RESUMEN
Histiocytic sarcoma is a neoplasm arising from the histiocytes. Histiocytic neoplasms are among the rarest malignancies of lymphatic tissues. Occurs in less than 1% of all malignancies affecting lymph nodes and soft tissues [1,2]. The exact incidence of histiocytic sarcoma has not been described so far. In this article, we report three patients with HS, who were treated at the departement of Internal medicine, haematology and oncology, Faculty Hospital Brno. Despite the fact that all these patients had the same disease, the treatment effects differ depending on the stage of the disease at the time of diagnosis.
Asunto(s)
Sarcoma Histiocítico/diagnóstico , Anciano , Biopsia , Terapia Combinada , Femenino , Histiocitos/patología , Sarcoma Histiocítico/patología , Sarcoma Histiocítico/terapia , Humanos , Ganglios Linfáticos/patología , Masculino , Persona de Mediana Edad , Imagen Multimodal , Estadificación de Neoplasias , Tomografía de Emisión de Positrones , Tomografía Computarizada por Rayos XRESUMEN
Castleman disease is a rare idiopathic non-neoplastic lymphoproliferative disorder with 2 clinical (unicentric and multicentric) and 3 histomorphological (hyaline-vascular, plasma-cell and mixed) forms identified. The case report given here describes the 3-year experience with therapy in a patient, male born 1961, diagnosed with multicentric plasma-cell Castleman disease (HIV and HHV-8 negative) with the finding of generalized lymphadenopathy and splenomegaly. During first line treatment (R-CHOP: rituximab, cyclophosphamide, doxorubicin, vincristine, prednisone, 3 cycles in total, 12/2008-2/2009) the development of bilateral upper and lower limb edemas with clinical manifestation of vasculitis occurred and a restaging computed tomography (CT) examination revealed a stable finding of the lymphadenomegaly. Greater success was achieved with thalidomide regimen (CTD: cyclophosphamide, thalidomide, dexamethasone, 10 cycles, 3/2009-1/2010) leading to reduction in the size of the hypervascularized lymph nodes (almost by 50%) as well as their radiopharmaceutical (fluorodeoxyglucose) uptake as seen on a combined positron emission tomography and computed tomography (PET/CT) scan imaging. Thalidomide was given daily at doses between 100 and 200 mg. We returned to the CTD regimen again in April 2010 after a short period of monoclonal antibody tocilizumab treatment (400 mg intravenous in 2-week intervals with 50% dose reduction due to a limited supply of the drug, 5 doses in total) during which edemas reoccurred with a CT scan finding of stable lymphadenomegaly. However, the renewed regimen with thalidomide was stopped after 2.5 cycles due to adverse effects of thalidomide (neuropathy) and corticoids (Cushing syndrome). In September 2010, after enrollment in the Celgenes Compassionate Use Program we were able to start treating the patient with the derivative of thalidomide, lenalidomide, at a dosage of 25 mg on days 1-21 in a 28-day cycle, 15 cycles in total (10/2010-12/2011). The monotherapy with lenalidomide was very well tolerated by the patient without any effects of myelotoxicity, thromboembolism or relapses of edemas and vasculitis, additionally now with apparent improvement of fatic disorder and the patients motor abilities. Thus, lenalidomide represents an attractive alternative agent for patients with Castleman disease after rituximab and cytostatics failures. It has a favourable safety profile and could be therefore considered for administering in first line treatment.
Asunto(s)
Enfermedad de Castleman/tratamiento farmacológico , Vasculitis/complicaciones , Enfermedad de Castleman/complicaciones , Enfermedad de Castleman/diagnóstico , Humanos , Masculino , Persona de Mediana EdadRESUMEN
Monoclonal gammopathy of undetermined significance (MGUS) is a precancerosis comprising two different kinds of cancer: lymphoid/lymphoplasmocytoid MGUS and plasma cell MGUS that represents about 85% of all MGUS cases. This type of MGUS has low but persistent tendency to transform to malignant disease, mainly multiple myeloma (MM), with frequency of about 1% per year. Using known risk stratification models based on clinical parameters, it is possible to identify patients' groups with average rates of progression as low as 0.26% and as high as 12% per year. However, due to the lack of clear genetic and/or phenotypic markers distinguishing MGUS from MM, we are not able to predict if and when MGUS will progress to MM in individual patients. There are partially overlapping molecular pathogenic events shared by MGUS and MM. Better understanding of pathogenesis of MGUS and MM using molecular-genetic approaches will help disclose the mechanisms of myeloma genesis; it can be also useful for identification of novel molecular targets. The ultimate goal for the near future is to develop better markers for definition of high-risk MGUS patients who will be candidates for early treatment intervention.
