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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with hemophagocytic lymphohistiocytosis who presented with acute liver failure are reported. Both presented with fever, hepatosplenomegaly, markedly elevated liver function tests, abnormal coagulation profiles, and an increase in serum ferritin. Both infants were diagnosed with neonatal hemochromatosis based on a clinical picture of hepatic insufficiency with hyperferritinemia and were referred for liver transplantation. The first patient died of liver failure and septicemia before transplantation. Review of autopsy material revealed a hepatitis-like pattern and extensive infiltration of liver and other organs including bone marrow by histiocytes, some of which were hemophagocytic. The second patient underwent liver transplantation but died 44 days thereafter from progressive hemophagocytic lymphohistiocytosis. Examination of the resected liver demonstrated a hepatitis-like pattern, proliferation of histiocytes, and hemophagocytosis, and the bone marrow revealed hemophagocytic histiocytosis. Hemophagocytosis recurred in the allograft. Hepatic manifestations are common in hemophagocytic lymphohistiocytosis and overt hepatic failure may occur, but initial presentation as fulminant hepatic failure is not well recognized. Elevated serum ferritin can make the distinction from neonatal hemochromatosis and other forms of neonatal liver failure difficult. Hemophagocytic lymphohistiocytosis should be considered in the differential diagnosis of neonatal liver disease, especially when it is accompanied by cytopenias.
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PMID:Hemophagocytic syndrome presenting as acute hepatic failure in two infants: clinical overlap with neonatal hemochromatosis. 1034 80

Congenital galactosaemia reveals usually in the second and third weeks of life with a severe liver dysfunction. We report on a case of congenital galactosaemia with, on the one hand, an early onset liver failure, without any free interval, and on the other hand, an hemophagocytic syndrome as a severe secondary outbreak with pulmonary haemorrhage. Appropriate diet led to normalisation of liver function. Hemophagocytosis, probably linked to an associated Klebsiella Pneumoniae sepsis, had a favourable outcome after antibiotic and corticosteroid therapy.
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PMID:[Congenital galactosaemia: an unusual presentation]. 1569 40

Macrophage activation syndrome is a form of secondary haemophagocytic lymphohistiocytosis seen in the context of rheumatic diseases. It is seen most frequently in association with systemic onset juvenile arthritis or childhood Still's disease. Hemophagocytosis is part of a sepsis-like clinical syndrome caused by hypercytokinemia due to a highly stimulated but ineffective immune response. Coagulopathy and hemorrhages, decreased white cell count, elevated levels of aspartate aminotransferase, fever, rash, hepatosplenomegaly and central nervous system dysfunction are some of diagnostic criteria of macrophage activation syndrome, but it is very difficult to diagnose due to the lack of specific clinical signs. We report a 8-year-old child who was admitted to the ICU with lethargy, fever, acute respiratory failure, coagulopathy, metabolic acidosis and multiorgan failure. Septic shock was suspected, but he was diagnosed with macrophage activation syndrome and treated with corticosteroids and intravenous immunoglobulin and later discharged from the ICU.
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PMID:[Multi-organ failure as first clinical sign of macrophage activation syndrome in childhood Still's disease]. 2064 91

Hemophagocytosis may be encountered in association with a variety of underlying conditions, including primary or familial disorders, and secondary forms induced by infections, malignancy, and metabolic disorders. It is usually observed in organs such as the spleen, lymph nodes, bone marrow, and central nervous system, but rarely in the skin. When hemophagocytosis coexists with a sepsis-like systemic disorder it is termed hemophagocytic syndrome or hemophagocytic lymphohistiocytosis. Recently, 2 cases with evidence of perivascular hemophagocytosis in skin biopsy specimens of patients without additional findings of hemophagocytic lymphohistiocytosis have been reported. We report 2 additional cases of patients with cutaneous lesions suggestive of leukocytoclastic vasculitis whose skin biopsies showed a perivascular and interstitial infiltrate of neutrophils with nuclear dust and extravasated erythrocytes, and the presence of histiocytes with phagocytized red blood cells and nuclear fragments. There was also evidence of fibrin in the walls of the venules. One patient presented with a sepsis-like clinical scenario, but an extensive work-up failed to demonstrate any underlying disease or infection. The second patient was asymptomatic at the time of presentation, but further work-up revealed an underlying B-cell lymphoma. Whether these histologic findings represent late lesions of leukocytoclastic vasculitis or an incomplete presentation as part of a hemophagocytic syndrome is debatable, because both are associated with activated immunity and cytokine release, which could account for the presence of hemophagocytosis. Although the histopathologic finding alone of hemophagocytosis is insufficient to label as a syndrome, it should incite the clinician for further systemic evaluation.
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PMID:Perivascular hemophagocytosis: report of 2 cases and review of the literature. 2085 80

Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a rare and frequently fatal disorder caused by an uncontrollable and ineffective systemic immune response. Patients initially present with fever, cytopenia, and hepatosplenomegaly, and subsequently develop multiorgan failure (MOF). Hemophagocytosis can be found on biopsy specimen but is not required. Acquired forms of HLH can occur in apparently healthy adults, while children present more often with an inherited form of the disease. Since HLH often presents with sepsis-like symptoms and organ dysfunction, patients are usually treated for presumed sepsis, which inevitably leads to delayed diagnosis and treatment. Intensivists need to have a low threshold for suspecting this disorder when previously healthy individuals present with a fulminant sepsis-like syndrome, which are unresponsive to conventional treatment. We present 3 patients with HLH who were admitted to our adult medical intensive care unit (MICU) over a 2-year period with fatal outcomes and emphasize the diagnostic importance of markedly elevated serum ferritin levels and the need for tissue biopsy in making an accurate diagnosis in a timely manner.
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PMID:What intensivists need to know about hemophagocytic syndrome: an underrecognized cause of death in adult intensive care units. 2596 95

Differential diagnosis of hemophagocytic lymphohistiocytosis (HLH; hemophagocytic syndrome) and sepsis is critically important because the life-saving aggressive immunosuppressive treatment, required in the effective HLH therapy, is absent in sepsis guidelines. Moreover, HLH may be complicated by sepsis. Hyperinflammation, present in both states, gives an overlapping clinical picture including fever and performance status deterioration. The aim of this review is to provide aid in this challenging diagnostic process. Analysis of clinical features and laboratory results in multiple groups of patients (both adult and pediatric) with either HLH or sepsis allows to propose criteria differentiating these two conditions. The diagnosis of HLH is supported by hyperferritinemia, splenomegaly, marked cytopenias, hypofibrinogenemia, low CRP, characteristic cytokine profile and, only in adults, hypertriglyceridemia. In the presence of these parameters (especially the most characteristic hyperferritinemia), the other HLH criteria should be assessed. Genetic analyses can reveal familial HLH. Hemophagocytosis is neither specific nor sensitive for HLH.
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PMID:Similar but not the same: Differential diagnosis of HLH and sepsis. 2847 37

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon and life-threating condition characterized by major immune activation and massive cytokine production by mononuclear inflammatory cells, due to defects in cytotoxic lymphocyte function. It is even more unusual in renal transplant recipients, in which it is often associated with uncontrolled infection. The mortality is high in HLH and differential diagnosis with sepsis is a challenge. The approach and management depend on the underlying trigger and comorbidities. We report a case of a 50-year-old renal transplant female admitted with fever and malaise 3 months post-transplant and presenting anemia, fever, hypertriglyceridemia, high levels of serum ferritin, and positive CMV antigenemia. Urine was positive for decoy cells and BKV-DNA. Graft biopsy showed CMV nephritis. Both blood and urine cultures where positive for E. coli. Hemophagocytosis was confirmed by bone marrow aspiration. Immunosuppression was reduced, and the patient received high-dose intravenous immunoglobulin and dexamethasone, with complete response after 3 weeks. We highlight the importance of early diagnosis and proper management of a rare and serious condition in a renal transplant patient, which can allow a favorable clinical course and improve survival rate.
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PMID:Hemophagocytic lymphohistiocytosis, a rare condition in renal transplant - a case report. 3032 66

Haemophagocytosis encompasses phagocytosis of erythrocytes, leukocytes, platelets and their precursors by macrophages in bone marrow and other tissues. Haemophagocytic lymphohistiocytosis (HLH) usually presents with high fever, pancytopenia, splenomegaly, lymphadenopathy, haemophagocytosis in bone marrow, liver, lymphnodes or CSF. We report coagulase negative Staphylococcus induced HLH in a 16 year old boy presenting with high grade fever, lymphadenopathy, hepatosplenomegaly, pancytopenia with neutropenic sepsis in the department of Medicine, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh on 28th November 2016. He responded to high dose dexamethasone and management of neutropenic sepsis. This might give a guidance in the management of haemophagocytic syndrome at the earliest time to prevent morbidity and mortality.
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PMID:Haemophagocytic Lymphohistiocytosis in a 16-Year-Old Boy. 3075 76