True T-cell chronic lymphocytic leukemia: a morphologic and immunophenotypic study of 25 cases [see comments]

We studied 25 T-cell chronic lymphocytic leukemia (T-CLL) cases collected over a 15-year period. Immunophenotypic analysis was performed in each case; 12 cases were evaluated by cytogenetics, and gene rearrangement studies were performed in 14 cases. The median age was 57 years with a male predominance (M:F, 15:10). The median presenting lymphocyte count was 36.3 x 10(9)/L (range, 3.9 to 438 x 10(9)/L). Fourteen patients (56%) had shotty adenopathy and ten (40%) had mild-to-moderate splenomegaly at presentation; four (16%) had erythematous skin lesions. The lymphocytes were predominantly small; some cases had a minor component of medium-sized cells (< 10%). The nuclear: cytoplasmic ratios were uniformly high with round to oval nuclei; however, a wide spectrum of nuclear outlines could be found, ranging from minimally to markedly convoluted. Nucleoli were either absent or small and inconspicuous. These lymphocytes did not have the morphology of prolymphocytes and did not contain cytoplasmic granules. Bone marrow infiltration was generally in an interstitial pattern; the degree of involvement ranged from 15% to 90%. Immunophenotyping showed that the lymphocytes were mature T-cells with a predominant CD4+ immunophenotype. Three cases displayed a CD8+ immunophenotype. The patients were treated with a variety of chemotherapeutic regimens with only a minimal response observed in two of 20 patients. We conclude that T-CLL is an uncommon chronic lymphoproliferative disorder (CLPD) that can be morphologically similar to B-CLL, is distinct from T- prolymphocytic leukemia, and has an aggressive clinical course that is refractory to therapy. It may also be difficult to distinguish T-CLL from other T-CLPD, especially the leukemic phase of peripheral T-cell lymphoma and some cases of Sezary syndrome.

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True T-cell chronic lymphocytic leukemia: a morphologic and immunophenotypic study of 25 cases [see comments]

Blood ◽

10.1182/blood.v86.3.1163.1163 ◽

1995 ◽

Vol 86 (3) ◽

pp. 1163-1169 ◽

Cited By ~ 74

Author(s):

JD Hoyer ◽

CW Ross ◽

CY Li ◽

TE Witzig ◽

RD Gascoyne ◽

...

Keyword(s):

T Cell ◽

Chronic Lymphocytic Leukemia ◽

Wide Spectrum ◽

Minor Component ◽

Skin Lesions ◽

Lymphocytic Leukemia ◽

Prolymphocytic Leukemia ◽

Male Predominance ◽

A Minor ◽

Cell Chronic Lymphocytic Leukemia

Abstract We studied 25 T-cell chronic lymphocytic leukemia (T-CLL) cases collected over a 15-year period. Immunophenotypic analysis was performed in each case; 12 cases were evaluated by cytogenetics, and gene rearrangement studies were performed in 14 cases. The median age was 57 years with a male predominance (M:F, 15:10). The median presenting lymphocyte count was 36.3 x 10(9)/L (range, 3.9 to 438 x 10(9)/L). Fourteen patients (56%) had shotty adenopathy and ten (40%) had mild-to-moderate splenomegaly at presentation; four (16%) had erythematous skin lesions. The lymphocytes were predominantly small; some cases had a minor component of medium-sized cells (< 10%). The nuclear: cytoplasmic ratios were uniformly high with round to oval nuclei; however, a wide spectrum of nuclear outlines could be found, ranging from minimally to markedly convoluted. Nucleoli were either absent or small and inconspicuous. These lymphocytes did not have the morphology of prolymphocytes and did not contain cytoplasmic granules. Bone marrow infiltration was generally in an interstitial pattern; the degree of involvement ranged from 15% to 90%. Immunophenotyping showed that the lymphocytes were mature T-cells with a predominant CD4+ immunophenotype. Three cases displayed a CD8+ immunophenotype. The patients were treated with a variety of chemotherapeutic regimens with only a minimal response observed in two of 20 patients. We conclude that T-CLL is an uncommon chronic lymphoproliferative disorder (CLPD) that can be morphologically similar to B-CLL, is distinct from T- prolymphocytic leukemia, and has an aggressive clinical course that is refractory to therapy. It may also be difficult to distinguish T-CLL from other T-CLPD, especially the leukemic phase of peripheral T-cell lymphoma and some cases of Sezary syndrome.

