scholarly journals T-Cell Prolymphocytic Leukemia: A Rare Disease With an Aggressive Clinical Course

2018 ◽  
Vol 150 (suppl_1) ◽  
pp. S105-S106
Author(s):  
Lin Zhang ◽  
Daphne Massey ◽  
Vanya Jaitly ◽  
Xiaohong Wang
1987 ◽  
Vol 5 (2) ◽  
pp. 208-215 ◽  
Author(s):  
E Dmitrovsky ◽  
M J Matthews ◽  
P A Bunn ◽  
G P Schechter ◽  
R W Makuch ◽  
...  

The clinical course of cutaneous T cell lymphoma (mycosis fungoides and Sezary syndrome) is generally indolent, but in occasional patients becomes fulminant. We found that biopsies from patients with accelerating disease can reveal cytologic transformation from previously observed small, convoluted lymphocytes to large cells that are similar to cells seen in large-cell lymphoma. The cerebriform nuclei characteristic of malignant T cells can only rarely be identified. Of 150 cutaneous T cell lymphoma patients we treated from 1976 to 1984, cytologic transformation was identified in 12 after review of peripheral blood smears and biopsies from skin, lymph nodes, and visceral sites. Patients who developed cytologic transformation were initially characterized by advanced stage (11 of 12), with lymph node effacement (seven of 11) and erythroderma (five of 12). The tumor cell DNA content after transformation was aneuploid (four of four), and the ability to form rosettes with sheep erythrocytes was retained in transformed cells (three of three). The median time from diagnosis of cutaneous T cell lymphoma to cytologic transformation was 21.5 months (range, 4 to 64), and the median survival from transformation was only 2 months (range, 0 to 19+). We conclude that cytologic transformation in cutaneous T cell lymphoma represents a distinct clinicopathologic entity, characterized by an aggressive clinical course.


Author(s):  
DAPHINE CAXIAS TRAVASSOS ◽  
DARCY FERNANDES ◽  
ELAINE MARIA SGAVIOLI MASSUCATO ◽  
CLAUDIA MARIA NAVARRO ◽  
MIRIAN APARECIDA ONOFRE ◽  
...  

2019 ◽  
Vol 2019 ◽  
pp. 1-10
Author(s):  
Ali Sakhdari ◽  
Guilin Tang ◽  
Lawrence E. Ginsberg ◽  
Cheryl F. Hirsch-Ginsberg ◽  
Carlos E. Bueso-Ramos ◽  
...  

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.


2006 ◽  
Vol 130 (12) ◽  
pp. 1859-1864
Author(s):  
Cherie H. Dunphy ◽  
Daphne E. DeMello ◽  
Gordon B. Gale

Abstract Context.—Anaplastic large cell lymphomas (ALCLs) are a heterogeneous group of CD30+ large cell lymphomas that, according to the World Health Organization classification, are defined as being of T-cell origin, based on immunophenotype, and/or the finding of a T-cell gene rearrangement by molecular studies. Most cases express cytotoxic granule-associated proteins. Relatively recent data have suggested that some T-cell ALCLs are derived from cytolytic CD4+ cells, γδ T cells, or natural killer–like (CD56+ or CD57+) T cells. We encountered a pediatric case of CD56+, anaplastic lymphoma kinase-positive ALCL of apparent natural killer–like T-cell origin (showing positivity for CD2, cytoplasmic CD3, surface CD3 partial positivity, CD7, CD8, CD56, TIA-1, and granzyme B). The patient had initial lymph node and multiple sites of cutaneous involvement and an aggressive clinical course with multiple recurrences after varying periods of complete remission. Objective.—To review the current pediatric literature regarding the incidence, differential diagnosis, and clinical course of such cases. Data Sources.—Relevant articles indexed in PubMed (National Library of Medicine) between 1975 and 2006. Conclusions.—Our review did not confirm a uniformly aggressive clinical course in pediatric cases of CD56+ ALCLs. Such cases suggest the usefulness of the analysis of CD56-positivity in additional cases of ALCL in an attempt to accrue additional information on this condition. Future accrual of such cases may address whether such cases should be treated more aggressively or with possible targeted therapeutic regimens.


2014 ◽  
Vol 55 (4) ◽  
pp. 304-306 ◽  
Author(s):  
Masahito Yasuda ◽  
Naoya Igarashi ◽  
Yayoi Nagai ◽  
Atsushi Tamura ◽  
Osamu Ishikawa

1990 ◽  
Vol 14 (11-12) ◽  
pp. 1071 ◽  
Author(s):  
Fumio Kawano ◽  
Atsuko Tsukamoto ◽  
Masahiko Satoh ◽  
Isao Sanada ◽  
Tadahiro Shido ◽  
...  

2019 ◽  
Vol 64 (3) ◽  
pp. 353-361
Author(s):  
L. G. Gorenkova ◽  
S. K. Kravchenko ◽  
M. A. Silaev ◽  
N. V. Ryzhikova

Introduction. Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) belongs to a rare group of skin lymphoproliferative disorders, which is characterised by panniculitis-like clinical manifestations, the α/β cytotoxic immunophenotype of tumour cells, as well as a multidirectional course from indolent to aggressive forms.Aim. To examine patients with SPTCL having an aggressive clinical course and characterised by refractoriness to several lines of chemotherapy.Results. We present two case reports of patients with a generalised lesions and unfavourable prognostic factors, who achieved complete long-term remission of the disease as a result of gemcitabine chemotherapy.Conclusion. Despite the fact that both observed SPTCL patients demonstrated refractoriness to at least three types of treatment, the use of gemcitabine allowed long-term complete remissions of the disease to be achieved. 


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