Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P02794 (ferritin)
 17,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a case of haemophagocytic syndrome caused by Histoplasma capsulatum reactivation in a patient with chronic lymphocytic leukaemia treated with fludarabine and alemtuzumab. He presented with fever, pancytopenia, increased serum ferritin, lactate dehydrogenase and soluble interleukin-2 receptor. A bone marrow aspirate showed haemophagocytosis and possibly a yeast infection. Treatment with cyclosporine, dexamethasone, etoposide and caspofungin was started. After initial improvement his condition deteriorated. A second bone marrow examination confirmed a Histoplasma infection. After treatment with amphotericin B, the fever resolved and blood counts normalised. Haemophagocytic syndrome is a critical condition with high mortality that requires immunosuppressive therapy. The underlying cause should be investigated and treated. In this case a Histoplasma reactivation is described in a severely immunocompromised host years after the patient had left the endemic area.
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PMID:Histoplasma capsulatum reactivation with haemophagocytic syndrome in a patient with chronic lymphocytic leukaemia. 2120 68

Two children presented with a history of fever and rash. Lab values revealed pancytopenia, elevated ferritin, coagulopathy, and elevated triglycerides. Both children quickly developed respiratory distress and hypotension requiring admission to the ICU. Bone marrow biopsies revealed hemophagocytosis. Studies for Ehrlichia returned positive. The patients were started on doxycycline and treated for hemophagocytic lymphohistiocytosis (HLH). Each made a full recovery. In both patients, testing for MUNC and perforin genes were found to have no mutation. These two cases demonstrate the importance of considering Ehrlichiosis as a possible trigger of HLH.
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PMID:Ehrlichia-induced hemophagocytic lymphohistiocytosis in two children. 2129 56

Hemophagocytic lymphohistiocytosis (HLH), also known as hemophagocytic syndrome (HS), is a life-threatening hyperinflammatory condition caused by uncontrolled proliferation of activated lymphocytes and histiocytes producing excessively proinflammatory cytokines. HLH can occur in all age groups. The most common syndromes are: prolonged fever, hepatosplenomegaly, unspecific neurological symptoms, pancytopenia, hemophagocytosis. Characteristic biochemical markers include high triglycerides, ferritin and decreased fibrinogen. There are two forms of HLH, primary (genetic) and acquired (secondary to infections, malignant and autoimmune diseases). Occurrence of both forms of HLH, acquired and genetic, is induced by infections, usually viral or by other triggering agents. Despite established diagnostic criteria many cases probably remain unrecognized. The main diagnostic difficulty is low specificity of symptoms, and in secondary form contemporary occurrence of symptoms of underlying disease. The aim of HLH treatment is to suppress hyperinflammation, what can be achieved by use of immunosuppressive/immunomodulating agents or cytostatics. Patients with genetic form of HLH require hematopoietic stem cell transplantation. Awareness of the symptoms and diagnostic criteria of HLH is important for early diagnosis and immediate application of life-saving therapy.
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PMID:[Hemophagocytic lymphohistiocytosis: diagnostic problems in pediatrics]. 2134 73

The myelodisplastic syndrome is a heterogeneous group of diseases, characterised by ineffective and dysplastic haematopoesis and pancytopenia in the peripheral blood, followed by progressive disturbance of differentiation of the haematopoetic stem cell, resulting in evolution of the disease towards acute leukaemia. According to the latest WHO classification, the term myelodisplastic syndrome includes diseases with an indolent course, as well as diseases with a fast evolution towards acute leukaemia. Because of this diversity, haematologists base their therapeutic decisions on prognostic scoring systems which incorporate all the significant factors with an influence on survival in this group of patients with myelodisplastic syndrome. Bearing in mind that anaemia is the most frequent form of cytopenia in patients with myelodisplastic syndrome, it is common that at some point of the disease almost every patient with myelodisplastic syndrome is transfusion-dependent. Frequently applied transfusions secure the correction of anaemia in these patients, giving them a good quality of life, but at the same time endangering them with the potential threat of iron overload, when the physiological mechanisms of iron excretion from the organism become insufficient. There is a clear correlation between transfusion dependence and the overall survival in patients with myelodisplastic syndrome. Chelators secure the lowering of the iron surfeit and are indicated in transfusion-dependant patients with myelodisplastic syndrome ( need for two blood units monthly, during one year ), when the ferritin level increases over 1000, in patients who are candidates for transplantation as well as in patients from good prognostic groups with median survival over one year. The therapy with chelators lasts as long as the patient is transfusion-dependant.
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PMID:Iron overload in patients with transfusion dependent myelodisplastic syndrome. 2182 96

