Pathogenesis of Respiratory Distress in Acute Leukemia with Hyperleukocytosis

BACKGROUND. In pediatric acute leukemias (AL), hyperleukocytosis (> 100 × 10⁹/L) is identified in 5–20 % of cases. It is very common in acute lymphoblastic leukemia (ALL). High count of circulating leukemic cells is associated with high proliferative potential of the tumor and underlies aggressive disease course. Leukemic infiltration of the lung tissue together with leukostasis contribute to respiratory distress, and pneumonia added to these disorders increases induction mortality. The therapy of this form of pediatric AL requires close cooperation with intensive care specialists.

AIM. To analyze clinical, radiology, and laboratory findings in pediatric AL with hyperleukocytosis and respiratory distress complications.

MATERIALS & METHODS. In the Research Institute of Pediatric Oncology and Hematology NN Blokhin National Medical Cancer Research Center, 208 children with newly diagnosed AL were treated from January 2022 to November 2023. In 26 (12.5 %) of them, the disease manifested with hyperleukocytosis onset. The leukocyte count varied from 123.9 to 994.77 × 10⁹/L (median 230 × 10⁹/L). Patients were aged from 1 month to 16 years. ALL was diagnosed in 22 (84.6 %) and acute myeloid leukemia in 4 (15.4 %) patients. Respiratory distress symptoms of varying severity were observed in 13 (50 %) out of 26 children.

RESULTS. In 5 (38.5 %) out of 13 patients with respiratory distress signs, artificial lung ventilation (ALV) appeared to be necessary. In 10 (76.9 %) children, respiratory distress symptoms were controlled by intensive supportive care combined with chemotherapy. No leukapheresis was used to manage hyperleukocytosis. Early mortality was 15.3 % (n = 2).

CONCLUSION. Lung damage with respiratory distress in AL children having hyperleukocytosis is a severe complication, which, along with acute tumor lysis syndrome, acute renal impairment and DIC syndrome, can cause death. The treatment of these patients is to begin with cytoreductive prephase with underlying massive supportive, infusion, and transfusion therapy. Bearing in mind all the above issues, it is advisable to start the treatment in an intensive care unit. It enables non-stop monitoring of vital functions and early use of assisted detoxification, leukapheresis, and ALV.

• Regina Ramilevna Tukhvatullina Research Institute of Pediatric Oncology and Hematology, NN Blokhin National Medical Cancer Research Center, 23 Kashirskoye sh., Moscow, Russian Federation, 115522 https://orcid.org/0000-0002-7126-2708 (unauthenticated)
• N.V. Matinyan Research Institute of Pediatric Oncology and Hematology, NN Blokhin National Medical Cancer Research Center, 23 Kashirskoye sh., Moscow, Russian Federation, 115522; NI Pirogov Russian National Research Medical University, 1 Ostrovityanova ul., Moscow, Russian Federation, 117513 https://orcid.org/0000-0001-7805-5616 (unauthenticated)
• T.T. Valiev Research Institute of Pediatric Oncology and Hematology, NN Blokhin National Medical Cancer Research Center, 23 Kashirskoye sh., Moscow, Russian Federation, 115522 https://orcid.org/0000-0002-1469-2365 (unauthenticated)
• O.A. Ignatenko Research Institute of Pediatric Oncology and Hematology, NN Blokhin National Medical Cancer Research Center, 23 Kashirskoye sh., Moscow, Russian Federation, 115522 https://orcid.org/0000-0003-2794-6886 (unauthenticated)
• N.V. Kokosadze PA Gertsen Moscow Oncology Research Institute, branch of the NMRC of Radiology, 3 2-i Botkinskii pr-d, Moscow, Russian Federation, 125284 https://orcid.org/0009-0004-8975-0568 (unauthenticated)
• S.R. Varfolomeeva Research Institute of Pediatric Oncology and Hematology, NN Blokhin National Medical Cancer Research Center, 23 Kashirskoye sh., Moscow, Russian Federation, 115522 https://orcid.org/0000-0001-6131-1783 (unauthenticated)

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