Unusual morphologic presentations of blastic plasmacytoid dendritic cell neoplasm: report of two cases misdiagnosed as melanoma and leprosy.

We present 2 cases of blastic plasmacytoid dendritic cell neoplasm (BPDCN) showing unusual histological features. One patient, a 73-year-old male, presented with a nonpruritic macular erythema of the skin on the anterior and posterior chest wall, the biopsy of which was originally diagnosed as malignant melanoma. The neoplastic cells were negative for S100 and HMB45 and strongly positive for CD45, CD4, CD56, and CD123. The final diagnosis was a BPDCN associated with abundant melanin pigment and numerous melanophages. The second patient was a 73-year-old male with a 5-month history of small, slowly enlarging, bruise-like plaques on his limbs and chest. Histologic examination of the skin biopsy revealed an atypical cellular/myxoid infiltrate with numerous macrophages, which was originally diagnosed as consistent with lepromatous leprosy. The atypical cells were immersed in an alcian blue-positive myxoid matrix at pH 2.5. The Fite-Faraco stain was negative. Positive immunoreactivity was demonstrated for CD4, CD56, and CD123. Based on the histopathology and immunohistochemistry findings, a diagnosis of BPDCN with prominent myxoid matrix was rendered.

[Primary bone anaplastic large cell lymphoma]. / Linfoma anaplásico de células grandes. Primario de hueso.

INTRODUCTION: Anaplastic large cell lymphoma (ALCL) is a neoplasia of T-cell or null-cell origin accounting for approximately 3% of non-Hodgkin lymphomas in adults and 10-30% of lymphomas in children. It involves the lymph nodes as well as various extranodal sites, but the involvement of bone as either the primary or secondary site of spread, is rare. CASE REPORT: A 47-year-old male presented with an ALCL and multiple bone lesions involving the vertebrae, ribs, and iliac bone, with no other site of involvement. Histologic findings included interstitial and focal bone infiltrate consisting of large pleomorphic cells with an inflammatory background and marked fibrosis. There was immunohistochemical expression of CD30, EMA, CD45RO, CD43, CD8, Glut-1 and ALK-1. DISCUSSION: There are 20 reported cases of ALCL (T/null cell) arising from the bone. To diagnose primary bone lymphoma it is necessary to first exclude systemic lymphoma of the lymph nodes or the skin with bone spread. The differential diagnoses include osteomyelitis, small round cell tumors, mainly Ewing sarcoma/PNET, metastatic carcinoma, melanoma, Hodgkin lymphoma and Langerhans' cell histiocytosis. In this case, the differential FDG accumulation in the lesions observed in the PET-CT was associated with the degree of Glut-1 expression.

Kinetics of hematopoiesis in bone marrow cultures from patients with chronic myeloid leukemia: effect of recombinant cytokines in dexter-type long-term cultures.

Chronic myeloid leukemia (CML) is a hematological neoplasia that results from the transformation of a hematopoietic stem cell. It is characterized by the expansion of the myeloid lineage, which results in the accumulation of mature and immature granulocytes in peripheral blood and bone marrow. However, when CML marrow cells are cultured in Dexter-type long-term cultures (LTMC) hematopoiesis is defective and can be sustained for only a few weeks. One possible explanation for the deficient growth of hematopoietic cells in CML LTMC is that some factors that act as key regulators of hematopoiesis are absent in this experimental system. Thus, we tested this hypothesis by adding recombinant cytokines to these cultures. As a first approach, we added recombinant human granulocyte-macrophage colony stimulating factor (rhGM-CSF), rhGranulocyte-CSF (rhG-CSF) and rhErythropoietin (rhEPO); each factor was added individually once a week. Addition of rhGM-CSF and rhG-CSF resulted in a significant increase in the levels of nucleated cells and myeloid progenitors; the highest effects were seen in the presence of rhGM-CSF. Interestingly, such a cytokine also induced a significant decrease in the levels of erythroid progenitors. Recombinant hEPO had no significant effects on nucleated cells or myeloid progenitors, however, it induced a significant, although transient, increase in the levels of erythroid cells. The above results indicate that the hematopoietic regulators used here (rhGM-CSF, rhG-CSF and rhEPO) are capable of stimulating the growth of hematopoietic cells in LTMC from CML patients. Thus, this study demonstrates that it is, indeed, possible to manipulate CML LTMC by the addition of recombinant cytokines; this observation may be of particular relevance, since this in vitro experimental system has already been used as a method for purging of leukemic cells in autologous transplant settings. By using specific recombinant hematopoietic modulators it might be possible to make LTMC a more efficient system for such a clinical purpose.

