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

Author(s):  
Anu Yarky ◽  
Vipan Kumar ◽  
Nidhi Chauhan ◽  
Neha Verma

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.

2021 ◽  
Vol 14 (1) ◽  
pp. e238183
Author(s):  
Nawar Suleman ◽  
Metin Ozdemirli ◽  
David Weisman

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.


2014 ◽  
Vol 6 (1) ◽  
pp. e2014067 ◽  
Author(s):  
Seval Ozen ◽  
Alper Dai ◽  
Enes Coskun ◽  
Serdar Oztuzcu ◽  
Sercan Ergun ◽  
...  

Background and objective: Hemophagocytic lymphohistiocytosis (HLH) is a life threatening hyper inflammatory disease. It is difficult to differentiate between primary and secondary HLH based on clinical findings at the onset of disease. We aimed to find parameters that can help to differentiate primary and secondary HLH at initial diagnosis especially for physicians working in developing countries.Patient and Method: We retrospectively analyzed data of 38 HLH patients who were admitted to the Pediatric Hematology Department of Gaziantep University between January 2009 and December 2013.Results: Of 38 patients, 20 were defined as primary and 18 were secondary HLH. The average age of primary and secondary HLH patients was 31±9 and 81±14 months, respectively (p=0.03). We found consanguinity rates significantly higher in primary HLH patients compared to secondary HLH patients (p=0.03). We found that total and direct bilirubin levels significantly increased in primary HLH patients compared to secondary HLH patients (p=0.006, p=0.044). Also, CRP levels were found markedly increased in secondary HLH patients compared to primary ones (p=0.017).Conclusion: We showed that cholestasis and hyperbilurubinemia findings of HLH patients at the initial diagnosis should be considered in favor of primary HLH and increased level of CRP should be considered in favor of secondary HLH.


2021 ◽  
pp. 64-66
Author(s):  
Athira P A ◽  
Indumathi K DCP ◽  
Theranirajan Theranirajan ◽  
Priyadharshini Swaminathan

Hemophagocytic lymphohistiocytosis is a hyper-inammatory condition that is either Familial (Primary) or Secondary to autoimmune diseases , infection, malignancy or other triggers.It is a cytokine storm syndrome where there inefcient antigen removal that leads to sustained cytokine release.It is a rare phenomenon occuring in adults that has got a specic trigger which is less documented and have a good response to steroids where as Familial form is a childhood disease due to genetic defects, both of which are life threatening and may need Allogenic bone marrow transplant. Macrophage activation syndrome is also a subtype of this entity that occurs in the treatment phase of SLE and Still's disease.We describe here 8 cases of secondary HLH, their primary triggers and treatment response.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Mei Meng ◽  
Limin Chen ◽  
Sheng Zhang ◽  
Xuan Dong ◽  
Wenzhe Li ◽  
...  

Abstract Background Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening hyperinflammatory event and a fatal complication of viral infections. Whether sHLH may also be observed in patients with a cytokine storm induced by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is still uncertain. We aimed to determine the incidence of sHLH in severe COVID-19 patients and evaluate the underlying risk factors. Method Four hundred fifteen severe COVID-19 adult patients were retrospectively assessed for hemophagocytosis score (HScore). A subset of 7 patients were unable to be conclusively scored due to insufficient patient data. Results In 408 patients, 41 (10.04%) had an HScore ≥169 and were characterized as “suspected sHLH positive”. Compared with patients below a HScore threshold of 98, the suspected sHLH positive group had higher D-dimer, total bilirubin, alanine aminotransferase, aspartate aminotransferase, blood urea nitrogen, serum creatinine, triglycerides, ferritin, interleukin-6, C-reactive protein, procalcitonin, lactate dehydrogenase, creatine kinase isoenzyme, troponin, Sequential Organ Failure Assessment (SOFA) score, while leukocyte, hemoglobin, platelets, lymphocyte, fibrinogen, pre-albumin, albumin levels were significantly lower (all P < 0.05). Multivariable logistic regression revealed that high ferritin (>1922.58 ng/mL), low platelets (<101 × 109/L) and high triglycerides (>2.28 mmol/L) were independent risk factors for suspected sHLH in COVID-19 patients. Importantly, COVID-19 patients that were suspected sHLH positive had significantly more multi-organ failure. Additionally, a high HScore (>98) was an independent predictor for mortality in COVID-19. Conclusions HScore should be measured as a prognostic biomarker in COVID-19 patients. In particular, it is important that HScore is assessed in patients with high ferritin, triglycerides and low platelets to improve the detection of suspected sHLH.


2018 ◽  
Vol 3 (2) ◽  

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


2020 ◽  
Vol 21 (2) ◽  
pp. 123-126
Author(s):  
Quazi Tarikul Islam ◽  
Hirinmoy Barman Sagor ◽  
Tasmina Chowdhury Tuli

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


2021 ◽  
pp. 1753495X2199991
Author(s):  
Camille Simard ◽  
Marie-Lou Tardif

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.


2016 ◽  
Vol 47 (1) ◽  
pp. 71-72
Author(s):  
Esra Tanyel ◽  
Mustafa Sunbul ◽  
Tom Fletcher ◽  
Hakan Leblebicioglu

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.


2015 ◽  
Vol 7 (3) ◽  
Author(s):  
Bachar Samra ◽  
Mohamad Yasmin ◽  
Sami Arnaout ◽  
Jacques Azzi

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.


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