Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 46-year-old man diagnosed as refractory anemia was hospitalized because of high fever and extensive erythema with ulceration in the femoral region. His peripheral blood examination showed marked leukocytosis (WBC 31,500/microliter:neutrophilic 90%) and anemia (Hb 8.6 g/dl. In spite of administration of antibiotics, the cutaneous ulcer rapidly extended to the right thigh and became necrotic. The bacterial culture of the cutaneous lesion showed no growth and a skin biopsy showed infiltration of neutrophils in the dermis. He became afebrile and his cutaneous lesion improved after administration of corticosteroid. When the dose of corticosteroid was decreased, cutaneous erythema and nodules appeared at other sites repeatedly, and disappeared after the dose of corticosteroid was increased. The cutaneous lesions had characteristics of both Sweet's syndrome and pyoderma gangrenosum. Moreover, the patient had immunological abnormalities and decreased neutrophilic functions (chemotaxis and O2- generation). Thus, it was suggested that the cutaneous lesions of this patient could be diagnosed as "neutrophilic dermatosis of MDS", and corticosteroid was recognized to be very effective in treating these skin lesions.
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PMID:[Neutrophilic dermatosis in a patient with refractory anemia]. 163 24

A 76-year-old male admitted to Surugadai Nihon University hospital complaining of general fatigue, slight fever and anorexia. The laboratory examination revealed anemia and an appearance of a few myeloblasts and 7% of monocytes in the peripheral blood. The nucleated cell count was 2 x 10(4)/microliters with 43% myeloblasts in the bone marrow aspirate. He was diagnosed as acute myelomonocytic leukemia. He did not receive any chemotherapy for leukemia because of his old age and smoldering disease. Pyoderma gangrenosum developed in the left submandibular and axillary regions about 6 months later. Three more month later, significant increase of myeloblast was recognized in the peripheral blood and the bone marrow. It has been reported that pyoderma gangrenosum precedes a remarkable increase of leukemic cells in the patients with acute leukemia in complete remission and with myelodysplastic syndrome. In our case, to, the same process was strongly suggested.
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PMID:[Smoldering leukemia with pyoderma gangrenosum]. 225 57

Pyoderma gangrenosum (PG) is a rare condition remarkable for its association with particular diseases, notably haemopathies and gastrointestinal diseases. As regards haemopathies, the associations most frequently encountered are with myeloid malignancies and monoclonal dysglobulinaemia. The association of PG with mainly inflammatory digestive tract diseases is also classical. The lack of publications concerning gastric atrophy and the dual haematological and gastric pathology which characterizes our case have prompted us to report it. A 60-year-old woman without significant history was admitted for PG on both knees, following vesiculo-bullous lesions. Laboratory examinations detected a normochromic anaemia tending to be macrocytic, a marked inflammatory syndrome and a monoclonal lambda light chain IgA peak at protein immunoelectrophoresis. Bone marrow biopsy, skeletal radiography and a search for Bence-Jones proteinuria were normal or negative. Colonoscopy showed no abnormality, but fibroscopy of the upper digestive tract revealed a severe gastric atrophy en plaques. Serum vitamin B12 level was moderately low, but there was no other sign of pernicious anaemia. After one month treatment with systemic corticosteroids, healing was obtained under replacement vitamin therapy. PG recurred a few months later; serum vitamin B12 level was normal, and the lesions healed after systemic corticosteroid treatment. In non-myelomatous dysglobulinaemia IgA is frequently found and there is no light chain predominance. PG often precedes dysglobulinaemia. Evolution towards a true myeloma seems to be exceptional. In a recent publication, 17 cases of association between PG and myeloma were mentioned, the IgA type being most common. Protein electrophoresis is indispensable in patients with PG. Five cases of congenital hypogammaglobulinaemia have been recorded, including three with IgA deficiency.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Pyoderma gangrenosum and IgA gammopathy. Association with atrophic gastritis]. 251 19