High doses of dexamethasone in adult patients with idiopathic thrombocytopenic purpura.

BACKGROUND: High-dose dexamethasone (DXM) has been used in treatment of patients with idiopathic thrombocytopenic purpura (ITP) who are refractory to other treatments such as prednisone and splenectomy; nevertheless, different studies show variable success rates, this postulated as possibly being due to racial differences. The objective of this study was to determine DXM effectiveness at high doses in Mexican mestizo adult patients diagnosed with ITP with and without splenectomy. METHODS: Nonhospitalized adult patients with ITP were included, eight patients previously splenectomized (group 1) and 11 who had not undergone splenectomy (group 2). Patients received DXM 40 mg/day intravenously (i.v.) during 4 consecutive days every 4 weeks until six cycles were completed. RESULTS: There were no differences between the two groups regarding age (mean 39 vs. 33 years of age) and initial platelet count (M 17 vs. 24 x 10(9)/L). Median evolution time was 84 months for group 1 and 7 months for group 2 (p = 0.002). Of 19 patients, nine achieved a favorable response (FR), six belonged to group 1, and three to group 2 (Fisher p = 0.07). Nevertheless, after 6 months only two group 1 patients and two group 2 patients maintained FR (Fisher exact test p = 1). Patients achieving FR to initiation of second cycle maintained FR at the end of six cycles. CONCLUSIONS: Thus, the previously mentioned high-dose DXM therapy appears to be useful for both patients with ITP with and without splenectomy and high-dose DXM appears to be a good alternative therapy for postsplenectomy and relapse patients. However, duration of FR to treatment was brief; therefore, other treatment plans might be required to achieve longer remission duration. Response was similar to that observed in other studies carried out in different populations; thus, apparently no genetic or racial variations exist. In addition, whether patients not responding after second cycle should continue until completing the 6-month plan or should try a different therapeutic approach must be considered in the treatment plan.

Valoración de los efectos indeseables de una heparina de bajo peso molecular, durante la profilaxia de enfermedad tromboembólica en pacientes de cirugía abdominal / Appraisal of the Undesirable effects of low molecular weight heparin during thromboembolic illeness prophylaxis in abdominal surgery patients

El objetivo del presente estudio fue determinar el tipo frecuencia e intensidad de los efectos indeseables de una heparina de bajo peso molecular (HBPM) en comparación con heparina estándar (HE), durante la profilaxia de enfermedad tromboembólica (ETE), en pacientes de cirugía mayor abdominal. Se estudiaron 27 hombres y 30 mujeres; todos con factores de riesgo para ETE, incluyendo neoplasias digestivas. Diecisiete (Grupo A) recibieron HBPM 20 mg-día; 20 (Grupo B) HE 5 mil U/12 horas y el resto (Grupo C) formó el grupo control. No se encontraron diferencias en lo referente a edad, variables antropométricos, tipo y duración de la cirugía; tampoco en el de eritrocitos, inicio de la ambulación y días de estancia posoperatoria, entre los tres grupos. En el posoperatorio, en el grupo A se determinó, aumento significativo de plaquetas (p=0.01) y acortamiento del tiempo de tromboplastina parcial (p=0.001) con respecto a los grupos B y C

Niveles séricos de eritropoyetina en sujetos sanos estudiados en el valle de México / Erythropoietin seric levels in healthy student subjects in the Valley of Mexico

