SICKLE CELL ACUTE CHEST SYNDROME ASSOCIATED WITH PARVOVIRUS B19 INFECTION: CASE SERIES AND REVIEW.

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.

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Subclinical Parvovirus B19 Infection in Children With Sickle Cell Anemia

Journal of Pediatric Hematology/Oncology ◽

10.1097/00043426-200305000-00007 ◽

2003 ◽

Vol 25 (5) ◽

pp. 387-389 ◽

Cited By ~ 18

Author(s):

Sherri A. Zimmerman ◽

Jacqueline S. Davis ◽

William H. Schultz ◽

Russell E. Ware

Keyword(s):

Sickle Cell ◽

Sickle Cell Anemia ◽

Parvovirus B19 ◽

Parvovirus B19 Infection

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Adverse Events in Sickle Cell Patients with Nocturnal Hypoxia.

Blood ◽

10.1182/blood.v106.11.3793.3793 ◽

2005 ◽

Vol 106 (11) ◽

pp. 3793-3793

Author(s):

Andrew D. Campbell ◽

Megumi Okumura ◽

Ndidi Unaka ◽

Sally Hutchinson ◽

Onyinye Onyekwere

Keyword(s):

Obstructive Sleep Apnea ◽

Sleep Apnea ◽

Sickle Cell Disease ◽

Adverse Events ◽

Sickle Cell ◽

Case Series ◽

Acute Chest Syndrome ◽

Cell Disease ◽

Obstructive Sleep ◽

The Relationship

Abstract The relationship between hemoglobin dexoygenation and sickling is well known. However, the relationship between hypoxia and severity of disease in sickle cell patients has not been well established. Recently, nocturnal hypoxemia has been associated with higher incidence of CNS events including strokes, and elevated TCDs. We present our case series on 13 patients(12 SS, 1 SC) with sickle cell disease (SCD) who have nocturnal hypoxia. Approximately 75 patients were screened at the University of Michigan Sickle Cell clinic for nocturnal hypoxia either by symptoms of obstructive sleep apnea or by longitudinal baseline clinic 02 saturations (02 Sat <92%). Of the 13 hypoxic pts, median baseline O2 Sat 90%(n=13, mean 90) and the median nocturnal O2sat (Nctnl 02 sat) 84%(n=13, mean 80%) with 10/13 with moderate-severe nocturnal hypoxia (O2sats<85%)based on sleep studies. Multiple adverse events noted in the cohort were pulmonary hypertension (PHTN TRJV>2.5, n=9, median 2.74/mean 2.74,) frequent pain episodes(>3visits to ER or hospitalizations/year, n=7, with 5 pts >5/year ), recurrent acute chest syndrome( ≥ 3 episodes, n= 10), CNS events (n=3 silent infarcts, vascular stenosis), priapism( (n=4, among 6 males ). Also reported were possible causes of the underlying hypoxia including obstructive sleep apnea(OSA)(n=7 of 11 pts), asthma(n= 10 of 13 pts), and chronic lung disease( n=8). In conclusion, the persistence of nocturnal hypoxia in pediatric sickle cell disease could possibly contribute to the development of severe complications of sickle cell disease. Treatment of underlying hypoxia (ie nighttime oxygen, maximize asthma treatment, T&A for OSA)may help prevent complications and lead to the improvement clinical symptoms. Further, chronic nocturnal hypoxia may complicate pulmonary disease and accelerated the development of PHTN. More studies are needed to clarify the mechanism of hypoxia in SCD. Table I. Clinical &Demographic Data of 13 SCD Patients with Nocturnal Hypoxia. Age:(6–22y/o, mean 15) Sex: M=6 F=7 Clinical: Total Mild Mod Sev. Genotype: SS=12, SC=1 Mean Range Nctnl Hypoxia(<%) 13 3 5(<85) 5(<80) Baseline O2 Sat(%) 90 +3.0 86–97 Obstr Sleep Apnea 7 3 3 1 Nctnl 02 sat (%) 80+8.4 59–87 Pulm HTN 9 4 4 1 Total #Apneic Events(11) 65.6+80 6–256 Rest. Lung Ds. 8 2 5 1 Obstr. Apneic Events(7) 27+68.5 0–221 # of Episodes <3 3–4 >4 Hypopneic Events(9) 32.5+38 0–132 ACS 2 5 5 TRJet Velocity 2.74+.42 2–3.5 Severe Pain Crises/yr 1 2 5

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Acute human parvovirus B19 infection in a 6-year old girl with sickle cell disease: a case report

Open Journal of Hematology ◽

10.13055/ojhmt_5_1_7.141007 ◽

2014 ◽

Vol 5 ◽

pp. 1

Author(s):

Daniele Don� ◽

Federica Visentin ◽

Eleonora Borgia ◽

Stefania Scanferla ◽

Carlo Giaquinto ◽

...

