Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0243026 (sepsis)
 52,417 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Azathioprine (AZA) is a commonly used immunosuppressant for systemic lupus erythematosus (SLE). Myelosuppression is a serious adverse reaction due to AZA and its metabolites. Thiopurine S-methyltransferase (TPMT) is the rate-limiting enzyme. Variations of TPMT enzyme activity may be responsible for myelosuppression. However, a correlation between certain mutant alleles of low TPMT enzyme activity and myelotoxicity has also been suggested as a factor. We describe herein a case of AZA-induced severe myelosuppression associated with TPMT*3C heterozygous mutant allele in a SLE patient. The patient presented with pancytopenia, sepsis, typhlitis and disseminated intravascular coagulopathy after a short period of AZA therapy. The patient had low TPMT activity and TPMT*3C genotype. Measurement of TPMT activity and determination of TPMT variant allele may identify patients at risk for AZA-induced myelosuppression.
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PMID:Azathioprine-induced fatal myelosuppression in systemic lupus erythematosus patient carrying TPMT*3C polymorphism. 1825 Jan 37

We report a Japanese case of human herpes virus-8 (HHV-8)-associated multicentric Castleman disease(MCD) complicated by hemophagocytic syndrome(HPS). A 60-year-old man presented with persistent fever and progressive pancytopenia in June 2004. On physical examination, anemia, icterus, hepatosplenomegaly, and generalized lymphadenopathy were detected. Laboratory findings showed elevated levels of serum ferritin and soluble interleukin-2 receptor. Anti-human immunodeficiency virus (HIV) antibody was negative. Bone marrow aspiration revealed a normocellular marrow with an increased number of hemophagocytic histiocytes. Biopsy of cervical lymph node disclosed pathological features compatible with the plasmablastic variant of Castleman disease. HHV-8 DNA was detected in the specimen from lymph node by polymerase chain reaction. Thus, the diagnosis of HHV-8-associated MCD complicated by HPS was made. The patient was treated with immunotherapy and subsequent chemotherapy. However, he died of bacterial sepsis after one-month therapy. This case report provides some evidence that HHV-8 may be a causative agent of MCD even in HIV-seronegative Japanese patients.
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PMID:[A Japanese case of human herpes virus-8-associated multicentric castleman disease complicated by hemophagocytic syndrome]. 1870 66

A 65-year-old white female patient with normal baseline renal function was referred to our hospital with nonoliguric renal failure requiring hemodialysis after progressive deterioration over the previous 6 months. Her past medical history was remarkable for easy fatigability, weight loss, low-grade fever, hypogammaglobulinemia and mild hepatosplenomegaly manifested over the past 6 years. Several liver and bone marrow biopsies during that period had shown a nonspecific polyclonal T-cell infiltration, and she was administered low-dose steroids for symptomatic relief. Physical examination, laboratory workup and imaging studies at presentation showed pancytopenia, hepatosplenomegaly, large symmetric kidneys with normal cortices and no evidence of obstructive uropathy, aseptic pyuria with neutrophils and lymphocytes and mild proteinuria. On biopsy the renal interstitium was infiltrated by large, granular CD3+CD8+CD56-CD57+ lymphocytes, clonal by molecular analysis, which established the diagnosis of T-cell large granular lymphocyte leukemia. Most urinary and peripheral blood lymphocytes bore the same T-LGL surface markers and were also clonal, as shown by flow-cytometry and PCR amplification of the T-cell receptor g-chain genes. A subsequent bone marrow biopsy revealed infiltration by lymphoma cells and excluded a myelodysplastic or hemophagocytic syndrome. After exclusion of an underlying EBV, CMV, HBV, HCV or HIV infection with negative serology and blood PCR the patient received one cycle of chemotherapy with cyclophosphamide, vincristine and prednisone. No improvement of renal function was achieved, while complication with a prolonged pulmonary infection and severe sepsis precluded further treatment. Our report indicates that the T-LGL leukemia should be considered in the differential diagnosis of renal failure with large-sized kidneys, especially when hepatosplenomegaly, pancytopenia and aseptic pyuria are also present. In the latter case, flow-cytometric and clonality analysis of the urine sediment can aid in establishing a diagnosis. Since renal function may deteriorate rapidly, chemotherapy should not be delayed.
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PMID:T-cell large granular lymphocyte leukemia presenting as end-stage renal disease: the diagnostic role of flow-cytometric and clonality analysis of the urine sediment. 1920 16

