Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In four patients a systemic vasculitis similar to polyarteritis nodosa developed within 2 years of the onset of hairy-cell leukaemia. Arteriographic studies in two patients revealed microaneurysms, and biopsy specimens in three patients revealed a vasculitis affecting medium-sized vessels. Blood neutrophilia and neutrophilic vascular infiltrate were absent. One patient had circulating immune complexes. Two patients responded to corticosteroids alone, one required cyclophosphamide as well as steroids, and one improved without chemotherapy. The association of vasculitis with hairy-cell leukaemia may provide insight into the pathogenesis of arteritis.
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PMID:Hairy-cell leukaemia with polyarteritis nodosa. 8 11

Multiple visceral aneurysms complicating periarteritis nodosa are considered characteristic, though not pathognomonic, on arteriography. This arteriographic pattern has been described with hairy-cell leukemia, collagen vascular disorders, and atrial myxoma, but, to our knowledge, has not been previously reported with subacute bacterial endocarditis. A patient with enterococcal endocarditis sustained separate intra-abdominal hemorrhages, 24 hours apart, from aneurysms of the middle colic and left colic arteries. Sterile vessel cultures with inflammatory infiltrates, decreased complement levels, positive rheumatoid factor, and arteriographic evidence of multiple visceral aneurysms suggest the vasculitis was immunologically mediated and not mycotic. Antibiotic therapy after control of hemorrhage controlled abdominal symptoms.
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PMID:Multiple mesenteric aneurysms complicating subacute bacterial endocarditis. 288 83

A polyarteritis nodosalike systemic vasculitis in hairy-cell leukemia was first reported in 1979, and since then at least 30 additional cases of this unusual association have been reported in the literature. Reported herein is, to my knowledge, the first known example of hairy-cell leukemia with necrotizing vasculitis limited to the testis only.
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PMID:Isolated polyarteritis of testis in hairy-cell leukemia. 289 38

Systemic vasculitis is an unusual but recently recognized complication of hairy cell leukemia. We studied this relationship further in an attempt to better understand pathogenetic mechanisms of vasculitis. We examined the records of 129 cases of hairy cell leukemia seen at the Mayo Clinic between 1976 and 1983, and identified 2 cases with evidence of systemic vasculitis. The first of these cases is discussed in detail. Immunologic studies were performed but we were unable to demonstrate the presence of shared antigen on hairy cells and endothelial cells. The literature is reviewed and reports of this association are summarized. Possible mechanisms of vascular injury are discussed.
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PMID:Vasculitis in hairy cell leukemia: review of literature and consideration of possible pathogenic mechanisms. 356 Jan 5

Vasculitis associated with hairy-cell leukemia (HCL) has been reported occasionally. We determined the clinical and biological significance of this association by the retrospective study of a series of 50 patients with HCL, including nine patients with vasculitis. The development of vasculitis was not related to a variant of HCL on the basis of hematologic findings and survival. Vasculitis could occur at any time during the course of HCL, and was the circumstance for the diagnosis of HCL in three cases. Clinical and immunohistologic findings were those of hypersensitivity vasculitis in the nine patients. Infection was found to be an associated factor. Thus, eight of nine patients were infected at vasculitis onset, four died of their infection with no remittance of the cutaneous lesions, and three recovered from both infection and vasculitis. The monocyte deficiency in HCL is known to favor intracellular pathogen infection; however, we could not demonstrate that it also correlates with a decrease in the clearance of IgG-sensitized erythrocytes. Finally, vasculitis in HCL appears to be associated with a lasting infection in most cases.
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PMID:Vasculitis in hairy-cell leukemia. 382 53

Biopsy of skin for etiologic evaluation of generalized erythematous eruptions in a 4 1/2 years old child, starting four months ago, led to diagnosis of acute lymphoblastic leukemia. Leukocyte counts five and eight weeks and four months after appearance of eruptions were normal and no blasts were found. A leukocyte count performed simultaneously with biopsy of skin also showed few (less than 10%) blasts. Histologic examination of skin revealed two types of pathologic lesions: a perivascular cuffing of lymphoblasts and a leukocytoclastic type of small vessel vasculitis in other parts. This type of vasculitis which have been reported in association with chronic lymphocytic and hairy cell leukemias, is uncommon in acute lymphoblastic leukemia.
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PMID:[Diffuse cutaneous vasculitis disclosing acute lymphoblastic leukemia]. 386 61

Thirty-seven patients with hairy-cell leukaemia were retrospectively reviewed for the presence of autoimmune disease. Ten definite and two probable cases were identified; these patients had positive serologies (immune complexes, antinuclear antibodies or rheumatoid factor) or biopsy-proven vasculitis. Clinically, two distinct syndromes were recognized. Six patients had joint symptoms, usually associated with nodular skin lesions; all responded promptly to therapy. Four additional cases had a more severe disease consisting of fevers, malaise, weight loss, skin rash, and variable visceral involvement; there was one death in this group. There appeared to be no relationship between presence of vasculitis and the severity or progression of the underlying malignant disease. We conclude that autoimmune disease is much more frequent in hairy-cell leukaemia than has previously been recognized, and that the outcome in these syndromes is usually good. Although the autoimmune syndrome generally responds promptly to splenectomy, corticosteroids, or cytotoxic therapy, failure to recognize this complication may lead to increased morbidity and occasional mortality.
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PMID:Autoimmune disease in hairy-cell leukaemia: clinical syndromes and treatment. 404 78

A case of hairy cell leukaemia complicated as a terminal event by massive retroperitoneal lymphadenopathy is described. The patient had recently been treated with lithium carbonate and had previously been demonstrated to suffer from a systemic vasculitis, either or both of which may have contributed to the development of this rare complication.
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PMID:Massive retroperitoneal lymphadenopathy as a terminal event in hairy cell leukaemia. 636 Apr 96

A patient is reported, in whom clinical and biochemical evidence of connective tissue disease preceded the diagnosis of hairy-cell leukaemia by at least 2 years. The pathogenetic mechanism(s) responsible for the coexistence of hairy-cell leukaemia with vasculitis and rheumatic disease is discussed. It is proposed that the 2 diseases may share a common predisposing factor, or that the hairy cells may elicit disturbances in immune homeostasis, implying a dysfunction of T suppressor cells. Hairy-cell leukaemia should be considered in the differential diagnosis in cases of unexplained febrile illness associated with pancytopenia and signs of connective tissue disease.
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PMID:Hairy-cell leukaemia simulating connective tissue disease. 672 97

Hairy-cell leukemia has been associated with a number of disorders of the immune system. At least 13 cases of vasculitis and hairy-cell leukemia have been reported. However, the occurrence of cryoglobulinemia and hairy-cell leukemia is rare. We report a case of a patient with the unusual combination of hairy-cell leukemia, vasculitis, and cryoglobulinemia. This case illustrates that hairy-cell leukemia should now be included in the differential diagnosis of patients with the appearance of vasculitis and cryoglobulinemia. The association presents a therapeutic challenge and emphasizes the need for individualized treatment in such patients.
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PMID:Hairy-cell leukemia with the appearance of mixed cryoglobulinemia and vasculitis. 673 90


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