Treatment of adult systemic mastocytosis with interferon-alpha: results of a multicentre phase II trial on 20 patients.

Systemic mastocytosis (SM) is characterized by proliferation of mast cells in various organs, which may release a wide variety of mediators, thereby explaining the broad clinical spectrum of disease manifestations. The potentially life-threatening systemic symptoms and tumoral proliferation are poorly controlled despite the use of several cytotoxic chemotherapies and/or symptomatic treatments. Twenty consecutive adult SM patients with histologically confirmed bone marrow (BM) involvement received interferon-alpha subcutaneously (1-5 million units/m2/d, with progressive dose intensification over the first month of treatment) and were evaluated after 6 months of therapy.

Hypercalcemia may indicate Richter's syndrome: report of four cases and review.

Background: Hypercalcemia has rarely been reported in patients with Richter's syndrome (RS). In this article, the authors report four cases with complementary pathophysiologic examinations.

Methods: Four patients with hypercalcemia that indicated RS were admitted to the study unit between 1980 and 1994 with the following diagnoses: transformation of mixed follicular lymphoma, Waldenstrom's macroglobulinemia, and chronic lymphocytic leukemia, respectively, in 3 men ages 60 to 73 years, and chronic lymphocytic leukemia in a woman 73 years of age.

Plasma cell leukaemia mimicking acute monocytic leukaemia in the course of multiple myeloma.

We report a case of acute leukaemia occurring early in the course of IgA multiple myeloma. Ultrastructural studies, immunophenotyping and karyotyping were required to identify the origin and clonality of the leukaemic cells. Although ultrastructural examination of the blast cells revealed both monocytoid and plasma cell features, all cells expressed the CD 38 antigen and intracytoplasmic kappa light chains, while karyotyping revealed a clone with numerous abnormalities, leading to the diagnosis of clonal plasma cell disease.

Juvenile chronic myelocytic leukemia with unusual cytogenetic clonal evolution.

Cytogenetic studies are reported in a case of juvenile chronic myelocytic leukemia with dysmyelopoiesis and skin involvement. The clonal evolution of a 6q-anomaly is described. Hematological and cytogenetic findings suggest a role of hematopoietic stem cell in this patient for whom the outcome was fatal.

Questions concerning plasma cell leukaemia.

The classical concept of plasma cell leukaemia (PCL) is reviewed. This disease invariably concerns myeloma, either in its terminal phase (secondary PCL) or particularly rapid and aggressive (primary PCL). A more precise definition of primary PCL would be given by the term leukaemic myelomatosis.