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 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.

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.

A great pre-eclampsia masquerader. Hemophagocytic lymphohistiocytosis (HLH) presenting in pregnancy

2020 ◽  
Vol 9 (1) ◽  
Author(s):  
Marika Toscano ◽  
Elizabeth Westen ◽  
Stefanie J. Hollenbach ◽  
Archibald S. Perkins ◽  
Loralei L. Thornburg
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Killer Cell ◽  
High Serum ◽  
Lymphocyte Function ◽  
Treatment Resistant ◽  
Cytotoxic Lymphocyte ◽  
Case Presentation ◽  
Macrophage Phagocytosis ◽  
Life Threatening ◽  
In Pregnancy

AbstractBackgroundHemophagocytic lymphohistiocytosis (HLH) is a rare and life-threatening disease. It is characterized by impaired natural killer cell and cytotoxic lymphocyte function leading to dysregulated macrophage phagocytosis of erythrocytes, leukocytes and platelets. Coupled with a massive release of cytokines this rapidly leads to multi-organ failure and mortality if not treated promptly.Case presentationWe report a case of pregnancy-associated, treatment-resistant HLH with no identified risk factors.ConclusionIt is likely that HLH is an unrecognized cause of intractable fever and disseminated intravascular coagulation (DIC) in young pregnant women and it should be included in the differential diagnosis when patients do not respond to typical therapies. High serum ferritin should further prompt consideration of this diagnosis. This rare disease is often not thought of and many gynecologist obstetricians have never heard of it, making this case report of essential importance to reignite the interest in HLH, particularly when it occurs during pregnancy.

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

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.

Acute liver failure in pregnancy due to autoimmune hepatitis

2021 ◽  
Vol 14 (8) ◽  
pp. e241355
Author(s):  
Sonia Sajja ◽  
Jay Idler ◽  
Jaber Saad ◽  
Ray Bahado-Singh
Keyword(s):  
Liver Failure ◽  
Acute Liver Failure ◽  
Autoimmune Hepatitis ◽  
Maternal Morbidity ◽  
Disease Remission ◽  
Maternal Morbidity And Mortality ◽  
Appropriate Therapy ◽  
Prompt Diagnosis ◽  
In Pregnancy ◽  
Made In

Autoimmune hepatitis is a diagnosis rarely made in pregnancy, especially in the setting of acute liver failure. If unrecognised and untreated, it can result in significant fetal and maternal morbidity and mortality. We report a case of acute liver failure in a patient presenting at 17 weeks’ gestation. She was diagnosed with autoimmune hepatitis via transjugular liver biopsy. Prednisone therapy was initiated, resulting in disease remission for the remainder of her pregnancy. Induction of labour at 37 weeks’ gestation resulted in delivery of a healthy small for gestational age neonate. Prompt diagnosis of a non-obstetrical aetiology for acute liver failure in pregnancy is critical to provide the appropriate therapy to achieve an optimal pregnancy outcome.

The Evolution of Lead Extraction

2009 ◽  
Vol 5 (1) ◽  
pp. 32
Author(s):  
Melanie Maytin ◽  
Laurence M Epstein ◽  
◽  
Keyword(s):  
Natural Selection ◽  
Lead Extraction ◽  
Significant Morbidity ◽  
Extraction Techniques ◽  
Extraction Technology ◽  
Device Management ◽  
Future Developments ◽  
The Past ◽  
New Methods ◽  
Life Threatening

Prior to the introduction of successful intravascular countertraction techniques, options for lead extraction were limited and dedicated tools were non-existent. The significant morbidity and mortality associated with these early extraction techniques limited their application to life-threatening situations such as infection and sepsis. The past 30 years have witnessed significant advances in lead extraction technology, resulting in safer and more efficacious techniques and tools. This evolution occurred out of necessity, similar to the pressure of natural selection weeding out the ineffective and highly morbid techniques while fostering the development of safe, successful and more simple methods. Future developments in lead extraction are likely to focus on new tools that will allow us to provide comprehensive device management and the design of new leads conceived to facilitate future extraction. With the development of these new methods and novel tools, the technique of lead extraction will continue to require operators that are well versed in several methods of extraction. Garnering new skills while remembering the lessons of the past will enable extraction technologies to advance without repeating previous mistakes.

Pancytopenia: A Challenging Problem for Treating Physician

2018 ◽  
Vol 2 (01) ◽  
pp. 22-28
Author(s):  
Md. Rezaul Karim Chowdhury ◽  
Amina Begum ◽  
Md. Haroon Ur Rashid ◽  
Md. Kamrul Hasan
Keyword(s):  
Bone Marrow ◽  
Physical Examination ◽  
Blood Smear ◽  
Peripheral Blood Smear ◽  
Bone Marrow Examination ◽  
Challenging Problem ◽  
Trephine Biopsy ◽  
Life Threatening ◽  
Underlying Pathology ◽  
Careful History

Pancytopenia is an important clinico-haematological entity and striking feature of many serious and life-threatening illnesses. Many haematological and non-haematological diseases involve the bone marrow primarily or secondarily and cause pancytopenia. Decrease in haemopoietic cell production, ineffective haemopoiesis and peripheral sequestration or destruction of the cells are the main pathophysiology of pancytopenia. The cause of pancytopenia thus may be lying in the bone marrow or in the periphery or both. Careful history, physical examination, simple blood work, review of the peripheral blood smear, sometimes bone marrow examination and trephine biopsy are required for diagnosis. Treatment and prognosis depend on the severity of pancytopenia and underlying pathology.

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.

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