[Coexistence of plasmablastic lymphoma, Kaposi sarcoma and Castleman disease in a patient with HIV infection]. / Coexistencia de linfoma plasmablástico, sarcoma de Kaposi y enfermedad de Castleman en un paciente con infección por virus de inmunodeficiencia humana.

Plasmablastic lymphoma (PL) is an uncommon B-cell lymphoma that is strongly associated with human immunodeficiency virus (HIV) infection, and displays distinctive affinity for extranodal presentation in the oral cavity. We report the case of a PL involving the stomach in a 36 year-old man HIV+ patient, associated with Kaposi sarcoma (KS) in sections adjacent to lymphoma. He had a positive history of Castleman disease and KS in a lymphoid node biopsy.

Coexistencia de linfoma plasmablástico, sarcoma de Kaposi y enfermedad de Castleman en un paciente con infección por virus de inmunodeficiencia humana / Coexistence of plasmablastic lymphoma, Kaposi sarcoma and Castleman disease in a patient with HIV infection

El linfoma plasmablástico (LP) es un linfoma de células B poco común que está fuertemente asociado con la infección por el virus de inmunodeficiencia humana (VIH), y muestra una afinidad característica de presentación extra-ganglionar en la cavidad oral. Informamos el caso de un LP afectando el estómago en un paciente masculino de 36 años de edad con infección por VIH, asociado con sarcoma de Kaposi (SK) en áreas adyacentes al linfoma. Tenía el antecedente de enfermedad de Castleman y SK en una biopsia de ganglio linfático.

Plasmablastic lymphoma (PL) is an uncommon B-cell lymphoma that is strongly associated with human immunodeficiency virus (HIV) infection, and displays distinctive affinity for extranodal presentation in the oral cavity. We report the case of a PL involving the stomach in a 36 year-old man HIV+ patient, associated with Kaposi sarcoma (KS) in sections adjacent to lymphoma. He had a positive history of Castleman disease and KS in a lymphoid node biopsy.

[Clinical and pathological features of Castleman's disease: experience at the Instituto Nacional de Cancerología, Mexico City]. / Características clinicopatológicas de la enfermedad de Castleman. Experiencia en el Instituto Nacional de Cancerología.

BACKGROUND: Castleman's disease (CD) is a rare, poorly understood pathological entity. We report our experience with this clinicopathological entity. METHODS: We retrospectively analyzed records of all patients with CD from 1996 to 2003. The disease was classified as unicentric if a solitary mass was present or multicentric if generalized lymphadenopathy was present. We further subdivided the disease into hyaline vascular (HV) and plasma cell (PC) histological variants. RESULTS: We found 11 patients with CD. Six patients had unicentric disease and five had multicentric disease. Median follow-up was 40 months. All patients with unicentric disease had the HV variant. Of the five patients with multicentric disease, four had the PC variant and one had the HV. Five patients with unicentric disease were treated surgically with complete resection, and only one patient was treated with chemotherapy. All remain alive without disease. Three patients with multicentric disease were treated with chemotherapy, and two patients received chemotherapy plus radiotherapy for residual disease. Two patients received second-line chemotherapy with a favorable outcome. Two patients with a comorbid condition had a poor outcome. CONCLUSIONS: Clinical characteristics, pathological features and treatment results are similar to that reported in other populations.

Características clinicopatológicas de la enfermedad de Castleman: experiencia en el Instituto Nacional de Cancerología / Clinical and pathological features of Castleman's disease: experience at the Instituto Nacional de Cancerología, Mexico City

Introducción: La enfermedad de Castleman es una entidad patológica poco comprendida, descrita originalmente en pacientes europeos. Informamos nuestra experiencia con esta entidad clinicopatológica en pacientes del Instituto Nacional de Cancerología de la Ciudad de México. Material y métodos: Analizamos retrospectivamente los expedientes de pacientes con enfermedad de Castleman de 1996 a 2003. La enfermedad fue monocéntrica si había solo un ganglio o multicéntrica si se encontraba linfoadenopatía generalizada. Además, se dividió en las variantes histológicas hialinovascular y de células plasmáticas. Resultados: Once pacientes con enfermedad de Castleman fueron diagnosticados en el periodo referido, seis tenían enfermedad monocéntrica y cinco multicéntrica. La mediana de seguimiento fue de 40 meses. Todos los pacientes con enfermedad monocéntrica tenían la variante hialinovascular. De los cinco con multicéntrica, cuatro tenían la variante de células plasmáticas y uno la hialinovascular. Cinco pacientes con enfermedad monocéntrica se trataron con cirugía y uno con quimioterapia; al momento de este informe todos permanecían vivos y sin enfermedad. Tres pacientes con enfermedad multicéntrica recibieron quimioterapia y dos, quimioterapia más radioterapia por enfermedad residual; a dos pacientes se les prescribió quimioterapia de segunda línea, con buena respuesta. Dos pacientes con una condición asociada evolucionaron desfavorablemente. Conclusiones: Las características clínicas, patológicas y los resultados del tratamiento son similares a los señalados en otras poblaciones.

