Cases reported "Disease Susceptibility"

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1/25. Two separate episodes of hemophagocytic syndrome at a two-year interval in an apparently immunocompetent male.

    We describe two separate episodes of hemophagocytic syndrome (HPS) at an interval of two years in a seemingly immunocompetent male. This case suggests the possible existence of an inherent predisposition to HPS, in which otherwise negligible self-limited viral infection may trigger HPS. Laboratory data for a 16-year-old boy admitted with persistent high grade fever and severe thrombocytopenia disclosed coagulation abnormality, liver damage, and hypercytokinemia. A bone marrow aspiration revealed a proliferation of histiocytes with fresh hemophagocytosis. We diagnosed that he was suffering from HPS. Responding to steroid pulse therapy, he recovered completely and was discharged. After two years of healthy life, he became febrile again and was readmitted. The fever was refractory to antibiotics and was associated with a sudden drop in platelet count. Laboratory data and the bone marrow picture were consistent with those of HPS. He was again successfully treated with steroid. After the second episode, he has been healthy for more than two years.
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ranking = 1
keywords = bone
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2/25. Treatment of dyskeratosis congenita with granulocyte-macrophage colony-stimulating factor and erythropoietin.

    dyskeratosis congenita (DC) is a rare inherited disorder characterized by reticulate skin pigmentation, nail dystrophy, mucosal leucoplakia, and bone marrow failure. pancytopenia is difficult to manage in patients with this disorder. We describe a 13-month-old-boy who presented with reticulate skin lesions, paleness, and hepatosplenomegaly. anemia and leukopenia developed by the age of 43 months. The patient was treated with granulocyte-macrophage colony-stimulating factor (GM-CSF) (5 microg/kg/d, subcutaneously) for 19 months and erythropoietin (150 U/kg 3 days in a week, subcutaneously) for 8 months, with excellent neutrophil and hemoglobin response. Recurrent infections were not developed after starting GM-CSF, and packed red blood cell transfusion was not given to the patient after starting erythropoietin. GM-CSF combined with erythropoietin may be used in the treatment of bone marrow failure in patients with DC without an HLA-identical donor.
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ranking = 134.32194986599
keywords = macrophage, bone
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3/25. arthritis associated with tuberculosis.

    There has been a resurgence in tuberculosis (TB) worldwide. Approximately 2 billion people have latent infection, 8 million would develop active TB annually, and 2-3 million would die due to TB. With this resurgence, cases with extrapulmonary TB have also shown an increase. Approximately 10-11% of extrapulmonary TB involves joints and bones, which is approximately 1-3% of all TB cases. The global prevalence of latent joint and bone TB is approximately 19-38 million.TB arthritis most commonly manifests as a monoarthritis of weight-bearing joints in the hip or the knee. Oligo- or polyarticular presentation is not rare and may cause diagnostic confusion with inflammatory arthritis. Owing to the low incidence in developed countries, the diagnosis of joint and bone TB is often delayed. A high degree of sensitivity to this diagnosis would prevent delays, permitting prompt institution of anti-TB therapy and preventing irreversible joint damage. Despite advances, confirmation of diagnosis still relies on lengthy microbiological techniques or invasive biopsy. Due to the frequency of isoniazid resistance, initial treatment at present typically includes a combination of four drugs: isoniazid, rifampicin, pyrazinamide and streptomycin or ethambutol. Antimicrobial therapy should be of at least 9 months duration, longer in children and immunocompromised hosts. Surgical procedures should be restricted to joints with severe cartilage destruction, large abscesses, joint deformity, multiple drug resistance or atypical mycobacteria.
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ranking = 1.5
keywords = bone
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4/25. Acute haematogenous anaerobic osteomyelitis in sickle cell disease. A case report and review of the literature.

    patients with sickle cell disease are more susceptible to acute anaerobic osteomyelitis due to focal gut mucosal ischaemia, translocation of bacteria, and seeding in infarcted bone marrow. Modulation of the immune system is also present. The isolation of anaerobic organisms requires a high index of suspicion, correct specimen collection procedures and meticulous specimen handling. bacteroides is the predominant organism isolated. Intra-osseous gas in the bone may be seen within four days and radiographs are therefore useful earlier than with aerobic osteomyelitis. Surgical debridement and intravenous antibiotics are the mainstay of treatment with the erythrocyte sedimentation rate being relied on heavily to guide conversion to oral antibiotics. Coexistence of septic arthritis is more common with anaerobic osteomyelitis.
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keywords = bone
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5/25. plasmodium falciparum causing hemophagocytic syndrome after allogeneic blood stem cell transplantation.

