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 report a 40-year-old man who presented with acute onset of hemophagocytic syndrome (HPS) after allogeneic bone marrow transplantation (alloBMT) for acute myelogenous leukemia. On day 8 after alloBMT, the patient suddenly manifested high-grade fever, transfusion-resistant severe anemia, and thrombocytopenia. Neither veno-occlusive disease nor thrombotic microangiopathy was documented. The level of ferritin in serum was elevated to 1192 ng/mL. A bone marrow aspiration test on day 16 showed a markedly increased number of activated macrophages showing massive hemophagocytosis. Serum levels of interferon-gamma, soluble interleukin-2 receptor, interleukin-6, tumor necrosis factor-alpha, and macrophage colony-stimulating factor (M-CSF) were elevated. From these findings, we determined his transfusion-resistant cytopenias to be attributable to HPS. No viruses (including cytomegalovirus, Epstein-Barr virus, human herpes-virus-6, parvovirus B19, and adenovirus B11) were detected in serum or urine by polymerase chain reaction amplification. We speculate that in addition to the administration of M-CSF, hypercytokinemia during the early phase post-alloBMT might have contributed to the onset of HPS in this patient. Methylprednisolone pulse therapy was very effective for the treatment of the HPS. This case reveals that HPS could develop after alloBMT, even when engraftment of hematopoietic cells is not confirmed.
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PMID:Early onset of hemophagocytic syndrome following allogeneic bone marrow transplantation. 1103 76

A 23-year-old man, admitted because of high fever, polyarthralgia, butterfly rash and chest pain, was diagnosed as systemic lupus erythematosus (SLE) from the findings of positive antinuclear antibody and anti-DNA antibody. He was treated with 60 mg prednisolone daily, but as reducing the dose, white blood cell counts and platelet counts were decreased and fever, polyarthralgia, decrease of complements, increase of ferritin, hepato-splenomegaly and liver dysfunction were observed. Bone marrow specimen revealed phagocytosis of blood cells by histiocytes and he was diagnosed as hemophagocytic syndrome(HPS) due to active SLE. Methylprednisolone pulse therapy was effective temporarily, HPS recurred while reducing steroid, and cyclosporin was added. After a temporary remission, marked extensive swelling in the face appeared suddenly. Facial skin biopsy showed necrosis of fat cells and hemophagocytosis by histiocytes. Accordingly, he was diagnosed as panniculitis due to HPS and was treated successfully with intravenous cyclophosphamide pulse therapy and high dose of gammaglobulin. Several cases of HPS due to SLE have been reported recently, but this is a rare case of cytophagic histiocytic panniculitis (CHP) due to SLE.
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PMID:[A case of systemic lupus erythematosus with hemophagocytic syndrome and cytophagic histiocytic panniculitis]. 1129 53

Macrophage activation syndrome (MAS) is a rare and potentially fatal complication of rheumatic disorders in children. We describe a 13-month-old boy in whom MAS developed as a complication of systemic juvenile rheumatoid arthritis (S-JRA). He suffered from fever and generalized rash followed by multiple joints swelling for four months before admission. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, coagulopathies resembling disseminated intravascular coagulation, anemia and thrombocytopenia. Hyperplasia of hemophagocytic macrophages was remarkable in his bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvements. This is the third case of MAS associated with S-JRA in Koreans, and the first one, in which hemophagocytic macrophages were proven in bone marrow.
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PMID:Macrophage activation syndrome in a child with systemic juvenile rheumatoid arthritis. 1610 Apr 70

