RESUMO
Erdheim-Chester disease (ECD) is a form of non-langerhans histiocytosis mainly affecting men, whose pathophysiology is poorly understood. Clinical picture is heterogeneous. The presence of hair kidney sign on Ct scan is pathognomonic. Diagnosis is based on anatomopathological examination. We here report the case of a 50-year old patient presenting with disorders of equilibrium and slow speech as well as general health deterioration Clinical examination showed cerebellar ataxia. Radiological and pathological investigations showed aggressive systemic ECD. cladribine-based treatment was initiated with satisfactory outcome. ECD is an extremely rare disease. Systemic forms are generally associated with poor prognosis and are refractory to treatment, unlike in the case of our patient who was treated with cladribine with good outcome.
Assuntos
Ataxia Cerebelar , Doença de Erdheim-Chester , Cladribina/uso terapêutico , Doença de Erdheim-Chester/diagnóstico , Doença de Erdheim-Chester/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Radiografia , Tomografia Computadorizada por Raios XRESUMO
Relapsed Hodgkin lymphoma with only breast involvement is rare. There is no specific clinical presentation. It can mimic another breast cancer or inflammatory disease. The histological profile is often misleading in the pauci-cellular forms where the inflammatory component predominates. We report a case of an 18-year-old female patient treated for mixed-cell Hodgkin lymphoma who was admitted 6 months after complete remission for a left breast inflammatory nodule. The first assessment showed breast abscess. As there was not a good outcome despite adapted antibiotic, more investigations were added and showed a relapsed Hodgkin lymphoma with only breast involvement. The patient is currently receiving a 4th therapeutic line regimen of chemotherapy. Breast Hodgkin lymphoma´s prognosis is poor in most cases reported in the literature. Therapeutic advances (targeted therapy) can improve patient outcomes.
Assuntos
Doença de Hodgkin , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Feminino , Doença de Hodgkin/diagnóstico , Doença de Hodgkin/tratamento farmacológico , Humanos , Recidiva Local de Neoplasia , Indução de Remissão , Terapia de Salvação , Resultado do TratamentoRESUMO
IgD myeloma is an extremely rare haemopathy with severe clinical presentation. It can be confused with non-secretory or free light chain myeloma. We here report the case of a 72-year old female patient presenting with bone pain and diffuse ecchymosis and deterioration of her general condition. Laboratory tests showed monoclonal gammopathy associated with severe acute renal failure and low total protein (TP) (48%) with factor X deficiency. Etiological assessment confirmed the diagnosis of IgD lambda myeloma stage IIIb, according to Durie and Salmon, International Staging System (ISS) score III unfavorable cytogenetics. Patient's outcome was favorable after treatment with proteasome inhibitor, anti-CD 38 and corticosteroid therapy. Adequate treatment of IgD myeloma, using new therapeutic approaches and hematopoietic stem cell autotransplantation, can improve the prognosis.