Hemophagocyctic lymphohistiocytosis developed in a Japanese boy with Chédiak-Higashi syndrome
| Autor: | Shumpei Yokota, Sakurako Izaki, Hisashi Yamanaka, Koichiro Ikuta, Takako Miyamae |
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| Rok vydání: | 2013 |
| Předmět: |
Male
Pathology medicine.medical_specialty Exacerbation Immunology Hepatosplenomegaly Lymphohistiocytosis Hemophagocytic hemic and lymphatic diseases Humans Immunology and Allergy Medicine Hemophagocytic lymphohistiocytosis biology business.industry Chédiak–Higashi syndrome Infant General Medicine medicine.disease Oculocutaneous albinism Ferritin Albinism biology.protein Primary immunodeficiency medicine.symptom Chediak-Higashi Syndrome business |
| Zdroj: | Japanese Journal of Clinical Immunology. 36:226-232 |
| ISSN: | 1349-7413 0911-4300 |
| Popis: | Chédiak-Higashi syndrome (CHS) is one of the primary immunodeficiency syndromes accompanied by oculocutaneous albinism. It is characterized by existence of giant granule of neutrophils, and development of symptoms of hemophagocytic lymphohistiocytosis. CHS is a rare disorder and recognition of the disease is indispensable for its diagnosis. In our case, a four-month-old boy, virus-associated hemophagocytic syndrome (VAHS) was suspected from generation of fever, hepatosplenomegaly, and existence of atypical lymphocytes on admission. However, elevation of serum AST, LDH and ferritin were quite slight as VAHS, and rapid exacerbation of the findings was not seen. Associated virus was undetected. He was finally diagnosed as CHS developing hemophagocyctic lymphohistiocytosis based on the existence of a giant granule of neutrophils in the peripheral blood smear and oculocutaneous albisum and laboratory findings. Clinical outcome was successful after receiving HLA-matched unrelated bone marrow transplantation. |
| Databáze: | OpenAIRE |
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