[Donor cell leukemia (DCL): A prospective study of its identification and treatment]. / Leucemia en células del donador (LCD): un estudio prospectivo de su identificación y tratamiento.

Donor-derived malignancies after allogeneic hematopoietic stem cell transplantation and after solid organ transplantation are considered as rare diseases. We have prospectively searched for donor cell leukemia in a 12-year period, in a single institution, in a group of 106 consecutive patients allografted because of leukemia. We have identified seven cases of donor cell leukemia; six were allografted because of relapsed acute lymphoblastic leukemia and one because of paroxysmal nocturnal hemoglobinuria/aplastic anemia. These figures suggest that the real incidence of donor cell leukemia has been underestimated. The six patients with lymphoblastic donor cell leukemia were treated prospectively with a pediatric-inspired combined chemotherapy schedule designed for de novo acute leukemia. A complete response was obtained in three out of six patients with lymphoblastic donor cell leukemia. It is possible to obtain favorable responses in donor cell leukemia patients employing combined chemotherapy. The long-term donor cell leukemia survivors remain as full chimeras and have not needed a second transplant.

Health-Related Quality of Life in leukemia Survivors of Allogeneic Hematopoietic Stem Cell Transplantation Employing the Mexican Reduced-Intensity Conditioning.

BACKGROUND: Quality of life (QOL) is an important consideration in the counseling, implementation, and post-treatment management of arduous treatments for life-threatening conditions such as allogeneic hematopoietic cell transplantation (allo-HCT). OBJECTIVE: To analyze the QOL of leukemia patients allografted with the Mexican reduced-intensity conditioning regimen in two Mexican academic medical centers. MATERIAL AND METHODS: By means of the quality metric short form 36 version 2 to measure generic health concepts, relevant QOL was analyzed in leukemia patients who underwent allo-HCT using reduced-intensity conditioning on an outpatient basis at either the Centro de Hematología y Medicina Interna de Puebla of the Clínica Ruiz or the Hematology Service of the Internal Medicine Department of the Hospital "Dr. José Eleuterio González" of the Universidad Autónoma de Nuevo León, and who had survived more than 12 months after the allograft, who could be approached, who were in a continued complete remission (with or without graft-versus-host disease), and who were willing to respond to the questionnaire. Thirty-five patients fulfilling these requirements were included, and a sex- and age-matched group of 35 reference subjects was also studied. RESULTS: Allografted patients were found to have a slightly better mental component summary than the reference subjects (53.23 vs. 48.66 points; p = 0.01), whereas the physical component summary did not show a difference (54.53 vs. 52.05 points; p = 0.59). Most of the differences between allografted individuals and reference subject controls were not significant. CONCLUSIONS: Despite several sources of bias, these data suggest that allografted individuals employing the Mexican reduced-intensity conditioning regimen enjoy a health-related QOL life similar to that of reference subjects, adding another advantage of this method of conducting stem cell allografts. However, more work needs to be done to elucidate the impact of reduced-intensity conditioning on post allo-HCT QOL.

Primary thrombophilia in México X: a prospective study of the treatment of the sticky platelet syndrome.

INTRODUCTION: The sticky platelet syndrome (SPS) is a common cause of thrombosis. There are no prospective studies concerning treatment. OBJECTIVE: To analyze changes in platelet hyperaggregability of patients with SPS who were given antiplatelet drugs and to assess its association with rethrombosis. METHODS: A total of 55 patients with a history of thrombosis and SPS phenotype were prospectively studied before and after treatment with aspirin and/or clopidogrel. RESULTS: Patients were followed for 1 to 129 months, median 13. Of 55 patients, 40 received aspirin, 13 received aspirin + clopidogrel, and 2 received only clopidogrel. The platelet aggregation response to adenosine diphosphate and epinephrine significantly diminished after treatment, and only 2 patients developed rethrombosis 52 and 129 months after starting therapy, with the freedom from rethrombosis rate of the patients being 96.4% at 129 months. CONCLUSION: Using antiplatelet drugs, the platelet hyperreactivity of patients with the SPS phenotype was reverted; and this translated into a substantial decrease in the rethrombosis rate.

Oral versus intravenous fludarabine as part of a reduced-intensity conditioning for allogeneic stem cell transplantation.

