RESUMO
The hypereosinophilic syndrome (HES) is a heterogeneous disease characterized by sustained eosinophilia for a period of at least six months with evidence of organ involvement. Its manifestations range from a benign disorder not requiring any therapy to an aggressive, malignant variety refractory to common treatments. Diverse therapies have been used, including steroids, hydroxyurea, and chemotherapy, with variable responses. Recently alpha-interferon therapy has been shown effective in this disorder. Of the various prognostic factors, elevated serum immunoglobulin E (IgE) levels is considered among the most favorable, with most patients presenting with a [quot ]benign[quot ] disorder, not requiring therapy. We describe a patient presenting with an aggressive variant of HES despite having elevated IgE levels. The patient had a dramatic and lasting response to alpha-interferon
Assuntos
Humanos , Masculino , Adulto , Antineoplásicos/uso terapêutico , Interferon-alfa , Síndrome Hipereosinofílica/tratamento farmacológico , Imunoglobulina E/sangue , Resistência a Medicamentos , Síndrome Hipereosinofílica/sangue , Síndrome Hipereosinofílica/diagnósticoRESUMO
Chronic lymphocytic leukemia (CLL) is a chronic, low-grade hematologic malignancy that can transform to a large cell non-Hodgkin's lymphoma (Richter's syndrome), which is associated with an unfavorable prognosis. A distinct Hodgkin's disease subgroup of lymphomatous CLL transformation has been well characterized. We describe a patient presenting with simultaneous manifestations of CLL and Hodgkin's disease. This patient is unique, presenting with separate sites of involvement of each disease within the same organ, in this case the bone marrow. The morphologic and immunohistochemical findings at diagnosis are correlated with the findings of the postmortem examination.
Assuntos
Doença de Hodgkin/complicações , Leucemia Linfocítica Crônica de Células B/complicações , Autopsia , Biópsia , Medula Óssea/patologia , Neoplasias da Medula Óssea/patologia , Feminino , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/patologia , Humanos , Imuno-Histoquímica , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/patologia , Pessoa de Meia-Idade , PrognósticoRESUMO
Chronic lymphocytic leukemia (CLL) is a chronic, low-grade hematologic malignancy that can transform to a large cell non-Hodgkin's lymphoma (Richter's syndrome), which is associated with an unfavorable prognosis. A distinct Hodgkin's disease subgroup of lymphomatous CLL transformation has been well characterized. We describe a patient presenting with simultaneous manifestations of CLL and Hodgkin's disease. This patient is unique, presenting with separate sites of involvement of each disease within the same organ, in this case the bone marrow. The morphologic and immunohistochemical findings at diagnosis are correlated with the findings of the postmortem examination
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Doença de Hodgkin/complicações , Leucemia Linfocítica Crônica de Células B/complicações , Autopsia , Biópsia , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/patologia , Imuno-Histoquímica , Leucemia Linfocítica Crônica de Células B/diagnóstico , Leucemia Linfocítica Crônica de Células B/patologia , Medula Óssea/patologia , Neoplasias da Medula Óssea/patologia , PrognósticoRESUMO
The hypereosinophilic syndrome (HES) is a heterogeneous disease characterized by sustained eosinophilia for a period of at least six months with evidence of organ involvement. Its manifestations range from a benign disorder not requiring any therapy to an aggressive, malignant variety refractory to common treatments. Diverse therapies have been used, including steroids, hydroxyurea, and chemotherapy, with variable responses. Recently alpha-interferon therapy has been shown effective in this disorder. Of the various prognostic factors, elevated serum immunoglobulin E (IgE) levels is considered among the most favorable, with most patients presenting with a "benign" disorder, not requiring therapy. We describe a patient presenting with an aggressive variant of HES despite having elevated IgE levels. The patient had a dramatic and lasting response to alpha-interferon.
Assuntos
Antineoplásicos/uso terapêutico , Síndrome Hipereosinofílica/tratamento farmacológico , Interferon-alfa/uso terapêutico , Adulto , Resistência a Medicamentos , Humanos , Síndrome Hipereosinofílica/sangue , Síndrome Hipereosinofílica/diagnóstico , Imunoglobulina E/sangue , Interferon alfa-2 , Masculino , Proteínas RecombinantesRESUMO
T-cell rich B-cell lymphomas (TCRBCL) are characterized as non-Hodgkin lymphomas with a minor population of malignant B-cells scattered among predominant, reactive T-lymphocytes. This entity can easily be confused with lymphocyte-predominant Hodgkin disease (HD-LP), resulting in inappropriate therapy and a poor outcome. Because of their similarity, the pathology of patients treated for HD-LP with an inadequate or short-lived response to therapy should always be reviewed by an expert hematopathologist. We describe the first reported patient in Puerto Rico with TCRBCL, originally diagnosed and treated as HD-LP. Although the patient received partial, substandard therapy for TCRBCL, an excellent prolonged complete response ensued, thus, giving further credence to the fact that malignant lymphomas and TCRBCL in particular, are a protean group of disorders which should be precisely and accurately classified before the proper therapeutic strategies can be outlined
Assuntos
Humanos , Masculino , Adulto , Doença de Hodgkin/diagnóstico , Linfoma de Células B/diagnóstico , Diagnóstico Diferencial , Doença de Hodgkin/terapia , Linfoma de Células B/terapia , Linfócitos T , Resultado do TratamentoRESUMO
T-cell rich B-cell lymphomas (TCRBCL) are characterized as non-Hodgkin lymphomas with a minor population of malignant B-cells scattered among predominant, reactive T-lymphocytes. This entity can easily be confused with lymphocyte-predominant Hodgkin disease (HD-LP), resulting in inappropriate therapy and a poor outcome. Because of their similarity, the pathology of patients treated for HD-LP with an inadequate or short-lived response to therapy should always be reviewed by an expert hematopathologist. We describe the first reported patient in Puerto Rico with TCRBCL, originally diagnosed and treated as HD-LP. Although the patient received partial, substandard therapy for TCRBCL, an excellent prolonged complete response ensued, thus, giving further credence to the fact that malignant lymphomas and TCRBCL in particular, are a protean group of disorders which should be precisely and accurately classified before the proper therapeutic strategies can be outlined.
Assuntos
Doença de Hodgkin/diagnóstico , Linfoma de Células B/diagnóstico , Adulto , Diagnóstico Diferencial , Doença de Hodgkin/terapia , Humanos , Linfoma de Células B/terapia , Masculino , Linfócitos T , Resultado do TratamentoRESUMO
Solid tumor cells are rarely seen in peripheral blood smears. When this occurs the term carcinocythemia is used. This report describes an 18-year-old female who presented with a painless lump in the labia majora associated with pancytopenia. Tumor cells were identified in the peripheral blood smear and the bone marrow aspirate showed a predominant population of small round vacuolated primitive cells, many of which formed clumps of varying sizes. Biopsies of the vulvar mass and bone marrow were interpreted as alveolar rhabdomyosarcoma. Review of the literature revealed 12 previously reported cases in whom carcinocythemia had been documented; rhabdomyosarcoma was the specific cell type involved in only two of these. The median time between detection of the leukemic phase of the tumor and death was 8.5 weeks, reflecting the fact that carcinocythemia, when it occurs, represents the terminal event of the disease. To our knowledge, our case is the third well documented case of rhabdomyosarcoma in leukemic phase so far reported. The clinical evolution as well as the management of this patient will be described in detail along with a review of the pertinent available literature.