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 17-year old female was admitted to our hospital with fever and systemic lymph node swelling. Laboratory data revealed pancytopenia, elevation of serum lactic acid dehydrogenase, triglyceride and ferritin levels. Bone marrow aspiration showed increased numbers of mature hemophagocytic histiocytes. We suspected virus-associated hemophagocytic syndrome (VAHS) but a biopsied lymph node revealed necrosis without the presence of hemophagocytosis by histiocytes and the diagnosis of subacute necrotizing lymphadenitis (SNL) was made. She recovered entirely after treatment with gamma-globulin, acyclovir and methylprednisolone. This patient was the second reported case of SNL with the clinical course mimicking VAHS and it is necessary to consider the relationship between these two disorders.
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PMID:[Subacute necrotizing lymphadenitis with a clinical course mimicking virus-associated hemophagocytic syndrome]. 806 22

A 15-year-old girl was admitted to our hospital because of fever, diarrhea, and right lower abdominal pain on November 11, 1997. Computed tomographic and ultrasound studies of the abdomen disclosed enlarged mesenteric lymph nodes. Hematologic and serologic findings included WBC 3000/microliter, LDH 550 IU/l, IFN-gamma 264 pg/ml, IL-6 9.74 pg/ml, sIL-2R 781 U/ml, and ferritin 720 ng/ml. Although the patient was treated with antibiotics, high fever and abdominal pain persisted with progressive anemia and leucocytopenia (1800/microliter). Bone marrow aspiration specimens revealed an increase of histiocytes with phagocytosis. Appendectomy and lymphadenectomy were performed on November 21. A lymph node specimen showed necrosis with infiltration by large mononuclear cells. The resected appendix revealed reactive lymphoid hyperplasia. Based on these findings, a diagnosis of necrotizing lymphadenitis (NL) was made. The postoperative course was satisfactory and systemic symptoms resolved gradually without specific treatment. However, high fever and abdominal pain recurred with right cervical lymph node swelling on December 15. The patient's general condition improved after treatment with prednisolone. In NL, lymphadenopathy is usually observed in the cervical region, and the involvement of intra-abdominal lymph nodes is quite rare. Our findings indicated the possibility that IFN-gamma may play an important role in the pathogenesis of NL with hemophagocytic syndrome.
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PMID:[Necrotizing lymphadenitis presenting as mesenteric lymphadenopathy]. 1049 41

A 26-year-old woman presented with general fatigue, persistent fever, nuchal lymphadenitis, thrombocytopenia, and liver damage. From the bone marrow finding, we diagnosed her condition as hemophagocytic syndrome. Steroid pulse therapy, cyclosporin A treatment, and combined chemotherapy generated no response. The patient showed severe mucosal bleeding, rapidly experienced multiple organ failure, and finally died of a brain hemorrhage on the 13th hospital day. Epstein-Barr virus, cytomegalovirus, human herpes virus type 6, human parvovirus B19, and herpes simplex virus were not detected. Autopsied samples of the spleen, bone marrow, and liver showed extreme proliferation of activated macrophages, so-called histiocytes, without lymphoid malignancy. The interferon gamma level at presentation was prominently high. The continuously elevated levels of ferritin and soluble interleukin 2 receptor were correlated with the catastrophic outcome. The disease in our case mimicked infantile hemophagocytic lymphohistiocytosis. However, there was neither a family history of the disease nor a mutation in the perforin gene. So, it is reasonable to categorize our case as macrophage activation syndrome. Although our patient lacked arthritis or eruption, we cannot deny the possibility that an oligoarthritis type of systemic-onset juvenile rheumatoid arthritis or, considering the patient's age, adult-onset Still disease lies at the base of our case.
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PMID:Fulminant hemophagocytic syndrome with a high interferon gamma level diagnosed as macrophage activation syndrome. 1523 1

