Haemophagocytosis

No abstract text is available yet for this article.

No abstract text is available yet for this article.

The difficulties and challenges of applying the HLH-2004 diagnostic criteria to early identification and diagnosis of haemophagocytic lymphohistiocytosis have been fully addressed in previous studies. However, the distribution of the diagnostic time lag of haemophagocytic lymphohistiocytosis and related patient characteristics remain unclear. This study investigated the time lags between symptom onset and diagnosis and between hospital admission and diagnosis among pediatric patients with haemophagocytic lymphohistiocytosis, and identified factors that associated with a shorter or longer diagnostic time lag...

Intravascular large B-cell lymphoma is a rare and aggressive EBV-negative large B-cell lymphoma with a dismal outcome. Here, we describe the case of a 76-year-old HIV-positive patient with an acute presentation of systemic symptoms and rapidly fatal outcome. Autopsy revealed a disseminated large B-cell lymphoma with an intravascular distribution involving the liver, lymph nodes, spleen, and bone marrow and associated to fibrin thrombi in hepatic capillary haemangiomas. The neoplastic B cells (CD79a + / - , CD20 + / - , CD30 + , MUM1 + , PD-L1 +) showed a Hodgkin and Reed-Sternberg-like morphology and were EBV-positive with a latency type II (LMP1 + , EBNA2-)...

CASE SUMMARY: A 14-year 3-month-old spayed female mixed-breed cat presented with jaundice, anaemia and thrombocytopenia. Haemophagocytic syndrome associated with lymphoma was suspected after cytological examination of the spleen. Despite treatment with prednisolone, L-asparaginase and nimustine, the cat died 176 days after the initial presentation. Necropsy revealed splenomegaly and hepatomegaly, without lymphadenopathy. Histopathologically, neoplastic lymphoid cells infiltrated the hepatic sinusoid and splenic sinus...

Haemophagocytic lymphohistiocytosis (HLH) is a rare condition of uncontrolled immune activation as a result of an inherited genetic defect or in response to malignancy, autoimmune disease, rheumatological disease, AIDS infection or post-transplant immunosuppression. Described here is the case of a 19-year-old Caucasian man who presented with complaints of worsening fever, new-onset jaundice and lethargy after failing treatment for suspected infectious mononucleosis. Physical examination was significant for fever and splenomegaly while laboratory results revealed transaminitis, cytopaenia, indirect hyperbilirubinaemia and elevated ferritin, raising the likelihood of both autoimmune haemolytic anaemia and HLH...

BACKGROUND: COVID-19 in children is usually mild or asymptomatic, but severe and fatal paediatric cases have been described. The pathology of COVID-19 in children is not known; the proposed pathogenesis for severe cases includes immune-mediated mechanisms or the direct effect of SARS-CoV-2 on tissues. We describe the autopsy findings in five cases of paediatric COVID-19 and provide mechanistic insight into the mechanisms involved in the pathogenesis of the disease. METHODS: Children and adolescents who died with COVID-19 between March 18 and August 15, 2020 were autopsied with a minimally invasive method...

BACKGROUND: Secondary haemophagocytic lymphohistiocytosis (sHLH) is characterised by a hyper activation of immune system that leads to multiorgan failure. It is suggested that excessive immune response in patients with COVID-19 could mimic this syndrome. Some COVID-19 autopsy studies have revealed the presence of haemophagocytosis images in bone marrow, raising the possibility, along with HScore parameters, of sHLH. AIM: Our objective is to ascertain the existence of sHLH in some patients with severe COVID-19...

Left ventricular assist devices provide circulatory support to heart failure patients while awaiting a suitable donor heart. However, with their increased duration of therapy, complications are seen frequently. Although coagulation disorders (bleeding and thrombosis) are the most common complications, infection is also a major complication associated with significant morbidity. We report a case of a 53-year-old male with a left ventricular assist device who presented with driveline infection. He subsequently developed pancytopenia and was diagnosed with haemophagocytosis...

Systemic lupus erythematosus (SLE)-associated haemophagocytic lymphohistiocytosis (HLH) is called acute lupus haemophagocytic syndrome (ALHS), which is relatively rare but life-threatening. We present the case of a 43-year-old woman diagnosed with SLE with panniculitis, pleuritis, and autoimmune hepatitis. She was treated with high-dose glucocorticoids. Although disease activity temporarily improved, she developed fever, elevation of liver enzymes, hyperferritinemia, severe inflammatory response, and thrombocytopenia a month after starting glucocorticoids...

