IMAGES IN HEMATOLOGY

A case of acute monocytic leukemia in an elderly patient

Un caz de leucemie acută monocitară la un pacient vârstnic

Abstract

Acute monocytic leukemia is a distinct subtype of acute mye­loid leukemia, with ≥80% blasts being of monocytic lineage, displaying a variable degree of maturation. We re­port the case of an elderly patient admitted in the Hema­to­lo­gy Unit of the Emergency University Hos­pi­tal Bucharest, Romania, with hyperleukocytosis, gingival hypertrophy, pa­la­tal hematoma, and moderate hepatosplenomegaly, that displayed on the peripheral blood smear a pre­do­mi­nance of monocytic precursors at a more mature stage – promonocytes. It is highlighted the importance of cor­rect discrimination between different monocytic cells, pro­mo­no­cytes being counted as blasts.



Keywords
acute monocytic leukemiapromonocyteshyperleukocytosis

Rezumat

Leucemia acută monocitară este un subtip distinct de leu­ce­mie acută mieloidă, în care ≥80% din blaști aparțin se­ri­ei monocitare, având un grad variabil de maturație. Ra­por­tăm cazul unei paciente vârstnice, internată în Clinica de hematologie a Spitalului Universitar de Urgență din Bu­cu­reşti, România, cu hiperleucocitoză, hipertrofie gingivală, he­ma­tom palatin și hepatosplenomegalie moderată, care a prezentat predominant la examenul frotiului de sânge pe­ri­fe­ric precursori monocitari într-un stadiu intermediar de ma­tu­ra­ție – promonocite. Este evidențiată importanța dis­cri­mi­nă­rii co­rec­te a diferitelor tipuri de celule monocitice, pro­mo­no­ci­tele fiind incluse în procentajul de blaști.

Cuvinte Cheie
leucemie acută monocitarăpromonocitehiperleucocitoză

A 76-year-old female patient presented in the Emergency Department of the of the Emergency University Hospital Bucharest, Romania, with fatigue, dyspnea, headache, dizziness, tender and bleeding gums.

The physical exam revealed gingival hypertrophy, palatal hematoma, and moderate hepatosplenomegaly.

Chest computed tomography with diffuse bilateral interstitial opacities was suggestive for pulmonary leukostasis.

Automatic complete blood count (CBC) indicated hyperleukocytosis (125 x 109/L), mild anemia (hemoglobin 10.1 g/dL) and severe thrombocytopenia (15 x 109/L).

Peripheral blood smear showed 85% large immature cells, most of them with folded nuclei, compatible with promonocytes (blast equivalents); only a few cells could be morphologically assigned to monoblasts (Figure 1).

Figure 1. Representative images of peripheral blood smear (100x magnification) depicting a population of large immature cells, most of them with indented or delicately folded nuclei, dispersed chromatin and nucleoli, moderate amount of blue-gray cytoplasm, finely granulated and vacuolated in some cells (promonocytes, A, B). A cell displaying round/ovalar nucleus, lace-like chromatin with nucleoli, moderate amount of basophilic cytoplasm, with vacuoles is shown in panel C (monoblast). For comparison, a normal monocyte (lobulated nucleus, condensed chromatin) is included in panel D
Figure 1. Representative images of peripheral blood smear (100x magnification) depicting a population of large immature cells, most of them with indented or delicately folded nuclei, dispersed chromatin and nucleoli, moderate amount of blue-gray cytoplasm, finely granulated and vacuolated in some cells (promonocytes, A, B). A cell displaying round/ovalar nucleus, lace-like chromatin with nucleoli, moderate amount of basophilic cytoplasm, with vacuoles is shown in panel C (monoblast). For comparison, a normal monocyte (lobulated nucleus, condensed chromatin) is included in panel D

Flow cytometry analysis performed in the peripheral blood revealed 80% blast cells, with the following phenotype: CD34-, HLA-DR+, CD33+, CD64+, CD36+, CD14+/-, CD11b+. According to the 2022 World Health Organization classification of myeloid neoplasms(1), the presence of ≥80% precursors expressing at least two monocytic markers, including CD11c, CD14, CD36 and CD64, establishes the diagnosis of acute monocytic leukemia.

Molecular testing using DNA fragment analysis detected both FLT3-ITD and NPM1 mutations.

Acute monocytic leukemia, subtype M5 in the French-American-British classification system, accounts for approximately 10% of all acute myeloid leukemia cases. Extramedullary infiltrates with monocytic blasts displaying a variable degree of maturation are relatively common, involving gums (gingival hypertrophy), skin (leukemia cutis), central nervous system, lymph nodes, liver or spleen(2,3).

NPM1 and/or FLT3-ITD mutations accompanied by marked leukocytosis leading to leukostasis are frequently encountered(4). As highlighted in the images, promonocytes should be correctly recognized and counted as blasts.   

 

Corresponding author: Cristina Mambet E-mail: cristina.mambet@gmail.com

Conflict of interest: none declared.

Financial support: none declared.

This work is permanently accessible online free of charge and published under the CC-BY licence.

Figure:

Bibliografie


  1. Fontenay M, Germing U, Gujral S, et al. The 5th Edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms. Leukemia. 2022;36(7):1703-1719. 
  2. Döhner H, Wei AH, Appelbaum FR, et al. Diagnosis and management of AML in adults: 2022 recommendations from an international expert panel on behalf of the ELN. Blood. 2022;140(12):1345-1377.
  3. McCloskeyJK, Depadova S, Koprivnikar JL, et al. Features and Outcome of Patients (Pts) with New Diagnosis of Acute Myeloid Leukemia (AML) with Monocytic Differentiation (AML-Mo) Following Standard Induction Therapy. Do We Need a Different Strategy?. Blood. 2016;128 (22): 4017.
  4. Bewersdorf JP, Zeidan AM. Hyperleukocytosis and Leukostasis in Acute Myeloid Leukemia: Can a Better Understanding of the Underlying Molecular Pathophysiology Lead to Novel Treatments?. Cells. 2020;9(10):2310.
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