A 54-year-old man of Persian origin presented to our department with a 1-year history of ulcers on the right leg that had been unresponsive to numerous topical treatments, accompanied by lymphedema of the right leg. Medical history included hypergonadotropic hypogonadism, which had not been further investigated. He was treated for 20 years with testosterone IM once monthly, which he stopped a year before the current hospitalization for unclear reasons. The patient reported no congenital lymphedema. Physical examination revealed two deep skin ulcers (Figure 1) on the right leg measuring 10 cm in diameter with raised irregular inflammatory borders and a boggy, necrotic base discharging a purulent hemorrhagic exudate. Bilateral leg pitting edema and right lymphangitis with lymphadenitis were noted. He had low head hair implantment, sparse hair on the body and head, hyperpigmentation on both legs, onychodystrophia of the toenails (mainly the large toe and less prominent on the other toes), which was atrophic lichen-planus-like in appearance and needed no trimming (Figure 2), normal hand nails, oral thrush, and angular cheilitis. Other physical findings were gynecomastia, pectus excavatum, small and firm testicles, long extremities, asymmetrical goiter, systolic murmur 2/6 in left sternal border, and slow and inappropriate behavior. The patient's temperature on admission was 39 degrees C. Blood cultures were negative for bacterial growth. Results of laboratory investigations included hemoglobin (11.2 g/dL), hematocrit (26.8%), normal mean corpuscular volume and mean corpuscular hemoglobin volume, and red blood cell distribution width (16%). Blood smear showed spherocytes, slight hypochromia, anisocytosis, macrocytosis, and microcytosis. Blood chemistry values were taken for iron (4 micro g/dL [normal range 40-150 micro g/dL]), transferrin (193 mg/dL [normal range 220-400 mg/dL]), ferritin (1128 ng/mL [normal range 14-160 ng/mL]), transferrin saturation (1.5% [normal range 20%-55%]), serum folate (within normal limits), and vitamin B12 (within normal limits). Direct Coombs' test equaled positive 2 + IgG. All these values indicated anemia of chronic diseases combined with hemolytic anemia. Further blood work-up tested antinuclear antibody (positive <1:80 homogeneous pattern), rheumatoid factors (143 IU/mL [positive >8.5 IU/mL]), C-reactive protein (286 mg/L [normal range 0-5 mg/L]), anticardiolipin IgM antibody (9.0 monophosphoryl lipid U/mL [normal range 0-7.00 MPL U/mL]) and antithrombin III activity (135% [normal range 74%-114%]). Results of other blood tests were within normal limits or negative, including lupus anticoagulant, beta2 glycoprotein, anticardiolipin IgG Ab, anti-ss DNA Ab, C3, C4, anti-RO, anti-LA, anti-SC-70, anti-SM Ab, P-ANCA, C-ANCA, TSH, FT4, anti-T microsomal, antithyroglobulin, protein C activity, protein S free, cryoglobulins, serum immunoelectrophoresis, VDRL, hepatitis C antibodies, hepatitis B antigen, and human immunodeficiency virus. Endocrinological work-up examined luteinizing hormone (22.9 mIU/mL [normal range for adult men 0.8-6 mIU/mL]), follicle stimulating hormone (49.7 mIU/mL [normal range for adult men 1-11 mIU/mL]), testosterone (0.24 ng/mL [normal range for adult men 2.5-8.0 ng/mL]), bioavailable testosterone (0.02 ng/mL [normal range for adult men >0.6 ng/mL]), and percent bioavailable test (8.1% [normal value >20%]). These results indicate hypergonadotropic hypogonadism. Plasminogen activator inhibitor 1 was 6 U (normal value 5-20 U/mL). Karyotyping performed by G-banding technique revealed a 47 XXY karyotype, which is diagnostic of Klinefelter's syndrome. Doppler ultrasound of the leg ulcers disclosed partial thrombus in the distal right femoral vein. X-rays and bone scan displayed osteomyelitis along the right tibia. Histological examination of a 4-mm punch biopsy from the ulcer border revealed hyperkeratosis, acanthosis, hypergranulosis, and mixed inflammatory infiltrate containing eosinophils compatible with chronic ulcer. Multiple vessels were seen, compatible with a healing process. Direct immunofluorescence of the biopsy revealed granular IgM in the dermo-epidermal junction. Indirect immunofluorescence was negative. Thyroid function tests showed normal thyroid stimulating hormone and free throxine4. Multinodular goiter was seen on thyroid scan and ultrasound. Thyroid fine needle aspiration was compatible with multinodular goiter (normal follicular cells, free colloid, macrophages with pigment). IV treatment with amoxicillin-clavulanic acid 1 g t.i.d. was administered for 2 weeks, with a decrease in temperature and normalization of the leukocyte level. Oral antibiotic treatment with amoxicillin-clavulanic acid was continued for 10 more days, followed by 25 days of ciprofloxacin for the osteomyelitis. Local treatment included saline soakings followed by application of Promogran (Johnson & Johnson, New Brunswick, NJ) and Kaltostat (ConvaTec Ltd., a Bristol-Myers Squibb Company, New York, NY) with slight improvement. At the same time, the patient was treated with warfarin sodium due to deep vein thrombosis under international normalized ratio 2-3. The patient was treated with IM testosterone once monthly for 1 year, which resulted in a reduction in the diameter and depth of the leg ulcers (Figure 3). Blood tests were not performed for follow-up of the immune state.
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PMID:Klinefelter's syndrome presenting with leg ulcers. 1536 65