La eritropoyetina (EPO) es el factor humoral directamente resposable de la eritropoyesis, y su secreción se relaciona con el grado de oxigenación tisular. En altitudes por arriba del nivel del mar; las concentraciones de oxígeno son menores, lo que podría influir en los niveles séricos de EPO y de esta manera explicar el aumento de las cifras de hemoglobina y hematócrito. Por otro lado, la determinación de sus niveles sanguíneos es de gran importancia tanto en el diagnóstico diferencial de las anemias, como en el estudio de la eritrocitosis, en especial la policitemia rubra vera, enfermedad en que sus niveles son normales o bajos. Por tal motivo estimamos conveniente determinar las cifras que podrían ser consideradas como valores de referencia en población residente en la ciudad de México. Se estudió un total de 220 sujetos sanos, 168 hombres y 52 mujeres. Los niveles de EPO sérica, determinados por radioinmunoanálisis resultaron, en todo el grupo, con una mediana (Md) de 7.5 mU/ml, con un intervalo percentilar, (IP) de 1 a 18; en el sexo masculino la Md fue de 7.6 con un IP de 1 al 18 y en el sexo femenino Md de 7.5 con un IP de 1 a 16.9

Rhabdomyolysis in patients with acute lymphoblastic leukemia

The objective of this paper is to report 5 cases of rhabdomyolysis (RML) in patients with acute leukemia (AL). This occurred consecutively after the administration of chemotherapy, during the ensuing period of myelosuppression. Thirty-six patients with AL received, in a three-month period, 51 cycles of combined chemotherapy which included, in all of them, cytosine arabinoside (ARA-C); among them, along with myelosuppression, five experienced fever, infectious complications, gastrointestinal tract symptoms and severe myalgias. Serum creatine kinase (CK), liver function tests and a light microscopy muscle biopsy were performed on all of them. Ten - 17 days after receiving chemotherapy, five patients (4 males and 1 female) with acute lymphoblastic leukemia developed incapacitating myalgias in neck, thighs and arms. CK and/or alanine aminotransferase and spartate aminotransferase were increased 5-24 times above the normal range in four of these patients, and the muscle byopsy showed focal RML in all five. Myalgias were self-limited and lasted 4-10 days. In addition to the chemotherapy, other factors known to be capable of producing RML, such as sepsis, other medications, and dehydration were found. In conclusion, myalgias were due to focal RML produced probably by a combination of factor, particularly the chemotherapy along with dehydration due to gastrointestinal complications, infection, and the use of diverse antibioticas. The endemic nature of the finding in such a short period of time is outstanding

Cinco episodios de anemia aplástica transitoria en tres enfermos / Five episodes of transiet aplastic anemia in three patients

Se informan cinco episodios de anemia aplástica (AA) grave en tres enfermos, dos mujeres y un varón, de 32, 56 y 41 años de edad, respectivamente, el último con tres episodios. Fueron relacionados con exposición a insecticidas, solventes y fármacos (trimetoprim con sulfametoxazol, pirimetamina y derivados de pirazolanas). Acudieron con padecimintos de dos semanas de evolución con anemia, púrpura mucocutánea y en tres episodios fiebre. Como tratamiento en tres casos se utilizó prednisona y danazol por 10 días, así como antibióticos. En todos los casos se observó recuperación espontánea al cabo de 16 a 45 días que no se correlacionó con infección viral, hemoglobinopatía o evidencia de proceso mieloproliferativo. Se diagnósticaron como AA transitoria, variante de la AA con recuperación temprana y completa de la hematopoyesis

