Cyclic Bicytopenia in a Patient with Shapiro Syndrome

Shapiro syndrome and periodic hypothermia have been reported approximately fifty times in the literature. Shapiro syndrome is defined as the constellation of periodic hypothermia and hyperhidrosis along with agenesis of the corpus callosum by Shapiro et al. in 1969. Periodic hypothermia is a more br...

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Main Authors: Lindsey E. Roeker, Vinay Gupta, Wilson I. Gonsalves, Alexandra P. Wolanskyj, Naseema Gangat
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Hematology
Online Access:http://dx.doi.org/10.1155/2013/231713
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author Lindsey E. Roeker
Vinay Gupta
Wilson I. Gonsalves
Alexandra P. Wolanskyj
Naseema Gangat
author_facet Lindsey E. Roeker
Vinay Gupta
Wilson I. Gonsalves
Alexandra P. Wolanskyj
Naseema Gangat
author_sort Lindsey E. Roeker
collection DOAJ
description Shapiro syndrome and periodic hypothermia have been reported approximately fifty times in the literature. Shapiro syndrome is defined as the constellation of periodic hypothermia and hyperhidrosis along with agenesis of the corpus callosum by Shapiro et al. in 1969. Periodic hypothermia is a more broad diagnosis with a number of proposed mechanisms; it occurs in patients without structural brain abnormalities. Hematologic abnormalities beyond iron-deficiency anemia have not been documented in any of the reported cases of Shapiro syndrome or periodic hypothermia. Though accidental and therapeutic hypothermia have been associated with thrombocytopenia, this is, to our knowledge, the first reported case of periodic intrinsic hypothermia causing bicytopenia. In this report, we present the case of a patient with Shapiro syndrome who experienced cyclic bicytopenia mirroring hypothermic episodes. We address the differential diagnosis of bicytopenia, review the mechanisms proposed for cytopenias related to hypothermia, and propose possible mechanisms for the finding in this case.
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spelling doaj-art-258c7ab64b1b4eccb1d7baf105458f442025-02-03T05:44:41ZengWileyCase Reports in Hematology2090-65602090-65792013-01-01201310.1155/2013/231713231713Cyclic Bicytopenia in a Patient with Shapiro SyndromeLindsey E. Roeker0Vinay Gupta1Wilson I. Gonsalves2Alexandra P. Wolanskyj3Naseema Gangat4Mayo Medical School, Mayo Clinic, Rochester, USADepartment of Hematology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USADepartment of Hematology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USAMayo Medical School, Mayo Clinic, Rochester, USADepartment of Hematology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USAShapiro syndrome and periodic hypothermia have been reported approximately fifty times in the literature. Shapiro syndrome is defined as the constellation of periodic hypothermia and hyperhidrosis along with agenesis of the corpus callosum by Shapiro et al. in 1969. Periodic hypothermia is a more broad diagnosis with a number of proposed mechanisms; it occurs in patients without structural brain abnormalities. Hematologic abnormalities beyond iron-deficiency anemia have not been documented in any of the reported cases of Shapiro syndrome or periodic hypothermia. Though accidental and therapeutic hypothermia have been associated with thrombocytopenia, this is, to our knowledge, the first reported case of periodic intrinsic hypothermia causing bicytopenia. In this report, we present the case of a patient with Shapiro syndrome who experienced cyclic bicytopenia mirroring hypothermic episodes. We address the differential diagnosis of bicytopenia, review the mechanisms proposed for cytopenias related to hypothermia, and propose possible mechanisms for the finding in this case.http://dx.doi.org/10.1155/2013/231713
spellingShingle Lindsey E. Roeker
Vinay Gupta
Wilson I. Gonsalves
Alexandra P. Wolanskyj
Naseema Gangat
Cyclic Bicytopenia in a Patient with Shapiro Syndrome
Case Reports in Hematology
title Cyclic Bicytopenia in a Patient with Shapiro Syndrome
title_full Cyclic Bicytopenia in a Patient with Shapiro Syndrome
title_fullStr Cyclic Bicytopenia in a Patient with Shapiro Syndrome
title_full_unstemmed Cyclic Bicytopenia in a Patient with Shapiro Syndrome
title_short Cyclic Bicytopenia in a Patient with Shapiro Syndrome
title_sort cyclic bicytopenia in a patient with shapiro syndrome
url http://dx.doi.org/10.1155/2013/231713
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