A 24-year-old woman was referred to our hospital with fever and cervical lymphadenopathy. On admission, her serum concentration of soluble IL-2 receptor had increased to 1,740 IU/ml, LDH was 2,889 IU/l, and ferritin was 2,389 ng/ml. Dyspnea became evident (PaO2 60 Torr) with a further increase of serum ferritin to 96,552 mg/ml on day 11. Chest CT findings showed the thickening of the interlobular septa, bilateral pleural effusion, and swelling of mediastinal lymph nodes, symptoms which were very similar to those associated with lymphangitis carcinomatosis. She received steroid pulse therapy (methylprednisolone, 1,000 mg/day for three days). This resulted in a rapid improvement of her dyspnea and disappearance of the lymphatic blockade-mediated pulmonary findings in two days. A cervical lymph node biopsy was performed on day 12, and revealed that she had subacute lymphadenitis (Kikuchi disease). This case suggests that acute respiratory failure should be considered in cases of Kikuchi disease when associated with a lymphatic blockade.
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PMID:[Subacute necrotizing lymphadenitis showing lymphatic blockade-mediated acute respiratory failure]. 1678 97

We studied clinical manifestations, laboratory results, treatment, recurrence, and complications in 69 patients diagnosed with Kikuchi's disease by lymph node pathology from January 1, 1998, to December 31, 2007. Subjects were 34 men and 35 women (median age: 28 years, range: 12 to 58 years). Of the 69, 67 were Japanese and 2 were Korean. Major clinical symptoms and signs were fever >37 degrees (71%) and lymphadenopathy (100%). Lymphadenopathy was cervical in 93% and systemic in 3%. Some experienced night sweats and weight loss. The median white blood cell count was 3800 microL (1,700-9,300 microl), and 50.7% of subjects had leukocytopenia (<4,000 microL). The median serum LDH concentration was 245U/L (129-923 U/L). The median ferritin concentration, measured in 26 cases, was 769 ng/mL (4.5-2,580 ng/mL). The median concentration of soluble IL-2 receptor, measured in 27 cases, was 639 U/mL (0.5-4,000 U/ml). Having observed several cases with abnormally high ferritin and soluble IL2 receptor, we note the importance of carefully considering differential diagnosis from Still's disease and malignant lymphoma. Treatment included no medication in 30% of subjects, nonsteroidal anti-inflammatory drugs alone in 37.7%, steroids alone in 7%, and combined nonsteroidal anti-inflammatory drugs and steroids in 22%. Of the 29% administered steroids, we mostly used prednisolone (0.5-1.0 mg/kg), tapering the dose as clinical features improved. Two developed aseptic meningitis and 2 systemic lupus erythematosus. In total, 75% improved in less than 3 months, whereas 6% showed improvement only after at least 6 months of continued treatment. All were cured, but the condition recurred in 8%. Recurrence was not associated initial the disease duration. Recurrence cannot be predicted but can occur. In some cases, we could not distinguish lymphadenitis from malignant lymphoma or tuberculous lymphadenitis based on clinical features or laboratory data. Diagnosis must thus be based on lymph node pathology. We also must consider the possibility of recurrence or attacks of systemic lupus erythematosus.
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PMID:[Medical study of 69 cases diagnosed as Kikuchi's disease]. 1969 71