Asunto(s)
Gammopatía Monoclonal de Relevancia Indeterminada , Progresión de la Enfermedad , Humanos , Inmunoglobulina M , Gammopatía Monoclonal de Relevancia Indeterminada/complicaciones , Gammopatía Monoclonal de Relevancia Indeterminada/diagnóstico , Mieloma Múltiple/etiología , Lesiones PrecancerosasRESUMEN
BACKGROUNDS: Osteolytic lesions are a common manifestation of multiple myeloma, though their healing is rare in these patients. Generally, during a complete remission, lesions only stop progressing; radiologically evident recalcification is exceptional. CASE: Herein we report a case of a male patient born in 1941 and diagnosed in 2005 with IgA multiple myeloma presenting with multiple osteolytic bone lesions. Administration of 4 cycles of VAD chemotherapy (vincristine, adriamycin, dexamethasone) with subsequent autologous peripheral blood stem cell transplantation and maintenance treatment with interferon alpha had resulted into a very good partial remission. In 2009, the disease relapsed with enlargement of osteolytic lesions evident on skiagrams. The largest lesion, reaching 24 x 10 mm in size, was located in the left femur. A complete remission of the disease was achieved with CVD senior regimen (cyclophosphamide, bortezomib, dexamethasone, 8 cycles in total). Bisphosphonates (zoledronate, ibandronate and, from 2007, clodronate) were administered as a long-term supportive therapy. A one-year follow-up skiagram of the left femur revealed over 50% regression of the osteolytic lesion (10 x 5 mm) documented in a set of pictures herein. CONCLUSION: A complete remission of the disease after an administration of bortezomib (Velcade)-based regimen in a long-term clodronate (Bonefos)-treated patient with relapsed multiple myeloma is radiographically apparent by clear healing signs of the osteolytic bone lesion.
Asunto(s)
Antineoplásicos/administración & dosificación , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Conservadores de la Densidad Ósea/uso terapéutico , Ácidos Borónicos/administración & dosificación , Ácido Clodrónico/uso terapéutico , Mieloma Múltiple/tratamiento farmacológico , Osteólisis/tratamiento farmacológico , Pirazinas/administración & dosificación , Anciano , Bortezomib , Fémur/diagnóstico por imagen , Humanos , Masculino , Mieloma Múltiple/complicaciones , Mieloma Múltiple/diagnóstico por imagen , Mieloma Múltiple/patología , Osteólisis/complicaciones , Osteólisis/diagnóstico por imagen , Radiografía , Recurrencia , Inducción de RemisiónRESUMEN
OBJECTIVES: The aim of the presented study was to evaluate the frequency-domain signal-averaged ECGs (SAECG) abnormalities in childhood and adolescence acute leukemia and lymphoma survivors treated either with or without anthracyclines (ANT) containing chemotherapy in comparison with healthy volunteers. BACKGROUND: The late development of chemotherapy-induced myocardial complications becomes an issue as the number of childhood cancer survivors is increasing. Underlying cardiac impairment may progress to serious cardiac diseases. Therefore, an early identification of myocardial injury is essential. PATIENTS AMD METHODS: Study population was divided into two treatment groups: ANT group (31 patients previously treated with ANT), and non-ANT group (32 patients who underwent chemotherapy without ANT, both more than 5 years ago). SAECG was added to routine cardiology examination in the whole population study and 32 controls. Using the frequency-domain analysis within the QRS complex a ratio (AR) of 20-50 (Hz)/0-20 (Hz) was calculated. RESULTS: AR 20-50/0-20 in SAECG was significantly higher in ANT and non-ANT groups, relative to controls (262.5 p < 0.00001 vs. 135.9 p < 0.001 vs. 74.7). The difference between both patient groups was also evident p < 0.01. CONCLUSION: Significant differences in frequency-domain SAECG parameters between patients (with or without anthracyclines) and controls might indicate the increased risk of electrical instability particularly in anthracycline-treated patients (Tab. 2, Fig. 1, Ref. 34).