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)

Microscopic and medical review of twenty-six patients with skin biopsy specimens that showed granulomatous vasculitis demonstrated vascular histiocytic granulomas with fibrinoid destruction of blood vessels in the dermis and panniculus. Cultures of the biopsy specimens were nonspecific. The skin lesions varied from erythema to papulonodular and vesicular eruptions; they were usually on the extremities but also involved the trunk. Eight patients had systemic lymphoproliferative diseases: three, lymphoma; two, angioimmunoblastic lymphadenopathy; two, preleukemia; and one, chronic granulocytic leukemia. Five of these eight patients died within 2 years after the onset of skin lesions. The four patients with systemic vasculitis died within 1 year after the onset of skin lesions. Five patients with arthritis, four with gastrointestinal disease, three with systemic sarcoidosis or sarcoidlike disease, and one with tuberculosis had a more favorable prognosis. The histologic pattern of cutaneous nonlymphomatoid granulomatous vasculitis is associated with significant systemic disease, especially lymphoproliferative disorders. Patients with lymphoproliferative disorders or systemic vasculitis have a much poorer prognosis than those with inflammatory or infectious granulomatous disease.
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PMID:Cutaneous granulomatous vasculitis: its relationship to systemic disease. 395 62

Pyoderma gangrenosum is a rare occurrence in patients with haematological malignancy. This characteristic but nonspecific inflammatory process with skin destruction occurred in 4 patients with myelodysplasia, in one with acute leukaemic transformation of myelofibrosis, and in de novo acute myeloblastic leukaemia in another. Clinically, the cutaneous lesion in these patients differed from that associated with inflammatory bowel disease, arthritis, or the idiopathic type of pyoderma gangrenosum by having the vesiculo-bullous borders. Histopathological differences were also evident since more superficial layers of the skin were involved in the ulceration than typically encountered in patients with non-malignant systemic disease. Despite the less penetrating nature of this variant, treatment of the pyoderma gangrenosum is unsatisfactory and in the absence of effective therapy for the underlying disease, healing occurred only in the patient with acute leukaemia who achieved complete remission in response to chemotherapy.
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PMID:Pyoderma gangrenosum in myelodysplasia and acute leukaemia. 403 58

Myelosarcoma (chloroma) is a rare primary condition in patients with either a myelodysplastic syndrome, or an acute or chronic leukemia. It is an extramedullary neoplasm which does not commonly present with changes in peripheral blood or bone marrow. The rarity and histomorphological similarity to malignant non-Hodgkin lymphoma renders the diagnosis notoriously difficult. Due to its coincidental or secondary manifestation followed by myelogenous leukemia, this tumor needs to be seen as a primary systemic disease. We present a 40 year old man with myelosarcoma of the jejunum and discuss this entity and its therapeutic options.
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PMID:[Myelosarcoma of the proximal jejunum. A rare primary condition of acute myelogenous leukemia]. 1243 65

Granulocytic sarcoma (chloroma) is a rare solid tumor resulting from the proliferation of myelogenous leukemia cells. Chloromas usually present as soft tissue or bony masses of the head and neck in patients with acute myelogenous leukemia (AML) of the French-American-British M2 subtype. Occasionally chloromas may occur in patients with myelodysplasia and other myeloproliferative disorders and rarely precede the development of systemic disease. It is distinctly rare for such tumors to cause epidural compression as a first manifestation of disease. Herein, we report the case of a man with a thoracic extradural chloroma whose presentation of progressive lumbar pain ultimately led to the diagnosis of M2 AML. Surgical intervention prior to the onset of paraplegia and the prompt initiation of chemotherapy resulted in an excellent neurological and hematological outcome. We also review the literature of previously reported cases of spinal cord-associated chloroma and focus on the clinical presentation and treatment of this disorder.
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PMID:Granulocytic sarcoma: an unusual complication of aleukemic myeloid leukemia causing spinal cord compression. A case report and literature review. 1469 30

Isolated central nervous system (CNS) angiitis are vasculitides of undetermined etiology in which only nervous system vessels are affected. In most cases there is no associated systemic disease. We report the case of a 67 years old man with previous hematologic diagnosis of myelodysplastic syndrome who developed an isolated CNS angiitis.
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PMID:[Isolated central nervous system angiitis and myelodysplastic syndrome: case report]. 1527 64

