Abstract
T cell prolymphocytic leukemia (T-PLL) is a rare mature T cell lymphoproliferative disease. It has been associated with an aggressive course, a poor response to conventional chemotherapy and a short median survival. Here we present a rare case of concurrent T-PLL and Kaposi sarcoma who achieved a complete hematologic and cytogenetic remission after a very short course of treatment with alemtuzumab. A review of T-PLL was done. In this review, clinical features, laboratory features and current therapeutic strategies of T-PLL are presented.
References
1.
Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, Sempere A, Catovsky D: Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia. Blood 1991;78:3269–3274.
2.
Robak T, Robak P: Current treatment options in prolymphocytic leukemia. Med Sci Monit 2007;13:RA69–RA80.
3.
Dearden CE, Matutes E, Cazin B, Tjønnfjord GE, Parreira A, Nomdedeu B, Leoni P, Clark FJ, Radia D, Rassam SM, Roques T, Ketterer N, Brito-Babapulle V, Dyer MJ, Catovsky D: High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H. Blood 2001;98:1721–1726.
4.
Melo JV, Catovsky D, Galton DA: The relationship between chronic lymphocytic leukaemia and prolymphocytic leukaemia. I. Clinical and laboratory features of 300 patients and characterization of an intermediate group. Br J Haematol 1986;63:377–387.
5.
Lan K, Murakami M, Choudhuri T, Tsai DE, Schuster SJ, Wasik MA, Robertson ES: Detection of Epstein-Barr virus in T-cell prolymphocytic leukemia cells in vitro. J Clin Virol 2008;43:260–265.
6.
Ravandi F, O’Brien S: Chronic lymphoid leukemias other than chronic lymphocytic leukemia: diagnosis and treatment. Mayo Clin Proc 2005;80:1660–1674.
7.
Magro CM, Morrison CD, Heerema N, Porcu P, Sroa N, Deng AC: T-cell prolymphocytic leukemia: an aggressive T cell malignancy with frequent cutaneous tropism. J Am Acad Dermatol 2006;55:467–477.
8.
Herling M, Valbuena JR, Jones D, Medeiros LJ: Skin involvement in T-cell prolymphocytic leukemia. J Am Acad Dermatol 2007;57:533.
9.
Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW: World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Lyon, IARC Press, 2008.
10.
Ravandi F, Kantarjian H, