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)

A 68-year-old man was admitted to our hospital because of fever, jaundice and hepatosplenomegaly. A diagnosis of diffuse large cell, B-cell type malignant lymphoma, associated with hemophagocytic syndrome (LAHS), was made. CT scan revealed lymphadenopathy in the abdominal cavity and multiple tumors in the spleen. Performance status and hepatic coma grade were 4 and II, respectively. Laboratory findings showed bicytopenia (Hb 9.9 g/dl, platelet 35 x 10(3)/microliter), severe liver dysfunction (ALP 1,115 U/l, gamma-GTP 437 U/l, T.Bil 15.4 mg/dl, D.Bil 12.8 mg/dl) and elevated levels of beta 2 microglobulin (12.9 mg/dl), ferritin (2,300 ng/ml) and sIL-2 receptor (36,900 U/ml). Plasma exchange (PE) and continuous hemodiafiltration (CHDF) enabled the patient to undergo diagnostic procedures, irradiation (total 34 Gy) and chemotherapy. Biopsy specimens revealed infiltration of lymphoma cells into the liver and bone marrow. We measured the blood concentrations of TNF-alpha, IL-6, and IL-8 before and after PE and CHDF by the ELISA method, and found normalization of hypercytokinemia after the procedure. It was suggested that initial treatment with PE and CHDF was effective for control of HPS, enabling us to perform chemotherapy for the lymphoma.
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PMID:[Plasma exchange and continuous hemodiafiltration as an initial treatment for diffuse large B-cell lymphoma-associated hemophagocytic syndrome]. 1186 63

Iron overload (IO) is associated with free radical generation and tissue damage. Our main objective was to ascertain if very high levels (VHL) of ferritin (>/=3000 microg/l) and transferrin saturation (TS) >/=100% during conditioning had an impact on overall survival (OS) and transplant-related mortality (TRM) after a haematopoietic stem cell transplantation (HSCT). Levels of ferritin and TS were measured at days -7 and -4, respectively, in 25 patients who underwent HSCT after CY/TBI. The group consisted of 20 men and five women with a median age of 40 years. Fifteen patients were autotransplanted and 10 allotransplanted. Nine of them had a diagnosis of AL, six of CML and 10 of lymphoma. Thirteen of them were in early and 12 in advanced status of disease. VHL of ferritin and TS >/=100% were associated with a decreased OS (P = 0.001 and P = 0.006, respectively) and an increased TRM (P = 0.003 and P = 0.004, respectively) in univariate survival analysis. Both variables remained significant at multivariate analysis for OS (P = 0.03 and 0.02, respectively) and TS was an independent factor for TRM (P = 0.01). Ferritin was very close to achieving statistical significance for TRM (P = 0.06) in multivariate analysis. In conclusion, VHL of ferritin and TS >/=100% at conditioning are associated with an increase in toxic deaths after transplant.
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PMID:Iron overload might increase transplant-related mortality in haematopoietic stem cell transplantation. 1209 67

TALL-1 is a member of the tumor necrosis factor family that binds to BCMA, TACI, and BAFF-R, three receptors mostly expressed by mature B lymphocytes. Previous studies have shown that the TALL-1 signaling is critically involved in B cell proliferation, maturation, and progression of lupus-like, autoimmune diseases. In this report, we performed cDNA subtractive hybridization experiments to identify downstream genes up-regulated by TALL-1. These experiments indicated that 10 genes, including interleukin (IL)-10, lymphocyte activation gene-1 (LAG-1), GCP-2, PBEF, ferritin, PIM-2, TFG, CD27 ligand, DUSP5, and archain, were up-regulated at the mRNA level by TALL-1 stimulation in B lymphoma RPMI-8226 cells and/or primary B lymphocytes. We also demonstrated that TALL-1 activated transcription of IL-10 and LAG-1 in a nuclear factor-kappaB-dependent manner in reporter gene assays. Moreover, our findings indicated BAFF-R, but not TACI, could dramatically up-regulate IL-10 secretion by RPMI-8226 cells. The identification of TALL-1-up-regulated genes will help explain the mechanisms of TALL-1-triggered biological and pathological effects and to identify molecular targets for intervention of lupus-like autoimmune diseases.
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PMID:Identification of downstream genes up-regulated by the tumor necrosis factor family member TALL-1. 1214 33

