Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026986 (myelodysplastic syndrome)
 14,926 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe ten cases of aortitis due to Salmonella that were treated at the University of Toronto-affiliated Hospitals between 1978 and 1997. Predisposing conditions included hypertension, diabetes mellitus, and myelodysplastic syndrome. Main presenting symptoms were fever and abdominal and back pain. The most frequent site involved was the abdominal aorta, followed by the thoracic aorta. All but one patient were treated with intravenous bactericidal antibiotics; seven also underwent surgery, four with axillobifemoral grafts and three with in situ grafts. Four of seven patients died within 1 month of the surgical procedure (three patients with in situ grafts and one patient with axillobifemoral graft). We also reviewed the pathogenesis, clinical and laboratory characteristics, and treatment of 140 cases of aortitis due to Salmonella reported in the literature since 1948. The use of bactericidal antibiotics, together with early surgical intervention and long-term suppressive antibiotic therapy, has led to improved survival.
 ...
PMID:Aortitis due to Salmonella: report of 10 cases and comprehensive review of the literature. 1058 4

Aortitis is the inflammation of the wall of the aorta and can occur from an infection or autoimmune disease. Myelodysplastic syndrome (MDS) is characterised by abnormal haematopoiesis and a dysfunctional immune system. Autoimmune manifestations have been described in MDS. Here a case of a patient with aortitis and MDS is presented and discussed. All possible aetiologies were ruled out. The patient's symptoms resolved after she received steroids.
 ...
PMID:Aortitis as a manifestation of myelodysplastic syndrome. 1116 Oct 81

Myelodysplastic syndrome (MDS) is frequently associated with autoimmune diseases such as polymyalgia, arthritis, and rarely, with systemic vasculitis. The pathogenesis of these autoimmune complications remains unknown, but there is increasing evidence of profound immune dysregulation in MDS. In the few cases reported so far, vasculitides associated with MDS affected mainly cutaneous vessels. Here we describe two cases of acute large-vessel vasculitis in association with MDS. The first patient is a 67-yr-old male presenting with a massive large-vessel arteritis as primary manifestation of refractory anemia with excess of blasts type 1 (RAEB-1). The second patient is a 60-yr-old male, who presented with acute thoracic aortitis after a 2-yr history of refractory anemia with ringed sideroblasts (RARS). Both patients received immunosuppressive treatment with steroids, leading to rapid improvement of systemic inflammatory symptoms, vessel wall injury and peripheral blood counts. Whereas the first patient displayed sustained favorable hematologic responses under long-term steroid therapy, there was a rapid transformation into secondary acute myeloid leukemia in the second patient. We conclude that large-vessel vasculitis should be added to the list of potential autoimmune complications in MDS. In this clinical setting, steroid therapy may alleviate inflammatory symptoms and result in beneficial hematologic responses.
 ...
PMID:Large-vessel arteritis and myelodysplastic syndrome: report of two cases. 1524 12

Several paraneoplastic inflammatory conditions, particularly autoimmune diseases, have been described in association with myelodysplastic syndromes (MDS). However, to date, recurrent acute pancreatitis has never been described in association with MDS. A 44-year-old man presented with prolonged fever and fatigue. Aortitis and pericarditis were diagnosed simultaneously with MDS, refractory anemia with excess blast type 2. His erythrocyte sedimentation rate and c-reactive protein were markedly elevated. The vasculitic syndrome responded rapidly to corticosteroids, but soon after tapering of corticosteroids, acute pancreatitis developed. Pain and pancreatic enzymes, however, improved rapidly with escalation of corticosteroid dosage. Multiple attempts at discontinuing the drug resulted in symptomatic flare-ups. Finally, his MDS transformed into acute myeloid leukemia (AML); severe acute pancreatitis closely accompanied. Induction chemotherapy and high-dose corticosteroids, however, controlled both conditions. A subsequent pancreatitis attack with pseudocyst formation occurred, but again was controlled with corticosteroids, although this was followed closely by another relapse of AML. All etiologies for recurrent acute pancreatitis were ruled out. The dramatic response of his pancreatitis attacks to immunosuppression suggested its autoimmune origin, while the close relationship in both the timing and severity of acute pancreatitis and MDS/AML suggested that the autoimmune pancreatitis was a paraneoplastic phenomenon related to MDS.
 ...
PMID:Recurrent steroid-responsive pancreatitis associated with myelodysplastic syndrome and transformations. 1562 95

Pyoderma gangrenosum (PG) is a chronic inflammatory disease of unknown cause that presents as an inflammatory and ulcerative disorder of the skin. PG is often associated with an underlying systemic disease. However, the frequencies of the underlying diseases are unclear in Japanese patients. In this retrospective, observational study, all patients diagnosed with PG who visited dermatology departments of nine regional hospitals in and around Ibaraki Prefecture were collected from 1982 to 2011 or 2014. The diagnoses of PG were based on the characteristic clinical and histological appearances and ruling out of infection. Sixty-two PG patients, including 29 males and 33 females, were identified. The ages of onset were 16-89 years, and the mean age was 50.2 years. Fifty (80%) of the 62 patients presented with an ulcerative PG, and the lower leg was the most common site (74%). Forty-six (74%) PG patients had underlying diseases. The most frequent was ulcerative colitis (32%), followed by myelodysplastic syndrome (11%), rheumatoid arthritis (6%) and aortitis syndrome (5%). For treatment, 54 cases (87%) received systemic corticosteroids and 10 received additional treatment with cyclosporin. There was no significant correlation between underlying diseases and response to the initial treatment. Multivariate analysis revealed that the number of affected sites negatively correlated with successful initial treatment. Fifteen (24%) of the 62 cases relapsed. In conclusion, ulcerative colitis and hematological disorders were frequently associated with PG while approximately a quarter of the cases were idiopathic.
 ...
PMID:Pyoderma gangrenosum and underlying diseases in Japanese patients: A regional long-term study. 2863 56