Treatment results of 23 cases of severe aplastic anemia with lymphocytapheresis

We report the results of 23 patients with aplastic anemia (AA) treated with a program of 14 lymphocytapheresis (LC). Treatments were performed with apheresis machines, models Haemonetics 30-S and Baxter CS3000, using the standard program. This procedure was done because AA in many cases appears as a result of the action of a T cell population that inhibits hematopoiesis. Theorically, removal of this clonal population would produce hematopoietic recovery. Of the total of 23 patients, 9 were excluded for final evaluation of treatment results because 7 died during or shortly after treatment (0.7-3 months); one patient abandoned treatment after three LC and another died 7 months later because of transformation to acute leukemia. The ramaining 14 patients were included in the final evaluation of treatment; seven females and seven males, average age 46.1 years (range 22-69); 13 with severe, and one with moderate AA; 11 with recently diagnosed, and 3 with chronica AA; 12 without previous treatment and two treated before with antilymphocyte globulin + oxymetholone (OXN) + cyclosporine A (CsA) with transiet partial remission (PR). Besides lymphocytapheresis, 13 patients received OXM; 4 of them GM-CSF ad one low dose CsA. Four patients had complete remission lasting >59.5 months (range 42-78); eight PR (average duration of >38.6 months), and two minimal remission (>37 and 29 months). Platelet, reticulocyte and granulocyte counts increased on average at 48.7, 73.3 and 91.4 days, respectively. In cocnlusion, 14 (60.8 perecent) of 23 patients with AA showed an improvement related to LC treatment, with a survival probability of 63 percent from the fourth month, the latter with and added beneficial effect of the other therapies used. Larger numbers of patients have to be treated with LC to determine its real usefulness, mechanism of action and the best conditions for its use

Storage histiocytes and hemophagocytosis: a common finding in the bone marrow of patients with active systemic lupus erythematosus

We report the frequency of the finding of storage and hemophagocytic histiocytes in the bone marrow of patients with systemic lupus erythematosus with one or more hemocytopenias. The study was performed on bone marrows of ten patients with systemic lupus erythematosus during an episode of hemocytopenia. Four patients were not receiving any treatment and six had been takin oral prednisone (3.5 ñ 1.5 mg/day) for the previous 6 months. Hemocytopenia found were lymphocytopenia in five, thrombocytopenia in three, and neutropenia and anemia in two each. The bone marrow had variable cell content; megakaryocytes, the myeloid:erythroid ratio, as well as lymphocyte, plasma cell, and reticular cell contents were usually increased. Seven bone marrow samples showed storage distiocytes (sea-blue histiocytes and other histiocytes that contained unidentified intracytoplasmic material). These same samples revealed histiocytes phagocytosing erythoblasts, erythrocytes, polymorphonuclear neutrophils, lymphocytes in all seven patients was related to a decrease in serum complement and with lupus disease activity as mesured with the SLEDAI index (X ñ SD = 2.1 ñ 1.5). The SLEDAI score of the remaining three patients, who had no histiocytes in their bone marrow, was 0, 0, and 1, respectively. the present study reveals that the bone marrow in patients with systemic lupus erythematosus and peripheral hemocytopenia contains storage and hemophagocytic histiocytes and the significance of these cells is discussed

Estudio de la actividad procoagulante de las plaquetas en el síndrome de Bernard Soulier / Procoagulant activity of platelets study in the Bernard Soulier's syndrome

En el síndrome de Bernard soulier (SBS) han sido demostradas alteraciones específicas que dan lugar a un trastorno en la adhesividad plaquetaria. También se le han atribuido otros defectos que condicionan una alteración en el actividad procoagulante de sus mismas plaquetas, los cuales no han logrado definirse satisfactoriamente. En el presente trabajo se estduió el efecto procoagulante de las plaquetas, en cuatro pacientes con SBS, comparándolo con los resultados en controles sanos y enfermos trombocitopénica, para tal fin se efectuaron modificaciones en el cosumo de protrombina (CP) y tiempo de recalcificación del plasma, para evaluar el efecto de la trombocitopenia y el de plasmas con deficiencias intensas y específicas de factores de coagulación V, VIII y XI sobre el mecanismo procoagulante de las plaquetas con SBS. Los resultados observados difieren de los informados por otros investigadores, pues no se detectó ningún defecto en la actividad procoagulante en las plaquetas de los pacientes con SBS, considerando que su actividad procoagulante es normal, o bien, que existan variante de dicho síndrome

Tratamiento de la hemoglobinuria paroxística nocturna con globulina antilinfocitaria / Antilymphocyte globulin therapy for paroxysmal nocturnal hemoglobinuria

