Marginal zone lymphoma of both spleen and kidney displaying transformation into large B-cell lymphoma.

We report a case of simultaneous involvement of the spleen and the left kidney in a marginal zone lymphoma with a monotypic lymphoplasmacytic cell component, which transformed into a diffuse large B-cell lymphoma of the immunoblastic type. PCR showed that the small and large B-cell populations carried the same type of immunoglobulin heavy chain gene rearrangement. This type of rearrangement was detected in the spleen, the latero-aortic lymphadenopathy and the kidney demonstrating that it is the same lymphoma that affected both organs and the lymph nodes. Primary renal lymphoma is very rare and only a few cases of renal marginal zone lymphoma, MALT type, have been reported. Involvement of simultaneous multiple sites has been described in MALT type lymphoma, but splenic involvement secondary to renal MALT lymphoma seems to have never been observed. Nevertheless, in our case the huge size of the spleen associated with splenic hilar node involvement is consistent with primary splenic marginal zone lymphoma. The extension into latero-aortic lymph nodes of this lymphoma can explain secondary kidney involvement. The nodal Kaposi's sarcoma observed in this patient of Mediterranean origin was probably coincidental.

Philadelphia-positive T-cell acute lymphoblastic leukemia with polymyositis, migratory polyarthritis and hypercalcemia following a chronic myeloid leukemia.

Transformation of chronic myeloid leukemia (CML) often results in acute myeloblastic or, less frequently, in precursor B-cell acute lymphoblastic leukemia (ALL). T-cell blast crisis is rare. Hypercalcemia has also been described as a rare complication of CML, but this usually occurs as a terminal event. Here we report a case of a 35-year-old woman who developed a CD4(+)/CD8(+) T-cell ALL 2 years after the diagnosis of a typical Ph(+) CML. Polymyositis and polyarthritis preceded by 4 months, and symptomatic hypercalcemia occurred just before blastic transformation, probably representing paraneoplastic manifestations of the disease.

Aggressive natural-killer cell lymphoma presenting with skin lesions, breast nodule, suprarenal masses and life-threatening pericardial and pleural effusions.

We report the clinical and laboratory findings of a patient with an aggressive Epstein-Barr virus positive CD2+/CD56+ natural killer-cell lymphoma with a high mitotic activity and complex chromosomal abnormalities presenting with life-threatening pericardial and pleural effusions, disseminated skin lesions, breast nodule and large suprarenal masses. The clinical course was characterized by resistance to chemotherapy and relapsing pericardial and pleural effusions with respiratory and haemodynamic failure. Death occurred 4 months after the first manifestations of the disease as a consequence of cardiac tamponade.

Different patterns of spleen involvement in systemic and malignant mastocytosis. A histological and immunohistochemical study of three cases.

Three cases of splenic involvement in three different types of generalized mastocytosis (systemic mast cell disease) are reported. The macroscopic, histological and ultrastructural modifications of the spleen are described. Each case exhibited a different morphological pattern. Giemsa staining, fluorescence after acridine orange staining and naphthol ASD chloracetate esterase reaction are shown to be valuable for diagnosis. By comparison, immunohistochemistry seemed not to be very useful, because no specific antigens are expressed. These findings are compared to previously published cases. Their value for the diagnosis and the prognosis are discussed.