ACUTE MEGAKARYOBLASTIC LEUKEMIA (AML-M7) IN 10-MONTH-OLD BABY BOY WITH DOWN SYNDROME

Tri Ratnaningsih(1*), Aji Bagus Widyantara(2)

(1) Clinical Pathology Departement, Medicine Public Health and Nursing Faculty, Universitas Gadjah Mada
(2) Universitas 'Aisyiyah Yogyakarta
(*) Corresponding Author

Abstract

ABSTRAK

Leukemia megakarioblastik akut (acute megakaryoblastic leukemia, AML-M7) merupakan subtipe AML dengan mayoritas megakarioblas. Kejadian  AML-M7 umumnya terjadi pada anak-anak dengan down syndrome. Bayi laki-laki berusia 10 bulan dengan down syndrome rujukan dari RSU PKU Muhammadiyah Yogyakarta datang dengan keterangan klinis prolonged fever  suspek keganasan akut hematologi seri mieloid. Pemeriksaan tanda vital nadi 102x/menit, suhu  36,7oC pernafasan 30x/menit. Pemeriksaan fisik: berat badan 7,1 kg, tinggi badan  66,1 cm. Hepar teraba 6 cm bawah arcus costae, limpa Schuffner II. Pemeriksaan laboratorium didapatkan lekositosis, netropenia, dan trombositopenia. Pemeriksaan morfologi darah tepi disimpulkan gambaran suspek keganasan akut seri mieloid. Gambaran aspirasi sumsum tulang mendukung diagnosis leukemia megakarioblastik akut atau  acute megakaryoblastic leukemia (AML-M7). Beberapa studi pada kelompok pediatrik menemukan sekitar setengah dari semua kasus AML-M7 terjadi pada anak-anak dengan down syndrome. Pada pemeriksaan fisik terdapat adanya organomegali. Pemeriksaan laboratorium didapatkan anemia, lekositosis, netropenia, dan trombositopenia. Pemeriksaan penunjang lain yang mendukung dalam penegakan diagnosis AML-M7 adalah morfologi darah tepi, aspirasi sumsum tulang, dan pengecatan sitokimiawi. Diagnosis acute megakaryoblastic leukemia (AML-M7) pada bayi dengan down syndrome. Diagnosis didasarkan gambaran klinis, anamnesis (alloanamnesa), pemeriksaan fisik, pemeriksaan laboratorium, dan pemeriksaan penunjang lainnya.

Kata Kunci: Acute megakaryoblastic Leukemia, AML-M7, Bayi, Syndrome Down

ABSTRACT

Acute megakaryoblastic Leukemia (AML-M7) is a subtype of AML where megakaryoblasts are the majority of the blasts. The incidence of AML-M7 is commonly seen in children with Down syndrome. A 10-month-old male baby with down syndrome, a referral from PKU Muhammadiyah General Hospital Yogyakarta, came with a prolonged fever suspected of acute hematological malignancy in the myeloid series. Vital signs examination of pulse rates: 102x / minute; temperature: 36.7oC; respiration: 30x / minute. On physical examination, the baby weighs 7.1 kgs, with a height of 66.1 cm. The palpable liver was 6 cm below the arcus rib, and the enlargement of the spleen was classified as Schuffner II. Laboratory tests explained leukocytosis, neutropenia, and thrombocytopenia. The peripheral blood morphology examination showed suspected acute malignancy with myeloid series. Bone marrow aspiration supported the diagnosis of acute megakaryoblastic Leukemia or acute megakaryoblastic Leukemia (AML-M7). Several studies in the pediatric group found that about half of all AML-M7 cases occurred in children with Down syndrome. On physical examination, there was organomegaly. Laboratory tests showed anemia, leukocytosis, neutropenia, and thrombocytopenia. Other supporting investigations in establishing the diagnosis of AMLM7 were peripheral blood morphology, bone marrow aspiration, and cytochemical staining. A diagnosis of acute megakaryoblastic Leukemia (AML-M7) in infants with Down syndrome. The diagnosis was based on clinical features, history (allo-anamnesis), physical examination, laboratory examination, and other supporting investigations.

 

Keywords: Acute megakaryoblastic leukemia, AML-M7, Baby, Down Syndrome

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