The development of central pontine myelinolysis (CPM) has rarely been reported in association with hemophagocytic syndromes (HPS). Here we report a unique case of Epstein-Barr Virus (EBV)-related HPS which was accompanied with CPM. A 72-year-old man who had no significant medical history was admitted to our hospital due to high fever and progressing dysphasia and dysarthria. Physical examination revealed anisocoria of the right pupil, fixed reaction to light, and paralysis of the left vagus nerves. Magnetic resonance imaging revealed low signal intensity on T1-weighted images and high signal intensity T2-weighted images in the patient's central midpontine lesion. Initial work-up showed anemia and thrombocytopenia with elevated levels of serum ferritin, lactate dehydrogenase, and soluble IL-2 receptor. Bone marrow aspiration revealed hemophagocytosis. The EBV genome was detected in the peripheral blood using the polymerase chain reaction method. He was diagnosed as having EBV-related HPS and CPM. Despite intensive treatment with methylpredonisolone, immunoglobulin, and etoposide, he died due to progressive disease and fungal septicemia. The etiology and relation between CPM and HPS are discussed.
Leuk Lymphoma 2002 Oct
PMID:EBV associated hemophagocytic syndrome accompanied by central pontine myelinolysis. 1248 7

The authors describe a 15-year-old boy with hepatosplenic gammadelta T-cell lymphoma associated with hemophagocytic syndrome (HPS) along with isochromosome 7q and trisomy 8. He presented with prolonged fever, mild anemia, thrombocytopenia, and hepatosplenomegaly. Physical examination, radiography, and ultrasound tomography revealed no lymphoadenopathy. He had elevated levels of serum ferritin, interferon-gamma, soluble interleukin-2 receptor, and interleukin-6. Bone marrow aspirate showed hypercellularity with 50% lymphoblasts and erythrophagocytosis of macrophage. A cytogenetic study of bone marrow revealed an abnormal karyotype, 47,XY,I(7q),+8, in 5/30 cells. Clonal rearrangement of the genes for T-cell receptor gamma and delta chains was elucidated by polymerase chain reaction. He achieved a complete remission after intensive chemotherapy and underwent splenectomy 18 months after diagnosis. Although the patient was clinically in remission, minimal residual disease (MRD) was detected in the removed spleen by polymerase chain reaction. This might mean that this type of lymphoma is refractory, as reported previously, and might indicate that marrow ablative therapy is needed to achieve a cure. The present case illustrates the usefulness of MRD analysis, and MRD studies in this group of disorders may be helpful in the decision of whether to continue a more aggressive therapeutic approach.
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PMID:Hemophagocytic syndrome and hepatosplenic gammadelta T-cell lymphoma with isochromosome 7q and 8 trisomy. 1516 51

We report a case of Epstein-Barr virus-associated aggressive natural killer cell leukemia/lymphoma with giant splenomegaly in which splenectomy resulted in progression of apoptosis and hemophagocytosis. The serum level of ferritin, triglycerides, interferon-gamma (IFN-gamma), interleukin 10 (IL-10), and soluble Fas ligand (sFasL) increased markedly, and liver damage progressed after splenectomy. The number of apoptotic cells in peripheral blood smears increased following splenectomy. In the present case, splenectomy caused disruption of the cytokine network, resulting in apoptosis of blood cells and hepatocytes, as well as phagocytosis.
Leuk Lymphoma 2004 Nov
PMID:Marked elevation of soluble fas ligand and cytokine secretion after splenectomy in aggressive natural killer cell leukemia/lymphoma. 1551 19

Haemophagocytic syndrome (HPS) secondary to viral infections usually has a variable course and can be life-threatening. We report a 53-year-old male patient who presented with fever, hepatosplenomegaly and pancytopenia. He had deranged liver function, abnormal clotting and markedly elevated serum ferritin. Bone marrow biopsy showed prominent haemophagocytosis. The patient was investigated thoroughly and found to have evidence of chronic hepatitis B-virus (HBV) infection by serological tests and liver biopsy. Other conditions associated with HPS such as lymphoma, malignancy and other viral or bacterial infections were not present. The patient did not respond to steroids, intravenous immunoglobulins or cyclosporin but responded to etoposide and became apyrexial. He also became HBV negative on lamivudine. The patient died of infection later on but there was no evidence of recurrence of HPS. To the best of our knowledge this is the first case report of HPS associated with isolated HBV infection.
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PMID:Haemophagocytic syndrome associated with hepatitis-B virus infection responding to etoposide. 1630 42