Jacobsen syndrome is a MCA/MR contiguous gene syndrome caused by partial deletion of the long arm of chromosome 11. To date, over 200 cases have been reported. The prevalence has been estimated at 1/100,000 births, with a female/male ratio 2:1. The most common clinical features include pre- and postnatal physical growth retardation, psychomotor retardation, and characteristic facial dysmorphism (skull deformities, hypertelorism, ptosis, coloboma, downslanting palpebral fissures, epicanthal folds, broad nasal bridge, short nose, v-shaped mouth, small ears, low set posteriorly rotated ears). Abnormal platelet function, thrombocytopenia or pancytopenia are usually present at birth. Patients commonly have malformations of the heart, kidney, gastrointestinal tract, genitalia, central nervous system and skeleton. Ocular, hearing, immunological and hormonal problems may be also present. The deletion size ranges from approximately 7 to 20 Mb, with the proximal breakpoint within or telomeric to subband 11q23.3 and the deletion extending usually to the telomere. The deletion is de novo in 85% of reported cases, and in 15% of cases it results from an unbalanced segregation of a familial balanced translocation or from other chromosome rearrangements. In a minority of cases the breakpoint is at the FRA11B fragile site. Diagnosis is based on clinical findings (intellectual deficit, facial dysmorphic features and thrombocytopenia) and confirmed by cytogenetics analysis. Differential diagnoses include Turner and Noonan syndromes, and acquired thrombocytopenia due to sepsis. Prenatal diagnosis of 11q deletion is possible by amniocentesis or chorionic villus sampling and cytogenetic analysis. Management is multi-disciplinary and requires evaluation by general pediatrician, pediatric cardiologist, neurologist, ophthalmologist. Auditory tests, blood tests, endocrine and immunological assessment and follow-up should be offered to all patients. Cardiac malformations can be very severe and require heart surgery in the neonatal period. Newborns with Jacobsen syndrome may have difficulties in feeding and tube feeding may be necessary. Special attention should be devoted due to hematological problems. About 20% of children die during the first two years of life, most commonly related to complications from congenital heart disease, and less commonly from bleeding. For patients who survive the neonatal period and infancy, the life expectancy remains unknown.
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PMID:Jacobsen syndrome. 1926 33

Acute graft-versus-host disease following orthotopic liver transplantation is a rare but feared complication arising in 1% to 2% of cases with a dismal prognosis. It most often presents as fever, rash, and diarrhea with or without pancytopenia. Patients die from complications of marrow failure such as sepsis or bleeding. Because of its low incidence, there is no clear treatment protocol for this complication. Both increasing and withdrawing immunosuppression have been attempted with variable success. Although anti-tumor necrosis factor alpha therapy has been widely used for the treatment of steroid-resistant acute graft-versus-host disease in the hematopoietic stem cell transplant setting, there previously have been no reported cases of its use in liver transplantation. The aim of this report is to review a case of acute graft-versus-host disease and the use of etanercept to manage this complication. Etanercept has never previously been used in liver transplantation complicated by acute graft-versus-host disease. In the hematology literature, the success of its use is offset by significant rates of serious infectious (especially fungal) complications. However, preliminary results are encouraging and offer insight into its use as a potentially viable therapeutic option. We report the first successful use of etanercept in liver transplantation-associated graft-versus-host disease, albeit complicated by invasive aspergillosis, and recommend concurrent antifungal prophylaxis when the drug is used in this setting.
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PMID:Acute graft-versus-host disease after liver transplant: novel use of etanercept and the role of tumor necrosis factor alpha inhibitors. 1932 15

The aim of this paper is retrospective analysis of data from patients in whom the indication for cyclophosphamide (CF) pulse therapy was established in our department. Indications for CF pulse treatment were lupus nephritis (LN) alone or associated with central nervous system lupus. CF was administred in the dose of 500-1000 mg/m2 intravenously once monthly for the 6 months and once every 3 months thereafter. Patients were treated with adequate dose of glucocorticoids and other symptomatic therapy. The effect of applied therapy has been analysed by monitoring proteinuria, serum creatinine concentration, creatinine clearance, ESR, ANF titer and total complement hemolytic activity. Initial therapeutic procedure has been completed in 25/30 patients. The reasons for discontinuation in 5/30 patients were as follows: end-stage renal failure in spite of therapy (1), psychosis and lost of compliance (1), recurrent pancytopenia and subsequent sepsis (1), death non related to SLE (1) and failure to show at follow-up (1). Significant improvement of all control parameters was observed in the majority of patients in whom the therapy was completely conducted. 16/25 patients continued therapy for the next 18 months and in only 1/16 patients therapy was discontinued because of end-stage renal failure. In other 15/16 patients further improvement of control parameters was observed, although not so expressed as in the first 6 months. The most frequent complications were infections (16 infections were microbiologically proved and there were probably more infections). Alopecia (2), haematuria (1) and amenorrhoea (1) were also observed. Relatively low incidence of complications may be explained by careful patient monitoring. Considering that therapeutic success is defined not only by the improvement of renal function, but by stopping of further progression of renal failure, it can be concluded that intermittent CF pulse therapy showed good effect on LN in patients with clear indication.
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PMID:[Intermittent intravenous cyclophosphamide application in patients with systemic lupus erythematosus]. 1965 69

