1. A Rare Case of Relapsed Pediatric Acute Promyelocytic Leukemia with Skin Involvement by Myeloid Sarcoma
- Author
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Luiz Arthur Calheiros Leite, Vitória Mikaelly da Silva Gomes, Luana Novaes Bomfim, Arthur Moacir Costa Sampaio Batinga, Amanda Katielly Firmino da Silva Gusmão, Nathalia Silva Araújo, Maria Rosa da Silva, Maria Jordana Rocha Gomes Alves, Célio Fernando de Sousa Rodrigues, Claudio José dos Santos Júnior, and Cyndi Myrelle da Silva Barros Romão
- Subjects
Acute promyelocytic leukemia ,Male ,Pathology ,medicine.medical_specialty ,Skin Neoplasms ,Biopsy ,Lesion ,03 medical and health sciences ,0302 clinical medicine ,Fatal Outcome ,Rare Diseases ,Leukemia, Promyelocytic, Acute ,immune system diseases ,Recurrence ,Antineoplastic Combined Chemotherapy Protocols ,Myeloid sarcoma ,medicine ,Humans ,Myeloid Cells ,Sarcoma, Myeloid ,Child ,neoplasms ,Scalp ,medicine.diagnostic_test ,business.industry ,General Medicine ,Articles ,medicine.disease ,Flow Cytometry ,Bone marrow examination ,Leukemia ,030220 oncology & carcinogenesis ,Cytarabine ,Sarcoma ,medicine.symptom ,business ,030215 immunology ,medicine.drug - Abstract
Patient: Male, 9 Final Diagnosis: Acute promyelocytic leukemia (APL) Symptoms: Bleeding Medication: — Clinical Procedure: — Specialty: Hematology Objective: Rare disease Background: 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.
- Published
- 2018