A case of longitudinally extensive transverse myelitis in an 80-year-old patient with systemic lupus erythematous and anti-aquaporin 4 antibodies.

BACKGROUND: Systemic Lupus Erythematous is a systemic autoimmune disease with multiorgan inflammation. Clinical manifestations are variable and may involve the Central Nervous System. Acute transverse myelitis is a rare complication. Recent studies have shown an association between SLE, transverse myelitis and presence of anti-aquaporin 4 antibodies. CASE PRESENTATION: We describe the case of an 80-year-old woman with a subacute onset of right hemiplegia followed by left-sided ataxia. Cervical MRI revealed longitudinally extensive transverse myelitis. Blood examinations showed positivity for anti-nuclear antibodies, anti-double-stranded DNA antibodies, anti-phospholipid antibodies and anti-aquaporin 4 antibodies. CONCLUSIONS: Anti-aquaporin 4 antibody testing is of paramount importance in order to reach a correct diagnosis and to treat patients with the best therapeutic approach.

Esophageal involvement as an uncommon modality of relapse of Hodgkin lymphoma.

We present a case of systemic Hodgkin's lymphoma, relapsed with esophageal involvement after 3 years of complete remission. The importance of an accurate diagnostic work-up is emphasized. Esophagectomy and chemotherapy followed by bone marrow transplantation allowed a complete response and the long-term survival of the patient.

Primary splenic lymphoma: does it exist?

The number of primary splenic lymphomas being reported is increasing despite the rarity of this malignancy, but what really constitutes a lymphoma arising primarily in the spleen is still a matter of discussion. The authors choose the "restrictive" definition of a lymphoma involving the spleen and the splenic hilar lymph nodes only. In this way, the risk of epidemiologic or clinical overestimation is avoided. The clinical features of this condition are characterized by non specific symptoms and signs, while the prevailing histology is that of a low-grade or intermediate-type lymphoma. Disease spreading outside of the spleen and its hilar lymph nodes is the single most important factor associated with an unfavorable prognosis. From this usual clinical picture, two distinct nosologic entities can be outlined on the basis of histologic and immunologic peculiarities: splenic lymphoma with circulating villous lymphocytes and marginal-zone splenic lymphoma. The former arises from follicular center cells and is characterized by hypersplenism, variable percentages of circulating villous lymphocytes and, frequently, a monoclonal gammopathy. The latter originates from a peculiar splenic B-cell structure separated by the mantle zone. The proliferating cells are medium-sized KiB3-positive lymphocytes with round or cleaved nuclei and pale cytoplasm, which surround follicular centers and infiltrate the mantle zone. It is interesting that marginal-zone lymphoma cells share some of the characteristics of the lymphocytes involved in both lymphomas of mucosa-associated lymphoid tissue and the B-monocytoid lymphomas. Splenectomy is still the most effective therapy for all primary splenic lymphomas.

Study of prognosis in Waldenström's macroglobulinemia: a proposal for a simple binary classification with clinical and investigational utility.

Prognostic evaluation of Waldenström's macroglobulinemia (WM) is unreliable, few studies considered prognostic factors in WM and only one was derived from a multivariate analysis. One hundred forty-four retrospective, previously untreated patients with clinically overt WM were studied to learn whether overall survival was related to any of the various clinical features presented at diagnosis. Patients were homogeneously treated with intermittent doses of chlorambucil for as long as this showed an effect on the monoclonal component. The population was randomly subdivided into a 90-patient exploratory sample, on whom investigation would be conducted, and in a 54-patient test sample, on whom the results would be validated. In the exploratory sample univariate analysis identified the following parameters as the most important for prognosis: age (< or > or = 70 years), platelet count (< or > or = 120 x 10(9)/L), presence or absence of an abnormal number of red blood cells in the urine, hemoglobin concentration (< or > or = 9 g/dL), erythrocyte sedimentation rate (< or > or = 110 mm at first hour), presence or absence of cryoglobulinemia and of weight loss. Cox multivariate analysis showed that only hemoglobin, age, weight loss, and cryoglobulinemia independently affected survival. These four clinical variables were also shown to be able to discriminate survival significantly in the test sample. Moreover, it was possible to demonstrate (both in the exploratory and the test sample) that clear-cut, albeit dichotomic, survival discrimination can be reached with the presence at diagnosis of either no more than one, or any two or more, of these four prognosticators. These simple clinical criteria could be the basis of an initial binary, prognostic classification of WM, which could help in differentiating therapy according to the severity of the disease, and in properly designing future clinical trials.