Macrophage activation syndrome (MAS) belongs to secondary hemophagocytic lymphohistiocytosis (HLH) syndrome. It is usually associated with rheumatic diseases. We retrospectively reviewed our hospital's medical records of 102 HLH/MAS patients from the past 20 years. Demographics, clinical data, treatment, and outcomes were analyzed. Among 102 patients, eight patients with underlying juvenile systemic lupus erythematous (two patients), mixed connective tissue disease (one patient), primary anti-phospholipid syndrome (one patient), and systemic type juvenile rheumatoid arthritis (sJRA; four patients) with 13 episodes of MAS were studied. Clinical manifestations of MAS included fever (100 %), hepatosplenomegaly (77 %), lymphadenopathy (38 %), skin rash (62 %), and neurological involvement (31 %). Laboratory features included leukopenia (54 %), anemia (46 %), thrombocytopenia (77 %), jaundice (27 %), hypofibrinogenemia (40 %), decreased erythrocyte sedimentation rate (67 %), and elevated liver enzymes (77 %), lactate dehydrogenase (100 %), ferritin (88 %), triglycerides (91 %), C-reactive protein (85 %), plasma D-dimer (50 %), and hemophagocytosis in bone marrow (83 %). The Epstein-Barr virus and adenovirus infection triggered MAS in two patients with sJRA. Methylprednisolone pulse therapy was effective in two out of three patients, and high-dose intravenous immunoglobulin (IVIG) was effective in two out of six patients. Patients with sJRA responded well to corticosteroids and cyclosporine. Complications included opportunistic infection with Pneumocystis jiroveci, multiple organ failure, and intensive care unit myopathy. The mortality rate was one out of eight (12.5 %). Our results showed that MAS could be fatal and complicate various pediatric autoimmune diseases. It generally has a good response to corticosteroids and IVIG. Prompt recognition and timely treatment can result in good outcomes.
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PMID:Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases. 2261 46

We report a rare case of rhabdomyolysis caused by peripheral T-cell lymphoma (PTCL) in skeletal muscle. A 62-year-old man was admitted with complaints of sudden muscle weakness. Laboratory abnormalities were identified including markedly elevated creatinine-phosphokinase, peaking at 62,640 IU/L and serum creatinine (Cr) at 5.0 mg/dL. Computed tomography scans revealed tumorous swelling of the right psoas major muscle and the obturator internus muscles. Consequently, he was diagnosed with acute renal failure caused by rhabdomyolysis and was treated with hydration and continuous hemodiafiltration, which resulted in significant improvement in renal function (Cr 1.79 mg/dL). However, the cause of the rhabdomyolysis remained unclear, and he suddenly developed a remittent fever and suffered from hemophagocytic syndrome. Serum ferritin level dramatically increased to 104,707.0 ng/mL and creatinine level to 4.09 mg/dL. We performed a biopsy of inguinal lymph nodes, leading to a diagnosis of PTCL. Finally, he was diagnosed with rhabdomyolysis caused by PTCL. Methylprednisolone pulse therapy markedly improved his general condition and renal function (Cr 1.48 mg/dL), and computed tomography scans revealed that tumorous swelling was greatly diminished. Except when the cause of rhabdomyolysis is readily apparent, such as in cases of trauma, drug and thrombophlebitis, one should consider that rhabdomyolysis may be a sequel of lymphoma.
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PMID:Rhabdomyolysis caused by peripheral T-cell lymphoma in skeletal muscle. 2381 71

Macrophage activation syndrome (MAS) is a severe and life-threatening complication of rheumatic disorders in children. We described a 9-year-old girl in whom MAS developed as a complication of systemic juvenile idiopathic arthritis (S-JIA) at onset with obvious hemophagocytosis presented in the marrow. She suffered from high fever and generalized rash subsequently joints swelling for two weeks before admission. Physical examination revealed mild cervical lymphadenopathy and hepatosplenomegaly. Laboratory findings were: abnormal liver enzymes, increased triglyceride and ferritin levels, anemia .Hyperplasia of hemophagocytic macrophages was remarkable in her bone marrow. Methylprednisolone and cyclosporin therapy resulted in clinical and laboratory improvement. It is unusual that hemophagocytosis presented in the marrow at onset of So-JIA without obvious abnormal coagulation profile, thrombocytopenia and leucopenia. It seemed that MAS may be occult at onset of SJIA. It may be integral to the pathogenesis of SJIA. The proper cyclosporine serum lever at the onset of MAS is as high as 200-300 ng/ml.
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PMID:Occult macrophage activation syndrome in systemic-onset juvenile idiopathic arthritic syndrome--a case report. 2414 17