[Epstein-Barr virus infection with severe consequences. EBV, haemophagocytic lymphohistiocytosis and Hodgkin lymphoma with Down syndrome]. 2014

Pieter C J Kuitert, and Floor C H Abbink, and Chantal J M Broers, and Paul van der Valk, and A Marceline van Furth, and Martijn van der Kuip
VU medisch centrum, afd. Kindergeneeskunde, Amsterdam.

BACKGROUND Over 90% of the population is infected with the Epstein-Barr virus (EBV). Following primary infection, the virus remains latent in B-lymphocytes. In isolated cases, especially in immunocompromised patients, the Epstein-Barr virus can result in a chronic active infection (CAEBV). METHODS We describe an 11-year-old boy with Down syndrome who was admitted because of fever of unknown origin during several periods. Serological findings (high VCA-IgG and absent EBNA-IgG) were suggestive of CAEBV, which was confirmed by the high circulating EBV viral load. During admission the clinical picture worsened and our patient developed pancytopenia, which led us to diagnose concurrent haemophagocytic lymphohistiocytosis and Hodgkin lymphoma. CONCLUSIONS CAEBV is the result of deficiency in cellular immunity, which in this patient could possibly be attributed to deficiencies in the immune system associated with Down syndrome. CAEBV is difficult to treat, and it can come with life-threatening complications such as haemophagocytic lymphohistiocytosis.

