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Histiocytosis‐lymphadenopathy plus syndrome revealed by repeated secondary hemophagocytic lymphohistiocytosis

Histiocytosis‐lymphadenopathy plus syndrome revealed by repeated secondary hemophagocytic... We report the follow‐up of an immunocompetent child with histiocytosis‐lymphadenopathy plus syndrome who had previously developed hemophagocytic lymphohistiocytosis (HLH), secondary to Parvovirus infection.1The patient first presented in 2019, at three years of age, with complaints of fever, rash, hepatosplenomegaly, and pancytopenia. She was the first‐born child of a non‐consanguineous marriage. The bone marrow biopsy revealed the presence of macro‐ovalocytes, histiocytes, and myelofibrosis. The serum anti‐parvoviral immunoglobulins were raised which established the diagnosis of parvoviral infection with secondary HLH. She improved on conservative treatment. On follow‐up, her counts were within normal limits, and she remained asymptomatic in the next 2 years.1The patient presented again in June 2021, with two months history of intermittent, high‐grade fever. Her nasal swab sample had tested positive for SARS‐CoV2, on polymerase chain reaction (PCR), in May 2021. Fever initially responded to oral dexamethasone (4 mg/day, given for seven days), before reappearing 3 weeks later, when she was retested and found positive for SARS‐CoV2 on PCR test (persistent positivity in the absence of a negative report). She maintained saturation on room air but was hospitalized elsewhere, for persistent fever and COVID positivity with a suspicion of post‐COVID multisystem inflammatory syndrome in children (MIS‐C), for which she received intravenous immunoglobulins http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Pediatric Allergy and Immunology Wiley

Histiocytosis‐lymphadenopathy plus syndrome revealed by repeated secondary hemophagocytic lymphohistiocytosis

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References (10)

Publisher
Wiley
Copyright
Copyright © 2022 John Wiley & Sons A/S
ISSN
0905-6157
eISSN
1399-3038
DOI
10.1111/pai.13787
Publisher site
See Article on Publisher Site

Abstract

We report the follow‐up of an immunocompetent child with histiocytosis‐lymphadenopathy plus syndrome who had previously developed hemophagocytic lymphohistiocytosis (HLH), secondary to Parvovirus infection.1The patient first presented in 2019, at three years of age, with complaints of fever, rash, hepatosplenomegaly, and pancytopenia. She was the first‐born child of a non‐consanguineous marriage. The bone marrow biopsy revealed the presence of macro‐ovalocytes, histiocytes, and myelofibrosis. The serum anti‐parvoviral immunoglobulins were raised which established the diagnosis of parvoviral infection with secondary HLH. She improved on conservative treatment. On follow‐up, her counts were within normal limits, and she remained asymptomatic in the next 2 years.1The patient presented again in June 2021, with two months history of intermittent, high‐grade fever. Her nasal swab sample had tested positive for SARS‐CoV2, on polymerase chain reaction (PCR), in May 2021. Fever initially responded to oral dexamethasone (4 mg/day, given for seven days), before reappearing 3 weeks later, when she was retested and found positive for SARS‐CoV2 on PCR test (persistent positivity in the absence of a negative report). She maintained saturation on room air but was hospitalized elsewhere, for persistent fever and COVID positivity with a suspicion of post‐COVID multisystem inflammatory syndrome in children (MIS‐C), for which she received intravenous immunoglobulins

Journal

Pediatric Allergy and ImmunologyWiley

Published: May 1, 2022

Keywords: H syndrome; HLH; intravenous immunoglobulin; multisystem inflammatory syndrome; post‐viral immune dysregulation; SARS‐CoV2

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