Hepatosplenic γδ T-cell lymphoma with ring chromosome 7, an isochromosome 7q equivalent clonal chromosomal aberration

Julia Tamaska, Emma Adam, Andras Kozma, Laszlo Gopcsa, H. Andrikovics, A. Tordai, Gabriella Halm, Laszlo Bereczki, E. Bagdi, L. Krenács

Research output: Contribution to journalArticle

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Abstract

Hepatosplenic T-cell lymphoma is a rare, clinically aggressive lymphoma. Most cases represent a neoplasm of mature non-activated γδ T cells. Isochromosome 7q i(7)(q10) is thought to be the primary cytogenetic abnormality of this disease. In this paper, we describe a hepatosplenic γδ T-cell lymphoma case, with clonal ring chromosome 7 exemplifying an isochromosome 7q equivalent clonal aberration. A 62-year-old female patient presented with thrombocytopenia, isolated hepatosplenomegaly, and extremely high levels of LDH. Bone marrow work-up demonstrated a sinusoidal cytotoxic T-cell infiltrate with blastic features, while molecular studies verified monoclonal rearrangement for both TCR γ and TCR δ genes. Cytogenetics revealed clonal abnormalities including ring chromosome 7, trisomy 8, and der(19), while FISH analysis detected 7q amplification with partial deletion of 7p in ring chromosome 7. To the best of our knowledge, this is the first reported T-cell lymphoma case with ring chromosome 7.

Original languageEnglish
Pages (from-to)479-483
Number of pages5
JournalVirchows Archiv
Volume449
Issue number4
DOIs
Publication statusPublished - Oct 2006

Fingerprint

Isochromosomes
T-Cell Lymphoma
Chromosome Aberrations
T-Lymphocytes
Cytogenetics
Thrombocytopenia
Lymphoma
Bone Marrow
Chromosome 7 ring syndrome
Genes
Neoplasms

Keywords

  • Cytogenetics
  • FISH
  • Hepatosplenic γδ T-cell lymphoma
  • Ring chromosome 7

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Hepatosplenic γδ T-cell lymphoma with ring chromosome 7, an isochromosome 7q equivalent clonal chromosomal aberration. / Tamaska, Julia; Adam, Emma; Kozma, Andras; Gopcsa, Laszlo; Andrikovics, H.; Tordai, A.; Halm, Gabriella; Bereczki, Laszlo; Bagdi, E.; Krenács, L.

In: Virchows Archiv, Vol. 449, No. 4, 10.2006, p. 479-483.

Research output: Contribution to journalArticle

Tamaska, Julia ; Adam, Emma ; Kozma, Andras ; Gopcsa, Laszlo ; Andrikovics, H. ; Tordai, A. ; Halm, Gabriella ; Bereczki, Laszlo ; Bagdi, E. ; Krenács, L. / Hepatosplenic γδ T-cell lymphoma with ring chromosome 7, an isochromosome 7q equivalent clonal chromosomal aberration. In: Virchows Archiv. 2006 ; Vol. 449, No. 4. pp. 479-483.
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AB - Hepatosplenic T-cell lymphoma is a rare, clinically aggressive lymphoma. Most cases represent a neoplasm of mature non-activated γδ T cells. Isochromosome 7q i(7)(q10) is thought to be the primary cytogenetic abnormality of this disease. In this paper, we describe a hepatosplenic γδ T-cell lymphoma case, with clonal ring chromosome 7 exemplifying an isochromosome 7q equivalent clonal aberration. A 62-year-old female patient presented with thrombocytopenia, isolated hepatosplenomegaly, and extremely high levels of LDH. Bone marrow work-up demonstrated a sinusoidal cytotoxic T-cell infiltrate with blastic features, while molecular studies verified monoclonal rearrangement for both TCR γ and TCR δ genes. Cytogenetics revealed clonal abnormalities including ring chromosome 7, trisomy 8, and der(19), while FISH analysis detected 7q amplification with partial deletion of 7p in ring chromosome 7. To the best of our knowledge, this is the first reported T-cell lymphoma case with ring chromosome 7.

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