Lamotrigine-associated hemophagocytic lymphohistiocytosis

2021 ◽  
Vol 14 (1) ◽  
pp. e238183
Author(s):  
Nawar Suleman ◽  
Metin Ozdemirli ◽  
David Weisman
Keyword(s):  
Immune System ◽  
Immune Activation ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Presentation ◽  
Paediatric Population ◽  
Laboratory Findings ◽  
Life Threatening ◽  
Rheumatological Disease ◽  
Prompt Diagnosis ◽  
Variable Clinical Presentation

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disorder of excessive immune activation. It is mostly seen in the paediatric population and is rarely observed in adults. HLH can be inherited or acquired and is commonly triggered by activation of the immune system by an underlying viral infection or in immune system deficiency such as malignancy or underlying rheumatological disease. HLH is a difficult entity to diagnose due to the rarity of this disorder, variable clinical presentation and non-specific clinical and laboratory findings. HLH carries a high mortality if left untreated, and therefore prompt diagnosis and initiation of immunosuppressive, immunomodulatory and cytostatic medications are critical to improve survival in affected patients. Here, we present a case of lamotrigine-associated HLH. To our knowledge, only eight other cases of lamotrigine-associated HLH have been reported in adult patients.

scholarly journals Hemophagocytic lymphohistiocytosis: a rare and life-threatening diagnosis

2021 ◽  
Vol 9 (12) ◽  
pp. 3705
Author(s):  
Anu Yarky ◽  
Vipan Kumar ◽  
Nidhi Chauhan ◽  
Neha Verma
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Presentation ◽  
Laboratory Findings ◽  
Delay In Diagnosis ◽  
Life Threatening ◽  
Sporadic Cases ◽  
Prompt Diagnosis ◽  
Excessive Activation ◽  
Variable Clinical Presentation ◽  
Secondary Hemophagocytic Lymphohistiocytosis

Hemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening syndrome of excessive activation of immune system. It frequently affects infants from birth to 18 months of age, but is also observed in children and adults of all ages. HLH can occur as a familial or sporadic disorder, and it is triggered by a variety of events, Infection being the most common trigger both in familial and in sporadic cases. Prompt treatment is very critical in cases of HLH, but the greatest barrier is often delay in diagnosis due to the rarity of this syndrome, variable clinical presentation, and lack of specificity of the clinical and laboratory findings. The key clinical features of HLH are high persistent fever, hepatosplenomegaly, blood cytopenia, elevated aminotransferase and ferritin levels, and coagulopathy. A diagnosis of HLH is mostly under-recognized, and is associated with high mortality, especially in adults; thus, prompt diagnosis and treatment are essential. We here present a rare case of HLH in an adult which was non-familial and infection being the trigger causing secondary hemophagocytic lymphohistiocytosis.

scholarly journals Complicated staphylococcal pericarditis in a child: a case report

2020 ◽  
Vol 7 (11) ◽  
pp. 2252
Author(s):  
Fehmida Sultana ◽  
Deepti Jujaray ◽  
Ravi P. V. Kiran
Keyword(s):  
Case Report ◽  
Pericardial Effusion ◽  
Early Identification ◽  
Clinical Presentation ◽  
Paediatric Population ◽  
Acute Pericarditis ◽  
Life Threatening

Although primary pericarditis is unusually experienced and diagnosed in paediatric population, it has probable life threatening sequel. This case report presents a case of complicated community acquired staphylococcal pericarditis, which illustrates how evasive the diagnosis of pericardial effusion can be. Early identification of pericarditis and pericardial effusion is vital to enable emergent intervention to enhance prognosis and alleviate mortality. The purpose of this report is to probe into the etiology of acute pericarditis and also to review the clinical presentation, the management and complications connected with acute pericarditis. 

scholarly journals Hemophagocytic Lymphohistiocytosis for the Internist and Other Primary Care Providers

2021 ◽  
Vol 12 ◽  
pp. 215013272110537
Author(s):  
Tanmayi Srinivas Pai ◽  
Fernando F. Stancampiano ◽  
Candido Rivera
Keyword(s):  
Primary Care ◽  
Immune System ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Immunosuppressive Treatment ◽  
Primary Care Providers ◽  
Outpatient Setting ◽  
Adult Patients ◽  
Diagnosis And Treatment ◽  
Care Providers ◽  
Life Threatening

Hemophagocytic lymphohistiocytosis (HLH) syndrome is a hyperinflammatory state that leads to life-threatening, disproportionate activation of the immune system and may be confused for and concomitantly exist with sepsis. However, its treatment differs from sepsis, requiring early initiation of immunosuppressive treatment. While HLH syndrome is more commonly diagnosed in children, internists and other primary care providers must be familiar with the diagnosis and treatment of adult patients with HLH in the hospital and outpatient setting. In this article, we review the essentials that an internist and other primary care providers managing adult HLH patients should know.

scholarly journals Clozapine-Induced Agranulocytosis Revealing a Hemophagocytic Lymphohistiocytosis: A Case Report

2018 ◽  
Vol 3 (2) ◽  
Keyword(s):  
Case Report ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Psychotic Symptoms ◽  
First Episode ◽  
Optimal Patient Outcome ◽  
Episode Psychosis ◽  
Threatening Condition ◽  
Life Threatening ◽  
Psychotic Features ◽  
Prompt Diagnosis

Context: The aim of this report is to illustrate a case of hemophagocytic lymphohistiocytosis (HLH) that was revealed in the context of treatment-resistant first-episode psychosis. The incidence of this life-threatening condition is increasing in adults. Prompt diagnosis and treatment are important for an optimal patient outcome. Case Report: The patient was a 20-year old female who had been admitted to the inpatient unit of a Psychiatric Hospital for disorganized behavior with psychotic features. Clozapine had been introduced as the clinical picture did not evolve despite several trials of antipsychotics. As severe agranulocytosis developed, the patient was transferred to a general hospital. A diagnosis of HLH was suggested. Patient responded well to treatment, and remission of psychotic symptoms was observed. Conclusion: This case demonstrates the occurrence of HLH in a complicated clinical scenario involving psychotic experiences and the use of clozapine. Further studies are necessary to understand the potential environmental and pathological factors related to HLH

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 in pregnancy evolving towards fulminant hepatitis

2021 ◽  
pp. 1753495X2199991
Author(s):  
Camille Simard ◽  
Marie-Lou Tardif
Keyword(s):  
Bone Marrow ◽  
Fulminant Hepatitis ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Significant Morbidity ◽  
Promising Alternative ◽  
Treatment Considerations ◽  
Life Threatening ◽  
Appropriate Therapy ◽  
Prompt Diagnosis ◽  
In Pregnancy

Background Hemophagocytic lymphohistiocytosis is a rare disorder, especially in pregnancy, characterized by excessive immune activation leading to hemophagocytic activity. Case Report A 34-year-old woman presented at 31 weeks’ gestation with fever, cytopenias, hyperferritinemia and fulminant hepatitis. Bone marrow biopsy was non-contributory, and no trigger was identified. Liver biopsy confirmed the diagnosis of hemophagocytic lymphohistiocytosis, and she received dexamethasone and etoposide, which was changed to anakinra to allow breastfeeding. Prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis is crucial to the survival of pregnant women, but no consensus exists regarding the most appropriate therapy during pregnancy. Conclusion Hemophagocytic lymphohistiocytosis is life-threatening and associated with significant morbidity and mortality. Important treatment considerations in pregnancy include maternal health, fetal prematurity and treatment toxicity. Anakinra has been successfully used in pregnancy and provides a promising alternative.

scholarly journals A case of endocarditis mimicking Crimean-Congo haemorrhagic fever

2016 ◽  
Vol 47 (1) ◽  
pp. 71-72
Author(s):  
Esra Tanyel ◽  
Mustafa Sunbul ◽  
Tom Fletcher ◽  
Hakan Leblebicioglu
Keyword(s):  
Endemic Area ◽  
Clinical Presentation ◽  
Delayed Diagnosis ◽  
Hemorrhagic Fever ◽  
Diagnostic Techniques ◽  
Viral Hemorrhagic Fever ◽  
Threatening Condition ◽  
Life Threatening ◽  
Crimean Congo Haemorrhagic Fever ◽  
Variable Clinical Presentation

Infective endocarditis (IE) is life-threatening condition with a highly variable clinical presentation. We report a case of acute IE with delayed diagnosis which resulted due to an initial misdiagnosis of Crimean Congo Hemorrhagic Fever (CCHF) in an endemic area. A case was due to Staphylococcus aureus and requiring valve replacement. They serve to emphasize the importance of careful history taking, physical examination and a broad range of different diagnostic techniques in the context of suspected viral hemorrhagic fever.

scholarly journals Familial Hemophagocytic Lymphohistiocytosis: A Rare Mutation of STXBP2 in Exon 19

2019 ◽  
Vol 09 (01) ◽  
pp. 066-068
Author(s):  
Femitha Pournami ◽  
Swati Upadhyay ◽  
Anand Nandakumar ◽  
Jyothi Prabhakar ◽  
Naveen Jain
Keyword(s):  
Immune System ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Laboratory Findings ◽  
Rare Mutation ◽  
Familial Hemophagocytic Lymphohistiocytosis ◽  
Immune System Activation ◽  
Missense Variation ◽  
System Activation ◽  
Progressive Disorder ◽  
Exon 19

AbstractFamilial hemophagocytic lymphohistiocytosis (FHLH) is a fulminant rapidly progressive disorder characterized by uncontrolled immune system activation. Over the last decade, STXBP2 mutations have been reported as causative. We report a baby with typical clinical features and supportive laboratory findings, who had a homozygous missense variation in exon 19 of STXBP2 that results in an amino acid substitution of aspartic acid for glycine. Adding to the currently scant literature on this variation may contribute to the database pool and help to confirm assertion of pathogenicity in FHLH.

scholarly journals Idiopathic hemophagocytic lymphohistiocytosis during pregnancy treated with steroids

2015 ◽  
Vol 7 (3) ◽  
Author(s):  
Bachar Samra ◽  
Mohamad Yasmin ◽  
Sami Arnaout ◽  
Jacques Azzi
Keyword(s):  
Pregnant Woman ◽  
Hemophagocytic Lymphohistiocytosis ◽  
Unknown Origin ◽  
Signs And Symptoms ◽  
Clinical Syndrome ◽  
Cell Transplant ◽  
Laboratory Findings ◽  
Hematopoietic Stem ◽  
First Case ◽  
Variable Clinical Presentation

Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe clinical syndrome characterized by a dysregulated hyperinflammatory immune response. The diagnosis of HLH during pregnancy is especially challenging due to the rarity of this condition. The highly variable clinical presentation, laboratory findings, and associated diagnoses accompanying this syndrome further complicate the problem. A pronounced hyperferritinemia in the setting of systemic signs and symptoms along with a negative infectious and rheumatological workup should raise suspicions for HLH. While treatment ideally consists of immunosuppressive chemotherapy and hematopoietic stem cell transplant, the potential toxicity to both the pregnant woman and the fetus poses a challenging decision. We report the first case of idiopathic HLH presenting as fever of unknown origin in a pregnant woman successfully treated with steroids.

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.

Sign in / Sign up

Export Citation Format

Share Document