| General |
Small to intermediate-size lymphoma cells preferentially infiltrate
the splenic red pulp cords and sinuses, hepatic sinusoids, and bone
marrow sinuses
Leukemic phase may develop as the disease progresses with circulating
tumor cells generally agranular
Cytoplasmic granules have been detected by electron microscopy in some
cases
Splenic infiltrates were primarily in the red pulp, with prominent
packing of the sinuses with frequent marked reduction or complete loss
of white pulp
Hepatic involvement was sinusoidal, with sparing of portal tracts in
most cases
Bone marrows had infiltrates were generally localized to the sinuses
and interstitium with occasional diffuse involvement and nodular aggregates
rarely
Lymphomatous infiltrates tended to partially involve the interstitium
and sinuses of lymph nodes
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| Hepatosplenic alpha-beta T-Cell Lymphomas A Report of
14 Cases and Comparison With Hepatosplenic gamma-delta T-Cell Lymphomas
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Am J Surg Pathol 2001;25:285-296
This study describes the clinicopathologic features of 14 hepatosplenic
alpha-beta T-cell lymphomas expressing alpha-beta TCR chains.
They occurred in 11 women and 3 men with a median age of 36 years.
Clinical presentation was similar to that described previously for hepatosplenic
gamma-delta T-cell lymphomas, except for the female preponderance and
age distribution (5 patients younger than 13 years of age and 5 patients
older than 50 years of age). Disease distribution was primarily in the
splenic red pulp and hepatic sinusoids, although liver infiltrates were
largely periportal in four cases. Bone marrow involvement, observed
in eight patients, was usually interstitial and/or within the sinuses.
Lymph nodes were involved in five patients, although lymphadenopathy
was demonstrable in only two.
Ten cases were composed of intermediate-size tumor cells with round/oval
nuclei, slightly dispersed chromatin, inconspicuous nucleoli, and scant
to moderate amounts of cytoplasm. Four lymphomas contained primarily
large cells with irregular nuclei, dispersed chromatin, discernible
nucleoli, and moderate to abundant cytoplasm. Tumor cells in all 14
lymphomas were cytotoxic T-cells; 13 co-expressed natural killer cell–associated
antigens and showed T-cell clonality. Three lymphomas were associated
with Epstein-Barr virus. Two of four cases had an isochromosome 7q.
Eleven patients are dead, eight within a year of diagnosis, and two
patients have maintained complete remissions after combination chemotherapy.
Conclusion:
These data show that hepatosplenic T-cell lymphomas include an alpha-beta-subtype.
This group, along with the previously recognized group, should be recognized
as phenotypically heterogeneous subtypes of the same disease
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|
Hepatosplenic gamma/delta T-Cell Lymphoma in Bone Marrow A Sinusoidal
Neoplasm With Blastic Cytologic Features
Francisco Vega, MD, PhD
L. Jeffrey Medeiros, MD
Carlos Bueso-Ramos, MD, PhD
Dan Jones, MD, PhD
Raymond Lai, MD, PhD
Rajyalakshmi Luthra, PhD
Lynne V. Abruzzo, MD, PhD
|
Am J Clin Pathol 2001;116:410-419 Abstract quote
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; 1 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.
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|
Hepatosplenic gammadelta-T-cell lymphoma with leukemic course after
renal transplantation.
Steurer M, Stauder R, Grunewald K, Gunsilius E, Duba HC, Gastl G,
Dirnhofer S.
Division of Haematology and Oncology, University Hospital Innsbruck,
Innsbruck; the Institute for Medical Biology and Human Genetics and
the Department of Pathology, University of Innsbruck, Innsbruck, Austria;
and the Institute of Pathology, University of Basel, Basel, Switzerland.
|
Hum Pathol 2002 Feb;33(2):253-258 Abstract quote
Hepatosplenic gammadelta-T-cell lymphoma (HSTCL) is a rare extranodal
T-cell non-Hodgkin's lymphoma (T-NHL) with only 46 well-documented cases
in medical literature. Notably, a relatively high number of these case
reports (15%) describe the occurrence of HSTCL after solid organ transplantation.
We describe the case of a 45-year-old man who developed a leukemic
HSTCL 5 years after renal transplantation and continous immunosuppression
with cyclosporine A and prednisolone. After a rapid clinical course,
the patient died and autopsy was performed. The malignant lymphocytes
showed a natural killer-like gammadelta-T-cell phenotype (CD2(+), CD3(+),
CD7(+), TCR gammadelta(+), CD56(+), TIA-1(+), CD4(-), CD8(-), and TCR
alphabeta(-)) and infiltrated the sinusoids of liver and the red pulp
of the spleen. Cytogenetically, an isochromosome 7q, trisomy 8, Y-loss,
and a translocation t(1;4) was detectable.
This case shows the difficulties of recognizing HSTCL early in the
clinical course and underlines that all types of T-NHL, nodal as well
as extranodal, have to be considered in the differential diagnosis of
posttransplantation lymphoproliferative disorders. Moreover, HSTCL seems
to occur as a specific late complication of solid organ transplantation.
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