Patient: Male, 9Final Diagnosis: Acute promyelocytic leukemia (APL)Symptoms: BleedingMedication: —Clinical Procedure: —Specialty: HematologyObjective:Rare diseaseBackground:Acute promyelocytic leukemia (APL) is a very rare leukemia in children. Extramedullary involvement by APL has been reported in between 3–5% of cases, mainly associated with cases of relapse. A rare case of relapse of APL in a 9-year-old child is presented with skin involvement with myeloid sarcoma.Case Report:A 9-year-old male child was admitted to the Oncology Service of the hospital complaining of fever, progressive fatigue, oral petechiae with severe bleeding in the oral cavity. Bone marrow examination showed some promyelocytes. Flow cytometry showed 86% immature myeloid cells with the t(15;17) translocation, and molecular analysis showed expression of the PML/RARα fusion protein, which confirmed the diagnosis of APL. The patient completed a course of daunorubicin, cytarabine, and AII trans-retinoic acid (ATRA) with complete remission. After six months, the patient was re-admitted to hospital with a violaceous lesion on the scalp, with relapse of APL. Histological and immunohistochemistry of the lesion involving the skin of the scalp showed a myeloid sarcoma invading the dermis.Conclusions:Myeloid sarcoma, also called granulocytic sarcoma, is an extramedullary tumor of immature myeloid cells, which very rarely presents in children with APL. The mechanisms that lead to myeloid sarcoma in children with APL and the possible association with ATRA therapy remain to be investigated.

Objetivo: Avaliar os aspectos clínico-epidemiológicos do câncer infantojuvenil em Alagoas. Métodos: Trata-se de um estudo transversal, de caráter retrospectivo e quantitativo. As informações referentes aos diagnósticos de neoplasias infantojuvenis no Estado de Alagoas foram coletadas a partir de consulta aos prontuários dos centros de referência estaduais para tratamento de câncer na população de 0 a 19 anos. Resultados: Quanto ao perfil dos acometidos, a incidência maior foi em indivíduos do sexo masculino entre 5-9 anos, pardos, sem escolaridade, procedentes da 1ª região de saúde. As neoplasias mais frequentes foram: tumores hematológicos (leucemias e linfomas), tumores do sistema nervoso central, renais, ósseos, retinoblastoma e partes moles. A terapêutica mais empregada no tratamento dos casos foi a quimioterapia. Houve aumento na média de diagnósticos de casos novos de câncer infantojuvenil na população do estado de Alagoas. A letalidade do câncer infantojuvenil no período foi de 14,6%. Conclusão: O câncer infantojuvenil em Alagoas, em concordância com o cenário nacional e internacional, continua sendo uma doença relativamente rara e de baixa incidência. O aumento dos casos verificado para o Estado pode estar associado a diversos fatores, entre estes, a maior capacitação profissional para triagem de sinais e sintomas clínicos, com consequente encaminhamento as unidades especializadas para investigação e diagnóstico precoce.

Patient: Female, 51Final Diagnosis: Blastic plasmacytoid dendritic cell neoplasmSymptoms: Pulmonary bleeding • small skin lesionMedication: Hyper-CVAD • methotrexate • cytarabineClinical Procedure: —Specialty: HematologyObjective:Rare diseaseBackground:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematodermic malignancy neoplasm with highly aggressive course and poor prognosis. This disease typically presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood.Case Report:Here, we report the case of a 51-year-old woman who presented a violaceus skin lesion on the lateral region of the right thigh, weight loss, fever, and lymphadenopathies. Computed tomography (CT) displayed thoracic and abdominal lymph node and alveolar bleeding. Flow cytometry from circulating blastic cells was compatible with BPDCN (CD4+, CD56+ and CD123+). She underwent 5 cycles of hyper-CVAD alternating with high-dose methotrexate and cytarabine, but the patient died due to alveolar bleeding and sepsis.Conclusions:We report a rare case of BPDCN characterized by an aggressive course, presence of atypical skin lesion, a finding suggestive of pulmonary infiltration, and nonresponse to induction chemotherapy, leading to late diagnosis and therapeutic management. Because of the late recognition of the skin lesion, neoplastic cells infiltrated the dermis and spread as the disease progressed rapidly to a fatal course.