UI MeSH Term Description Entries
D008297 Male Males
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002908 Chronic Disease Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2). Chronic Condition,Chronic Illness,Chronically Ill,Chronic Conditions,Chronic Diseases,Chronic Illnesses,Condition, Chronic,Disease, Chronic,Illness, Chronic
D004314 Down Syndrome A chromosome disorder associated either with an extra CHROMOSOME 21 or an effective TRISOMY for chromosome 21. Clinical manifestations include HYPOTONIA, short stature, BRACHYCEPHALY, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, single transverse palmar crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213) Mongolism,Trisomy 21,47,XX,+21,47,XY,+21,Down Syndrome, Partial Trisomy 21,Down's Syndrome,Partial Trisomy 21 Down Syndrome,Trisomy 21, Meiotic Nondisjunction,Trisomy 21, Mitotic Nondisjunction,Trisomy G,Downs Syndrome,Syndrome, Down,Syndrome, Down's
D006689 Hodgkin Disease A malignant disease characterized by progressive enlargement of the lymph nodes, spleen, and general lymphoid tissue. In the classical variant, giant usually multinucleate Hodgkin's and REED-STERNBERG CELLS are present; in the nodular lymphocyte predominant variant, lymphocytic and histiocytic cells are seen. Granuloma, Hodgkin,Granuloma, Malignant,Hodgkin Lymphoma,Lymphogranuloma, Malignant,Granuloma, Hodgkin's,Granuloma, Hodgkins,Hodgkin Lymphoma, Adult,Hodgkin's Disease,Hodgkin's Lymphoma,Hodgkins Disease,Lymphocyte Depletion Hodgkin's Lymphoma,Lymphocyte-Rich Classical Hodgkin's Lymphoma,Mixed Cellularity Hodgkin's Lymphoma,Nodular Lymphocyte-Predominant Hodgkin's Lymphoma,Nodular Sclerosing Hodgkin's Lymphoma,Adult Hodgkin Lymphoma,Disease, Hodgkin,Disease, Hodgkin's,Disease, Hodgkins,Hodgkin Granuloma,Hodgkin's Granuloma,Hodgkins Granuloma,Hodgkins Lymphoma,Lymphocyte Rich Classical Hodgkin's Lymphoma,Lymphogranulomas, Malignant,Lymphoma, Hodgkin,Lymphoma, Hodgkin's,Malignant Granuloma,Malignant Granulomas,Malignant Lymphogranuloma,Malignant Lymphogranulomas,Nodular Lymphocyte Predominant Hodgkin's Lymphoma
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D051359 Lymphohistiocytosis, Hemophagocytic A group of related disorders characterized by LYMPHOCYTOSIS; HISTIOCYTOSIS; and hemophagocytosis. The two major forms are familial and reactive. Familial Hemophagocytic Lymphocytosis,Hemophagocytic Lymphohistiocytosis, Familial,Hemophagocytic Syndrome, Infection-Associated,Hemophagocytic Syndrome, Reactive,Hemophagocytic Syndromes,Reactive Hemophagocytic Syndrome,Erythrophagocytic Lymphohistiocytosis, Familial,Familial Erythrophagocytic Lymphohistiocytosis,Familial Hemophagocytic Histiocytosis,Familial Hemophagocytic Lymphohistiocytosis,Familial Hemophagocytic Reticulosis,Familial Histiocytic Reticulosis,Hemophagocytic Lymphohistiocytosis Familial -1,Hemophagocytic Lymphohistiocytosis, Familial, 1,Hemophagocytic Reticulosis, Familial,Hemophagocytic Syndrome,Primary Hemophagocytic Hymphohistiocytosis,Primary Hemophagocytic Lymphohistiocytosis,Reticulosis, Familial Histiocytic,Erythrophagocytic Lymphohistiocytoses, Familial,Familial Erythrophagocytic Lymphohistiocytoses,Familial Hemophagocytic Histiocytoses,Familial Hemophagocytic Lymphocytoses,Familial Hemophagocytic Lymphohistiocytoses,Familial Hemophagocytic Reticuloses,Familial Histiocytic Reticuloses,Hemophagocytic Histiocytoses, Familial,Hemophagocytic Histiocytosis, Familial,Hemophagocytic Hymphohistiocytoses, Primary,Hemophagocytic Hymphohistiocytosis, Primary,Hemophagocytic Lymphocytoses, Familial,Hemophagocytic Lymphocytosis, Familial,Hemophagocytic Lymphohistiocytoses,Hemophagocytic Lymphohistiocytoses, Familial,Hemophagocytic Lymphohistiocytoses, Primary,Hemophagocytic Lymphohistiocytosis,Hemophagocytic Lymphohistiocytosis Familial 1,Hemophagocytic Lymphohistiocytosis, Primary,Hemophagocytic Reticuloses, Familial,Hemophagocytic Syndrome, Infection Associated,Histiocytic Reticuloses, Familial,Histiocytic Reticulosis, Familial,Histiocytoses, Familial Hemophagocytic,Histiocytosis, Familial Hemophagocytic,Hymphohistiocytoses, Primary Hemophagocytic,Hymphohistiocytosis, Primary Hemophagocytic,Infection-Associated Hemophagocytic Syndrome,Lymphocytoses, Familial Hemophagocytic,Lymphocytosis, Familial Hemophagocytic,Lymphohistiocytoses, Familial Erythrophagocytic,Lymphohistiocytoses, Familial Hemophagocytic,Lymphohistiocytoses, Hemophagocytic,Lymphohistiocytoses, Primary Hemophagocytic,Lymphohistiocytosis, Familial Erythrophagocytic,Lymphohistiocytosis, Familial Hemophagocytic,Lymphohistiocytosis, Primary Hemophagocytic,Primary Hemophagocytic Hymphohistiocytoses,Primary Hemophagocytic Lymphohistiocytoses,Reticuloses, Familial Hemophagocytic,Reticuloses, Familial Histiocytic,Reticulosis, Familial Hemophagocytic
D019562 Viral Load The quantity of measurable virus in a body fluid. Change in viral load, measured in plasma, is sometimes used as a SURROGATE MARKER in disease progression. Viral Burden,Virus Titer,Burden, Viral,Load, Viral,Titer, Virus
D020031 Epstein-Barr Virus Infections Infection with human herpesvirus 4 (HERPESVIRUS 4, HUMAN); which may facilitate the development of various lymphoproliferative disorders. These include BURKITT LYMPHOMA (African type), INFECTIOUS MONONUCLEOSIS, and oral hairy leukoplakia (LEUKOPLAKIA, HAIRY). EBV Infections,Epstein-Barr Virus Infection,Herpesvirus 4 Infections, Human,Human Herpes Virus 4 Infections,Human Herpesvirus 4 Infections,Infections, EBV,Infections, Epstein-Barr Virus,EBV Infection,Epstein Barr Virus Infection,Epstein Barr Virus Infections,Infection, EBV,Infection, Epstein-Barr Virus,Virus Infection, Epstein-Barr,Virus Infections, Epstein-Barr

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