    We describe a case of plasmodium falciparum infection in a 25-year-old male patient with a myelodysplastic syndrome, who underwent allogeneic peripheral blood stem cell transplantation (PBSCT) in September 2003. Conditioning regimen consisted of total body irradiation (10 Gy) and cyclophosphamide 60 mg/kg for 2 days. A dose of 4 x 10(6) CD34 cells/kg was transfused. Engraftment was well documented on day 17 post-transplantation. Spiking fevers occurred on days 19 and 21, associated with a pancytopenia, hepatosplenomegaly and neurological signs. P. falciparum parasites were found on the peripheral blood smear (parasitemia = 23%). Marrow aspiration showed P. falciparum parasites and proliferation of mature histiocytes with hemophagocytosis. quinine 10 mg/kg i.v. three times a day for 10 consecutive days was given. The fever subsided within 3 days, and pancytopenia vanished in 14 days. parasitemia cleared in 6 days. The patient left the unit on day 46 with no further complications. The screening of donors showed that infection was acquired from two blood units (from a single donor) given 5 days before transplantation. We report the first case of profound hemophagocytosis in immunosuppressed patient with malaria of high parasitemia after a bone marrow transplant.
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ranking = 0.5
keywords = bone
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6/25. SBDS mutations and isochromosome 7q in a patient with Shwachman-diamond syndrome: no predisposition to malignant transformation?

    Shwachman-diamond syndrome (SDS) is a genetic disorder characterized by pancreatic hypoplasia, recurrent infection, and bone marrow (BM) dysfunction. SDS-patients have an increased frequency of myelodysplasia and leukemic transformation. Unspecific cytogenetic aberrations are a common finding in SDS. However, in a rising number of patients abnormalities of chromosome 7 have been reported, especially an i(7)(q10), which seems to be a non-random chromosome abnormality. Recently, the SDS gene has been mapped at locus 7q11 and subsequently cloned; recurrent mutations have been found. We report a case of SDS with an i(7)(q10) in the BM and two different mutations in the SBDS gene. At the age of 25 years, the patient suffers from mild aplastic anemia but does not show any clinical sign of myelodysplasia or leukemic transformation.
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ranking = 0.5
keywords = bone
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7/25. Acute polymyositis after donor lymphocyte infusion.

    polymyositis usually occurred along with other manifestations of chronic graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (HSCT) had been reported. However, polymyositis with a sole manifestation of acute GVHD following donor lymphocyte infusion (DLI) is rare. We reported a 45-yr-old man of acute lymphoid leukemia post-allogeneic HSCT 6 months developed acute polymyositis after DLI. He did not develop any symptoms, signs of acute or chronic GVHD following allogeneic HSCT, despite withdraw of immuosuppresive agents, cyclosporin A (CsA). As the dna-STR of bone marrow analysis showed mixed chimerism, he received the DLI for remission on May 16, 2002. Acute polymyositis developed following DLI 22 d later. The clinical presentation of polymyositis is compatible with a manifestation of acute GVHD following DLI. It also responds to the treatment of steroid and CsA.
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ranking = 0.5
keywords = bone
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8/25. Acquired predisposition to mycobacterial disease due to autoantibodies to IFN-gamma.

    Genetic defects in the IFN-gamma response pathway cause unique susceptibility to intracellular pathogens, particularly mycobacteria, but are rare and do not explain mycobacterial disease in the majority of affected patients. We postulated that acquired defects in macrophage activation by IFN-gamma may cause a similar immunological phenotype and thus explain the occurrence of disseminated intracellular infections in some patients without identifiable immune deficiency. macrophage activation in response to IFN-gamma and IFN-gamma production were studied in whole blood and PBMCs of 3 patients with severe, unexplained nontuberculous mycobacterial infection. In all 3 patients, IFN-gamma was undetectable following mitogen stimulation of whole blood, but significant quantities were detectable in the supernatants of PBMCs when stimulated in the absence of the patients' own plasma. The patients' plasma inhibited the ability of IFN-gamma to increase production of TNF-alpha by both autologous and normal donor PBMCs, and recovery of exogenous IFN-gamma from the patients' plasma was greatly reduced. Using affinity chromatography, surface-enhanced laser desorption/ionization mass spectrometry, and sequencing, we isolated an IFN-gamma-neutralizing factor from the patients' plasma and showed it to be an autoantibody against IFN-gamma. The purified anti-IFN-gamma antibody was shown to be functional first in blocking the upregulation of TNF-alpha production in response to endotoxin; second in blocking induction of IFN-gamma-inducible genes (according to results of high-density cDNA microarrays); and third in inhibiting upregulation of HLA class II expression on PBMCs. Acquired defects in the IFN-gamma pathway may explain unusual susceptibility to intracellular pathogens in other patients without underlying, genetically determined immunological defects.
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ranking = 26.664389973198
keywords = macrophage
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9/25. Virus-associated hemophagocytic syndrome following bone marrow transplantation.

    Multiple-organ infiltration by mature, benign erythrophagocytic histiocytes is the pathologic hallmark of the virus-associated hemophagocytic syndrome (VAHS). Although VAHS has been described in a number of clinical settings, it has been reported following bone marrow transplantation (BMT) only once previously. Our report identifies the clinical and laboratory features associated with VAHS and compares the immune defects described in VAHS with those known to occur following BMT.
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ranking = 2.5
keywords = bone
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10/25. Topical voriconazole solution for cutaneous aspergillosis in a pediatric patient after bone marrow transplant.

    Invasive aspergillosis seems to be on the rise, especially in immunocompromised children. Historically, only systemic amphotericin b has been effective against aspergillus. Development of newer antifungal agents, such as voriconazole and caspofungin, has improved the treatment options available for aspergillosis, although no definitive management strategy has been established. Here we describe the use of topical voriconazole combined with systemic antifungal agents for cutaneous aspergillosis in a pediatric patient after bone marrow transplant.
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keywords = bone
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