scholarly journals A pediatric patient with sickle cell disease presenting with severe anemia and splenic sequestration in the setting of COVID‐19

2020 ◽  
Vol 67 (12) ◽  
Author(s):  
Smrithy Jacob ◽  
Aviva Dworkin ◽  
Eleny Romanos‐Sirakis
Author(s):  
Madison Irwin ◽  
William Gunther ◽  
Patricia Keefer ◽  
D'Anna Saul ◽  
Sharon Singh ◽  
...  

2021 ◽  
Author(s):  
Delaney Sheehan ◽  
Belinda Mantle ◽  
Ashley Kraft ◽  
Randall Craver ◽  
Christopher Arcement ◽  
...  

Author(s):  
Mohamed Almuqamam ◽  
◽  
Swetha Madhavarapu ◽  
Nataly Apollonsky ◽  
◽  
...  

Sickle Cell Disease (SCD) is an inherited hemoglobinopathy, which results in production of abnormal hemoglobin S. HbSC disease is a variant of SCD, which shares a similar clinical complication profile to HbSS disease, but often thought to be a milder condition. In patients with SCD, Hb S in deoxygenated state undergoes polymerization, leading to hemolysis, vaso-occlusive events, and eventually end-organ damage. Among other complications in patients with SCD is increased risk of complications caused by parvovirus B19. We present a case of a 14-year-old female with HbSC disease who presented to the emergency room with complaint of abdominal pain and found to have splenic sequestration. Splenic sequestration progressed rapidly, Hemoglobin (hb) dropped to 4.6 g/dl and acute chest syndrome (ACS) developed. She was treated following the ACS protocol, received 4 units of Packed Red Blood Cells (PRBC) and subsequently underwent a single volume PRBC exchange transfusion. Considering her unusual presentation, with severe ARDS from alveolar hemorrhage requiring mechanical ventilation and multi-organ injury, several autoimmune and infectious conditions with a cytokine storm component including COVID-19 disease, were considered. Results of viral testing revealed parvovirus B19 IgM antibodies signifying an acute infection. She fully recovered with supportive care and was discharged home. Multisystem involvement simulating connective tissue disorders or malignancies with acute parvovirus B19 infection has been reported and is considered extremely rare. To our knowledge, there were no reports of pediatric patients with SC disease presenting with splenic sequestration and ACS in the setting of parvovirus B19 multisystem disease. Keywords: sickle cell disease; acute respiratory distress syndrome; acute chest syndrome; parvovirus B19.


2000 ◽  
Vol 30 (12) ◽  
pp. 830-833 ◽  
Author(s):  
S. Sheth ◽  
C. Ruzal-Shapiro ◽  
S. Piomelli ◽  
W. E. Berdon

2020 ◽  
Vol 80 ◽  
pp. 102374
Author(s):  
Monia Ben Khaled ◽  
Monia Ouederni ◽  
Yosra Mankai ◽  
Samia Rekaya ◽  
Ilhem Ben Fraj ◽  
...  

Blood ◽  
1995 ◽  
Vol 86 (2) ◽  
pp. 776-783 ◽  
Author(s):  
FM Gill ◽  
LA Sleeper ◽  
SJ Weiner ◽  
AK Brown ◽  
R Bellevue ◽  
...  

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.


2015 ◽  
Vol 37 (4) ◽  
pp. 311-314 ◽  
Author(s):  
Natalia Maximova ◽  
Davide Zanon ◽  
Lorella Pascolo ◽  
Floriana Zennaro ◽  
Massimo Gregori ◽  
...  

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