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Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation
Published on: November 26, 2018
[IBL like T cell lymphoma associated with hemolytic anemia and polyploidy chromosomal abnormality--a case report]
Y Sasagawa1, R Koyama, T Nagai
1Department of Internal Medicine, Hokkaido Preferectual Sapporo Kitano Hospital.
Insights
This study reports a rare case of an Epstein-Barr virus-associated lymphoproliferative disorder (IBL) like T cell lymphoma in a 56-year-old man. The lymphoma exhibited unique polyploidy chromosomal abnormalities and was associated with hemolytic anemia.
Area of Science:
- Hematology
- Oncology
- Immunology
Background:
- Epstein-Barr virus-associated lymphoproliferative disorders (IBL) are rare, particularly T cell lymphomas.
- T cell lymphomas can present with diverse clinical and pathological features.
Observation:
- A 56-year-old male presented with fever, ascites, pleural effusion, lymphadenopathy, and hepatosplenomegaly.
- Laboratory results revealed Coombs' positive hemolytic anemia, leukocytosis, polyclonal hypergammaglobulinemia, and positive serological markers for autoimmune conditions.
- Histological examination of a lymph node biopsy showed features of IBL-like T cell lymphoma with specific surface markers (CD2+, CD8+, CD4-).
Findings:
- Chromosomal analysis identified polyploidy in all chromosomes except chromosome 14, with XXY sex chromosomes and specific markers (mar1, mar2, mar3).
- Gene rearrangement for the beta-chain of the T cell receptor was confirmed in the lymphoma cells.
- The patient was treated with chemotherapy but succumbed to respiratory failure.
Implications:
- This case highlights a rare presentation of T cell lymphoma with significant chromosomal abnormalities.
- The co-occurrence of IBL-like morphology, T cell lymphoma, polyploidy, and hemolytic anemia presents a unique diagnostic and therapeutic challenge.
- Further research into the pathogenesis and optimal treatment strategies for such rare hematologic malignancies is warranted.
Abstract:
A 56-year-old man was admitted to Sapporo Kitano Hospital on May 30, 1987 because of fever, retention of ascites and pleural effusion, generalized lymphnode swelling and hepatosplenomegaly. Laboratory findings showed Coombs' positive hemolytic anemia, leukocytosis and polyclonal hypergammaglobulinemia. Serological test included positive RA factor, anti-DNA 16 U/ml, thyroglobulin Ab 1600 x and microsome Ab 3200 x. A cervical lymphnode specimen exhibited typical histologic picture of IBL like T cell lymphoma. The surface markers of lymphoma cells were CD2(+), CD8(+) and CD4(-). Chromosomal analysis of these cells revealed polyploidy abnormality for all chromosomes except for No. 14's which are disomy. All lymphoma cells have XXY sex chromosome and mar1, mar2, mar3. Gene rearrangement for beta-chain of T cell receptor was proved in these lymphoma cells. He was treated with prednisolone, vincristine, cyclophosphamide, adriamycin etc, but died of respiratory failure 171 days after admission. We reported a rare case of IBL like T cell lymphoma with polyploidy chromosomal abnormality accompanied with hemolytic anemia.