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Rearrangement and expression of T cell antigen receptor genes in B cell chronic lymphocytic leukemia

Blood ◽

10.1182/blood.v71.1.178.bloodjournal711178 ◽

1988 ◽

Vol 71 (1) ◽

pp. 178-185

Author(s):

JD Norton ◽

J Pattinson ◽

AV Hoffbrand ◽

H Jani ◽

JC Yaxley ◽

...

Keyword(s):

T Cell ◽

Chronic Lymphocytic Leukemia ◽

B Cell ◽

Germ Line ◽

Lymphocytic Leukemia ◽

Gene Transcript ◽

Prolymphocytic Leukemia ◽

Beta 2 ◽

Beta Gene ◽

Cell Chronic Lymphocytic Leukemia

Fifty-nine patients with B cell chronic lymphocytic leukemia (B-CLL) were screened for clonal rearrangement of T cell receptor (TCR) beta and gamma chain genes. Four were found with rearranged TCR beta genes, but none had detectable rearrangement of TCR gamma genes. One typical patient with B-CLL had a TCR beta gene structure consistent with a variable-diversity-joining rearrangement into the C beta 2 gene on one allele. An apparently identical rearrangement pattern was seen in a second patient, which suggested that there may be a restriction on the repertoire of possible TCR beta gene recombinations in mature B cells. Two further patients had a simple deletion of sequences, consistent with a diversity-joining rearrangement into C beta 2 on one allele. All four patients had rearrangements of immunoglobulin heavy- and light- chain genes typical of mature B cell malignancies. However, on review of clinical, morphological, and immunophenotype data, two had features consistent with B cell prolymphocytic leukemia or B lymphoma, and a third had progressed to a prolymphocytic transformation. Low-level expression of a predominantly 1.0- to 1.2-kilobase germ line TCR beta gene transcript was detected in several B-CLLs and at a comparable level in the four with rearranged TCR beta genes. This, together with the low frequency of TCR gene rearrangement, suggests that most B-CLL cases arise at a developmental stage when factors required for TCR gene activity are not operative.

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Similarities between T-cell chronic lymphocytic leukemia and the small- cell variant of T-prolymphocytic leukemia [letter; comment]

Blood ◽

10.1182/blood.v87.8.3520.bloodjournal8783520 ◽

1996 ◽

Vol 87 (8) ◽

pp. 3520-3521 ◽

Cited By ~ 21

Author(s):

E Matutes ◽

D Catovsky

Keyword(s):

T Cell ◽

Chronic Lymphocytic Leukemia ◽

Small Cell ◽

Lymphocytic Leukemia ◽

Prolymphocytic Leukemia ◽

Small Cell Variant ◽

Letter Comment ◽

Cell Variant ◽

Cell Chronic Lymphocytic Leukemia

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Unusually indolent T-cell prolymphocytic leukemia associated with a complex karyotype: Is this T-cell chronic lymphocytic leukemia?

American Journal of Hematology ◽

10.1002/ajh.10221 ◽

2002 ◽

Vol 71 (3) ◽

pp. 224-226 ◽

Cited By ~ 12

Author(s):

Lorinda Soma ◽

Dennis B. Cornfield ◽

David Prager ◽

Peter Nowell ◽

Adam Bagg

Keyword(s):

T Cell ◽

Chronic Lymphocytic Leukemia ◽

Lymphocytic Leukemia ◽

Complex Karyotype ◽

Prolymphocytic Leukemia ◽

Cell Chronic Lymphocytic Leukemia

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Rearrangement and expression of T cell antigen receptor genes in B cell chronic lymphocytic leukemia

Blood ◽

10.1182/blood.v71.1.178.178 ◽

1988 ◽

Vol 71 (1) ◽

pp. 178-185 ◽

Cited By ~ 33

Author(s):

JD Norton ◽

J Pattinson ◽

AV Hoffbrand ◽

H Jani ◽

JC Yaxley ◽

...

Keyword(s):

T Cell ◽

Chronic Lymphocytic Leukemia ◽

B Cell ◽

Germ Line ◽

Lymphocytic Leukemia ◽

Gene Transcript ◽

Prolymphocytic Leukemia ◽

Beta 2 ◽

Beta Gene ◽

Cell Chronic Lymphocytic Leukemia

Abstract Fifty-nine patients with B cell chronic lymphocytic leukemia (B-CLL) were screened for clonal rearrangement of T cell receptor (TCR) beta and gamma chain genes. Four were found with rearranged TCR beta genes, but none had detectable rearrangement of TCR gamma genes. One typical patient with B-CLL had a TCR beta gene structure consistent with a variable-diversity-joining rearrangement into the C beta 2 gene on one allele. An apparently identical rearrangement pattern was seen in a second patient, which suggested that there may be a restriction on the repertoire of possible TCR beta gene recombinations in mature B cells. Two further patients had a simple deletion of sequences, consistent with a diversity-joining rearrangement into C beta 2 on one allele. All four patients had rearrangements of immunoglobulin heavy- and light- chain genes typical of mature B cell malignancies. However, on review of clinical, morphological, and immunophenotype data, two had features consistent with B cell prolymphocytic leukemia or B lymphoma, and a third had progressed to a prolymphocytic transformation. Low-level expression of a predominantly 1.0- to 1.2-kilobase germ line TCR beta gene transcript was detected in several B-CLLs and at a comparable level in the four with rearranged TCR beta genes. This, together with the low frequency of TCR gene rearrangement, suggests that most B-CLL cases arise at a developmental stage when factors required for TCR gene activity are not operative.

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Composite Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma and T-Prolymphocytic Leukemia Presenting with Lymphocytosis, Skin Lesions, and Generalized Lymphadenopathy

Case Reports in Pathology ◽

10.1155/2019/4915086 ◽

2019 ◽

Vol 2019 ◽

pp. 1-10

Author(s):

Ali Sakhdari ◽

Guilin Tang ◽

Lawrence E. Ginsberg ◽

Cheryl F. Hirsch-Ginsberg ◽

Carlos E. Bueso-Ramos ◽

...

Keyword(s):

T Cell ◽

Chronic Lymphocytic Leukemia ◽

Clinical Course ◽

Skin Lesions ◽

Lymphocytic Leukemia ◽

Rare Type ◽

Prolymphocytic Leukemia ◽

High Relapse Rate ◽

Dismal Prognosis ◽

Generalized Lymphadenopathy

Chronic lymphocytic leukemia (CLL) is the most common type of leukemia in Western countries with an incidence of 3-5 cases per 100,000 persons. Most patients follow an indolent clinical course with eventual progression and need for therapy. In contrast, T-prolymphocytic leukemia (T-PLL) is a rare type of T-cell leukemia with most patients having an aggressive clinical course and a dismal prognosis. Therapies are limited for T-PLL patients and there is a high relapse rate. Morphologically, the cells of CLL and T-PLL can show overlapping features. Here, we report the case of a 61-year-old man who presented with a clinically indolent CLL and T-PLL, initially diagnosed solely and followed as CLL, despite the presence of an associated but unrecognized aberrant T-cell population in blood. After 2 years, the T-PLL component became more apparent with cutaneous and hematologic manifestations and the diagnosis was confirmed by immunophenotypic and cytogenetic analysis. Fluorescencein situhybridization demonstrated anATMdeletion in both CLL and T-PLL components. Retrospective testing demonstrated that composite CLL and T-PLL were both present in skin and lymph nodes as well as in blood and bone marrow since initial presentation. This case is also unique because it highlights that a subset of T-PLL patients can present with clinically indolent disease. The concomitant detection ofATMmutation in CLL and T-PLL components raises the possibility of a common pathogenic mechanism.

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