T-Cell Prolymphocytic Leukemia Involving Extramedullary Sites

Jose R. Valbuena, MD; Marco Herling, MD; Joan H. Admirand, MD; Anthony Padula, MD; Dan Jones, MD, PhD; L. Jeffrey Medeiros, MD

Disclosures

Am J Clin Pathol. 2005;123(3):456-464. 

In This Article

Materials and Methods

During the last 6 years, 41 patients with T-PLL have been evaluated at The University of Texas M.D. Anderson Cancer Center, Houston. The criteria used to establish the diagnosis of T-PLL are those specified in the World Health Organization classification.[1] From this group, we identified 14 patients who underwent biopsy of 1 or more extramedullary sites.

At the time of initial diagnosis, all patients had involvement of peripheral blood and bone marrow by small lymphocytes with cytologic and immunophenotypic features consistent with T-PLL. The peripheral blood WBC count ranged from 17,000 to 350,000/µL (17.0-350.0 × 109/L). Bone marrow aspiration and biopsy showed involvement by T-PLL representing 20% to 80% of the total cellularity. All patients received single or multiagent chemotherapy, immunotherapy (anti-CD52 monoclonal antibody, CAMPATH-1H), or both.

Flow cytometry immunophenotypic analysis was performed on bone marrow aspirate material from all cases with an extensive panel of antibodies as previously described.[5] All neoplasms expressed T-cell antigens and were negative for TdT. Twelve neoplasms were CD4+/CD8–, 1 was CD4+/CD8+ (case 14), and 1 was CD4–/CD8– (case 10). Conventional cytogenetic studies were performed on 9 cases using methods previously described,[6] and the results are summarized in Table 1 .

All extramedullary biopsy specimens were obtained at the time of disease progression. The term disease progression, as used in the present study, includes development of disease relapse after complete clinical remission or uncontrolled peripheral blood lymphocytosis or growth of disease at an extramedullary site (eg, new-onset lesions or expansion of preexisting lesions) despite standard therapy.

Each biopsy specimen was fixed in formalin, processed routinely, and H&E-stained slides were prepared. Immunohisto-chemical studies were performed on all specimens using fixed, paraffin-embedded tissue sections and a variable panel of antibodies, including CD3, CD4, CD5, CD8, CD20, CD57, S-100, T-cell leukemia–1 (TCL-1), and TdT as described previously.[7,8] Flow cytometry immunophenotypic studies (see "Case Selection") were performed on cell suspensions of 4 specimens.

Each biopsy specimen was fixed in formalin, processed routinely, and H&E-stained slides were prepared. Immunohisto-chemical studies were performed on all specimens using fixed, paraffin-embedded tissue sections and a variable panel of antibodies, including CD3, CD4, CD5, CD8, CD20, CD57, S-100, T-cell leukemia–1 (TCL-1), and TdT as described previously.[7,8] Flow cytometry immunophenotypic studies (see "Case Selection") were performed on cell suspensions of 4 specimens.

In 4 biopsy specimens from 3 patients (cases 2, 5, and 10), molecular studies to assess for clonality of the T-cell receptor (TCR) g chain gene were performed using fixed, paraffin-embedded tissue samples from extramedullary biopsy sites. In case 2, the TCR g chain gene was assessed using denaturing gradient electrophoresis and staining with ethidium bromide as described by Theodorou et al.[9] In cases 5 and 10, the TCR g chain gene was assessed using fluorescently labeled consensus variable region primers, unlabeled joining region primers, and a polymerase chain reaction method followed by capillary electrophoresis and GeneScan (PE/Applied Biosystems, Foster City, CA) analysis as described previously.[10]

In 1 patient (case 5), conventional cytogenetics studies (see "Case Selection") were performed on a cell suspension of tissue obtained from a subcutaneous tissue mass on the right thigh.

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