Acute Myeloid Leukemia (M4)/Myeloid Sarcoma Presenting with Hyperleukocytosis, 47,XX + mar Case

Hyperleukocytosis is defined as a blood blast count >100.000/ mm3 and may occur most commonly in monocytic (M4) acute myeloid leukemia (AML). Hyperleukocytosis is a medical emergency associated with lung and central nervous system leukostasis. An 18-month-old female patient was brought to our clinic with anemia, thrombocytopenia, and leukocytosis with bilateral palpebral swelling/proptosis, a palpable mass in the midline, and lymphadenopathy in the neck. Myeloid sarcoma was suspected. The patient had AML M4 morphological features, and 47,XX + mar was detected in bone marrow karyotype analysis. She had complications such as hyperleukocytosis, leukostasis (blurring of consciousness, tachypnea/tachycardia), tumor lysis syndrome, and disseminated intravascular coagulation. In addition to low-dose cytoreduction chemotherapy, leukocyte apheresis was performed. All symptoms and complaints of the patient disappeared. Therefore, myeloid sarcoma should be considered in young children with orbital swelling/ proptosis, an abdominal mass, high leukocyte count at diagnosis, AML M4 morphological features, and diffuse intravascular coagulation.

Keywords:

acute myeloid leukemia, myeloid sarcoma, hyperleukocytosis disseminated intravascular coagulation,

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1. Emadi A, Baer MR. Acute myeloid leukemia in adults. In: Greer JP, Arber DA, Glader B, List AF, Means Jr RT, Paraskevas F, Rodges GM. Wintrobe’s Clinical Hematology, 13th ed. Philadelphia: Lippincott Williams & Wilkins. 2014: 35913695.
2. Creutzig U, Ritter J, Budde M, Sutor A, Schellong G. Early deaths due to hemorrhage and leukostasis in childhood acute myelogenous leukemia. Associations with hyperleukocytosis and acute monocytic leukemia. Cancer. 1987; 60:3071.
3. Beckmann L, Rolling CC, Voigtländer M, Mäder J, Klingler F, Schulenkorf A et al. Bacitracin and rutin regulate tissue factor production in inflammatory monocytes and acute myeloid leukemia blasts. Cancers (Basel). 2021 Aug 4; 13(16):3941
4. Dicke C, Amirkhosravi A, Spath B, Jiménez-Alcázar M, Fuchs T, Davila M et al. Tissue factor-dependent and -independent pathways of systemic coagulation activation in acute myeloid leukemia: a single-center cohort study. Exp Hematol Oncol. 2015 Aug 6; 4:22.
5. Bruserud Ø, Liseth K, Stamnesfet S, Cacic DL, Melve G, Kristoffersen E et al. Hyperleukocytosis and leukocytapheresis in acute leukaemias: experience from a single centre and review of the literature of leukocytapheresis in acute myeloid leukaemia. Transfus Med. 2013 Dec; 23(6):397-406.
6. Ganzel C, Becker J, Mintz PD, Lazarus HM, Rowe JM. Hyperleukocytosis, leukostasis and leukapheresis: practice management. Blood Rev. 2012; 26:117.
7. Oberoi S, Lehrnbecher T, Phillips B, Hitzler J, Ethier MC, Beyene J et al. Leukapheresis and low-dose chemotherapy do not reduce early mortality in acute myeloid leukemia hyperleukocytosis: a systematic review and meta-analysis. Leuk Res. 2014 Apr; 38(4):460-8.
8. Dusenbery KE, Howells WB, Arthur DC, Alonzo T, Lee JW, Kobrinsky N et al. Extramedullary leukemia in children with newly diagnosed acute myeloid leukemia: a report from the Children’s Cancer Group. J Pediatr Hematol Oncol. 2003; 25:760.
9. Shimizu H, Saitoh T, Hatsumi N, Takada S, Yokohama A, Handa H et al. Clinical significance of granulocytic sarcoma in adult patients with acute myeloid leukemia. Cancer Sci. 2012; 103:1513.
10. Johnston DL, Alonzo TA, Gerbing RB, Lange BJ, Woods WG. Superior outcome of pediatric acute myeloid leukemia patients with orbital and CNS myeloid sarcoma: a report from the Children’s Oncology Group. Pediatr Blood Cancer. 2012; 58:519.
11. Hall MD, Chen YJ, Schultheiss TE, Pezner RD, Stein AS, Wong JY. Treatment outcomes for patients with chloroma receiving radiation therapy. J Med Imaging Radiat Oncol. 2014; 58:523.
12. Liehr T, Claussen U, Starke H. Small supernumerary marker chromosomes (sSMC) in humans. Cytogenet Genome Res. 2004; 107:55-67.