[Multiple Myeloma Treatment Guidelines by the Portuguese Group of Multiple Myeloma]. / Recomendações do Grupo Português do Mieloma Múltiplo para Tratamento do Mieloma Múltiplo.

The treatment of multiple myeloma has profoundly changed with the introduction of several innovative therapies. The optimization of therapeutic sequencing through the combined use of the various drugs developed in recent years and the attention given to the characteristics of patients have allowed the reduction of toxicities and increased survival and quality of life of patients with multiple myeloma. These treatment recommendations from the Portuguese Multiple Myeloma Group offer guidance for first-line treatment and progression/relapse situations. These recommendations are given highlighting the data that justify each choice and referring to the respective levels of evidence that support these options. Whenever possible, the respective national regulatory framework is presented. These recommendations constitute an advance towards the best treatment of multiple myeloma in Portugal.

O tratamento do mieloma múltiplo tem sido amplamente alterado com introdução de várias terapêuticas inovadoras. A otimização da sequenciação terapêutica através do uso combinado dos vários fármacos desenvolvidos nos últimos anos e a atenção dada às características dos doentes têm permitido diminuir toxicidades e aumentar a sobrevivência dos doentes, bem como aumentar a sua qualidade de vida. As presentes recomendações terapêuticas do Grupo Português do Mieloma Múltiplo oferecem orientações para o tratamento de primeira linha e progressão/recaída. As recomendações são fundamentadas evidenciando os dados que justificam cada escolha e referindo os respetivos níveis de evidência que suportam essas opções. Sempre que possível é apresentado o respetivo enquadramento regulamentar nacional. Estas recomendações constituem um avanço para o melhor tratamento do mieloma múltiplo em Portugal.

Severe Aplastic Anemia as First Manifestation of Classical Hodgkin Lymphoma.

Autoimmune cytopenia, a known paraneoplastic complication of lymphoid neoplasms, may occur before, concurrently, at relapse, or even years after completion of lymphoma treatment. In the case of Hodgkin lymphoma (HL), it is thought that immune dysregulation, typical of this neoplasm, may be involved in the genesis of these manifestations. We report a 57-year-old male presenting with stage IIIA, International Prognostic Score (IPS) 4, nodular sclerosis HL, and severe AA (SAA) confirmed on the histologic exam of the bone marrow that showed severe marrow hypoplasia due to a decrease in the elements of the three cell linages with left shift of the myeloid maturation. Immunosuppression with steroids and cyclosporine A was started. Eltrombopag and G-CSF were also added. In spite of prompt initiation of immunosuppressive therapy, the patient presented an unfavorable outcome with progressive pancytopenia and severe acute cerebral hemorrhagic event. The patient died 59 days after admission. Although autoimmune disorders are described in HL, its concomitant diagnosis is extremely rare. Our case shows a rare instance of SAA as the first manifestation of HL.