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Kikuchi's disease: a case report with emphasis on flow cytometric studies
M Lima1, F Silvestre, J Correia
1Service of Clinical Hematology, Sto Antonio's Hospital, Porto Portugal.
Summary
This case study details Kikuchi-Fujimoto disease in a 27-year-old female, highlighting its diverse symptoms and diagnostic methods. The findings emphasize the role of activated CD8 T lymphocytes in the disease pathology.
Area of Science:
- Immunology
- Pathology
- Hematology
Background:
- Kikuchi-Fujimoto disease (KFD) is a rare, benign, self-limiting disorder of unknown etiology.
- It typically presents with cervical lymphadenopathy, fever, and constitutional symptoms.
Observation:
- A 27-year-old female presented with a complex clinical picture including fever, urinary tract infections, rash, polyarthritis, lymphadenopathy, pancytopenia, liver dysfunction, and coagulopathy.
- Histopathologic examination of lymph nodes revealed histiocytic necrotizing lymphadenitis, characteristic of KFD.
Findings:
- Immunophenotypic analysis via flow cytometry demonstrated a significant expansion of activated CD8 T lymphocytes in affected lymph nodes, bone marrow, and peripheral blood.
- Elevated serum lactate dehydrogenase was noted, correlating with disease activity.
Implications:
- This case underscores the importance of integrating histopathologic and immunophenotypic findings for accurate KFD diagnosis.
- The observed T-cell expansion provides insights into the potential immunopathogenesis of Kikuchi-Fujimoto disease.
- Early recognition and diagnosis are crucial for appropriate management and to rule out other lymphoproliferative disorders.