BACKGROUND: Castleman's disease (CD) is a rare, poorly understood pathological entity. We report our experience with this clinicopathological entity. METHODS: We retrospectively analyzed records of all patients with CD from 1996 to 2003. The disease was classified as unicentric if a solitary mass was present or multicentric if generalized lymphadenopathy was present. We further subdivided the disease into hyaline vascular (HV) and plasma cell (PC) histological variants. RESULTS: We found 11 patients with CD. Six patients had unicentric disease and five had multicentric disease. Median follow-up was 40 months. All patients with unicentric disease had the HV variant. Of the five patients with multicentric disease, four had the PC variant and one had the HV. Five patients with unicentric disease were treated surgically with complete resection, and only one patient was treated with chemotherapy. All remain alive without disease. Three patients with multicentric disease were treated with chemotherapy, and two patients received chemotherapy plus radiotherapy for residual disease. Two patients received second-line chemotherapy with a favorable outcome. Two patients with a comorbid condition had a poor outcome. CONCLUSIONS: Clinical characteristics, pathological features and treatment results are similar to that reported in other populations.

Allografting in patients with severe, refractory aplastic anemia using peripheral blood stem cells and a fludarabine-based conditioning regimen: the Mexican experience.

We studied the effectiveness of a fludarabine/cyclophosphamide-based conditioning regimen without anti-thymocyte globulin in 23 aplastic anemia patients who had no response to previous conventional pharmacologic treatment. Patients received oral busulphan 4 mg/kg/day/2 days, IV cyclophosphamide 350 mg/m(2)/day/3 days, and fludarabine 30 mg/m(2)/day/3 days. For GVHD prophylaxis, patients received MTX 5 mg/m(2) days +1, +3, +6, and +11 and oral cyclosporin A (CyA) 5 mg/kg/day, starting on day -1. Peripheral blood stem cell products were used with a median dose of 5.5 x 10(6) CD34(+)/kg. The patients were followed for an average of 25 months. By a median of day +11, an ANC > 0.5 x 10(9)/L was reached; and by day +12, the platelet count had reached >20,000 x 10(9)/L. Acute grade I-II GVHD occurred in 4 patients, whereas limited chronic GVHD presented in 6 cases. Twenty-one patients (91.3%) achieved engraftment. Two patients failed to engraft, and 4 developed late rejection; 2 of these individuals died, 2 have survived with high transfusion requirements, whereas 2 received a second peripheral blood stem cell infusion and achieved sustained engraftment. Currently 21 (91%) of the 23 patients are alive, whereas 19 of 21 (90%) remain in complete remission. The average cost was about USD 15,000 for this kind of reduced-intensity allotransplant. Reduced-intensity stem cell transplantation represents an affordable alternative to traditional more cytotoxic conditioning for severe aplastic anemia (SAA) patients. Long-term effects however, remain to be evaluated.

[Primary testicular lymphomas. Clinicopathologic analysis of 10 cases. Experience at the National Institute of Cancerology in Mexico City]. / Linfomas primarios de testículo. Análisis clínicopatológico de 10 casos. Experiencia en el Instituto Nacional de Cancerología.

UNLABELLED: Non-Hodgkin lymphoma is the most common primary testicular neoplasm of older men but is rare in young men. The vast majority of primary testicular lymphomas (PTL) are intermediate- to high-grade lymphomas. OBJECTIVE: To describe the clinical, morphologic, and immunophenolypic characteristics of PTL seen in a referral center. MATERIAL AND METHODS: We reviewed the cases of PTL seen from 1986 to 1999. We obtained data of laboratory, tests, clinical course, treatment, and immunohistochemical studies. RESULTS: 10 patients with diagnosis of PTL were identified. Median age was 62.3 years (range 42-81 years), and nine patients underwent orchiectomy as initial therapeutic procedure. Other treatment modalities after diagnosis included combination chemotherapy (60%) and combination radiotherapy (20%). Histologically, testes showed diffuse dense infiltration of large lymphoma cells. All cases were classified as diffuse large cell lymphoma according to REAL classification. Eight cases were studied with use of paraffin-section immunoperoxidase, 7/8 tumors were B-lineage lymphomas, and one was a T-lineage lymphoma.