Objetivo. Evaluar la utilidad de la globulina antilinfocitos T humanos (GAL) en el tratamiento de la hemoglobinuria paroxística nocturna (HPN). Diseño. Estudio prospectivo, no controlado de casos clínicos. Ubicación. Servicio de Hematología, Hospital de Especialidades Centro Médico Nacional Siglo XXI, IMSS, México. Pacientes. Se incluyeron seis pacientes, tres varones y tres mujeres con edad promedio de 37.5 años. El diagnóstico de HPN se estableció por datos clínicos y pruebas de Ham, sucrosa e inulina positivas. Se consideró grave en cuatro enfermos en virtud de su actividad hemolítica continua e intensa, y los requerimientos transfusionales altos (más de dos concentrados de hematíes por mes); cinco de ellos no habían tenido mejoría con esteroides y/o andrógenos; uno no tenía tratamiento previo. Intervención. Se aplicó GAL (Lymphoglobuline Mérieux, Lyon, Francia; lote E 0034) 10 mg/kg/día por cuatro días, en infusión de 20 horas; también recibieron metilprednisolona, 500 mg/día, en infusión de dos horas cambiando, al cabo de siete días, a prednisona de 1 mg/kg y en dosis decrecientes hasta suspenderla en 30 días. Criterios de respuesta. Se valoró por medio del aumento en el nivel de homoglobina, la disminución o ausencia en los requerimientos transfusionales de glóbulos rojos, y la mejoría de la cuenta de plaquetas, granulocitos o ambas, en los tres meses posteriores al tratamiento con GAL. Resultados. En dos enfermos la primera dosis de GAL provocó una reacción anafiláctica y fueron excluidos del estudio. De los cuatro enfermos restantes, sólo dos mostraron respuesta a las 12 semanas post-tratamiento, uno total y otro mínima; ambas respuestas fueron transitorias. En un seguimiento promedio de 12.5 meses, ninguno de los pacientes mostraba mejoría. En los cuatro sujetos hubo necesidad de reiniciar tratamiento en danazol y prednisona. Conclusiones. El paciente con HPN en actividad intensa, la terapéutica con GAL, en las dosis y tiempo dados, no ofreció ningún beneficio. En consecuencia, se deben buscar otras alternativas para tratar a estos enfermos

Resultado del tratamiento de la púrpura trombocitopénica idiopática crónica con ácido ascórbico / Results of treatment in chronic idiopathic thrombocytopenic purpura with ascorbic acid

El objetivo de este trabajo es referir los resultados del tratamiento con ácido ascórbico en un grupo de enfermos con púrpura trombocitopénica idiopática crónica (PTIC) y resumir las experiencias de su empleo publicadas en la literatura respectiva. El grupo constó de trece enfermos refractarios a otras modalidades terapéuticas del trastorno, que recibieron ácido ascórbico 2g diariamente en la mañana por la vía bucal, durante un mínimo de ocho semanas. Al término del período de observación sólo cuatro enfermos (30 por ciento) experimentaron remisión parcial; en los demás no se demostró reacción favorable. Publicaciones previas informaron remisión completa y parcial en 11 y 19 por ciento respectivamnte en promedio. De acuerdo con tales resultados y los del presente estudio, se concluye que el empleo del ácido ascórbico carece de utilidad en el tratamiento de la PTIC

Tratamiento de la hemorragia intracraneana en la púrpura trombocitopénica idopática crónica / Treatment of intracraneal hemorrhage in chronic idiopathic thrombocytopenic purpura

Se informan los casos clínicos de dos pacientes con púrpura trombocitopénica idiopática crónica (PTIC) y hemorragia petequial intracraneana en las que el tratamiento convencional de esta complicación fue ineficaz; en cambio, en coincidencia con la aplicación de la vincristina se observó desaparición inmediata de sus manifestaciones neurológicas y aumento de las plaquetas con recuperación clínica total. Esta evolución contrasta con la informada en enfermos similares tratados con corticosteroides o esplenectomía, entre los que la mortalidad en ocasiones llegó a ser de 100 por ciento. La reacción favorable fue atribuída al efecto de la vincristina. Llama la atención, sobre todo, la mejoría inmediata de las manifestaciones neurológicas. Se propone su utilización en cojunto con las medidas tradicionalmente empleadas en el control de esta grave complicación, en particular en pacientes con complicaciones para la esplenectomía urgente.