AIMS: Haemophagocytosis in the bone marrow of patients who have succumbed to coronavirus disease 19 (COVID-19) has not been widely studied. The aims of the present study were to perform morphological analyses and morphometry of haemophagocytosis in the bone marrow of patients with severe COVID-19, and to correlate the findings with the clinical course of the disease. METHODS AND RESULTS: In this single-centre study performed at the University Hospital Jena, bone marrow specimens of 15 deceased patients who had experienced a severe course of COVID-19 were sampled from the vertebral column during autopsy...

BACKGROUND: Severe COVID-19 has a high mortality rate. Comprehensive pathological descriptions of COVID-19 are scarce and limited in scope. We aimed to describe the histopathological findings and viral tropism in patients who died of severe COVID-19. METHODS: In this case series, patients were considered eligible if they were older than 18 years, with premortem diagnosis of severe acute respiratory syndrome coronavirus 2 infection and COVID-19 listed clinically as the direct cause of death...

HLH is a rare, life-threatening, hematologic disorder resulting from prolonged and excessive activation of antigen presenting cells (macrophages, histiocytes) and CD8+ T cells. It is characterized by fever, pancytopenia,splenomegaly and haemophagocytosis in bone marrow,liver or lymph node. This hyperinflammatory condition is often triggered by a variety of agents or events, mostly genetic or infectious. HLH secondary to TB, have 100 % mortality in absence of anti-tubercular treatment .Since it mimics other disorders, its timely diagnosis remains a challenge...

Background: Haemophagocytic lymphohistiocytosis (HLH) is an uncommon but serious systemic inflammatory response with high mortality rates. It can be triggered by malignancy or infectious agents, often in the context of immunosuppression. Literature covering HLH in heart transplantation (HTx) is scarce. Case summary: A 25-year-old male with a history of celiac disease underwent HTx at Sahlgrenska Hospital in 2011 due to giant cell myocarditis and was treated with tacrolimus, mycophenolate mofetil (MMF), and prednisolone...

AIM: To evaluate clinical, laboratory, imaging findings, and outcomes of adult patients with bone marrow haemophagocytosis (BMH) who meet the diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH) with those who do not meet the criteria. MATERIALS AND METHODS: A pathology database search was performed from 2009 to 2019 to identify adult patients with BMH. Electronic medical records of 41 patients were reviewed to distinguish those who fulfil the HLH-2004 diagnostic guidelines, which identified 22 patients (11 men; mean age, 53...

In critically ill COVID-19 patients, the failure of the cardiorespiratory system can be due to one of the following: (1) cytokine storm, haemophagocytosis – septic shock, (2) unmanageable hypoxemia, (3) isolated organ failure or as part of multi-organ failure. Herein we give an overview of the therapeutic options for treating or preventing these disease states. In recent years, CytoSorb-haemoperfusion to remove cytokines has shown promising results in the treatment of septic shock. Inhalational nitric oxide (iNO), inhalational epoprostenol and veno-venous extracorporeal membrane oxygenation (ECMO) are options in severe hypoxemia that is unresponsive to conventional mechanical ventilation...

Macrophage activation syndrome is a state of hyper-inflammation that results from increased secretion of proinflammatory cytokines, responsible of the inappropriate activation and proliferation of cells from the lymphohistiocyte lineage. It associates clinical signs, biological abnormalities and images of haemophagocytosis. It is a rare but serious attack, that can be "primary" or "secondary" to an infection, neoplasia, or autoimmune disease. Cancer etiology by solid tumor is exceptional...

A 63-year-old man presented to the hospital with generalised weakness, fatigue and a 22 kg weight loss 4 months after being diagnosed with sarcoidosis on a mediastinal lymph node biopsy, with minimal improvement in symptoms on prednisone and methotrexate therapy. On arrival, he was found to have a haemoglobin of 57 g/L and platelet count of 82×109 /L. Further work-up revealed six of eight diagnostic criteria for haemophagocytic lymphohistiocytosis (HLH): fever >38.9°C, splenomegaly, cytopaenia, hypertriglyceridaemia, haemophagocytosis and elevated ferritin >31 000 ng/mL...

Various agents including Ca. Piscichlamydia salmonis, Ca. Branchiomonas cysticola, Desmozoon lepeophtherii, Paramoeba perurans and salmon gill poxvirus may be associated with complex gill disease in Atlantic salmon. Co-infections involving two or more of these agents are common and histopathological interpretation of lesions is therefore challenging. In this study, we developed a semi-quantitative scoring system for examination of histopathological gill lesions in sea-farmed Atlantic salmon suffering from gill disease...

No abstract text is available yet for this article.