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Blood Res 2021; 56(2):

Published online June 30, 2021

https://doi.org/10.5045/br.2021.2021030

© The Korean Society of Hematology

Leukoerythroblastosis with dyserythropoiesis in an adolescent with SARS-CoV-2-associated macrophage activation syndrome

Mahtab Fakhari1, Jeremy M. Loberger2, Geling Li1,3

1Department of Pathology, 2Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Alabama at Birmingham, 3Department of Pathology and Laboratory Medicine, Children’s of Alabama, Birmingham, AL, USA

Correspondence to : Geling Li, M.D., Ph.D., Department of Pathology and Laboratory Medicine, Children’s of Alabama, University of Alabama, 1600 7th Avenue South, Birmingham, AL 35233, USA, E-mail: geling.li@childrensal.org

Received: February 13, 2021; Revised: March 4, 2021; Accepted: March 11, 2021

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

A 15-year-old, previously healthy male with low-risk obesity and recent SARS-CoV-2 exposure presented with severe hyperglycemic hyperosmolar syndrome, metabolic encephalopathy, and hypovolemic shock necessitating mechanical ventilation. Biochemical tests showed elevated ferritin (3,542 mg/L), triglycerides (10.5 mmol/L), and glucose (32.2 mmol/L). His CBC showed mildly elevated leukocytes (13,900/mL) and neutrophils (11,920/mL). An ultrasound showed hepatomegaly. He rapidly developed anemia (Hb 7.6 g/dL), thrombocytopenia (platelets, 57,000/mL), and monocytosis (5,900/mL) with continued worsening shock requiring multiple inotropes, dialysis, and increased ventilatory support. After initially improving, he again developed significant lung disease along with a new fever. He was then diagnosed with SARS-CoV-2 via PCR and severe acute respiratory distress syndrome. Ultimately, he was cannulated on to veno-venous extracorporeal life support for refractory hypoxemic respiratory failure. He was also diagnosed with macrophage activation syndrome secondary to the SARS-CoV-2 infection. For this, he was treated with steroids and 6 days of Anakinra. Laboratory testing showed increased ferritin (19,894 mg/L), sCD25 (1,770 U/mL), inflammatory cytokines (IL-6, IL-8, IL-10, IL-18, and CXCL9), and decreased CD107a (4%). A PB smear showed numerous nucleated RBC (352/100 WBC), including 30% dysmorphic nRBCs (long arrows), immature granulocytes (long dotted arrows) (10%), vacuolated monocytes (arrowheads) (22%), and occasional bilobed neutrophils (short arrows) (A and B). Ultimately, he developed intracranial hemorrhagic complications, and support was withdrawn.

Article

Image of Hematology

Blood Res 2021; 56(2): 59-59

Published online June 30, 2021 https://doi.org/10.5045/br.2021.2021030

Copyright © The Korean Society of Hematology.

Leukoerythroblastosis with dyserythropoiesis in an adolescent with SARS-CoV-2-associated macrophage activation syndrome

Mahtab Fakhari1, Jeremy M. Loberger2, Geling Li1,3

1Department of Pathology, 2Division of Pediatric Critical Care Medicine, Department of Pediatrics, University of Alabama at Birmingham, 3Department of Pathology and Laboratory Medicine, Children’s of Alabama, Birmingham, AL, USA

Correspondence to:Geling Li, M.D., Ph.D., Department of Pathology and Laboratory Medicine, Children’s of Alabama, University of Alabama, 1600 7th Avenue South, Birmingham, AL 35233, USA, E-mail: geling.li@childrensal.org

Received: February 13, 2021; Revised: March 4, 2021; Accepted: March 11, 2021

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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A 15-year-old, previously healthy male with low-risk obesity and recent SARS-CoV-2 exposure presented with severe hyperglycemic hyperosmolar syndrome, metabolic encephalopathy, and hypovolemic shock necessitating mechanical ventilation. Biochemical tests showed elevated ferritin (3,542 mg/L), triglycerides (10.5 mmol/L), and glucose (32.2 mmol/L). His CBC showed mildly elevated leukocytes (13,900/mL) and neutrophils (11,920/mL). An ultrasound showed hepatomegaly. He rapidly developed anemia (Hb 7.6 g/dL), thrombocytopenia (platelets, 57,000/mL), and monocytosis (5,900/mL) with continued worsening shock requiring multiple inotropes, dialysis, and increased ventilatory support. After initially improving, he again developed significant lung disease along with a new fever. He was then diagnosed with SARS-CoV-2 via PCR and severe acute respiratory distress syndrome. Ultimately, he was cannulated on to veno-venous extracorporeal life support for refractory hypoxemic respiratory failure. He was also diagnosed with macrophage activation syndrome secondary to the SARS-CoV-2 infection. For this, he was treated with steroids and 6 days of Anakinra. Laboratory testing showed increased ferritin (19,894 mg/L), sCD25 (1,770 U/mL), inflammatory cytokines (IL-6, IL-8, IL-10, IL-18, and CXCL9), and decreased CD107a (4%). A PB smear showed numerous nucleated RBC (352/100 WBC), including 30% dysmorphic nRBCs (long arrows), immature granulocytes (long dotted arrows) (10%), vacuolated monocytes (arrowheads) (22%), and occasional bilobed neutrophils (short arrows) (A and B). Ultimately, he developed intracranial hemorrhagic complications, and support was withdrawn.

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