Fatal infectious mononucleosis in a family
Two male siblings, one aged five and a half months (SB), and the other aged six months (VB), with fatal infectious mononucleosis phenotype of the X-linked lymphoproliferative syndrome, which resulted in the death of both infants, are presented. Both patients had been healthy, one until the ag...
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| Format: | Article |
| Language: | English |
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Hacettepe University Institute of Child Health
1990-04-01
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| Series: | The Turkish Journal of Pediatrics |
| Online Access: | https://turkjpediatr.org/article/view/3799 |
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| author | O Sanal G Kale F Ersoy S Göğüş Y Coşkun M Cağlar A I Berkel |
| author_facet | O Sanal G Kale F Ersoy S Göğüş Y Coşkun M Cağlar A I Berkel |
| author_sort | O Sanal |
| collection | DOAJ |
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Two male siblings, one aged five and a half months (SB), and the other aged six months (VB), with fatal infectious mononucleosis phenotype of the X-linked lymphoproliferative syndrome, which resulted in the death of both infants, are presented. Both patients had been healthy, one until the age of five and a half months, and the other until the age of six months. Then, they developed a maculopapular rash, hepatosplenomegaly and lymphadenopathy. In one sibling, the serum IgG level was low, the IgM and IgA levels were high, and the proportion of E-rosette forming cells (E-RFC) and in vitro proliferative response to PHA were normal. In the other sibling, however, the serum IgG level was normal, the IgM and IgA levels were high and the stimulation index for proliferative response to PHA was reduced due to increased spontaneous blastogenesis. Anti-EBV antibodies were negative in both siblings, except for the IgM anti-VCA in V.B. A lymph node specimen could be studied in one infant and was found to be positive for the EBV genome. Postmortem histopathological findings included the absence of cortico-medullary differentiation and identifiable Hassal's corpuscles in the thymus and depletion of T-dependent regions of lymph nodes and spleen in V.B. Atypical mononuclear cell infiltration was detected in the portal areas of the postmortem liver biopsy in S.B.
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| format | Article |
| id | doaj-art-35c2fe481bfb4d019930651786fdfd9f |
| institution | DOAJ |
| issn | 0041-4301 2791-6421 |
| language | English |
| publishDate | 1990-04-01 |
| publisher | Hacettepe University Institute of Child Health |
| record_format | Article |
| series | The Turkish Journal of Pediatrics |
| spelling | doaj-art-35c2fe481bfb4d019930651786fdfd9f2025-08-20T02:59:41ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64211990-04-01322Fatal infectious mononucleosis in a familyO Sanal0G KaleF ErsoyS GöğüşY CoşkunM CağlarA I BerkelDepartment of Pediatrics, Hacettepe University Faculty of Medicine, Ankara. Two male siblings, one aged five and a half months (SB), and the other aged six months (VB), with fatal infectious mononucleosis phenotype of the X-linked lymphoproliferative syndrome, which resulted in the death of both infants, are presented. Both patients had been healthy, one until the age of five and a half months, and the other until the age of six months. Then, they developed a maculopapular rash, hepatosplenomegaly and lymphadenopathy. In one sibling, the serum IgG level was low, the IgM and IgA levels were high, and the proportion of E-rosette forming cells (E-RFC) and in vitro proliferative response to PHA were normal. In the other sibling, however, the serum IgG level was normal, the IgM and IgA levels were high and the stimulation index for proliferative response to PHA was reduced due to increased spontaneous blastogenesis. Anti-EBV antibodies were negative in both siblings, except for the IgM anti-VCA in V.B. A lymph node specimen could be studied in one infant and was found to be positive for the EBV genome. Postmortem histopathological findings included the absence of cortico-medullary differentiation and identifiable Hassal's corpuscles in the thymus and depletion of T-dependent regions of lymph nodes and spleen in V.B. Atypical mononuclear cell infiltration was detected in the portal areas of the postmortem liver biopsy in S.B. https://turkjpediatr.org/article/view/3799 |
| spellingShingle | O Sanal G Kale F Ersoy S Göğüş Y Coşkun M Cağlar A I Berkel Fatal infectious mononucleosis in a family The Turkish Journal of Pediatrics |
| title | Fatal infectious mononucleosis in a family |
| title_full | Fatal infectious mononucleosis in a family |
| title_fullStr | Fatal infectious mononucleosis in a family |
| title_full_unstemmed | Fatal infectious mononucleosis in a family |
| title_short | Fatal infectious mononucleosis in a family |
| title_sort | fatal infectious mononucleosis in a family |
| url | https://turkjpediatr.org/article/view/3799 |
| work_keys_str_mv | AT osanal fatalinfectiousmononucleosisinafamily AT gkale fatalinfectiousmononucleosisinafamily AT fersoy fatalinfectiousmononucleosisinafamily AT sgogus fatalinfectiousmononucleosisinafamily AT ycoskun fatalinfectiousmononucleosisinafamily AT mcaglar fatalinfectiousmononucleosisinafamily AT aiberkel fatalinfectiousmononucleosisinafamily |