[Polychemotherapy in multiple myeloma. The COMPA protocol]. / Poliquimioterapia en mieloma múltiple. Esquema C.O.M.P.A.

Ninety three patients with multiple myeloma (MM) were treated with cyclophosphamide, vincristine, melphalan, prednisone and adriamycin (C.O.M.P.A.). Their median age was 60.9 years and sixty five were males. Seven patients were in stage I-A; 25 in II-A; 33 in III-A and 28 in III-B. Complete remission (CR) was achieved in 61 (65.6%), partial remission (PR) in 18 (19.3%) and no response in 14 (15%). At present, the mean survival of the CR group, is 32.3 months (10 to 78), and of the PR 11.2 months (6 to 18). The actuarial survival of the CR group is 37.9 months. A hemoglobin level lower than 8.5 g/dL, serum creatinine higher than 2.0 mg/dL, and stage III disease were factors that together negatively influenced in both response to treatment and survival. Proteinuria did not affect response, but it was a negative factor for survival. Thirty percent of deaths were due to infection, and 24.5% to myeloma activity associated with infection. We conclude that this five drug combination (C.O.M.P.A.) achieves a high percentage of complete remissions, but does not differ significantly from other reported schemes in the mean survival obtained for multiple myeloma.

Low doses of high-potency antithymocyte globulin (ATG) in severe aplastic anemia: experience with the Mexican ATG.

Twenty patients with severe aplastic anemia (SAA) were treated with low doses (1-5 mg/kg/day) of a high-potency antithymocyte globulin (ATG) produced in Mexico, shown to have at least a 10-fold potency as compared with other globulins of commercial sources. Patients received ATG within a 10-day period, every other day (5 doses) at a dose of 1 mg/kg/day (4 courses), 2 mg/kg/day (12 courses) or 5 mg/kg/day (8 courses). Four patients received 2 consecutive courses of different doses of ATG. A response rate of 42% was recorded in the group, assessed by means of increases in reticulocytes, granulocytes or platelets. One patient showed a complete remission. The 570-day survival of the group was 51%. It is concluded that the domestically produced ATG is useful in the treatment of some patients with SAA in Mexico.

[Natural history of paroxysmal nocturnal hemoglobinuria in adolescents,adults, and children: the Mexican experience]. / Historia natural de la hemoglobinuria paroxística nocturna en adolescentes, adultos y en la edad pediátrica: la experiencia mexicana.

PURPOSE: To compare the clinical patterns and survival of young and adult (AP) versus paediatric (PP) patients with paroxysmal nocturnal haemoglobinuria (PNH). PATIENTS AND METHODS: The clinical records of 117 patients (82% AP, 18% PP) seen in four cities of the Mexican Republic were analysed, the clinical course and survival of both groups being compared. RESULTS: No sex difference was found in the two patient-groups: 51% and 52% males, 49% and 48% females in AP and PP, respectively. The onset of PNH had similar distribution for the two groups of patients: aplastic form, 45% in AP and 62% in PP; cytopenias, 24% in AP versus 27% in PP; haemolysis, 28% in AP and 9% in PP, and thrombosis, 3% in AP versus 0% in PP. The clinical features with significant difference were: anaemic+haemorrhagic syndrome (39 AP (40%) vs 14 PP (67%), p = 0.02), initial diagnosis of immunologic thrombocytopenic purpura (7 AP (7%) vs 7 PP (33%), p = 0.003), and death rate (17 AP (18%) vs 8 PP (38%), p = 0.04). The actuarial survival curves showed significant differences between both groups (p = 0.045, Cox-Mantel), with estimated 10-year survival of 81% for AP and 55% for PP, and 15-year survivals of 64% for AP and 55% for PP. CONCLUSIONS: Seemingly, PNH in paediatric age has poorer prognosis than in adults, which is associated to higher incidence of fatal haemorrhages due to thrombocytopenia.

Identificación de dos genes distintos de Talasemia ß en una familia mexicana / Identification of 2 distinct genes of beta thalassemia in a Mexican family

Una mujer de 21 años de edad con antecedentes de 11 años de anemia hemolítica fue diagnosticada como heterocigota compuesta para 2 genes diferentes de talasemia ß: ß- y ß+. La madre ß-/ß con Hb A2 elevada y el padre ß+/ß con Hb A2 normal mostraron elevación en la relación de cadenas globínicas alfa/no-alfa (1.39 y 1.34, respectivamente), lo que confirmó una deficiencia de síntesis de cadenas ß. 2 hermanas con el mismo genotipo ß-/ß+, presentaron también anemia hemolítica, otras 2 fueron heterocigotas simples ß+/ß y 3 más homocigotos normales. Este estudio demuestra por primera vez la presencia del gene ß+ con Hb A2 normal en población mexicana

Trombocitopenia y enfermedad tiroidea: presentación de 11 casos y revisión de la literatura / Thrombocytopenia and thyroid disease: report of 11 cases and review of the literature

Se informan once casos de trombocitopenia asociada a enfermedad tiroidea. Todos ellos mujeres. En 5 pacientes apareció la enfermedad tiroidea antes de la trombocitopenia, en 3 se trató de hipotiroidismo que se manifestó 13 años antes y en ellos se encontró el nivel más alto de inmunoglobulina "G" asociada a las plaquetas. En 4, se presentaron ambas enfermedades al mismo tiempo y en todas se trató de hipertiroidismo. En dos se resolvieron ambos problemas con tratamiento médico y en dos fue necesaria la esplenectomía. Tres recibieron Iodo radioactivo. En 2 pacientes la trombocitopenia precedió a la enfermedad tiroidea y tuvieron hipertiroidismo las dos, en una el cuadro clínico fue característico de tiroiditis de Hashimoto que se confirmó por histopatología después de cirugía de tiroides. No recibieron Iodo radioactivo y tienen el tiempo de observación más largo (8 años), sin evidencia de enfermedad autoinmune o linfoproliferativa. Se discuten los casos a la luz de los informes de la literatura y los mecanismos etiopatogénicos propuestos para tal asociación