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      Edema palpebral bilateral: un primer signo infrecuente de la leucemia mieloide aguda Translated title: Bilateral eyelid edema: an infrequent first sign of acute myeloid leukemia

      case-report

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          Abstract

          Resumen El sarcoma granulocítico es una manifestación extramedular de la leucemia mieloide aguda (LMA) poco frecuente. Es más común en la población pediátrica y puede presentarse en cualquier momento en el curso de la enfermedad. En ocasiones, puede preceder a la aparición clínica de leucemia mieloide aguda. Se presenta el caso de un niño de tres años con infiltración orbitaria bilateral que precipitó el diagnóstico de LMA.

          Translated abstract

          Abstract Granulocytic sarcoma is an unusual extramedullary manifestation of acute myeloid leukemia (AML). It is more common in the pediatric population and it may present at any time in the course of the disease. Occasionally, it may precede the clinical onset of acute myeloid leukemia. The case of a 3-year-old male child with a bilateral orbital infiltration that precipitated the diagnosis of AML is presented.

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          Most cited references8

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          Management of extramedullary leukemia as a presentation of acute myeloid leukemia.

          Extramedullary involvement is considered to be an uncommon presentation of acute myeloid leukemia (AML), although some data suggest it may be present in up to 30% of patients. Extra-medullary involvement by AML can present in a variety of clinical manifestations, most notably in the form of myeloid sarcoma, leukemia cutis, and central nervous system involvement. Each presents a unique clinical scenario in terms of symptoms and management. Extramedullary disease in any form presenting without evidence of bone marrow disease is still considered evidence of systemic disease and is usually treated as such. Most commonly, extramedullary disease presents concurrently with bone marrow disease, and although it may require additional local therapy in the form of intrathecal chemotherapy or radiation, the principles of systemic treatment remain unchanged. The prognostic impact of extramedullary disease is unclear. Specifically, whether hematopoietic stem cell transplantation should be considered in first remission irrespective of other prognostic factors has not been established. Patients who undergo transplantation have similar outcomes as patients without extramedullary disease, although they do have a higher rate of extramedullary relapse. More research is needed to define the molecular basis for extramedullary disease, its prognostic impact, and optimal management.
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            Orbital and facial granulocytic sarcoma (chloroma): a case report.

            Granulocytic sarcoma is an uncommon manifestation of myelogenous leukemias in which focal masses of immature myeloid cells from the granulocytic lineage infiltrate bone and soft tissue. It is most common in the pediatric population and may present at any time in the course of the disease, either concurrently with the onset of leukemia or during a remission or relapse. Occasionally, it may precede the clinical onset of acute myelogenous leukemia (AML), presenting a diagnostic challenge.
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              A case of myeloid sarcoma presenting with an orbital mass, hearing loss, and multiple cranial neuropathies.

              Lim SH, Nam HN, Lim KI, Jeon IS. A case of myeloid sarcoma presenting with an orbital mass, hearing loss, and multiple cranial neuropathies. Turk J Pediatr 2018; 60: 322-325. Primary myeloid sarcoma occurring in multiple sites; orbit, ear, brain, and spinal cord is a rare clinical entity. A 15-year-old male adolescent presented with bilateral orbital mass, hearing difficulty, and clinical signs of multiple cranial nerves palsy. Approximately 6 weeks later, acute myeloid leukemia was confirmed. This case alerts us that in patients with diverse sarcomatous lesions, acute myeloid leukemia presenting as myeloid sarcoma should be considered.
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                Author and article information

                Journal
                pap
                Pediatría Atención Primaria
                Rev Pediatr Aten Primaria
                Asociación Española de Pediatría de Atención Primaria (Madrid, Madrid, Spain )
                1139-7632
                June 2020
                : 22
                : 86
                : 165-168
                Affiliations
                [1] Albacete orgnameComplejo Hospitalario Universitario de Albacete orgdiv1Servicio de Pediatría España
                Article
                S1139-76322020000300008 S1139-7632(20)02208600008
                559fdc76-9663-45a2-a090-0620ae0b64ec

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 8, Pages: 4
                Product

                SciELO Spain

                Categories
                Notas Clínicas

                Acute myeloid leukemia,Sarcoma mieloide,Leucemia mieloide aguda,Myeloid sarcoma

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