Myeloid sarcoma: an unusual presentation of acute promyelocytic leukemia causing spinal cord compression / Miyeloid Sarkom: Akut Promyelositik Loseminin Spinal Kord Kompresyonuna Neden Olan Olagandtsi Bir Klinik Tablosu

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Authors: Tay Za Kyaw, Jayaranee A.S. Maniam, Ping Chong Bee, Edmund Fui Min Chin, Veera Sekaran Nadarajan and Hemalatha Shanmugam
Date: Sept. 2012
From: Turkish Journal of Hematology(Vol. 29, Issue 3)
Publisher: Galenos Yayinevi Tic. Ltd.
Document Type: Article
Length: 2,272 words

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Abstract

Acute promyelocytic leukemia with concurrent myeloid sarcoma is a rare clinical event. Herein we describe a patient that presented with back pain and bilateral leg weakness caused by spinal cord compression due to extramedullary deposition of leukemic cells. Acute promyelocytic leukemia was suspected based on immunophenotypic findings of malignant cells in bone marrow aspirate. The diagnosis was confirmed by the presence of PML-RARa fusion copies. MRI showed multiple hyperintense changes on the vertebral bodies, together with intraspinal masses causing spinal cord compression. The patient immediately underwent radiotherapy, and was treated with all-trans retinoic acid and idarubicin. Reassessment MRI showed complete resolution of all intraspinal masses and the disappearance of most of the bony lesions. Post-treatment bone marrow aspirate showed complete hematological and molecular remission. The motor power of his legs fully recovered from 0/5 to 5/5; however, sensory loss below the T4 level persisted.

Key Words: Myeloid sarcoma, Promyelocytic leukemia, Cord compression, Chloroma

Ozet

Es zamanli miyeloid sarkomlu akut promiyelositik losemi nadir bir klinik olaydir. Burada ekstrameduller losemik hucre birikimi nedeniyle spinal kord kompresyonu sonucunda bel agrisi ve bilateral bacak zayifligi ile gelen bir hastayi tanimliyoruz Kemik iligi aspiratinda malign hucreler immunofenotipik bulgulari temelinde akut promiyelositik losemiden suphelenildi. Tani PML-RAR[alpha] fuzyon kopyalarinin varligiyla dogrulandi. MRG, vertebral cisimlerde cok sayida hiperintens degisiklik ve beraberinde spinal kord kompresyonuna neden olan intraspinal kitleler gosterdi. Hastaya hemen radyoterapi baslandi ve all-trans retinoik asit ve idarubisin ile tedavi edildi. Tekrar degerlendirmede MRG tum intraspinal kitlelerin tam olarak gectigini ve kemik lezyonlarinin cogunun kayboldugunu gosterdi. Tedavi sonrasi kemik iligi aspirati tam hematolojik ve molekuler remisyon gosterdi. Bacaklarda motor guc 0/5'ten 5/5'e tam olarak iyilesti ancak T4 seviyesi altindaki duyu kaybi devam etti.

Anahtar Sozcukler: Miyeloid sarkom, Promiyelositik losemi, Kord kompresyonu, Kloroma

Introduction

Myeloid sarcoma (MS) is a rare extramedullary tumor consisting of immature myeloid cells (1). MS may develop de novo or concurrently with acute myeloid leukemia (AML), myeloproliferative disorders, or myelodysplastic syndrome. It may proceed or coincide with the occurrence of AML or may present as the initial manifestation of relapse in a previously treated AML patient in remission (2). MS may involve any organ system, from the more common involvement of the skin, bone, soft tissue of the head and neck (frequently the orbits) and lymph nodes, to rare cases involving the heart or small intestine (3). The occurrence of MS in an acute promyelocytic leukemia (APL) patient is a rare clinical event. Herein we report a patient with APL that presented with spinal cord compression due to underlying MS.

Case Presentation

Written informed consent was obtained from the patient. A 26-year-old male was referred to our hospital due to suspected AML in August 2010. He presented with progressive back pain and bilateral leg weakness, and was unable to walk. Initial neurological examination showed paraparesis with a power of 0/5 in both lower limbs, presence of the Babinski sign, and loss of pain and sensory perception below the T4 level. There were no other abnormal findings on physical examination. Urgent MRI of the...

Source Citation

Source Citation
Kyaw, Tay Za, et al. "Myeloid sarcoma: an unusual presentation of acute promyelocytic leukemia causing spinal cord compression / Miyeloid Sarkom: Akut Promyelositik Loseminin Spinal Kord Kompresyonuna Neden Olan Olagandtsi Bir Klinik Tablosu." Turkish Journal of Hematology, vol. 29, no. 3, 2012, p. 278+. Accessed 23 Oct. 2020.
  

Gale Document Number: GALE|A345621018