Literature DB >> 31005860

Hunter syndrome with persistent thrombocytopenia.

Inusha Panigrahi1, Manoj Dhanorkar1, Siyaram Didel1, Raja Ashok Koganti1.   

Abstract

A case of Hunter syndrome, 6½-year-old boy presented with persistent thrombocytopenia and bleeding diathesis. However, cytopenia is not a usual presentation in patients with mucopolysaccharidosis II. After ruling out other causes of severe thrombocytopenia, a clinical possibility of chronic Epstein-Barr virus (EBV) infection was considered. He was treated with intravenous immunoglobulin for refractory thrombocytopenia and intracranial bleed. This was followed by oral prednisolone. The EBV serology was found positive. Platelet counts gradually recovered and no recurrence of bleeds was observed. EBV infection usually causes haematological abnormalities, mainly atypical lymphocytosis, which is a feature of infectious mononucleosis, and uncomplicated cases often present with mild decreases in platelet counts. Severe thrombocytopenia is an extremely rare complication of acute or chronic EBV infection. In Asians, EBV infection should be considered in children presenting with thrombocytopenia, bleeding diathesis and anaemia along with organomegaly. © BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.

Entities:  

Keywords:  MPS II; anaemia; bleeding; chronic EBV infection; platelets

Mesh:

Substances:

Year:  2019        PMID: 31005860      PMCID: PMC6506028          DOI: 10.1136/bcr-2018-226518

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  8 in total

1.  Intravenous gammaglobulin treatment for immune thrombocytopenia associated with infectious mononucleosis.

Authors:  E M Cyran; J M Rowe; R E Bloom
Journal:  Am J Hematol       Date:  1991-10       Impact factor: 10.047

2.  Hunter syndrome and new onset idiopathic thrombocytopenic purpura in a young patient.

Authors:  Burak Uz; Haluk Demiroglu; Osman Ilhami Ozcebe
Journal:  Ann Hematol       Date:  2011-05-10       Impact factor: 3.673

3.  Acute Epstein-Barr virus infection complicated by severe thrombocytopenia.

Authors:  M L Pipp; N D Means; J W Sixbey; K L Morris; C L Gue; L M Baddour
Journal:  Clin Infect Dis       Date:  1997-11       Impact factor: 9.079

4.  Acute disseminated encephalomyelitis and thrombocytopenia following Epstein-Barr virus infection.

Authors:  Muhammad Saeed; Omar Dabbagh; Muhammad Al-Muhaizae; Hesham Dhalaan; Aziza Chedrawi
Journal:  J Coll Physicians Surg Pak       Date:  2014-11       Impact factor: 0.711

5.  NK and B cell deficiency in a MPS type II family with novel mutation in the IDS gene.

Authors:  Leuridan Cavalcante Torres; Diogo Cordeiro de Queiroz Soares; Leslie Domenici Kulikowski; Jose Francisco Franco; Chong Ae Kim
Journal:  Clin Immunol       Date:  2014-07-16       Impact factor: 3.969

Review 6.  Atypical manifestations of Epstein-Barr virus in children: a diagnostic challenge.

Authors:  Vasileios Bolis; Christos Karadedos; Ioannis Chiotis; Nikolaos Chaliasos; Sophia Tsabouri
Journal:  J Pediatr (Rio J)       Date:  2016-01-20       Impact factor: 2.197

7.  Survival in idursulfase-treated and untreated patients with mucopolysaccharidosis type II: data from the Hunter Outcome Survey (HOS).

Authors:  Barbara K Burton; Virginie Jego; Jaromir Mikl; Simon A Jones
Journal:  J Inherit Metab Dis       Date:  2017-09-08       Impact factor: 4.982

8.  Severe thrombocytopenia as a complication of acute Epstein-Barr virus infection.

Authors:  Robert Likic; Dusko Kuzmanic
Journal:  Wien Klin Wochenschr       Date:  2004-01-31       Impact factor: 2.275

  8 in total
  3 in total

1.  Comparisons Between Infectious and Autoimmune Encephalitis: Clinical Signs, Biochemistry, Blood Counts, and Imaging Findings.

Authors:  Chen-Na Huang; Xiao-Bing Tian; Shu-Min Jiang; Sheng-Hui Chang; Nan Wang; Ming-Qi Liu; Qiu-Xia Zhang; Ting Li; Lin-Jie Zhang; Li Yang
Journal:  Neuropsychiatr Dis Treat       Date:  2020-11-04       Impact factor: 2.570

2.  The Value of Case Reports in Systematic Reviews from Rare Diseases. The Example of Enzyme Replacement Therapy (ERT) in Patients with Mucopolysaccharidosis Type II (MPS-II).

Authors:  Miguel Sampayo-Cordero; Bernat Miguel-Huguet; Andrea Malfettone; José Manuel Pérez-García; Antonio Llombart-Cussac; Javier Cortés; Almudena Pardo; Jordi Pérez-López
Journal:  Int J Environ Res Public Health       Date:  2020-09-10       Impact factor: 3.390

Review 3.  Non-cardiac Manifestations in Adult Patients With Mucopolysaccharidosis.

Authors:  Karolina M Stepien; Andrew Bentley; Cliff Chen; M Wahab Dhemech; Edward Gee; Peter Orton; Catherine Pringle; Jonathan Rajan; Ankur Saxena; Govind Tol; Chaitanya Gadepalli
Journal:  Front Cardiovasc Med       Date:  2022-03-07
  3 in total

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