Chronic myelogenous leukemia in the monosomic cell line of a fertile turner syndrome mosaic (45,X/46,XX)

Malignancy in patients with constitutional chromosome abnormality is of interest not only because it permits insights into the relationship between chromosome abnormality and cancer, but also because it provides opportunities to address such questions as the clonality and evolution of tumors. We report Ph¹-positive chronic myelogenous leukemia (CML) in a 50-year-old mosaic (45,X/46,XX) Turner syndrome patient whose leukemia was restricted to the monosomic cell line. Our extensive cytogenetic studies of this patient demonstrated that non-leukemic normal cells persisted in the marrow and were able to proliferate during a period of temporary suppression of the leukemic clone following aggressive treatment.     

Primary splenic lymphoma with filiform ultrastructure.

A case of primary large cell splenic lymphoma of B lineage exhibiting filiform cell appearance is reported. The patient presented with massive splenomegaly, and following spontaneous splenic rupture, died of adult respiratory distress syndrome. The clinical aspects of the case, notably a lymphoma arising as a primary tumour in the spleen, with spontaneous spleen rupture and rapid fatal outcome, in combination with the filiform appearance of the lymphoma on electron microscopic examination, constitute an unusual combination of features. As far as is known, this B cell neoplasm is only the second primary splenic lymphoma of filiform type to be recorded.