T‐prolymphocytic leukaemia with spontaneous remission

Shichishima, Tsutomu; Kawaguchi, M; Machii, Takashi; Matsuoka, Reiko; Ogawa, Kaoru; Maruyama, Yukio

doi:10.1046/j.1365-2141.2000.01836.x

Cited by 11 publications

(5 citation statements)

References 10 publications

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“…The frequency of 14q32 abnormalities on conventional cytogenetic analysis ranged from 25 to 76.6% in cases with T-PLL [1][2][3]. A total of 30 T-PLL cases, including 11 in the present study, have been reported in Japan [21,[26][27][28][29][30][31][32][33][34]; however, the frequency of typical chromosomal abnormalities involving 14q32 and Xq28 is low (3.3%) according to conventional methods. These data confirm a previous report [21].…”

Section: Discussionmentioning

confidence: 61%

TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients

et al. 2011

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T-cell prolymphocytic leukemia (T-PLL) is a rare type of peripheral T-cell leukemia. In this study, we examined the clinical and biological characteristics of 11 Japanese patients with T-PLL. Median age was 74 years, with male predominance. Median lymphocyte frequency was 85.3% in blood. Physical characteristics were splenomegaly (36.4%), tiny lymph adenopathy (63.6%), skin lesion (9.1%) and pleural effusion (27.3%). Median survival was 30.1 months, despite treatment with various chemotherapeutic modalities. Although complex chromosomal abnormalities were observed in 5 of 11 cases (45.5%), typical 14q32 and Xq28 abnormalities were not detected. TCL1A mRNA expression was observed in 6 of 11 cases (54.5%) on real-time quantitative PCR. In 5 of these 6 cases, flow cytometric analysis and/or immunohistochemistry confirmed the expression of TCLA1 protein. Split signals for the TCL1 region on fluorescence in situ hybridization confirmed rearrangement in 3 out of 7 cases evaluated. These cases corresponded to cases that were positive for TCL1A expression, suggesting that rearrangement of the TCL1 region induced high expression of TCL1A gene. In summary, a substantial number of T-PLL cases in Japan had abnormal expression of TCL1A, probably due to rearrangement of TCL1 region. Expression and/or rearrangement of TCL1A may, therefore, be a useful marker for diagnosing T-PLL, regardless of chromosomal abnormalities.

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Section: Discussionmentioning

confidence: 61%

TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients

et al. 2011

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“…A total of 48% of treated patients for whom response data were available showed an initial response, but all eventually progressed. Surprisingly, the disease in one patient spontaneously regressed and stayed in remission for the period of follow‐up (55 months) . The cause of death was disease progression in 86% of patients who died during the follow‐up period.…”

Section: Systematic Review Of Previous Cases Of T‐pll‐svmentioning

confidence: 98%

T‐cell chronic lymphocytic leukemia or small‐cell variant of T‐cell prolymphocytic leukemia: a historical perspective and search for consensus

Rashidi

Fisher

2015

European J of Haematology

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There is a rich history behind the extinct entity 'T-cell chronic lymphocytic leukemia (T-CLL)' and the nowestablished replacement, small-cell variant of T-cell prolymphocytic leukemia (T-PLL-sv). Herein, we review the history of the events, observations, and discussions that led to this replacement. We also provide a systematic analysis of all previously reported cases of T-PLL-sv as well as our four new additional cases. Despite the higher frequency of a normal karyotype and perhaps an overrepresented CD4 À CD8À immunophenotype among these patients (compared to T-PLL in general) as well as bland morphology (that makes them superficially appear more similar to B-CLL), we argue that the current World Health Organization (WHO)-based classification as T-PLL-sv is adequate and should continue for the time being. Morphologically, T-PLL-sv represents approximately one-fifth of all T-PLL cases. However, morphology alone does not determine the clinical course and should not be the basis for clinical decision making and prognostication. We propose a clonal evolution model in which mature T-cell leukemias classified in the past as T-CLL are perhaps T-PLL diagnosed early in the course of the disease. Future research using nextgeneration sequencing, comparative genomic hybridization, and molecular array studies, including serial analyses of individual cases over time, is needed to better identify this rarely diagnosed, inherently controversial form of T-cell leukemia.

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“…Some authors have noted atypical features in patients with leukaemia undergoing spontaneous remission. In a report of a patient with T‐prolymphocytic leukaemia, Shichishima et al suggested that absence of a CD7 positive phenotype and lack of structural abnormalities of 14q might have contributed to the patient's subsequent spontaneous complete remission 10 . It is important to note that remission is by no means synonymous with cure; in many cases the remission is short lived 8 and development of second malignancies has been reported with high frequency – 40% in patients with spontaneous remission of chronic lymphocytic leukaemia 7 .…”

Section: Discussionmentioning

confidence: 99%

“…Spontaneous remission of leukaemia is rare. This phenomenon has been reported in most leukaemias, including chronic myeloid leukaemia, 6 chronic lymphocytic leukaemia, 7 acute myeloid leukaemia, 8 and rarer disorders such as hairy cell leukaemia 9 and T‐prolymphocytic leukaemia 10 . The mechanism is not well understood.…”

Section: Discussionmentioning

confidence: 99%

Spontaneous Remission of Large Granular Lymphocytic Leukaemia

Sosin

Handa

2003

Int J Clinical Practice

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SUMMARYThe phenomenon of spontaneous remission is one of the less well known aspects of the leukaemias. Spontaneous remission has been reported in both acute and chronic leukaemia. The mechanism is unclear but may involve immune modulation related to an intercurrent infection or transfusion of blood products. We present a rare case of spontaneous complete remission of T‐cell large granular lymphocytic (T‐LGL) leukaemia occurring in an 82‐year‐old man. The patient died two years later of an unrelated condition, with no evidence of recurrence of T‐LGL leukaemia. Elucidation of the mechanism of the phenomenon of spontaneous remission may lead to advances in the treatment of this diverse group of disorders.

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T‐prolymphocytic leukaemia with spontaneous remission

Cited by 11 publications

References 10 publications

TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients

TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients

T‐cell chronic lymphocytic leukemia or small‐cell variant of T‐cell prolymphocytic leukemia: a historical perspective and search for consensus

Spontaneous Remission of Large Granular Lymphocytic Leukaemia

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