Linfomas primarios de testículo: Análisis clínicopatológico de 10 casos. Experiencia en el Instituto Nacional de Cancerología

El linfoma no-Hodgkin (LNH) es la neoplasia testicular más común en hombres viejos y rara en hombres jóvenes. La gran mayoría de los linfomas primarios de testículo (LPT) es clasificada como linfomas de grado intermedio o alto. Objetivo: describir las características clínicas, morfológicas e inmunofenotípicas de los LPT atendidos en un hospital de referencia. Material y métodos: de 1986 a 1999 se revisaron los casos de LPT. Se analizaron los datos clínicos, estudios de laboratorio, evolución, tratamiento y se realizaron cortes en blanco para estudios de inmunohistoquímica (IH). Resultados: se incluyeron 10 pacientes con diagnóstico de LPT. El promedio de edad fue de 62.3 años (margen 42-81), en nueve pacientes se realizó orquiectomía como modalidad terapéutica inicial. Otros tratamientos posteriores al diagnóstico fueron; quimioterapia (60%) y radioterapia (20%). Histológicamente, los testículos mostraron infiltración difusa por células grandes de estirpe linfoide. Basados en la clasificación de la REAL, todos los casos fueron clasificados como linfomas difusos de células grandes. En ocho casos se realizó estudio de IH; 7/8 tumores expresaron inmunofenotipo B y sólo un caso inmunofenotipo T.

Non-Hodgkin lymphoma is the most common primary testicular neoplasm of older men but is rare in young men. The vast majority of primary testicular lymphomas (PTL) are intermediate- to high-grade lymphomas. Objective: to describe the clinical, morphologic, and immunophenotypic characteristics of PTL seen in a referral center. Material and Methods: we reviewed the cases of PTL seen from 1986 to 1999. We obtained data of laboratory tests, clinical course, treatment, and immunohistochemical studies. Results: 10 patients with diagnosis of PTL were identified. Median age was 62.3 years (range 42-81 years), and nine patients underwent orchiectomy as initial therapeutic procedure. Other treatment modalities after diagnosis included combination chemotherapy (60%) and combination radiotherapy (20%). Histologically, testes showed diffuse dense infiltration of large lymphoma cells. All cases were classified as diffuse large cell lymphoma according to REAL classification. Eight cases were studied with use of paraffin-section immunoperoxidase, 7/8 tumors were B-lineage lymphomas, and one was a T-lineage lymphoma.

Delayed administration of G-CSF until day +7 after autologous peripheral blood stem cell transplant is as effective in accelerating hematopoietic as day 0.

PURPOSE: To compare the hematopoietic recovery and the clinical outcome after high dose chemotherapy and peripheral blood stem cell (PBSC) transplant between patients who received recombinant granulocyte colony-stimulating factor (G-CSF) starting on day 0 and those who received it starting on day +7. PATIENTS AND METHODS: Thirty-five consecutive patients received high dose chemotherapy with ifosfamide, etoposide and carboplatin (ICE) and autologous PBSC transplant. PBSC mobilization was achieved with G-CSF. In 19 transplants done in 1994, G-CSF was started on day 0 and in 16 procedures done in 1995, G-CSF was started on day +7. In both groups G-CSF was given until the absolute neutrophil count was 1 x 10(9)/L. RESULTS: The median number of days to reach an absolute neutrophil count > 0.1 x 10(9)/L, was 11 in the patients who started the G-CSF on day 0 and 11 days in the patients with delayed administration of G-CSF and to reach > 0.5 x 10(9)/L 14 days and 13 respectively. The median number of days to reach a platelet count > 20 x 10(9)/L was 14 and 13. Comparison of early vs. delayed administration of G-CSF did not show any significant differences regarding the time to achieve platelet independence, number of days with fever, median days of hospital stay, number of red cell or platelet transfusions. CONCLUSIONS: Starting G-CSF on day +7 after high dose ICE chemotherapy and autologous transplant of PBSC is as effective in restoring the hematopoietic function as is starting its administration on day 0.

Allogeneic peripheral blood stem cell transplantation. Safety, hematologic recovery, kinetics, and complications.