Hemophagocytic syndrome (HPS) has been associated with infections, hematological malignancies and autoimmune conditions. Malaria is rarely reported to cause HPS. We report a case of an 11-month-old infant with fever, hepatosplenomegaly, pancytopenia, high serum ferritin, hypertriglyceridemia, and bone marrow hemophagocytosis, consistent with hemophagocytic syndrome. Gametocytes of plasmodium falciparum were identified on bone marrow aspiration. Rapid recovery was observed after treatment with antimalarials.
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PMID:Hemophagocytic syndrome associated with Plasmodium falciparum infection. 2193 31

An immunocompetent Nigerian developed a fulminant hemophagocytic lymphohistiocytosis due to Epstein-Barr virus reactivation. The patient initially presented with fever, hepatosplenomegaly and pancytopenia. The clinical status of our patient deteriorated quickly despite treatment with corticoids. Escalation of immunosuppressive treatment was not possible. He died of lung, liver and circulatory failure in our intensive care unit.Hemophagocytic lymphohistiocytosis is a rare disease characterized by inflammation due to prolonged and excessive activation of antigen-presenting cells. High plasma ferritin levels and phagocytosis of hematopoetic cells in bone marrow, spleen and liver lead to the diagnosis. Hemophagocytic lymphohistiocytosis should therefore be included in the differential diagnosis in patients with persistent fever, hepatosplenomegaly and cytopenia.
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PMID:[Unexplained fever and B-symptoms in a young male Black African]. 2195 27

We report the case of a 26-year-old patient who was victim of severe head trauma following a trafic road accident. Two days after trauma, she had fever, pancytopenia and multi-organ failure. The haemophagocytic lymphohistiocytosis syndrome was suspected. This diagnosis was confirmed by biologic findings (elevated serum levels of ferritin and triglycerid) and histological findings (examination of bone marrow smears showing histiocytes phagocytosing blood cells). The evolution was unfavorable despite of corticotherapy and symptomatic measures.
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PMID:[Haemophagocytic lymphohistiocytosis syndrome: a possible cause of multi-organ failure in severe head trauma?]. 2197 80

A 15 month-old girl was admitted after a couple of months' history of illness with remittent fever, increasing pallor and a swollen abdomen. On admission she was highly febrile, with palpably enlarged liver and spleen. Blood tests revealed pancytopenia, a high CRP level and a high serum ferritin level. We describe the diagnostic evaluation, interpretation and treatment.
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PMID:A 15-month-old girl with fever and pancytopenia. 2217 Jan 37

Hemophagocytic lymphohistiocytosis occurring as a primary or acquired disorder is a condition of chaotic and uncontrolled immune system stimulation. Cytotoxic cells and macrophages cause multiorgan damage, hemophagocytosis, and severe systemic inflammation. Clinical manifestations include a fever, organ enlargement, and weight loss. Laboratory tests show bicytopenia or pancytopenia, cytolysis and cholestasis, serum ferritin elevation, and clotting disorders. The reference standard for the diagnosis remains the presence in histological specimens of hemophagocytic macrophages, which may be lacking early in the disease, leading to diagnostic challenges. Inherited forms produce symptoms in early childhood and are fatal in the absence of specific treatment. In adults, the clinical spectrum ranges from mild and self-limited hemophagocytic lymphohistiocytosis to rapidly fatal multiorgan failure. Many questions remain unresolved regarding the diagnosis and treatment in adults. This update is an attempt at providing answers.
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PMID:Hemophagocytic lymphohistiocytosis in adults: diagnosis and treatment. 2246 18

Hemophagocytic syndrome (HPS) may be provoked by infections, malignancies and autoimmune diseases. We report on a 56-year-old woman with long-lasting systemic lupus erythematosus (SLE) who presented with malar rash, inflammatory livedo reticularis, fever, weight loss, pancytopenia and mild splenomegaly with cervical lymphadenopathy. She had criteria for SLE flare-up (malar rash, high antinuclear antibody titer, complement consumption, pathological urinary sediment, and retinal vasculitis). Despite high-dose glucocorticoid therapy, pancytopenia and fever worsened. Important elevations of triglycerides and ferritin were also found. Bone marrow aspirate demonstrated hemophagocytosis, which confirmed the coexistence of HPS and SLE. The treatment with glucocorticoids, immunoglobulins, cyclophosphamide, filgrastim and antimicrobial therapy was unsuccessful. After one month, the patient developed Pneumocystis jirovecii pneumonia with fatal outcome. Bone marrow biopsy, taken 5 days before death, showed high grade diffuse large B-cell (CD20+, Ki-67+) non-Hodgkin's lymphoma (DLBCL). We are the first to report the association of both SLE and non-Hodgkin's lymphoma complicated by HPS. We showed that, based on clinical and laboratory data, it was difficult to distinguish the early phase of HPS from SLE flare-up and new-onset DLBCL. Therapy of such a complex case of HPS has not been standardized, and opportunistic infections remain a difficult issue.
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PMID:Systemic lupus erythematosus progressing to non-Hodgkin's lymphoma complicated by fatal hemophagocytic syndrome: case report. 2250 70


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