Hepatosplenic gamma/delta T-cell lymphoma in bone marrow

A sinusoidal neoplasm with blastic cytologic features

Francisco Vega, L. J. Medeiros, C. Bueso-Ramos, D. Jones, R. Lai, R. Luthra, L. V. Abruzzo

Research output: Contribution to journalArticle

81 Citations (Scopus)

Abstract

We report 8 cases of hepatosplenic T-cell lymphoma (HSTCL) involving bone marrow and correlate histologic findings with disease progression. Immunophenotypic analysis demonstrated mature, aberrant gamma/delta T-cell immunophenotypes. Isochromosome 7q was identified in 4 cases; 1 case showed the t(7;14)(q34;q13). Seven of 7 cases tested had monoclonal TCR gamma gene rearrangements. The initial diagnostic bone marrow biopsy specimens were hypercellular with a frequently subtle, predominantly sinusoidal infiltrate of atypical small to medium-sized lymphoid cells. In all cases, aspirate smears at diagnosis and in subsequent specimens contained malignant cells that resembled blasts, some with fine cytoplasmic granules. With progression, the pattern of HSTCL in bone marrow biopsy specimens became increasingly interstitial, and the neoplastic cells became larger. In aspirate smears, the proportion of blasts increased. Seven patients died; I was lost to follow-up. Autopsy performed on 1 patient demonstrated malignant cells within vascular channels in all organs sampled, with relatively little tumor formation, resembling intravascular lymphoma at these sites. HSTCL often can be recognized in bone marrow by its unique combination of a sinusoidal pattern in core biopsy specimens and blastic cytology in aspirate smears.

Original languageEnglish
Pages (from-to)410-419
Number of pages10
JournalAmerican Journal of Clinical Pathology
Volume116
Issue number3
DOIs
StatePublished - Sep 27 2001
Externally publishedYes

Fingerprint

Somatostatin-Secreting Cells
T-Cell Lymphoma
Bone Marrow
Biopsy
Neoplasms
Isochromosomes
Cytoplasmic Granules
Gene Rearrangement
Lost to Follow-Up
Blood Vessels
Cell Biology
Disease Progression
Autopsy
Lymphoma
Lymphocytes
T-Lymphocytes

Keywords

  • Bone marrow
  • Gamma/delta T-cell receptor
  • Hepatosplenic T-cell lymphoma
  • Immunophenotype

ASJC Scopus subject areas

  • Pathology and Forensic Medicine

Cite this

Hepatosplenic gamma/delta T-cell lymphoma in bone marrow : A sinusoidal neoplasm with blastic cytologic features. / Vega, Francisco; Medeiros, L. J.; Bueso-Ramos, C.; Jones, D.; Lai, R.; Luthra, R.; Abruzzo, L. V.

In: American Journal of Clinical Pathology, Vol. 116, No. 3, 27.09.2001, p. 410-419.

Research output: Contribution to journalArticle

Vega, Francisco ; Medeiros, L. J. ; Bueso-Ramos, C. ; Jones, D. ; Lai, R. ; Luthra, R. ; Abruzzo, L. V. / Hepatosplenic gamma/delta T-cell lymphoma in bone marrow : A sinusoidal neoplasm with blastic cytologic features. In: American Journal of Clinical Pathology. 2001 ; Vol. 116, No. 3. pp. 410-419.
@article{b68ee9f391494e138b196089bec51435,
title = "Hepatosplenic gamma/delta T-cell lymphoma in bone marrow: A sinusoidal neoplasm with blastic cytologic features",
abstract = "We report 8 cases of hepatosplenic T-cell lymphoma (HSTCL) involving bone marrow and correlate histologic findings with disease progression. Immunophenotypic analysis demonstrated mature, aberrant gamma/delta T-cell immunophenotypes. Isochromosome 7q was identified in 4 cases; 1 case showed the t(7;14)(q34;q13). Seven of 7 cases tested had monoclonal TCR gamma gene rearrangements. The initial diagnostic bone marrow biopsy specimens were hypercellular with a frequently subtle, predominantly sinusoidal infiltrate of atypical small to medium-sized lymphoid cells. In all cases, aspirate smears at diagnosis and in subsequent specimens contained malignant cells that resembled blasts, some with fine cytoplasmic granules. With progression, the pattern of HSTCL in bone marrow biopsy specimens became increasingly interstitial, and the neoplastic cells became larger. In aspirate smears, the proportion of blasts increased. Seven patients died; I was lost to follow-up. Autopsy performed on 1 patient demonstrated malignant cells within vascular channels in all organs sampled, with relatively little tumor formation, resembling intravascular lymphoma at these sites. HSTCL often can be recognized in bone marrow by its unique combination of a sinusoidal pattern in core biopsy specimens and blastic cytology in aspirate smears.",
keywords = "Bone marrow, Gamma/delta T-cell receptor, Hepatosplenic T-cell lymphoma, Immunophenotype",
author = "Francisco Vega and Medeiros, {L. J.} and C. Bueso-Ramos and D. Jones and R. Lai and R. Luthra and Abruzzo, {L. V.}",
year = "2001",
month = "9",
day = "27",
doi = "10.1309/BM40-YM6J-9T3X-MH8H",
language = "English",
volume = "116",
pages = "410--419",
journal = "American Journal of Clinical Pathology",
issn = "0002-9173",
publisher = "American Society of Clinical Pathologists",
number = "3",

