Occurrence of a Ki-1-positive anaplastic large-cell lymphoma in a patient with Ph' positive chronic myelogenous leukemia successfully treated by alpha-interferon.

We describe a patient with Philadelphia-chromosome-positive (Ph' +) chronic myelogenous leukemia (CML), who developed an anaplastic large cell lymphoma (ALCL) with T-phenotype, after 43 months successful treatment with alpha-interferon (IFN). Characterization studies of lymphoma cells showed positivity for Ki-1 monoclonal antibody, T-cell surface markers, T-cell receptor beta chain rearrangement, and germline configuration of the BCR gene. At the time of lymphoma diagnosis, the patient had achieved complete hematologic remission from CML with partial karyotypic conversion (50% Ph' + cells). After twelve weekly courses of polychemotherapy, he obtained complete remission from lymphoma. At present, five years from CML diagnosis, the patient has a remarkably stable disease, being in remission from lymphoma and in well controlled CML chronic phase. Our case thus represents the first well documented description of a T-cell non-Hodgkin's lymphoma developed during the course of CML.

Long-term remission of T-lymphoid extramedullary blast crisis of chronic myelogenous leukemia following allogeneic bone marrow transplantation.

Extramedullary blast crisis (EBC) with T-lymphoid phenotype has been reported rarely and generally associated with extremely poor prognosis. We describe a case of T-lymphoid EBC in which long-lasting remission was observed following intensive chemotherapy and allogeneic bone marrow transplantation (BMT). Characterization of bone marrow (BM) and lymph node (LN) cells was performed by means of morpho-cytochemical, cytogenetic, immunophenotypic and molecular analyses. These showed, together with a marrow picture consistent with typical Ph'+ chronic myelogenous leukemia, the expansion of an early T-lymphoid (CD7+/TdT+) LN cell population exhibiting the same bcr rearranged pattern and an additional Ph' chromosome. At the present time, 33 months after BMT, the patient is alive and well, with persistent clinical, hematological, cytogenetic and molecular evidence of complete remission.