A Clinical and Ultrastructural Study of Fechtner Syndrome in Two Japanese Families
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- OHATA Masahiko
- Department of Clinical Laboratory, Shizuoka Red Cross Hospital
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- SUGIURA Kousaku
- Department of Internal Medicine, Shizuoka Red Cross Hospital
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- OTSUKA Syoichi
- Department of Clinical Laboratory, Shizuoka Red Cross Hospital
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- NONAKA Kiyoshi
- Department of Clinical Laboratory, Shizuoka Red Cross Hospital
Bibliographic Information
- Other Title
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- Fechtner症候群の2家系8症例の臨床的・電顕的検討
- Fechtner ショウコウグン ノ 2 カケイ 8 ショウレイ ノ リンショ
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Description
This is a report of Fechtner syndrome in two Japanese families. Six members of family I and three members of family II were studied. All but one had macrothrombocytopenia and leukocyte inclusion bodies, four had deafness, four had persistent proteinuria and none had cataracts. Under a diagnosis of ITP, two of them had splenectomy which resulted in no response. History reveolod, other family members with deafness and/or nephritis were confirmed in both families. Ultrastructural studies of leukocytes showed oval inclutson bodies with unclear borders containing many fine ribosome like granules and randomly scattered filaments. Ultrastructural studies of macrothrombocytes were unremarkable except for a well-developed opencanalicular system. More than half of megakaryocytes had uneven basophilic speckles in the cytoplasm, which were positive for Unna-Pappenheim staining. Ultrastructuraly, widening of demarkating systems and remaining ribosomes were noted in the cytoplasma of mature megakaryocytes.
Journal
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- Rinsho Ketsueki
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Rinsho Ketsueki 33 (2), 139-147, 1992
The Japanese Society of Hematology
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Details 詳細情報について
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- CRID
- 1390001205026458240
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- NII Article ID
- 130004919110
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- NII Book ID
- AN00252940
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- COI
- 1:STN:280:DyaK38zktFCktw%3D%3D
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- ISSN
- 18820824
- 04851439
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- NDL BIB ID
- 3763075
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- PubMed
- 1321917
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- Text Lang
- ja
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- Data Source
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- JaLC
- NDL
- PubMed
- CiNii Articles
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- Abstract License Flag
- Disallowed