RESUMO
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that affects numerous organs, particularly the digestive tract (33-52% of cases). However, isolated ascites has rarely been described in patients with PAN. We observed a patient who had developed ascites revealing PAN, and because of suspected malignancy laparotomy and abdominal surgery were performed, including systematic total abdominal hysterectomy, bilateral salpingo-oophrectomy, submesocolic omontectomy and appendicectomy. Macroscopic examination revealed a nodule involving the left ovary, while histological examination of biopsy specimens demonstrated characteristic damage of necrotizing vasculitis involving the appendix, omentum, uterus, fallopian tubes and right ovary, consistent with PAN. The patient had a favourable outcome of clinical manifestations related to PAN after initiation of combined therapy of prednisone and cyclophosphamide. Our case report therefore reinforces the possibility of an unusual presentation of PAN. As ascites may precede other signs of PAN, we suggest that when unexplained ascites is noted an evaluation for misdiagnosed vasculitis should be carried out, including a search of the constitutional symptoms (e.g. fever, weight loss). Patients undergoing laparoscopy for peritoneal involvement of unknown origin should also have tissue specimens taken for pathologic studies, notably to exclude an underlying vasculitis.
Assuntos
Ascite/etiologia , Poliarterite Nodosa/complicações , Idoso , Apêndice/patologia , Ciclofosfamida/administração & dosagem , Quimioterapia Combinada , Tubas Uterinas/patologia , Feminino , Humanos , Omento/patologia , Ovário/patologia , Poliarterite Nodosa/diagnóstico , Poliarterite Nodosa/tratamento farmacológico , Poliarterite Nodosa/patologia , Prednisona/administração & dosagem , Útero/patologiaRESUMO
Intravascular large B-cell lymphoma (ILBCL) is a rare subtype of diffuse large B-cell lymphoma (as currently recognized by the World Health Organization classification) and is characterized by proliferation of mature B-cells within the lumina of small and medium vessels. We report on a 66-year-old man who presented with a fever of undetermined origin, a splenomegaly, and an elevated lactate dehydrogenase level. The diagnosis of ILBCL was established by a bone marrow biopsy that showed CD20+ tumor cells confined within the lumina of sinuses. A karyotypic analysis obtained from the bone marrow aspirate showed a hypotetraploid clone. Magnetic resonance imaging of the brain revealed multiple high-signal areas in the periventricular white matter above the tentorium. Focal dural enhancement (pachymeningitis) close to the medium third of the superior sagittal sinus was also observed and was related to a partial superior sagittal sinus thrombosis as confirmed by venous magnetic resonance angiography. After 8 courses of a CHOP (cyclophosphamide/doxorubicin/vincristine/prednisone) plus rituximab regimen, normalization of the superior sagittal sinus and of the bone marrow was obtained. With a follow-up of 15 months, the patient is still considered in complete remission. This observation highlights an unusual vascular aspect of ILBCL and the efficacy of the current standard treatment for this age group (CHOP/rituximab) in this particularly aggressive lymphoma subtype.