BACKGROUND: Hematopoietic stem cell transplants are an accepted treatment for several malignant and nonmalignant hematologic diseases. Recently, the peripheral blood, after mobilization of stem cells with growth factors, has become the source of choice for hematopoietic stem cells. We report on a series of patients who received peripheral blood stem cell transplants at the Instituto Nacional de Cancerologia (INCAN) in Mexico City. METHODS: Between May 1995 and December 1999, 33 patients received peripheral blood stem cell transplants to treat hematologic diseases. Sixty percent of our patients had chronic myelocytic leukemia (CML). All had a matched related donor. Patients were conditioned with one of five different conditioning regimens and subsequently received one of two different graft-vs.-host disease prophylaxis regimens. Stratified Wilcoxon rank-sum, chi square, and Mann-Whitney tests were used to analyze the results. RESULTS: In our series, median time to achieve a total neutrophil count of 0.5 x 10(9)/L was 14 days and to achieve a platelet count of 20 x 10(9)/L, 15 days. Acute graft-vs.-host disease occurred in seven patients. Chronic graft-vs.-host disease occurred in 69% of surviving patients. Survival for low-risk patients was 67% and for the high-risk group, 9%. CONCLUSIONS: Peripheral blood stem cells produce a faster hematopoietic recovery. The rate of acute graft-vs.-host disease is not increased using the peripheral blood as source of stem cells; however, chronic graft-vs.-host disease continues to be a significant problem. Donors tolerated the procurement procedure without complications.

Delayed administration of G-CSF until day +7 after autologous peripheral blood stem cell transplant is as effective in accelerating hematopoietic as day 0

PURPOSE: To compare the hematopoietic recovery and the clinical outcome after high dose chemotherapy and peripheral blood stem cell (PBSC) transplant between patients who received recombinant granulocyte colony-stimulating factor (G-CSF) starting on day 0 and those who received it starting on day +7. PATIENTS AND METHODS: Thirty-five consecutive patients received high dose chemotherapy with ifosfamide, etoposide and carboplatin (ICE) and autologous PBSC transplant. PBSC mobilization was achieved with G-CSF. In 19 transplants done in 1994, G-CSF was started on day 0 and in 16 procedures done in 1995, G-CSF was started on day +7. In both groups G-CSF was given until the absolute neutrophil count was 1 x 10(9)/L. RESULTS: The median number of days to reach an absolute neutrophil count > 0.1 x 10(9)/L, was 11 in the patients who started the G-CSF on day 0 and 11 days in the patients with delayed administration of G-CSF and to reach > 0.5 x 10(9)/L 14 days and 13 respectively. The median number of days to reach a platelet count > 20 x 10(9)/L was 14 and 13. Comparison of early vs. delayed administration of G-CSF did not show any significant differences regarding the time to achieve platelet independence, number of days with fever, median days of hospital stay, number of red cell or platelet transfusions. CONCLUSIONS: Starting G-CSF on day +7 after high dose ICE chemotherapy and autologous transplant of PBSC is as effective in restoring the hematopoietic function as is starting its administration on day 0.

GST-pi expression in BCR-ABL+ and BCR-ABL- cells from CML patients

En nuestro laboratorio durante varios años se ha estudiado a la enzima glutatión-sulfhidrilo-transferasa-pi (GST-pi). Experimentos recientes mostraron que la GST-pi es expresada en diferentes etapas de maduración celular durante la hematopoyesis en cultivo líquido de CPMO de pacientes con LMC candidatos a alotrasplante. Sugiriendo que la expresión de la GST-pi fue en células malignas. En el presente trabajo, confirmamos lo anterior mediante la detección por inmunofluorescencia de la GST-pi en células BCR-ABL+ y BCR-ABL- evaluadas por FISH en SP de 30 pacientes con LMC, durante diferentes etapas clínicas: tratamiento (T), recaída hematológica (R), crisis blástica (CB) y post-alotrasplante (PT). Así como en SP de 30 donadores del Banco de Sangre del Instituto. Los resultados, expresados como porcentaje de células, fueron: BCR-ABL+ GST-pi+: T= 1-67 por ciento, R= 33-69 por ciento, BC= 90-100 por ciento y PT= 1-2 por ciento; BCR-ABL- GST-pi+: T= 2-31 por ciento, R= 5-18 por ciento, BC= 0-10 por ciento y PT= 2-5 por ciento; BCR-ABL- GST-pi-: T= 2-97 por ciento, R= 13-62 por ciento, BC= 0 por ciento y PT= 93-96 por ciento; BCR-ABL+ GST-pi-: T= 0 por ciento, R= 0 por ciento, BC= 0 por ciento y PT= 0 por ciento. La GST-pi no se expresó en las células de los donadores. Los resultados obtenidos confirman nuestras observaciones previas y sugieren que la expresión de la GST-pi podría usarse para evaluar la enfermedad mínima residual en los pacientes con LMC.