A 15-year-old boy with AML develops a fulminant candida krusei sepsis complicated by acute blindness due to enophthalmitis and subsequent bleeding during prolonged pancytopenia after induction therapy. Despite a low dose prophylaxis with oral nystatine and i. v. amphotericin B (ampho B) three times a week (0.8 mg/kg). Under an early intensified therapy with ampho B (1.5 mg/kg/d) combined with 5-flucytosin (160 mg/kg/d)the sepsis could be controlled and visual acuity slowly improved. A vitrectomy is due to the bleeding unavoidable. Despite a therapy delay of 4 weeks and omission of two cycles of the intensification treatment the patient is in continuous complete remission for longer than 10 years after diagnosis. If it is within the treatment protocols manageable to detect patients with increased sensitivity against cytostatic drugs and correspondingly highly sensitive leukemic cells, such complications could be avoided due to primary treatment adaptation.
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PMID:Long time survival after reduced chemotherapy ina 15-year-old patient with AML and Candida krusei sepsis and eye involvement. 1989 Jul 93

Graft-versus-host disease (GVHD) is a dreaded complication of bone marrow and solid organ transplantation. Commonly affected organs include skin, liver, and the gastrointestinal tract, with bone marrow and renal involvement occurring more rarely. GVHD is less commonly seen with solid organ transplants. Fewer than 100 cases of GVHD have been reported in the literature following liver transplantation. We report a case of a 53-year-old woman who required a multiorgan transplant after a complicated postoperative course following paraduodenal hernia repair. She developed isolated pancytopenia approximately 4 months after receiving an en bloc transplant involving the liver, kidney, small bowel, and pancreas. No evidence of skin, gastrointestinal, or hepatic involvement was discovered. HLA typing of the peripheral blood revealed that 28% of patient peripheral blood was composed of donor lymphocytes. Bone marrow biopsy showed a markedly hypocellular marrow with 23% donor lymphocytes and 80% of the T-cell population from the donor as well. The patient began treatment for GVHD, including high-dose steroids, basiliximab, and rituximab. Unfortunately, she developed overwhelming sepsis and subsequently died. This case describes an instance of GVHD manifested by isolated pancytopenia after en bloc transplantation of multiple solid organs. GVHD is a rare, but serious complication of solid organ transplantation that can result in death. Although isolated bone marrow involvement is uncommon, it must be considered early to avoid a delay in diagnosis. This case also highlights an association of GVHD with multiorgan transplants, although this is incompletely characterized in the current literature.
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PMID:Graft-versus-host disease presenting with pancytopenia after en bloc multiorgan transplantation: case report and literature review. 2000 17

Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of cellular blood component transfusion that produces a graft-versus-host clinical picture with concomitant bone marrow aplasia. We report the case of 2 patients with severe combined immunodeficiency (SCID) who developed TA-GvHD. Both patients had been given nonirradiated erythrocyte suspension before the diagnosis of SCID. Although one of them was aged 12 months, he had still not been diagnosed as having severe T-cell deficiency at the time of transfusion. Both patients presented similar signs and symptoms (fever, skin rash, diarrhea, pancytopenia, and icterus). Skin biopsies demonstrated Grade II GVHD involvement. In both cases, sepsis and septic shock developed, with progression to multiorgan failure. Unfortunately, the 2 patients died, despite prompt, appropriate sepsis treatment and immunomodulatory therapy. TA-GVHD must be considered in the differential diagnosis of patients who present fever, pancytopenia, diarrhea, skin rash and icterus, and the transfusion history must be questioned.
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PMID:Transfusion-associated graft-versus-host disease in severe combined immunodeficiency. 2046 70

A case of severe and irreversible pancytopenia secondary to acute primary cytomegalovirus infection in an immunocompetent woman is described. The patient presented with thrombocytopenia, lymphopenia, anemia, and abnormal liver function tests. Treatment with corticosteroids and intravenous immunoglobulin was ineffective in reconstituting hemopoiesis. The patient developed severe sepsis and eventually expired.
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PMID:Fulminant pancytopenia due to cytomegalovirus infection in an immunocompetent adult. 2056 46


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