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an unusual type of skin lymphoma, characterized by subcutaneous soft tissue infiltration with pleomorphic T-cells and benign macrophages that mimic panniculitis. Hemophagocytic lymphohistiocytosis is a rare but potentially fatal disorder which is thought to result from uncontrolled activation and proliferation of T-cells and excessive activation of macrophages. Hemophagocytic lymphohistiocytosis have been reported as the presenting feature in 37% of SPTCL patients. High-resolution ultrasonographic appearance of SPTCL with a characteristic finding has been described in only one patient. We report a 28-year-old woman with hemophagocytic lymphohistiocytosis presented with nonremitting fever, thrombocytopenia, hypofibrinogenemia, hyperferritinemia, increased serum levels of soluble interleukin (IL)-2 receptors and hemophagocytosis in bone marrow examination. Markedly elevated level of serum IL-18 was also demonstrated in our patient. The fever subsided and the ferritin level declined to normal after treatment with oral steroid and etoposide. No evidence of hemophagocytosis was revealed in a follow-up bone marrow examination. However, nonremitting high fever and panniculitis-like skin lesions over her back, arm, lower abdominal wall, and bilateral pretibial regions developed 1 month later. A high-resolution ultrasonography revealed thickening of the subcutaneous fat layer with homogeneous hyperechogenicity pattern and poor-defined margin over the skin lesions. An ultrasound-guided excision biopsy of the nodular lesion over left upper arm was performed and the histopathology showed neoplastic cells with hyperchromatic nucleoli, which extended from deep dermis to subcutaneous fat. The immunochemical stain showed a T-cell lineage of tumor cells. SPTCL was diagnosed and the skin lesions subsided gradually after treatment with anthracycline-based combination chemotherapy.
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PMID:Subcutaneous panniculitis-like T-cell lymphoma presenting with hemophagocytic lymphohistiocytosis and skin lesions with characteristic high-resolution ultrasonographic findings. 1646 78

Macrophage activation syndrome (MAS), which can also be considered as reactive hemophagocytic syndrome (HPS), is a rare and potentially fatal complication of rheumatic diseases. We describe a 42-year-old woman in whom MAS developed as a complication of ankylosing spondylitis (AS). She suffered from fever and low back pain before admission. Laboratory findings were pancytopenia, abnormal liver enzymes, increased ferritin levels, and positive for B27. Hyperplasia of hemophagocytic macrophages was confirmed in her bone marrow. High-dose steroids therapy resulted in clinical and laboratory improvements. In this patient, there was no possible causative factor of HPS (such as viral infection, lymphoma, and systemic lupus erythematosus) except the presence of AS. There have been no previously reported cases describing the relationship between AS and HPS. This case indicates that attention should be given to the possibility that certain patients with AS-associated cytopenia may display accompanying intramedullary hemophagocytic phenomena.
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PMID:Ankylosing spondylitis presenting with macrophage activation syndrome. 1723 50

The aim of the study was to evaluate the role of hypochromic erythrocytes (HYPO%) compared to "traditional" and novel markers of iron status and erythropoiesis in recognizing iron-restricted erythropoiesis (IRE) and predicting response to erythropoietin (rHuEPO) in anemic patients with myeloma and lymphoma. Forty-one newly diagnosed patients who received epoetin-beta at a subcutaneous weekly dose of 30,000 IU for 6 weeks were studied. Response to rHuEPO was observed in 27 patients (65.8%). Twelve non-responders received, additionally, 200 mg of IV iron sucrose, weekly, for 4 weeks. Evaluation of markers was performed at baseline and on weeks 1, 2 and 6 for all patients and also on weeks 7-10 for non-responders to rHuEPO. Baseline HYPO%, at a cut-off value of <5%, and an increment in reticulocyte absolute number (RETICS-AB) >or= 50,000/microl and reticulocyte hematocrit (RETICS-Hct) >or= 50%, between baseline and week 2, were independent predictive factors for response to rHuEPO. We found that these markers had superior predictive value for response to rHuEPO than four widely used predictive models. Furthermore, a baseline HYPO% count of above 5% proved superior over serum ferritin < 100 ng/ml and transferrin saturation < 20% in recognizing IRE. In conclusion, baseline HYPO% either alone or in combination with RETICS-AB or RETICS-Hct after 2 weeks of rHuEPO treatment could be reliably used in predicting response to rHuEPO. Additionally, HYPO% has proved a reliable marker for recognizing IRE before rHuEPO treatment and, thus, could be used for identifying patients who will benefit from IV iron supplementation.
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PMID:Hypochromic erythrocytes (%): a reliable marker for recognizing iron-restricted erythropoiesis and predicting response to erythropoietin in anemic patients with myeloma and lymphoma. 1737 2


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