A new case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with a malignant hemopathy is presented. The blood disease was a chronic myelomonocytic dysmyelopoiesis which was discovered during the eruption and resulted in the patient's death within a few months, probably through acutization. The skin lesions were atypical, bullous and ulcerated. On this occasion, the international literature concerning all cases of Sweet's syndrome associated with malignant or premalignant hemopathies is reviewed. Several concepts emerge from this study: the association is frequent (about 20 p. 100 of all published cases of Sweet's syndrome); there is a strong predominance of granulocytic hemopathies over lymphoplasmocytic and monocytic hemopathies; the blood disease is revealed by the skin eruption in some 50 p. 100 of the patients; there are frequent chronological relations between Sweet's syndrome and the events that occur in the course of the hemopathy; finally, the association is usually of poor prognosis. A comparison with Sweet's syndrome unassociated with a blood disease showed only three significant points: the frequency of bullous lesions, of the initial anaemia (the most important element) and of extreme figures in leucocyte counts (leucopenia or major hyperleukocytosis). The atypical character of the skin lesions in the patient presented here incites to discuss the nosological relationship between Sweet's syndrome and bullous pyoderma, an entity closely associated with hemopathies. It has recently been suggested by several authors that this anatomico-clinical kinship should be turned into a wide spectrum of acute neutrophilic dermatoses, with typical Sweet's syndrome at one end and Pyoderma gangrenosum at the other end. The interface between this spectrum and haemopathies seems to be maximum at its intermediate stage: the bullous and superficially ulcerated lesions. The aetiology and pathogenesis of this new nosological entity are uncertain. The presence of chemoattractants or of polymorphonuclear cell abnormalities is still open to discussion. The relationship between the entity and leukocytoclastic vasculitis has recently been questioned.
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PMID:[Acute febrile neutrophilic dermatosis and malignant hematologic diseases: report of a new bullous case and review of the literature]. 305 44

Atypical bullous pyoderma gangrenosum was diagnosed during the course of a myeloid malignancy in three patients. One patient had chronic myeloid leukaemia, one acute myeloid leukaemia, and the third, refractory anaemia with excess of blasts. This atypical form of pyoderma gangrenosum has been specifically associated with myeloid malignancies. The atypical appearance of the skin lesions and the clinical context in which they arose caused the true diagnosis to be delayed in all cases. Treatment with steroids was associated with rapid healing of the skin lesion. The histopathological changes in the skin biopsy specimens from these cases were non-specific, and although the histology was considered to be atypical of pyoderma gangrenosum in one case, the unusual features could be attributed to the patient's neutropenia. (Skin biopsy was performed to exclude other specific pathology). Atypical bullous pyoderma gangrenosum is an uncommon association of the myeloid malignancies. It may remain unrecognised and should be considered more often.
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PMID:Atypical bullous pyoderma gangrenosum associated with myeloid malignancies. 347 88

The authors describe three cases of refractory anaemia with an excess of myeloblasts in the bone marrow (RAEM), associated with pyoderma gangrenosum (PG) and vasculitis. The first patient was an 85-year old man whose RAEM had begun in 1979. In 1985, he developed pyoderma gangrenosum in the popliteal fossa and on the right heel. Histology confirmed the diagnosis. A direct immunofluorescence test on the biopsy specimen was negative. Apart from the haematological syndrome, there were no laboratory abnormalities. The cutaneous lesions disappeared after 7 weeks of treatment with prednisolone 20 mg per day. The blood disease remained unchanged. The second patient was a 71-year old woman with RAEM since 1982. In 1984, she presented with lesions of cutaneous vasculitis located on the anterior aspect of the upper third of her left leg. There was neither arthralgia nor fever, and no history of drug toxicity or infection. Beside RAEM, polyclonal hyperglobulinaemia was present. Histological examination of the skin showed evidence of vasculitis with fibrinoid necrosis of vascular walls and perivascular lympho-histiocytic infiltrate with granulocytes and slight leucocytoclasia. Direct immunofluorescence testing of the skin demonstrated intravascular complement deposits. There were no circulating immune complexes; measurements of complement and complement fractions gave normal values; no cryoglobulin was found. The cutaneous lesions recurred on two occasions in 6 months, although no drug toxicity or infection could be elicited and the haematological syndrome was unaffected. The third patient was a 67-year old man with RAEM since 1982. In 1983, an ulcero-necrotic lesion spontaneously developed on his right leg.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Skin manifestations of refractory anemia with excess of blasts (RAEB)]. 357 13

