RESUMEN
The body cavities are rarely involved in hairy cell leukaemia. Here we report a patient who had pancytopenia, hepatosplenomegaly, massive haemorrhagic ascites, pleural effusion at the left hemithorax and increased CA 125 serum level at the time of initial diagnosis. Laparoscopy showed multiple nodular white, opaque lesions on the omentum and on the parietal peritoneum. Laparoscopic biopsy of these lesions, and a bone marrow biopsy revealed a diffuse cellular infiltrate of tartrate-resistant acid phosphatase staining mononuclear cells. These mononuclear cells with irregular cytoplasmic protrusions were also found in the peripheral blood, in the ascites fluid and in the pleural effusion. The patient was treated with cladribine 0.1 mg/kg/day with continuous infusion for seven days. Three months after the treatment, the patient achieved a complete remission with normalisation of the peripheral blood count, bone marrow findings, CA 125 serum level, with no detectable ascites and/or pleural effusion.
Asunto(s)
Ascitis/patología , Antígeno Ca-125/sangre , Leucemia de Células Pilosas/diagnóstico , Derrame Pleural/diagnóstico , Ascitis/diagnóstico , Humanos , Leucemia de Células Pilosas/patología , Masculino , Persona de Mediana Edad , Derrame Pleural/patologíaRESUMEN
We report a 19-year-old woman who was presented with B-symptoms, massive splenomegaly, hepatomegaly and hypersplenism. She underwent diagnostic/therapeutic splenectomy. Microscopically, the spleen showed a vaguely micronodular and diffuse proliferation of lymphoid cells in the white pulp that also involved the red pulp. On immunohistochemical staining, this proliferation consisted predominantly of CD3(+), CD7(+) small T cells with the presence of a minor population of CD15(-),CD30(-), CD20(+) large atypical B cells. A liver biopsy also showed a similar morphology to that seen in the spleen. After splenectomy, only the pancytopenia improved. A combined immunochemotherapy regimen (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone) was utilized, which resulted in a complete remission.
Asunto(s)
Hiperesplenismo/etiología , Linfoma de Células B/complicaciones , Neoplasias del Bazo/patología , Linfocitos T/patología , Adulto , Anticuerpos Monoclonales/administración & dosificación , Anticuerpos Monoclonales de Origen Murino , Protocolos de Quimioterapia Combinada Antineoplásica/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Ciclofosfamida/administración & dosificación , Doxorrubicina/administración & dosificación , Femenino , Hepatomegalia/etiología , Histocitoquímica , Humanos , Linfoma de Células B/diagnóstico , Linfoma de Células B/tratamiento farmacológico , Linfoma de Células B/patología , Pancitopenia/etiología , Pancitopenia/terapia , Prednisona/administración & dosificación , Rituximab , Neoplasias del Bazo/complicaciones , Neoplasias del Bazo/tratamiento farmacológico , Vincristina/administración & dosificaciónRESUMEN
We report on a young female who had presented with fatigue, bilateral knee pain and gait disturbance. Primary hyperparathyroidism was diagnosed together with splenomegaly and anemia. Bone marrow biopsy revealed myelofibrosis. A parathyroid adenoma was excised during surgical intervention. As early as three months after the operation, hematologic parameters improved along with bone markers without any other intervention. The control bone marrow biopsy demonstrated well marked regression in marrow fibrosis. Her spleen has also gradually decreased in size. These findings indicate that her myelofibrosis was the result of primary hyperparathyroidism. Anemia associated with primary hyperparathyroidism may be due to bone marrow fibrosis.
Asunto(s)
Hiperparatiroidismo/diagnóstico , Mielofibrosis Primaria/etiología , Anemia/diagnóstico , Anemia/patología , Biopsia con Aguja , Médula Ósea/patología , Femenino , Humanos , Lactante , Mielofibrosis Primaria/patología , Esplenomegalia/diagnóstico , Esplenomegalia/patologíaAsunto(s)
Radioisótopos de Yodo , Yodobencenos , Timo/diagnóstico por imagen , 3-Yodobencilguanidina , Adulto , Medios de Contraste , Reacciones Falso Positivas , Femenino , Humanos , Neoplasias del Mediastino/diagnóstico por imagen , Neoplasias del Mediastino/secundario , Feocromocitoma/diagnóstico por imagen , Feocromocitoma/secundario , CintigrafíaRESUMEN
We report on a family with two sons affected with tetra-amelia, cleft lip-palate, bilateral agenesis of lungs, and heart defects. These two cases support the previous suggestions that this complex entity may indeed represent a new syndrome. However, the mode of inheritance is still not clarified.
Asunto(s)
Anomalías Múltiples , Fisura del Paladar , Ectromelia , Cardiopatías Congénitas , Pulmón/anomalías , Labio Leporino , Feto/anomalías , Humanos , Masculino , SíndromeRESUMEN
Adult lipid storage disorders with pulmonary involvement are rare and usually diagnosed at autopsy. We report a patient with splenomegaly and reticulonodular pattern on lung computed tomography. Bronchoalveolar lavage was performed and revealed the presence of lipid-containing foamy cells, with the demonstration of both periodic acid-Schiff (PAS) and scharlach red stain positive vacuoles in the cytoplasm of alveolar macrophages. The same cells were found in bone marrow biopsy. As in other rare disorders, bronchoalveolar lavage may be of diagnostic value in lipid storage disorders with pulmonary involvement.
Asunto(s)
Líquido del Lavado Bronquioalveolar/citología , Células Espumosas/patología , Enfermedad de Gaucher/diagnóstico , Enfermedades Pulmonares/diagnóstico , Enfermedades de Niemann-Pick/diagnóstico , Adulto , Humanos , Enfermedades Pulmonares/etiología , MasculinoRESUMEN
Using a silver staining technique, nucleolar organizer region-associated proteins (Ag-NORs) have been studied in paraffin sections of 76 non-Hodgkin's lymphomas, five normal lymph nodes, and five <