experiencia con 22 donadores de médula ósea / Experience with 22 bone marrow donors

Describimos nuestra experiencia en el estudio protocolizado de donadores sanos del programa de trasplante alogénico de médula ósea del Instituto Nacional de Cancerología de México. Se estudiaron 22 donadores sanos y 23 procedimientos de colección de médula ósea. Fueron 7 hombres y 15 mujeres, con edades de 16 a 47 años. Todos ellos fueron negativos a VIH, HB y HC. Se colectaron volúmenes de médula ósea de 750 a 1500 mL. Catorce donadores presentaron reducción de hemoglobina > 3.0 g/dL pero sólo la mitad de ellos requirieron transfuusión autóloga. Todos recibieron un programa de analgésicos con meperidina, dextropropoxifeno y ketoprofeno. Sólo dos donadores presentaron complicaciones (9 por ciento). Actualmente todos se encuentran en buenas condiciones de salud. Concluímos que la donación de médula ósea mostró ser un procedimiento seguro, con la complicación más frecuente de reducción de hemoglobina circulante que es fácilmente corregible con transfusión de sangre autóloga

Chronic myelocytic leukemia in accelerated phase with i(17) (q10) and loss p53 gene. Case report

Chronic myelogenous leukemia (CML) is a clonal disorder that presents with a stable period followed by an accelarated phase. The most frequent chromosomal abnormality described is t(9;22). Alterations of chromosome 17, where p53 is located, have been described during transformation. We studied a 23-year-old male who presented with chronic myelogenous leukemia. The karyotype demonstrated 46,XY,t(9;22) (q34;qll) in 12 percent of mitoses and hyperdiploidy in 43 percent. Forty six months later the patient suffered a blast crisis characterized by absolute basophilia; the cytogenetic study demonstrated 48,XY,+8,t(9;22(q34;qll), +der(22)t(9;22)(q34;qll),+i(17)(q10) in 18 percent of the mitoses, 46,XY,t(9;22) (q34;qll) in 34 percent and hyperdiploidy in 23 percent. Since there was i(17)(q10) during this stage, a retrospective DNA study of the biopsy material before and after the transformacition was performed. In the chronic phase, p53 was present in normal amounts, during transformation there was loss of genetic material from the p53 region. The protein product of suppressor gene p53 normally works holding the proliferation of cells. When there is the formation of an isochromosome, genetic material is lost; thus, in this patient, p53 was delted upon the observation of i(17). Lastly, this case shows how DNA can be extracted from slides; this technique is novel and can be used for retrospective studies when parafin block or fresh tissue are not available

Prevención de mielotoxicidad en un caso de transplante medular con factor estimulante de colonias granulacito-macrófago asociado a ganciclovir / Use of GM-CSF as prophylaxis of ganciclovir-induced severe bone marrow suppression

Se informa la primera experiencia mexicana con el uso de factor estimulante de colonia granulocito-macrófago (GM-CSF) com profilaxis de la neutropenia secundaria a ganciclovir, en la prevención de la enfermedad por citomegalovirus (CMV) en un paciente CMV sero-positivo con leucemia mieloide aguda en primera remisión, trasplantando con donador HLA idéntico y CMV sero-positivo. La toma de injerto ocurrió el día 14. Se inició ganciclovir 5 mg/kg/3 veces por semana) en el día 35 acompañandose de toxicidad medular secundaria 28 días después con neutropenia grave que remitió de manera espontánea posterior a la suspensión del mismo. A fin de concluir el esquema de profilaxis, se inició GM-CSF a dosis de 300 mg/kg/día concomitante al ganciclovir a dosis de 5 mg/kg/día con lo cual fue posible conluir tratamiento sin que se reindujera toxicidad medular. No hubo evidencia de enfermedad de injerto contra huésped ni de infección por CMV. La evolución del paciente fue satisfactoria durante un año, posterior al cual, presentó recaída de su enfermedad de base mueriendo por complicaciones secundarias a leucemia