Histiocytic necrotizing lymphadenitis (also known as Kikuchi-Fujimoto's disease) is an uncommon, benign and self-limiting lymph disease. We report three patients aged 14, 22 and 50 years, who presented with fever and cervical lymphadenopathy, accompanied by skin lesions and joint pain in two of the three cases. One of the patient's sister suffered from histiocytic necrotizing lymphadenitis. Laboratory abnormalities varied and findings included leucopenia, relative neutrophilia, elevated C reactive protein, erythrocyte sedimentation rate and ferritin. Basic laboratory screening tests were performed on all patients to rule out autoimmune and infectious diseases. Lymph node biopsy and subsequent pathological examination were essential to establish the diagnosis. All patients received antibiotics at some point of their hospital stay. Two patients required glucocorticoid treatment, while the remaining case experienced a spontaneous recovery. Its pathogenesis is still unknown, but clinical and histopathological studies suggest a connection with autoimmune diseases. There is no established treatment, but apparently the disease responds to the administration of glucocorticoids.
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PMID:[Histiocytic necrotizing lymphadenitis: report of 3 cases]. 2408 82

Kikuchi-Fujimoto disease (KFD) is a benign cause of self-limiting subacute necrotizing lymphadenitis. KFD is rarely complicated with reactive hemophagocytic lymphohistiocytosis (HLH), and the clinical features of the simultaneous occurrence of these conditions are uncertain. A 30-year-old Japanese man with a persistent fever and sore throat presented to our hospital for treatment. Laboratory analysis showed bicytopenia, and radiological studies showed systemic lymphadenopathy accompanied by splenomegaly. A bone marrow examination showed hemophagocytic macrophages, suggesting HLH. Malignant lymphoma was suspected as a possible underlying disease, but the histology of the lymph nodes led to a final diagnosis of KFD and treatment with prednisolone (1 mg/kg/day), resulting in clinical improvement. This case highlighted the importance and difficulty of differentiating KFD from malignant lymphoma as an underlying condition of HLH. The literature review showed that patients with HLH-associated KFD may have higher serum ferritin and lactate dehydrogenase levels compared to typical KFD cases. Definite diagnosis based on pathological examination is essential for a better understanding of this rare disease. The presence of systemic lymphadenopathy does not exclude the possibility of KFD. This case serves to remind physicians that KFD is a potential etiology of HLH.
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PMID:Kikuchi-Fujimoto Disease Complicated with Reactive Hemophagocytic Lymphohistiocytosis. 2777 31

Periodic fever with pharyngitis, aphthous stomatitis and cervical adenitis (PFAPA syndrome) is a common cause of periodic fever in children and usually manifests as episodes of fever recurring with a clockwork periodicity. Although rare after adolescence, adult patients with PFAPA syndrome may present with a wider range of symptoms and may lack the clockwork periodicity of fever. A 24-year-old patient presented with a 4-year history of periodic fever with pharyngitis and cervical adenitis. She also complained of vomiting, fatigue and sporadically presented with aphthous stomatitis. During crises, laboratory evaluation showed a moderate elevation of inflammatory markers. Blood cultures and ANA titres were negative. Immunoglobulins and serum ferritin levels were normal. After other causes of periodic fever had been excluded, a diagnosis of PFAPA syndrome was made.
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PMID:Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults. 3093 Dec 75