Erythropoietin plus granulocyte colony-stimulating factor in the treatment of myelodysplastic syndromes. Identification of a subgroup of responders. The Spanish Erythropathology Group.

BACKGROUND AND OBJECTIVE: Anemia leading to transfusion is probably the most important problem in patients with myelodysplastic syndromes (MDS). Human recombinant erythropoietin (rHuEpo) and granulocyte colony-stimulating factor (G-CSF) have been used to treat patients with anemia of MDS, but fewer than 50% respond. The aim of this work was to evaluate the benefit of rHuEpo +/- G-CSF treatment and to isolate the response predictive variables in a group of selected patients with MDS. DESIGN AND METHODS: A non-randomized multicenter trial was carried out in 32 patients with MDS. The inclusion criteria were age >= 18 years, refractory anemia (RA) or refractory anemia with ringed sideroblasts, Hb <= 100 g/L or receiving transfusions and serum erythropoietin <= 250 U/L. These patients were treated with subcutaneous rHuEpo (300 U/kg) three times a week for 8 weeks. In the case of partial response (PR) or no response (NR) subcutaneosly administered G-CSF (1 microg/kg) three times a week was added to the rHuEpo for 8 more weeks. If the patient achieved complete response (CR) or PR in the second phase, he was included in a follow-up phase of 24 weeks in which the dose of growth factors was tapered down. Several variables, including the score published by the Scandinavian-American group, were used as possible predictive variables. RESULTS: An erythroid response was observed in 16 patients (50%); in 12 it was a CR and in 4 it was a PR. During the period of rHuEpo administration, 7 CR and 4 PR (34.4%) were documented. Of the 14 patients in whom G-CSF was added to rHuEpo, 7 (50%) responded (3 CR and 4 PR). No major side-effects associated with growth factors were observed. The multivariate analysis showed that of the different variables evaluated only the Scandinavian-American response score was significant with a relative probability of response of 11.8 (95% confident intervals: 2.5-53) when this score was > +1 (77% of cases responded). In contrast, when this score was <= 1 only 15 % of the cases responded. INTERPRETATION AND CONCLUSIONS: Use of the Scandinavian-American response score is to be recommended in a patient-oriented approach to treating MDS cases with the Epo and G-CSF. Treatment with rHuEpo and G-CSF is safe, its main drawback being its cost. However, a long-term study evaluating the regimen's cost-benefit ratio is warranted.

Detection of a t(8;21)(q22;q22) in a case of M5 acute monoblastic leukemia.

Although the translocation (8;21) is the single most common structural rearrangement reported in acute myeloblastic leukemia (AML), it is rarely seen in AML FAB type M5. We describe a case of a 51-year-old male with a diagnosis of acute monoblastic leukemia (AML M5b with hemophagocytic component) whose karyotype showed at (8;21)(q22;22). To our knowledge, this is the first report of this translocation in an AML M5b. The t(8;21) has been associated with a good prognosis, but our patient suffered a fast and fatal evolution.

[Splenic lymphoma with circulating hairy lymphocytes lymphoma. Clinical cytological study of 4 patients]. / Linfoma esplénico con linfocitos vellosos circulantes. Estudio clínico citológico de cuatro pacientes.

PURPOSE: The splenic lymphoma with circulating villous lymphocytes (SLCVL) is an infrequent disease included within the low grade non Hodgkin's lymphoma, B-cell type. The results of the study of four patients are reported. PATIENTS AND METHODS: Clinical, cytological, immunophenotypic, ultrastructural, evolutive and therapeutic data have been revised in all the cases. RESULTS: Two males and 2 females of 76, 66, 68 and 62 years, respectively were diagnosed as having SLCVL. The initial symptoms were scarce, basically asthenia, and a big spleen without significant lymphadenopaty was the most relevant physical finding in each of them. In peripheral blood leukocyte count was normal with a slight lymphocytosis and a variable percentage of villous circulating lymphocytes. The immunophenotype of peripheral blood obtained by flow cytometry was according with a mature B-cell lymphocyte population, CD 5 and CD 25 negative. The cells were positive to acid phosphatase with a diffuse pattern of variable intensity; the reaction was inhibited by tartaric acid. All the patients had BM infiltration, studied with aspiration and biopsy. One case (M,66) had an IgM monoclonal gammopathy. The ultrastructural study, performed in 3 cases, showed thin and short villous prolongations. After splenectomy, a low degree lymphoma therapy has been employed in all the cases. The follow-up ranges between, 4 years and 4 months, all the patients being alive. CONCLUSIONS: The SLCVL is a definitive entity regarding the clinical, morphologic and immunophenotype features. A long clinical evolution and a good prognosis after splenectomy are common.

[Bone marrow aplasia and gold salts. Review of the literature apropos of 2 cases]. / Aplasia medular y sales de oro. Actualización de la literatura a propósito de dos casos.

Bone marrow aplasia remains the most serious adverse effect with gold therapy. Its prevalence is still a controversial issue and at present it is not possible to give accurate figures on its frequency. Two patients diagnosed of rheumatoid arthritis are reported. They underwent chrysotherapy and developed bone marrow aplasia within a 2-month period of therapy. The pathogenic mechanism of blood dyscrasias secondary to gold salts is still unknown. The best available method in the prevention of this serious condition is the periodical obtention of complete blood counts.

A new Spanish family with Hb Louisville.

The clinical, hematological, and biochemical characteristics of a new family with heterozygous hemoglobin (Hb) Louisville are described. The family showed a decrease in both oxygen affinity and cooperativity with the normal Bohr effect. This family has the greatest number of affected members reported to date. Among the descendants, two first cousins (III-10 and III-11), both of whom are affected by the heterozygous trait of Hb Louisville, had had three abortions of undetermined causes.