Keyword(s):

Case Report ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Parvovirus B19 ◽

Human Parvovirus B19 ◽

Cell Disease ◽

Parvovirus B19 Infection

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Sickle cell anemia, Aplastic crisis with Parvovirus B19 Infection

Indian Journal of Medical & Health Sciences ◽

10.21088/ijmhs.2347.9981.3216.11 ◽

2016 ◽

Vol 3 (2) ◽

pp. 135-137

Author(s):

Muhammad Kamal ◽

◽

Bakshi S.K. ◽

Keyword(s):

Sickle Cell ◽

Sickle Cell Anemia ◽

Parvovirus B19 ◽

Aplastic Crisis ◽

Parvovirus B19 Infection

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The detection, treatment of parvovirus B19 infection induced anemia in solid organ transplants: a case series and literature review of 194 patients

Transfusion Clinique et Biologique ◽

10.1016/j.tracli.2021.12.005 ◽

2022 ◽

Author(s):

Qiang Zhong ◽

Jun Zeng ◽

Tao Lin ◽

TuRun Song

Keyword(s):

Literature Review ◽

Parvovirus B19 ◽

Case Series ◽

Solid Organ ◽

Organ Transplants ◽

Parvovirus B19 Infection

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Acute Chest Syndrome Is Strongly Associated Parvo Virus B19 Seroconversion in Patients with Hemoglobin SC Disease.

Blood ◽

10.1182/blood.v104.11.1664.1664 ◽

2004 ◽

Vol 104 (11) ◽

pp. 1664-1664

Author(s):

Thomas D. Coates ◽

Carlton Dampier ◽

Karen Kalinyak ◽

Alice Lail ◽

William Mentzer ◽

...

Keyword(s):

Sickle Cell ◽

Odds Ratio ◽

Causal Relation ◽

Parvovirus B19 ◽

Statistical Significance ◽

Geographic Area ◽

Acute Chest Syndrome ◽

Aplastic Crisis ◽

Increased Risk ◽

Hemoglobin Sc Disease

Abstract Parvovirus B19 replicates principally in human erythrocyte precursors resulting in temporary cessation of erythropoiesis. In patients with sickle hemoglobinopathies, parvovirus B19 infection has been associated with acute chest syndrome and may lead to transient aplastic crisis. Evidence of prior infection with parvovirus B19 is found in 50 to 60% of adults. Infection appears to be followed by lifelong immunity. The objectives of the Study of Seroprevalence and Incidence of Parvovirus B19Infection in Children with Sickle Hemoglobinopathies are to determine the prevalence of parvovirus B19 antibodies in children less than 16 years old; determine the rate of parvovirus B19 seroconversion; and determine whether parvovirus B19 seroconversion is associated with the occurrence of aplastic crisis or acute chest syndrome. Between April 2000 and June 2002 a total of 1039 children from eight Comprehensive Sickle Cell Centers (CSCC) were enrolled in the study and 898 had at least one follow up visit. Participants ranged in age from <1 month to 16 years at enrollment (mean age 7.2, + 4.5 years), 47% were female, 53% were male. 67% had homozygous Hb SS disease, 24% had Hb SC disease, 6% had Hb SB+ disease, 3% had Hb SB0 disease, and <1% had other S sickle cell disease. The seroprevalence of parvovirus B19 in the cohort was 35%. Prevalence varied significantly by age, ranging from a low of 8% for children 1–2 years of age to 69% for children 13+ years of age (p<0.0001). The greatest increase in seroprevalence occurred between 6 and 7 years of age. Adjusted for age, there were no significant differences in seroprevalence by gender, clinical center, geographic area of residence (northeast, southeast, midwest, and west) sickle hemoglobinopathy, number of siblings, hemoglobin level, reticulocyte %, or among recipients of treatment with transfusion or hydroxyurea. Parvovirus B19 seroconversion was strongly associated with an increased risk of aplastic crisis in both SS (odds ratio of 29, 95% CI (11.3, 74.4)) and SC patients (odds ratio of 110, 95% CI (9.6, 1270.4)), even when controlling for age. Seroconversion was associated with an increased risk of acute chest syndrome in SC patients, (odds ratio of 6.4, 95% CI (1.9, 21.9)) but did not reach statistical significance in SS patients. These data show a clear causal relation between parvovirus B19 seroconversions and acute chest syndrome in subjects with hemoglobin SC disease and confirm the previously known association aplastic crisis.

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