We evaluated 10 patients with hidradenitis suppurative or acne conglobata who developed arthritis. In contrast to patients with acne fulminans and arthritis, all our subjects were adults over 22 years of age; nine were black; and four were women. Nine patients had episodic inflammatory oligoarthritis affecting mainly larger joints of the upper and lower extremities. Eight patients had roentgenographic evidence of peripheral arthritis, four with erosions. Nine had clinical axial arthropathy, but roentgenograms showed abnormalities of the axial skeleton in all 10 patients. Pyoderma gangrenosum, erythema nodosum, conjunctivitis, urethritis, and oral and penile ulcers occurred in some patients. Rheumatoid factor was negative in all patients; the erythrocyte sedimentation rate was elevated in nine; five had chronic anemia; four had circulating immune complexes; and complement components were elevated in four. There was no increased incidence of HLA-B27 or other HLA-B7 cross-reacting antigens. A temporal relation of skin and joint disease activity was suggested. We report a spondyloarthropathy associated with hidradenitis suppurativa and acne conglobata. Clinical and laboratory manifestations suggest the arthropathy may be reactive to chronic cutaneous infection.
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PMID:Spondyloarthropathy associated with hidradenitis suppurative and acne conglobata. 621 80

A 58-year-old man with chronic "idiopathic' pyoderma gangrenosum, recurrent secondary staphylococcal infection, nail candidiasis, markedly impaired lymphocyte function and a serum blocking factor is described. Despite an initially favourable response to immunosuppressive therapy, the patient later relapsed whilst on high dose systemic corticosteroids and azathioprine. Immune function remains abnormal 3 years cessation of azathioprine. Attempted immunostimulation with Corynebacterium parvum proved unsuccessful. A striking but transient increase in lymphocyte function followed parenteral iron therapy for an unexplained iron-deficiency anaemia. Gradual reduction in prednisone dosage to 5 mg daily has coincided with a period of clinical resolution and modest improvement in lymphocyte reactivity. Prolonged immunosuppressive therapy may be inadvisable in chronic idiopathic pyoderma gangrenosum associated with defective cellular immunity.
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PMID:Idiopathic pyoderma gangrenosum and impaired lymphocyte function: failure of azathioprine and corticosteroid therapy. 723 16

The authors present a 42-year-old man's case in whom the onset of chronic ulcerative colitis and pyoderma gangrenosum was simultaneous. Pyoderma gangrenosum began as bullae on his chest and lower limb. The main symptoms were fever, anaemia, hypoproteinaemia beyond the skin lesions, and the abdominal symptoms characterizing chronic ulcerative colitis were absent. The thorough internal check-up and the micribiologic and histologic examinations of the skin disorders made clear that the lesions mentioned were the skin symptoms of a systemic disease. Sulfasalazin and prednisolone therapy of the underlying disease and the local non-specific treatment of the cutaneous lesions resulted together in the patient's rapid improvement. The authors give a brief review of pyoderma gangrenosum and of the connection between pyoderma gangrenosum and chronic ulcerative colitis.
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PMID:[Ulcerative colitis associated with pyoderma gangrenosum]. 790 38

A markedly obese 54 year old woman with seropositive rheumatoid arthritis, anaemia, dyspepsia, controlled hypothyroidism and depression presented with a seven month history of large pyoderma gangrenosum ulcers on the shins. Routine dressings for the ulcers had been ineffective. Her arthritis was being treated with azathioprine and NSAID's. Initial treatment with clobestasol propionate and disodium cromoglycate under occlusion produced only partial healing. Introduction of Cyclosporin A and continuation of topical therapy, with the addition of triamcinolone acetonide injections, led to progressive healing which was complete after seven months. There has been no relapse to date. Cyclosporine can be combined with azathioprine and local therapy for successful treatment of pyoderma gangrenosum.
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PMID:Cyclosporine, azathioprine and local therapy for pyoderma gangrenosum. 799 94


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