Vasculite associada à ANCA e leucemia linfocítica crônica: uma rara associação

Lucena, Juliana Miranda de; Callegari, Amanda Garbin; Barbosa, Fabíola Brasil; Sarinho, José Celso Giordan Cavalcanti; Casagrande, Renielly; Souza, Branca Dias Batista de

doi:10.1016/j.rbr.2014.01.005

Cited by 2 publications

(1 citation statement)

References 10 publications

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“…The association of eyehole cellulites with the rhino sinusal lesions on the same region (Fig. 2 ) completed the clinical aspect with elements which are related to Wegener’s granulomatosis, confirmed by biopsy and by positive c-ANCAs, a situation which is rarely described in the literature [ 4 , 5 ] in association with chronic lymphocytic leukemia. The diagnosis of granulomatosis would have not been possible without the pathological anatomical result.…”

Section: Case Presentationmentioning

confidence: 54%

A rare association of chronic lymphocytic leukemia with c-ANCA-positive Wegener’s granulomatosis: a case report

Bîrluțiu

Rezi²,

Bîrluţiu

et al. 2016

World J Surg Onc

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BackgroundWegener’s granulomatosis is a systemic vasculitis of the small- and medium-sized vessels, produced by the action of ANCA, which involves the respiratory tract, kidneys, and eyes, with a potential for lethal evolution in the first year after diagnosis. Its association with chronic lymphocytic leukemia is rarely described in the literature, and it may be difficult to diagnose and to treat this association.Case presentationWe present the case of a 73-year-old Caucasian patient, a rare case in which Wegener’s granulomatosis is associated in a patient with chronic lymphocytic leukemia, who is admitted in the Infectious Disease Department for fever, diplopia, headache, purulent and hemorrhagic nasal secretions, intense asthenia, and weight loss. The patient had associated eyelid edema; scleritis; chemosis; subconjunctival hemorrhage at the left eye; swelling of the left region of the eyehole, of the base of the nasal pyramid, and of the left zygomatic region; anterior nasal bleeding; pustulous non-itching lesions at the cervical region and posterior thorax; enlarged bilateral axillary lymph nodes; hepatomegaly; and moderate splenomegaly. During the surgical treatment of the pansinusitis, a biopsy from the tissue is taken; the biopsy fragments of the nasal mucosa pleads for Wegener’s granulomatosis. The c-ANCA were positive. The patient’s evolution was favorable under treatment with meropenem, teicoplanin, fluconazole, transfusions of platelet concentrates, and methylprednisolone.ConclusionsThe real dimension of the association between chronic lymphocytic leukemia and Wegener’s granulomatosis is not known; it may be useful to evaluate the vasculitis by testing ANCA routinely in patients with chronic lymphocytic leukemia and by histopathological examinations of the lesions.

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Section: Case Presentationmentioning

confidence: 54%

A rare association of chronic lymphocytic leukemia with c-ANCA-positive Wegener’s granulomatosis: a case report

Bîrluțiu

Rezi²,

Bîrluţiu

et al. 2016

World J Surg Onc

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ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives

Folci

Ramponi

Shiffer

et al. 2019

Journal of Immunology Research

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The purpose of this paper is to collect and summarize all evidences relating to an association between ANCA-associated vasculitides (AAVs) and hematologic malignancies, in the form of either a paraneoplastic vasculitis or leukemias and lymphomas developing on a preexisting vasculitis. Additionally, the role of cyclophosphamide in vasculitis treatment has been assessed and compared to rituximab. Paraneoplastic AAV seems to be an uncommon presentation of hemopathies. Hematologic malignancy risk in AAV is more likely to be increased by cyclophosphamide, although not yet definitely proven. Furthermore, the pathogenesis of ANCA-associated vasculitis has been reviewed with particular emphasis on the role of proteinase 3 (PR3) in fuelling granulomatosis with polyangiitis (GPA) inflammation. PR3 is a bactericidal protein expressed by neutrophilic granules and on their plasma membrane. Derangements in its expression and function have been linked to leukemias and GPA alike. PR3-derived PR1 peptide is being studied as an immunotherapy target in leukemia and multiple myeloma. This study is aimed at bringing together various evidences from the field of immunological and hematological research, at exposing contradictions, and at revealing novel insights on the association between ANCA-associated vasculitis and hematologic malignancies.

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Vasculite associada à ANCA e leucemia linfocítica crônica: uma rara associação

Cited by 2 publications

References 10 publications

A rare association of chronic lymphocytic leukemia with c-ANCA-positive Wegener’s granulomatosis: a case report

A rare association of chronic lymphocytic leukemia with c-ANCA-positive Wegener’s granulomatosis: a case report

ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives

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