CWBHF, Almmune Therapeutics Inc AIMT, Medical PPTYS TR Inc. MPW, Caretrust Reit Inc CTRE, ANGI Homeservices Inc (Shareholder)Bristil-Myers Squibb/Medarex, Crispr therapeutics, IDEXX Laboratories, Johnson & Johnson, Pfizer, Procter & Gamble, Vertex (Shareholder)Falk Foundation, Incyte, Takeda (Other Financial or Material Support, Travel, Accommodations, Expenses)Jazz Pharmaceuticals, Incyte, Morphosys, Alexion Pharmaceuticals, Karyopharm Therapeutics, Seattle Genetics (Consultant)Jazz Pharmaceuticals, Pharmacyclics, Incyte, AstraZeneca (Grant/Research Support)Novartis, Insys Therapeutics, Abbvie, GW Pharmaceuticals, Cardinal Health, Clovis Oncology, Cellectis, CVS Health, Celgene, Bluebird Bio (Shareholder)

scholarly journals How I treat hemophagocytic lymphohistiocytosis

Blood ◽  
2011 ◽  
Vol 118 (15) ◽  
pp. 4041-4052 ◽  
Author(s):  
Michael B. Jordan ◽  
Carl E. Allen ◽  
Sheila Weitzman ◽  
Alexandra H. Filipovich ◽  
Kenneth L. McClain
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Diagnostic Testing ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Signs And Symptoms ◽  
Disease Recurrence ◽  
Clinical Signs And Symptoms ◽  
Time Required ◽  
Risk Of Treatment ◽  
Variable Presentation

Abstract Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, occurring as either a familial disorder or a sporadic condition, in association with a variety of triggers. This immune dysregulatory disorder is prominently associated with cytopenias and a unique combination of clinical signs and symptoms of extreme inflammation. Prompt initiation of immunochemotherapy is essential for survival, but timely diagnosis may be challenging because of the rarity of HLH, its variable presentation, and the time required to perform diagnostic testing. Therapy is complicated by dynamic clinical course, high risk of treatment-related morbidity, and disease recurrence. Here, we review the clinical manifestations and patterns of HLH and describe our approach to the diagnosis and therapy for this elusive and potentially lethal condition.

scholarly journals Wolman’s disease presenting with secondary hemophagocytic lymphohistiocytosis: a case report from Saudi Arabia and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Fahad Alabbas ◽  
Ghaleb Elyamany ◽  
Talal Alanzi ◽  
Tahani Bin Ali ◽  
Fatma Albatniji ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Metabolic Disorder ◽  
Clinical Signs ◽  
Failure To Thrive ◽  
Severe Infection ◽  
Signs And Symptoms ◽  
Immune Deficiency Syndrome ◽  
Autoimmune Disorder ◽  
Deficiency Syndrome ◽  
Homozygous Deletion

Abstract Background Hemophagocytic lymphohistiocytosis (HLH) is a rare and potentially fatal syndrome that is characterized by strong activation of the immune system from hyperinflammatory cytokines. Symptoms of HLH patients include fever, hepatosplenomegaly, cytopenia, and hyperferritinemia. Inherited HLH is classified as primary, whereas secondary HLH (sHLH) occurs when acquired from non-inherited reasons that include severe infection, immune deficiency syndrome, autoimmune disorder, neoplasm, and metabolic disorder. Wolman’s disease (WD) is a rare and fatal infantile metabolic disorder caused by lysosomal acid lipase deficiency, that exhibits similar clinical signs and symptoms as HLH. This paper reports the case of an infant diagnosed with WD and who presented with sHLH. Case presentation A 4-month-old infant presenting with hepatosplenomegaly, failure to thrive, and other abnormalities. WD diagnosis was confirmed by the presence of the LIPA gene homozygous deletion c.(428 + 1_967-1)_(*1_?)del. The infant also met the HLH-2004 diagnostic criteria. Conclusions Metabolic disorder such as WD should be investigated in infants fulfilling the HLH criteria to diagnose the underlying condition. More studies are needed to understand the link between WD and sHLH and to identify appropriate therapies.

scholarly journals Dementia and Major Neurocognitive Disorders: Some Lessons Learned One Century after the first Alois Alzheimer’s Clinical Notes

Geriatrics ◽  
2021 ◽  
Vol 6 (1) ◽  
pp. 5
Author(s):  
Donatella Rita Petretto ◽  
Gian Pietro Carrogu ◽  
Luca Gaviano ◽  
Lorenzo Pili ◽  
Roberto Pili
Keyword(s):  
Young Woman ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Neurocognitive Disorders ◽  
Lessons Learned ◽  
Alzheimer Dementia ◽  
Clinical Notes ◽  
Alois Alzheimer ◽  
Clinical Signs And Symptoms

Over 100 years ago, Alois Alzheimer presented the clinical signs and symptoms of what has been later called “Alzheimer Dementia” in a young woman whose name was Augustine Deter [...]

scholarly journals Correction to: Levoketoconazole improves clinical signs and symptoms and patient-reported outcomes in patients with Cushing’s syndrome

Pituitary ◽  
2020 ◽  
Author(s):  
Eliza B. Geer ◽  
Roberto Salvatori ◽  
Atanaska Elenkova ◽  
Maria Fleseriu ◽  
Rosario Pivonello ◽  
...  
Keyword(s):  
Cushing’S Syndrome ◽  
Patient Reported Outcomes ◽  
Clinical Signs ◽  
Signs And Symptoms ◽  
Cushing's Syndrome ◽  
Original Version ◽  
Patient Reported ◽  
Author Group ◽  
Clinical Signs And Symptoms

The original version of the article unfortunately contained an error in the first name and the surname of one of the authors in the author group. The last author name was incorrectly published as ‘F. Pecori Giraldi’ and the corrected name is ‘Francesca Pecori Giraldi’ (First name: Francesca; Surname: Pecori Giraldi).

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