More than a third of non-gastric malt lymphomas are disseminated at diagnosis: a single center survey.

Mucosa-associated lymphoid tissue (MALT) lymphomas are extranodal B-cell tumors that generally follow an indolent course. The gastrointestinal tract is the most common site of MALT lymphoma, comprising 50% of all cases. The tissue lesions are often localized, have high therapeutic response rates with late relapses with a long overall survival (OS). The patients with non-gastric lesions may follow a different clinical course and many of them present with disseminated disease. This study reports a series of 51 patients with non-gastric MALT lymphoma. Twenty patients (39.2%) presented with disseminated disease, seven (13.7%) patients had two MALT mucosal sites involved and eight (15.7%) had involvement of three or more mucosal sites. At presentation, 17 (33.3%) patients had the lymph node and 12 (23.5%) the bone marrow involvement. Following various combinations of treatment, complete remission was achieved in 40 (81.6%), and partial remission in three of the 49 treated patients with no difference in response rates between different disease stages. Relapse occurred in 12/43 (27.9%) patients among whom eight (18.6%) recurred in the presenting organ system. Five patients (9.8%) died because of a rapid disease progression after a median follow-up of 56 months; two patients with primary lung lesions, 1 patient with secondary intestinal disease, and 2 patients suffered transformation to diffuse large B-cell lymphoma. No significant difference in survival was found between localized and disseminated disease (log rank 0.05, df = 1, P = 0.81). A patient age > or = 60 yr at diagnosis and presentation with the nodal disease were found to be statistically significant negative prognostic factors (P < 0.05). Median OS was not reached after 145 months of follow-up, with the estimated OS being 88% at 2 yr, and 78% at 5 yr.

[Hepatosplenic candidiasis in acute leukaemia patients].

INTRODUCTION: Hepatosplenic candidiasis is a disseminated invasive fungal infection that may affects patients with acute leukaemia. The main clinical manifestation is a persistent fever in patients recovered from prolonged neutropenia after recent chemotherapy. CASE OUTLINE: The authors present three patients, two women and one men, aged 23, 26 and 33 years, with acute leukaemia; one with acute myeloblastic and two with acute lymphoblastic leukaemia who developed hepatosplenic candidiasis. The diagnosis was based on prolonged fever, elevated serum bilirubin and alkaline phosphatase, as well as characteristic lesions on computed tomography, nuclear magnetic resonance and ultrasonographic findings and positive blood culture in one patient. The antifungal treatment was successful in one patient only. Two patients died due to progression of leukaemia. CONCLUSION: If leukaemia patient in remission after chemotherapy develops a prolonged fever of unknown origin, hepatosplenic candidiasis has to be considered and all efforts should be done to diagnose it. The diagnosis is based on clinical presentation and imaging techniques. The positive cultures of fungi are not usually possible and are not mandatory. The antifungal treatment may be prolonged, sometimes 2 to 3 months or even more.

Nephrotic syndrome and acute renal failure in non-Hodgkin lymphoplasmacytic lymphoma.

Two patients with lymphoplasmacytic lymphoma, and monoclonal proteins of IgM in one, and IgG and lambda light chains in the second patient, nephrotic syndrome and acute renal failure are reported. A 58-year-old man previously treated for pre-B acute lymphoblastic leukemia, developed 3 years later nephrotic syndrome as a complication of lymphoplasmacytic lymphoma and high-paraprotein IgM kappa type. Immunofluorescent analysis of kidney biopsy showed extensive IgM and light kappa chain deposits, which caused membranoproliferative glomerulonephritis. Treatment with cyclophosphamide was ineffective and patient died 2 months later. The second patient is a 42-year-old female diagnosed with lymphoplasmacytic lymphoma and paraprotein IgG lambda type. The course of the disease was fulminant with developing nephrotic syndrome and fatal acute renal failure. Immunofluorescent and light microscopic studies of kidney biopsy showed signs of immunotactoid glomerulonephritis with deposits of IgG and C3. Hemodyalises and cytostatic therapy were without response and she died after 45 days.