BACKGROUND: In 2003, oral fludarabine was introduced in the USA for the treatment of patients with hematologic malignancies as an alternative to its intravenous (i.v.) formulation; in 2008, it was introduced in México while the i.v. formulation was withdrawn. Accordingly, i.v. fludarabine had to be replaced by oral fludarabine as part of the conditioning regimen employed to conduct allogeneic stem cell transplantation in México. METHODS: Nonrandomized retrospective analysis of 55 patients conditioned with oral fludarabine compared with 113 patients conditioned with the i.v. formulation. In addition to fludarabine, the conditioning regimen included oral busulfan and i.v. cyclophosphamide. Donors were HLA-matched siblings. RESULTS: The clinical features of the two groups were comparable. There were no statistical differences in time to neutrophil engraftment, time to platelet engraftment, acute graft versus host disease rate and nonrelapse mortality at day 100. The overall survival of patients allografted with oral fludarabine was better than those allografted with i.v. fludarabine: 62 and 33% at 67 months, respectively (p = 0.0006). DISCUSSION: Oral fludarabine can replace its i.v. formulation as part of reduced-intensity conditioning regimens with no deleterious effect on any of the early transplantation outcomes.

Poor performance of the total kappa/lambda light chain quantification in the diagnosis and follow-up of patients with multiple myeloma.

BACKGROUND: The gold standard for paraproteinemia screening in plasma cell disorders has been serum protein electro- phoresis (SPE) with immunofixation electrophoresis (IFx); serum total and free light chain quantifications have also been used. OBJECTIVE: To define the role of SPE, IFx and serum total light chain (sLC) determinations in patients with multiple myeloma (MM), both at diagnosis and at maximum response during treatment follow-up. MATERIAL AND METHODS: These serological studies were performed in a group of 62 patients with MM at diagnosis, and in a subset of 29 patients at the point of maximum response to treatment. RESULTS: At diagnosis, we found an abnormal SPE in 58%, an abnormal IFx in 92% and an abnormal sLC in 45% of the 62 patients; 64% had simultaneously abnormal results in all three serological studies. IFx alone proved to be the most sensitive of all three assays, followed by SPE, which was redundant in most instances with sLC and IFx. At maximum response, the abnormal SPE normalized in 7 cases, the abnormal IFx in 7 cases and the abnormal sLC in 7 cases. There were 12 instances in which an abnormal IFx was found despite normal sLC, and one case in which a normal IFx was found in the presence of abnormal sLC. The association between IFx and sLC was highly significant (r = 0.9274611, p < 0.000001), despite instances where a positive result for IFx was associated to a normal sLC. CONCLUSION: All three serological methods should ideally be simultaneously performed in patients with MM both at diagnosis and throughout therapy. In this series, the total sLC assay was not more sensitive than IFx neither at diagnosis nor during follow-up.

Systemic immunoglobulin light-chain amyloidosis (AL) in Mexico: a single institution, 30-year experience.

MATERIAL AND METHODS: In a 30-year period in a single institution, 23 cases of systemic immunoglobulin light chain amyloidosis (AL) were identified, within a group of 1,388 individuals with some form of a hematological malignancy. RESULTS: AL is 14 times less frequent in Mexico than in Caucasians and it represents 15% of all monoclonal gammopathies. Median age was 57 years (range 39-98); there were 11 males and 12 females. The histologic diagnosis was done in the periumbilical fat in 39%, the bone marrow in 30%, the kidney in 13%, the gastrointestinal tract in 13% and in a lymph node in one case. The nephrotic syndrome was present in 61% of cases, heart failure in 35%, sensorimotor peripheral neuropathy in 26% and weight loss in 6%. Anemia was present in 14% of cases at diagnosis; median hemoglobin was 11 g/dL. An abnormal monoclonal spike in the peripheral blood was present in 70% of cases; it had a median of 1.2 g/dL (range 0.2-3.6); there were 7 cases of light-chain only disease and five in whom an abnormal paraproteinemia was not found. Six cases were associated with overt multiple myeloma. Seventeen individuals (74%) were followed for more than 3 months (range 90 to 5190 days, median 210); their overall survival (OS) was 71% at 173 months, whereas the median OS has not been reached, being above 173 months. Eight patients were treated with melphalan/predisone and five were given high dose chemotherapy and an autologous stem cell transplantation; the others were given other treatments. CONCLUSIONS: AL is less frequent in Mexican mestizos and probably underrecognized; the clinical features of the disease are not significantly different from those informed from other populations.