Karyotype and prognosis in adult Spanish acute lymphoblastic leukemia.

The aim of the study was to define the frequency and prognostic significance of acquired chromosomal abnormalities in our adult population and to ascertain whether karyotype represents a significant prognostic factor in adult patients with acute lymphoblastic leukemia (ALL) independently of the new intensive chemotherapy programs and initial clinical characteristics.

Iron depletion by phlebotomy with recombinant erythropoietin prior to allogeneic transplantation to prevent liver toxicity.

Iron overload may induce liver toxicity after hematopoietic stem cell transplantation (HSCT), but it is not known if iron depletion prior to HSCT can reduce the risk of severe toxicity in this setting. We used subcutaneous recombinant erythropoietin (EPO) (25 UI/kg) three times a week and phlebotomy once a week, to prevent liver toxicity in a patient with advanced acute leukemia and liver disease due to severe iron overload, previous drug toxicity and hepatitis C viral infection. Over the 9 months prior to allogeneic HSCT, 34 phlebotomies were carried out. Serum ferritin dropped from 2964 to 239 microg/l and the ALT dropped to near normal values. At allogeneic HSCT no liver toxicity was observed, suggesting that iron depletion in the pretransplant period may contribute to reducing transplant-related toxicity in selected cases.

The use of an expanded circulating hematopoietic progenitor cell pool associated with bone marrow fibrosis for the support of autologous transplantation in IgD multiple myeloma.

Leukoerythroblastosis and myelofibrosis were observed at presentation in a patient with IgD myeloma. Interestingly, a 1000-fold increase in peripheral blood progenitor cells (PBPC) was found in the steady state without signs of any underlying myeloproliferative disorder. The myeloma was resistant to conventional therapy. The expanded PBPC were collected in the steady state and used to support two consecutive myeloablative courses. A complete remission of the myeloma was achieved, with resolution of myelofibrosis. Furthermore, the unprimed PBPC expanded as a result of myelofibrosis, provided a sustained hematopoietic reconstitution. This indicated that their hematopoietic potential was equivalent to that of PBPC mobilized by chemotherapy or growth factors.

Centroblastic transformation of chronic lymphocytic leukaemia with primary skin involvement--cutaneous presentation of Richter's syndrome.

The development of a large-cell non-Hodgkin's lymphoma in a patient suffering from chronic lymphocytic leukaemia is known as Richter's syndrome, representing one of the possible anaplastic transformations of the leukaemia. Cutaneous involvement is an extremely rare event. The case of a 45-year-old man with B-cell chronic lymphocytic leukaemia is reported. Five years later multiple cutaneous nodules developed on his extremities. Biopsies of the skin showed a diffuse large-cell lymphoma, bearing the same cell surface immunoglobulin light chain isotype as the leukaemia, suggesting the original relationship between both neoplasms.

Cyclosporine in the treatment of pure red cell aplasia.

We present a patient with severe idiopathic pure red cell aplasia who responded spectacularly to cyclosporin after failure of corticoid treatment and cyclophosphamide. We conclude that cyclosporin should be considered a viable therapeutic alternative to cytotoxic immunosuppressive treatments in this disease.

Controlled and uncontrolled thrombocytosis. Its clinical role in essential thrombocythemia.

Only two of 19 patients with spontaneously evolving essential thrombocythemia remained asymptomatic in a 421 patient-month observation. The rest of the patients showed hemorrhagic diathesis (four patients), nonspecific neurological semiology (two patients), and occlusive vascular illness in cerebral, myocardic, arterial, and often multiple locations (total, 12 patients). Peripheral neuropathy was found in five of 10 patients studied. In this series the incidence of cerebral ischemia in the uncontrolled condition was 180 times higher than the epidemiologic expectancy in a population not affected by the disorder. Of 35 ischemic attacks, 22 occurred when the platelet count was more than than 1000 X 10(9)/l, 13 when the count ranged from 650 to 990 X 10(9)/l, and none occurred at counts of less than 650 X 10(9)/l. In contrast, therapeutic control of the thrombocytosis caused all complications to disappear. These findings point out the danger of the natural course of the illness and justify active therapy. At the same time they call into question some of the most commonly used criteria in the diagnosis of essential thrombocythemia.

Six new cases of prolymphocytic leukemia with heterogeneous prognosis. Clinical and immunologic features, light microscopy, and ultrastructural findings.

The clinical features and diagnostic evaluation of six patients affected by prolymphocytic leukemia (PLL), are described. Some of the cases deviate from a relatively uniform and aggressive clinical course of the disease entity. An actuarial survival analysis of 60 cases gathered from the literature and the authors' experience indicate that the cases showing the most prolongated evolution may reach 30% of the total. The different aggressiveness in the clinical course of these patients does not depend on the efficiency of the therapies applied. The percentage of prolymphocytes (PL) in peripheral blood throughout the clinical course was variable and not depending on the treatments used. This fact, in conjunction with (1) the presence of cellular types deviated from the typical PL and detected both at optical and ultrastructural levels and (2) the existence of cases of PL with minimal splenomegaly and leukocyte counts of less than 50 X 10(9)/liter, could lead to an underdiagnosis of PLL and hinder the actual cognition of the natural medical history of the disease.

Signet-ring cell lymphoma. Ultrastructural and immunohistochemical features of three varieties.

An ultrastructural and immunohistochemical study was performed on three malignant lymphomas of follicular center cell origin with signet-ring cells. The first case corresponded to a diffuse lymphoma with follicular center cells and plasmacytic features; the other two cases corresponded to the nodular centrocytic-centroblastic category. The neoplastic lymphocytes contained Russell bodylike inclusions, clear vacuoles, and ill-defined eosinophilic masses, respectively, which produced a displacement of the nuclei to the periphery. Ultrastructurally, there were three different morphologic types because of the following cytoplasmic changes: the accumulation of electron-dense granular material in the cisternae of the rough endoplasmic reticulum; the formation of clear vacuoles with a smooth limiting membrane; and, the presence of granular-fibrillar and crystalline material in the hyaloplasm without a limiting membrane. The first type of signet-ring cell contained monoclonal immunoglobulin (IgM-K). No immunoglobulins were found in the clear cytoplasmic vacuoles of the second type; however, the rim of cytoplasm stained for IgG, kappa and lambda. Finally, the third type showed IgG and kappa light chain in the hyaloplasmic inclusions. It is concluded that the signet-ring cell appearance is due to an abnormal production and secretion of immunoglobulins.