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

A case of myelodysplastic syndrome (MDS) who suffered alternating proptosis was reported. A 57-year-old Japanese man with MDS developed acute painful left proptosis with eyelid edema, conjunctival injection, visual disturbance, and restriction of eye movements. This lasted for 3 weeks. Computerized tomography (CT) showed swelling of the lateral and medial rectus muscles and the optic nerve, as well as a retrobulbar mass. Later, a similar painful right proptosis with severe conjunctival chemosis developed. CT showed only bilateral mild optic nerve swelling. The visual acuity of the right eye decreased to null even after remission of the proptosis. The patient died due to myocardial infarction and underwent autopsy. The right optic nerve stroma was found to be increased over that of the left. Cellular infiltration was noted in the left optic nerve and the retrobulbar connective tissue. This self-limiting and relapsing proptosis was apparently related to MDS. This is only the second MDS case with such severe ophthalmological symptoms reported in the literature.
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PMID:Exophthalmos in myelodysplastic syndrome. 187 49

A 57-year-old man presented with proptosis of the right eye and findings consistent with bilateral angle-closure glaucoma. Subsequent evaluation revealed severe bilateral uveal effusions and associated nonrhegmatogenous retinal detachments, which were felt to be related to orbital pseudotumour and associated scleritis. Hematologic studies were consistent with a diagnosis of myelodysplastic syndrome. Although myelodysplastic syndrome has been reported in one patient with orbital inflammation and myositis, to our knowledge ciliochoroidal effusion and secondary angle closure have not previously been reported in myelodysplastic syndrome.
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PMID:Angle-closure glaucoma as initial presentation of myelodysplastic syndrome. 224 68

The clinical and hematological characteristics of ten children with myelodysplastic syndromes diagnosed and followed up over a 3 year period are presented. All of them had anemia and a low platelet count whilst the white blood cell count was variable. Presentation with bilateral proptosis and acute febrile neutrophilic dermatosis (Sweet's syndrome) were unique features observed in one case each. None of these cases could afford specific therapy and thus serve to illustrate the natural history of the disease in pediatric practice.
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PMID:Clinico-hematological profile and natural history of childhood myelodysplastic syndromes. 826 94

We analysed retrospectively our clinical experience with 36 cases of mucormycosis. They were seen during the last 15 years. The diagnosis suspected on clinical grounds, was confirmed in 31 cases by finding the hyphae in hematoxylin-eosin stained material obtained from aspirated or tissue biopsy or by isolation of the fungus in culture. Rhinocerebral mucormycosis was diagnosed in 22 patients. Diabetes was the underlying disorder in 20 cases, kidney failure in one and myelodysplastic syndrome in one. Nine had stable and 11 unstable diabetes (ketoacidosis in 10 and hyperosmolar coma in 1). The earliest sign was facial edema, followed by proptosis, chemosis and extraocular muscle paresis. They were treated by extensive surgical debridement, insulin and antifungal drugs with 69% of survival rate. The disseminated mucormycosis was diagnosed at the autopsy in 5 cases, acute leukemia was the underlying disease in 2 of them. Pulmonary mucormycosis was diagnosed in 2 cases, cutaneous form in 2, sinuorbital form in 4 and brain abscess in one patient. Eight of these 9 cases survived after therapy. We emphasize the importance of an early diagnosis. This can only be made in the presence of a typical clinical setting confirmed by finding the hyphae in tissue or culture. Antifungal drugs along with treatment of the underlying disorder and aggressive surgical debridement must follow.
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PMID:Rhinocerebral and systemic mucormycosis. Clinical experience with 36 cases. 898 Dec 94

Myelodysplastic syndrome (MDS) is a disease of elderly patients but, rarely can be encountered in pediatric age group. The present case of MDS (RAEB subtype) was reported in a child who presented with proptosis and unique and rare feature of haemophagocytosis by myeloid series of cells.
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PMID:Refractory anaemia with excess blasts (RAEB) in a child--an unusual clinicohaematological presentation--a case report. 1202 39

PURPOSE. To report an elderly patient with a primary myelodysplastic syndrome (MDS) type refractory anemia with excess of blasts (RAEB) who developed an orbital granulocytic sarcoma (GS) as a harbinger of an acute myelogenous leukemia (AML). METHODS. A 77-year-old man was diagnosed as having a MDS type RAEB. Eight months later he developed a progressive painless proptosis in his left orbit. A computed tomography (CT) scan revealed a large irregular mass involving the orbit. It showed heterogeneous soft tissue density and no osseous cortical destruction was observed. A diagnostic lateral orbitotomy with an excisional biopsy were performed. RESULTS. Histopathology showed sheets of immature granulocytic cells. Immunohistochemical staining was positive for markers for myeloperoxidase, which supported the diagnosis of GS. CONCLUSIONS. Reports of a GS complicating the course of a MDS are few. Sites of detection of the extramedullary tumors (EMT) in MDS vary, although cutaneous sites predominate. An orbital site is a very rare localization of this tumor in adults. The case we report is the first one with an orbital granulocytic sarcoma in an MDS-type RAEB in an elderly patient.
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PMID:Orbital granulocytic sarcoma in a myelodysplastic syndrome. 1204 74

Granulocytic sarcoma is a rare orbital complication of acute leukemia. It concerns primarily children under 10 years of age suffering from primitive acute myeloid leukemia. As this type of symptom can sometimes affect the elderly during the acute phase of myelodysplastic syndrome, it raises a problem with diagnosis and consequently with therapeutic treatments. We report the case of a 77-year-old female patient who had acute inflammatory proptosis during regressive right ethmoiditis. The diagnosis was reached by computed tomography showing an extraconical intraorbital tumor sprouting from the sinus, and by clinical examination and confirmed by the hematological investigation. Acute proptosis developing in an acute myeloblastic leukemia context heavily swayed diagnosis towards orbital granulocytic sarcoma. Chemotherapy by cytarabine and hydroxycarbamide associated with intravenous corticoid therapy resulted in complete disappearance of the proptosis within 10 days. This case report is a reminder of this peculiar sign of tumoral syndrome in acute myeloid leukemia. We also discuss different diagnostic methods and various therapeutic approaches, and analyze the disease course and patient follow-up.
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PMID:[Orbital granulocytic sarcoma revealing acute myeloblastic syndrome: a case study]. 1502 50

Thiazolidinediones (TZDs) are frequently used pharmacotherapeutics for type II diabetes mellitus, which exert their effect through peroxisomal proliferator agonist receptor (PPAR) mediated increased insulin sensitivity. TZDs are known to cause marrow suppression and to stimulate adipogenesis. Case and cohort studies show TZDs worsen thyroid-associated orbitopathy. We present a case consistent with earlier reports of marrow suppressive pancytopenia manifesting as myelodysplastic syndrome, a new implication of hypoerythropoetinemia, and non-Graves'-associated proliferative proptosis.
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PMID:Marrow suppression with myelodysplastic features, hypoerythropoetinemia, and lipotrophic proptosis due to rosiglitazone. 2013 89

Invasive aspergillosis originating from the paranasal sinuses is a rare disease that is associated with high mortality rates. We report on a 77-year-old patient with myelodysplastic syndrome and progressive left-sided proptosis persisting for 5 days. Antibiotic therapy and endonasal orbital decompression yielded insufficient improvement. Only upon application of complementary antifungal therapy was remission observed. Rapid diagnosis and treatment are essential in order to prevent life-threatening complications.
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PMID:[Invasive orbital aspergillosis: Rare but important differential diagnosis in the case of immunosuppression]. 2527 Aug 34