Simultaneous acute splenic sequestration and transient aplastic crisis in children with sickle cell disease

Yates, Amber Mayfield; Hankins, Jane S.; Mortier, Nicole A.; Aygün, Banu; Ware, Russell E.

doi:10.1002/pbc.22035

Cited by 19 publications

(26 citation statements)

References 18 publications

(28 reference statements)

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“…Rarely, the presence of splenic enlargement with reticulocytopenia in the setting of severe anemia may suggest the simultaneous development of both complications. (54) Most affected patients will require a simple transfusion administered slowly with careful monitoring for the development of congestive heart failure. Appropriate isolation measures should be taken for patients with SCD and suspected aplastic crisis, especially in the presence of pregnant and immunocompromised caregivers.…”

Section: Discussionmentioning

confidence: 99%

Sickle Cell Disease in the Emergency Department: Atypical Complications and Management

Brandow

Liem

2011

Clinical Pediatric Emergency Medicine

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Sickle cell disease is the most common inherited blood disorder in the United States. This disorder of hemoglobin structure leads to a chronic hemolytic anemia and complex chronic disease manifested by sudden, severe, and life-threatening complications. These acute complications can occur in any organ system beginning in early childhood and lasting throughout life. The intermittent nature and acuity of these complications lend the emergency department to be an important site of care. The hallmark of sickle cell disease is the vasoocclusive painful event. Other more “typical” complications include fever, acute chest syndrome, priapism, and ischemic stroke. Children with sickle cell disease can also present with other “atypical” complications that can have devastating consequences if they are unrecognized. Detailed discussion of these “atypical” sickle cell disease complications, organized by organ system involved, will be the focus of this article.

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Section: Discussionmentioning

confidence: 99%

Sickle Cell Disease in the Emergency Department: Atypical Complications and Management

Brandow

Liem

2011

Clinical Pediatric Emergency Medicine

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“…B19V is a potent inhibitor of hematopoiesis because it lytically infects erythroid progenitor cells (8), and in immunodeficient individuals, chronic B19V infection can result in chronic anemia (9). In individuals with underlying chronic hemolytic disorders, transient aplastic crisis following B19V infection can be a life-threatening complication (10). Finally, there is some evidence to suggest that B19V may infect other cell types, suggesting a possible involvement in the pathogenesis of a broad range of medical conditions, including idiopathic arthritis, vasculitis, meningoencephalitis, hepatitis, and myocarditis (11).…”

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confidence: 99%

Identification of Past and Recent Parvovirus B19 Infection in Immunocompetent Individuals by Quantitative PCR and Enzyme Immunoassays: a Dual-Laboratory Study

et al. 2014

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P arvoviruses (family Parvoviridae) are small (18-to 26-nm), nonenveloped, icosahedral viruses encapsidating a linear single-stranded DNA genome of approximately 5,000 nucleotides (1). Parvovirus B19, currently designated B19V, is a member of the family Parvoviridae, genus Erythrovirus (2). The B19V viral capsid has an icosahedral structure and is comprised of two proteins, VP1 (84 kDa) and VP2 (58 kDa). VP2 is the major protein (95% of the capsid composition) and contains receptor-and coreceptor-binding domains which together with self-assembly domains lead to the formation of highly stable particles (3). The minor capsid protein, VP1, differs from VP2 only in an N-terminal unique region (VP1u) composed of an additional 227 amino acids and has phospholipase A 2 (PLA 2 ) activity (4). VP1u elicits a dominant immune response (5).B19V infection in many instances is subclinical or presents as the mild and self-limiting fifth disease, or erythema infectiosum, typically manifesting as a "slapped cheeks" rash (6). Infection by B19V during pregnancy can result in several serious complications in the foetus, such as anemia, nonimmune hydrops, and ultimately intrauterine death (7). B19V is a potent inhibitor of hematopoiesis because it lytically infects erythroid progenitor cells (8), and in immunodeficient individuals, chronic B19V infection can result in chronic anemia (9). In individuals with underlying chronic hemolytic disorders, transient aplastic crisis following B19V infection can be a life-threatening complication (10). Finally, there is some evidence to suggest that B19V may infect other cell types, suggesting a possible involvement in the pathogenesis of a broad range of medical conditions, including idiopathic arthritis, vasculitis, meningoencephalitis, hepatitis, and myocarditis (11).Initially, the laboratory diagnosis of B19V infection was hampered by the lack of a cell culture system to grow the virus, necessitating the use of diagnostic tests lacking in sensitivity (12). Recently, recombinant DNA technology using prokaryotic (e.g., Escherichia coli) or eukaryotic (e.g., insect cells) expression systems has been applied to produce selected B19V antigens, in particular, VP1 and VP2. Prokaryotically expressed VP1/VP2 antigens undergo denaturation, and the antigen epitopes expressed are linear, whereas eukaryotic expression using baculovirus vectors generates empty capsids that are antigenically analogous to the native virus and include conformational epitopes. Assays using linear and those using conformational B19V VP1/VP2 can produce different results, leading to the general conclusion that conformational epitopes should be used for diagnostic purposes (13). Such assays need to be designed so that the presentation of conformational epitopes is optimized (14). Antibodies to linear VP2 epitopes are found mostly during acute infections and early convalescence, whereas those to conformational epitopes persist

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“…IVIg administration itself may trigger splenic sequestration of red‐cells, but has only previously been associated with splenic rupture in association with primary cytomegalovirus infection . Parvovirus infection has rarely been associated with splenic erythrocyte sequestration, but usually only in children with underlying sickle cell disease and this presentation is not associated with splenic rupture . In our patient, EMH was also seen within the spleen.…”

Section: Commentsmentioning

confidence: 99%

Atraumatic splenic rupture following IVIg for parvovirus B19 pure red cell aplasia post renal transplant

Tiong

Casan

McLean

2019

Transplant Infectious Dis

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Parvovirus B19 (PB19) associated pure red cell aplasia (PRCA) is an uncommon but well described complication of immunosuppression post solid organ transplantation. We report a unique case of a renal transplant patient with PB19 associated PRCA who developed a spontaneous splenic rupture after receiving IVIg for persistent anemia. He subsequently required splenectomy. Within the spleen we subsequently identified PB19 affected cells.

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Simultaneous acute splenic sequestration and transient aplastic crisis in children with sickle cell disease

Cited by 19 publications

References 18 publications

Sickle Cell Disease in the Emergency Department: Atypical Complications and Management

Sickle Cell Disease in the Emergency Department: Atypical Complications and Management

Identification of Past and Recent Parvovirus B19 Infection in Immunocompetent Individuals by Quantitative PCR and Enzyme Immunoassays: a Dual-Laboratory Study

Atraumatic splenic rupture following IVIg for parvovirus B19 pure red cell aplasia post renal transplant

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