Successful remission induction with deoxycoformycin in elderly patients with T‐helper prolymphocytic leukaemia

El-Agnaf, M.; Ennis, K. E.; Morris, T. C. M.; Robertson, J. H.; Markey, G. M.; Alexander, H. Denis

doi:10.1111/j.1365-2141.1986.tb07499.x

Cited by 34 publications

(6 citation statements)

References 20 publications

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“…T-PLL is typically an aggressive disease, resistant to chemotherapy, and is associated with short survival. However, a few cases with an unusually indolent clinical course have been reported (Corwin et al, 1983;Lauria et al, 1985;El'Agnaf et al, 1986;Catovsky et al, 1996). In all of them the stable course of the disease transformed into aggressive stage after a variable period.…”

Section: Discussionmentioning

confidence: 99%

Indolent course as a relatively frequent presentation in T‐prolymphocytic leukaemia

Goasguen²,

et al. 1998

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T‐prolymphocytic leukaemia (T‐PLL) is a rare disorder with a poor outcome. Presentation features were studied in 78 T‐PLL cases. Although 53 patients (group A) presented with typical progressive disease including rapidly increasing leucocytosis, 25 patients (group B) experienced an initial indolent clinical course with stable moderate leucocytosis. The morphology and antigenic profile of abnormal cells were similar in both groups, except for a lower incidence of CD45RO+ CD45RA− pattern in group B. A high incidence of inv(14)(q11;q32), t(14;14)(q11;q32) and i(8)(q10) chromosomal abnormalities were found in both groups. After an initial indolent phase (median 33 months; 6–103 months), 16 group B patients progressed to an aggressive stage with clinical and laboratory features similar to group A. Moreover, median survival after progression was short in both groups. In conclusion, T‐PLL may start as an indolent disease similar to that reported in ataxia telangectasia. In this rare genetic disorder, some patients develop stable T‐cell clones which progress toward T‐PLL‐like leukaemia. Moreover, ATM gene mutations have been reported in T‐PLL. Thus, both diseases are likely to be closely related.

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Section: Discussionmentioning

confidence: 99%

Indolent course as a relatively frequent presentation in T‐prolymphocytic leukaemia

Goasguen²,

et al. 1998

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“…Costanza (CA-37 135); Columbia Uni- T-cell malignancies cannot be ascertained for lack of an accurate denominator; however, three patients with Tcell phenotypes had responses of 2, 7, and 11 months. Others have noted significant response rates in cutaneous T-cell lymphomas [ 12,131, T-cell prolymphocytic leukemia [14], and various T-cell malignancies . Future studies should include careful phenotyping of lymphoid malignancies and targeted trials in specific T-cell malignancies.…”

Section: Discussionmentioning

confidence: 99%

2′ deoxycoformycin (pentostatin) for refractory non‐Hodgkin's lymphoma: A calgb phase II study

Duggan

Anderson²,

Dillman

et al. 1990

Med. Pediatr. Oncol.

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Seventy-six eligible patients with relapsed or refractory non-Hodgkin's lymphoma (NHL) were treated with 2'-deoxycoformycin (pentostatin) at a dose of 4 mg/m2 intravenously weekly for three weeks and then every other week for a minimum of five total treatments. All patients had measurable disease, near normal hematologic, renal, and hepatic function, and a performance status of 0 or 1. Severe hematologic toxicity was observed in 13% of patients; severe renal or neurologic toxicity was observed in less than 5% of patients. There were no treatment-related deaths. Objective therapeutic responses were seen in 16% of patients (five complete response [CR] and seven partial response [PR]). However, in three of the patients achieving CR and one patient achieving PR, dexamethasone was employed as an anti-emetic, making the response of these patients to pentostatin difficult to evaluate. There were eight responses (3 CR) in patients with diffuse histologies and four responses (2 CR) in patients with nodular or mixed histologies. Three responses were in patients with a T-cell phenotype. Three of five patients with diffuse well-differentiated lymphoma (IWF A) responded. We conclude that 2' deoxycoformycin is only minimally active at this dose and schedule against refractory or relapsed NHL. The possibility that low grade B- and T-cell malignancies are more sensitive to 2' deoxycoformycin deserves further investigation.

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“…However, T-PLL transformation has not been reported previously. Regarding T-PLL, a few cases with an unusually indolent clinical course have been reported [6][7][8][9], although stable disease has been known to become aggressive. Some indolent T-PLL cases might have similar clinical features to B-PLL [10].…”

Section: Discussionmentioning

confidence: 99%

A case of T cell prolymphocytic leukemia involving blast transformation

et al. 2011

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We report a case of T cell prolymphocytic leukemia (T-PLL) involving blast transformation. At the initial diagnosis, most peripheral blood cells demonstrated proliferation of indolent T cell small cell variants, i.e., small to medium prolymphocytes with inconspicuous nucleoli and a normal karyotype. These cells were positive for surface CD4, CD5, and CD7, and cytoplasmic CD3, but negative for surface CD3 and CD8 and cytoplasmic terminal deoxynucleotidyl transferase (TdT). The T cell receptor (TCR) Cβ1 gene was rearranged in the cells. Large prolymphocytes with prominent nucleoli, irregular nuclei, and cytoplasmic vacuoles that exhibited chromosome 8 trisomy were observed about 1.5 years later. The CD4+CD8- single positive effector memory T cells transformed into surface CD4+CD8+ double positive precursor T cells. The clonal TCR gene rearrangement patterns of these cells were identical throughout the clinical course, suggesting clonal blast transformation. The CD4+CD8+ cells demonstrated increased chromosome 8 trisomy combined with complex chromosome abnormalities with t(14;14)(q11.2;q32) containing a 14q32 chromosome after transformation. T cell leukemia 1a (TCL1a) (14q32.1) may be implicated in this case. The TCL1a oncoprotein is expressed in approximately 70% of T-PLL cases. The disease gradually developed resistance to chemotherapy, and the patient died of the disease. It is known that indolent T-PLL can become aggressive. Therefore, similar transformations may occur in other aggressive T-PLL cases, particularly those involving trisomy 8 and TCL1a.

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Successful remission induction with deoxycoformycin in elderly patients with T‐helper prolymphocytic leukaemia

Cited by 34 publications

References 20 publications

Indolent course as a relatively frequent presentation in T‐prolymphocytic leukaemia

Indolent course as a relatively frequent presentation in T‐prolymphocytic leukaemia

2′ deoxycoformycin (pentostatin) for refractory non‐Hodgkin's lymphoma: A calgb phase II study

A case of T cell prolymphocytic leukemia involving blast transformation

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