We report a case of diffuse plane normolipemic xanthomatosis (DPNX) which showed poorly demarcated, uncommon, yellow macules symmetrically distributed on the nape, axillae and inguinal folds accompanied by severe, persistent itching. Histopathological and ultrastructural studies of skin biopsy specimens revealed the existence of some foamy cells and the deposition of neutral fat in the upper papillary dermis. Laboratory investigations and bone marrow aspirate smears showed that our patient had myelodysplastic syndrome (MDS) associated with pancytopenia and monoclonal gammopathy of undetermined significance. Because our patient had neither a malignant hematological disorder nor a severe systemic disease, monoclonal gammopathy might explain the pathogenesis of DPNX in the present case.
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PMID:A case of diffuse plane normolipemic xanthomatosis associated with pancytopenia and monoclonal gammopathy. 1646 89

Neutrophilic panniculitis is rare and is classified as a panniculitic member of the neutrophilic dermatoses spectrum. In affected patients, an underlying systemic disease, such as myelodysplasia, is often present. We describe an infant with juvenile rheumatoid arthritis who developed neutrophilic panniculitis. Neutrophilic panniculitis clinically mimics other panniculitides and biopsy specimen can be diagnostic. Identification of this entity can aid the diagnosis of the underlying systemic process.
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PMID:Neutrophilic panniculitis in infancy: a cutaneous manifestation of juvenile rheumatoid arthritis. 1793 28

We report the case of a 10-month-old girl who presented with a spontaneous ulcer on the left buttock which failed to heal despite antibiotic therapy. Histology showed changes consistent with pyoderma gangrenosum and the ulcer resolved rapidly with super-potent topical steroids under occlusion. Blood tests revealed a persistent neutropenia. Immunoglobulin G (IgG) antineutrophil antibodies were detected in the serum, directed against human neutrophil antigen (HNA)-1a. Bone marrow studies showed normocellular marrow with no evidence of dysplasia. T and B cell subsets and karyotype analysis were normal. Autoimmune neutropenia is an uncommon self-limiting condition in young children. Pyoderma gangrenosum is rare in infants, although the buttocks are a common site of involvement in this age group. Pyoderma gangrenosum in infancy can be associated with systemic disease as in adults, particularly myelodysplasia and leukemia, arthritis and inflammatory bowel disease. However, the association of pyoderma gangrenosum and autoimmune neutropenia of infancy has not previously been reported.
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PMID:Pyoderma gangrenosum in association with autoimmune neutropenia of infancy. 1906 67

Sweet's syndrome or acute neutrophilic febrile dermatosis is a systemic disease of unknown etiology characterized by the appearance of skin lesions produced by a neutrophilic dermal infiltrate, fever and peripheral leukocytosis. It may be associated with hematologic diseases, including leukemia, with immune diseases as rheumatoid arthritis, or can occur in isolation. The myelodysplasias are hematological disorders characterized by one or more cytopenias secondary to bone marrow dysfunction. We present the case of a patient with Sweet's syndrome associated with myelodysplastic syndrome and treated with glucocorticoids who did not present a good clinical outcome. We discuss the different treatment of these diseases because in most cases glucocorticoids, which are the treatment of choice in Sweet's syndrome, may be insufficient.
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PMID:Sweet syndrome associated with myelodysplastic syndrome: report of a case. Review of the literature. 2274 28

The new onset of pancytopenia often creates a diagnostic dilemma to the treating physician and leads to bone marrow biopsy and aspiration. To determine the distribution of bone marrow findings in such cases of new-onset pancytopenia in a tertiary academic medical center, we evaluated 250 recent bone marrow aspirates and biopsies performed in the setting of new-onset pancytopenia in patients without previously diagnosed hematologic neoplastic disease. Of the 250 bone marrow studies, 193 were performed in adults and 57 were performed in children. In children, the most prevalent bone marrow finding was B-lymphoblastic leukemia, followed by nonspecific changes attributed clinically to a variety of factors including multifactorial, autoimmune, inflammatory, and infectious etiologies. In adults, hematologic neoplastic causes of pancytopenia were the most prevalent diagnoses, with the cases divided mostly between acute myeloid leukemia and myelodysplastic syndrome, with fewer numbers of cases of acute lymphoblastic leukemia, myeloproliferative neoplasms, and lymphomas. Many bone marrow findings demonstrated nonspecific changes that were attributed clinically to a variety of etiologies such as myelodysplastic syndrome, multifactorial causes, hypersplenism, drugs, and systemic disease. Overall, in both the pediatric and the adult population, new-onset pancytopenia was most commonly associated with neoplasia, although the neoplasm differed by age group. Although in most cases, a definitive diagnosis could be made based solely on bone marrow aspirate and biopsy interpretation, a significant fraction of cases in both children and adults demonstrated nonspecific marrow findings that required clinical follow-up and/or repeat biopsy for definitive diagnosis.
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PMID:Bone marrow evaluation in new-onset pancytopenia. 2485 52


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