Jesús Soler

Aggressive natural killer cell leukaemia/lymphoma in two patients with lethal midline granuloma

Summary. We report two patients with leukaemic proliferations of large granular lymphocytes. The immunophenotype study showed that the leukaemic cells were positive for CD2, CD38, CD56 and anti‐HLA‐DR monoclonal antibodies and negative for other T‐cell (CD3, CD4, CD8) and B‐cell markers (CD19, CD20 and surface immunoglobulins). The clinical course was acute and a diagnosis of aggressive natural killer cell leukaemia/lymphoma was made. No clonal rearrangements of either Cβ T‐cell receptor or JH immunoglobulin genes were found. Functional studies done in one patient demonstrated non‐restricted cytotoxic activity after activation with IL‐2. Lethal midline granuloma had been previously diagnosed in both patients. A possible relationship between this entity and the natural killer cell leukaemia is discussed.

Spontaneous splenic rupture as the initial manifestation of acute lymphoblastic leukaemia: Immunophenotype and cytogenetics

SummarySpontaneous splenic rupture is a rare, though life-threatening complication of some hematological malignancies and is even more infrequent as the initial symptom of acute lymphoblastic leukemia. We describe the fourth case, to our knowledge, of acute lymphoblastic leukemia presenting as splenic rupture, and for the first time the immunophenotype and cytogenetic pattern observed.

Acute Erythroblastic Leukemia

We report the clinical, cytological, immunophenotypic, and cytogenetic findings in one patient with acute erythroblastic leukemia. Blast cells were identified by their reactivity with the early erythr

Immunophenotype of blast cells in acute myelofibrosis

The immunophenotype of peripheral blood blast cells from six patients with acute myelofibrosis was studied using a panel of monoclonal antibodies directed against granulocytic, erythroid, megakaryocytic and lymphoid antigenic determinants. In all patients most of the blast cells were labeled with anti-HLA-DR and with the early myelomonocytic antibodies My7 (CD13), My9 (CD33) and B1-3C5 (CD34) (3/3). In three cases, platelet antibodies Edu3 (CD41) and GPIIIa (CD61) reacted with about 30% of blast cells. TdT was positive in two out of six samples studied. Lymphoid markers T3 (CD3), Leu9 (CD7), J5 (CD10), B4 (CD19) and B1 (CD20) were negative in all cases. These results suggest that blast cells are mainly of immature myelocytic origin. However, the coexistence of megakaryoblasts cannot be ruled out in the cases with a proportion of cells that are positive with Edu3 and GPIIIa antibodies.

Coincident Multiple Myeloma and Non-Hodgkin’s Lymphoma with 2 Serum Monoclonal Immunoglobulins

A case with features of both multiple myeloma and non-Hodgkins lymphoma at the moment of diagnosis is presented. The patient had lytic bone lesions and biclonal gammopathy, IgM kappa and IgA kappa. In the bone marrow biopsy, there was a diffuse infiltration by atypical plasma cells coexisting with an interstitial and nodular infiltration by poorly differentiated lymphoid cells. Immunofluorescence studies showed positive staining with alpha and kappa antisera in the cytoplasm of plasma cells and with mu and kappa antisera on the surface of lymphoid cells. After the beginning of chemotherapy, the IgA kappa monoclonal protein disappeared and the IgM kappa monoclonal protein remained constant.

Hematopoietic growth factor (G-CSF or GM-CSF) after salvage chemotherapy in refractory or relapsed adult de novo acute leukemia

Nineteen adults with primary refractory or relapsed acute leukemia (12 ALL and 7 ANLL) were treated with an intensive salvage chemotherapy (intermediate-dose ara-C, intermediate-dose methotrexate, vindesine, cyclophosphamide, mitoxantrone and prednisolone) followed by a hematopoietic growth factor (HGF), either granulocyte colony-stimulating factor (5 micrograms/kg) or granulocyte-macrophage colony-stimulating factor (10 micrograms/kg). Both were given from the day after chemotherapy ended and until the neutrophil count rose above 1 X 10(9)/l for three consecutive days. Eleven patients (58%, 95% CI 33% to 82%) achieved complete remission, and 15 courses of salvage therapy were given to these complete responders. In a historical control group that did not receive HGF, 23 out of 38 patients (60%, 95% CI 44% to 77%) achieved complete remission, and 27 courses of therapy were delivered to complete responders. Treatment with a HGF accelerated the recovery of neutrophils to 0.5 X 10(9)/l significantly, shortening it from a mean of 28 to 22 days (p = .0002), with no effect on platelet recovery. There were no differences in the rates of documented and fatal infections, which were relatively high in both groups. In the patients with ANLL, there was no evidence that HGF accelerated leukemic regrowth. We conclude that HGF accelerates neutrophilic recovery following intensive salvage chemotherapy for acute leukemia, although no reduction in documented infections was found. Many factors, including the small patient sample, may have contributed to this latter finding.