}

TY - JOUR

T1 - Hepatosplenic gamma/delta T-cell lymphoma in bone marrow

T2 - A sinusoidal neoplasm with blastic cytologic features

AU - Vega, Francisco

AU - Medeiros, L. J.

AU - Bueso-Ramos, C.

AU - Jones, D.

AU - Lai, R.

AU - Luthra, R.

AU - Abruzzo, L. V.

PY - 2001/9/27

Y1 - 2001/9/27

N2 - We report 8 cases of hepatosplenic T-cell lymphoma (HSTCL) involving bone marrow and correlate histologic findings with disease progression. Immunophenotypic analysis demonstrated mature, aberrant gamma/delta T-cell immunophenotypes. Isochromosome 7q was identified in 4 cases; 1 case showed the t(7;14)(q34;q13). Seven of 7 cases tested had monoclonal TCR gamma gene rearrangements. The initial diagnostic bone marrow biopsy specimens were hypercellular with a frequently subtle, predominantly sinusoidal infiltrate of atypical small to medium-sized lymphoid cells. In all cases, aspirate smears at diagnosis and in subsequent specimens contained malignant cells that resembled blasts, some with fine cytoplasmic granules. With progression, the pattern of HSTCL in bone marrow biopsy specimens became increasingly interstitial, and the neoplastic cells became larger. In aspirate smears, the proportion of blasts increased. Seven patients died; I was lost to follow-up. Autopsy performed on 1 patient demonstrated malignant cells within vascular channels in all organs sampled, with relatively little tumor formation, resembling intravascular lymphoma at these sites. HSTCL often can be recognized in bone marrow by its unique combination of a sinusoidal pattern in core biopsy specimens and blastic cytology in aspirate smears.

AB - We report 8 cases of hepatosplenic T-cell lymphoma (HSTCL) involving bone marrow and correlate histologic findings with disease progression. Immunophenotypic analysis demonstrated mature, aberrant gamma/delta T-cell immunophenotypes. Isochromosome 7q was identified in 4 cases; 1 case showed the t(7;14)(q34;q13). Seven of 7 cases tested had monoclonal TCR gamma gene rearrangements. The initial diagnostic bone marrow biopsy specimens were hypercellular with a frequently subtle, predominantly sinusoidal infiltrate of atypical small to medium-sized lymphoid cells. In all cases, aspirate smears at diagnosis and in subsequent specimens contained malignant cells that resembled blasts, some with fine cytoplasmic granules. With progression, the pattern of HSTCL in bone marrow biopsy specimens became increasingly interstitial, and the neoplastic cells became larger. In aspirate smears, the proportion of blasts increased. Seven patients died; I was lost to follow-up. Autopsy performed on 1 patient demonstrated malignant cells within vascular channels in all organs sampled, with relatively little tumor formation, resembling intravascular lymphoma at these sites. HSTCL often can be recognized in bone marrow by its unique combination of a sinusoidal pattern in core biopsy specimens and blastic cytology in aspirate smears.

KW - Bone marrow

KW - Gamma/delta T-cell receptor

KW - Hepatosplenic T-cell lymphoma

KW - Immunophenotype

UR - http://www.scopus.com/inward/record.url?scp=0034843939&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0034843939&partnerID=8YFLogxK

U2 - 10.1309/BM40-YM6J-9T3X-MH8H

DO - 10.1309/BM40-YM6J-9T3X-MH8H

M3 - Article

VL - 116

SP - 410

EP - 419

JO - American Journal of Clinical Pathology

JF - American Journal of Clinical Pathology

SN - 0002-9173

IS - 3

ER -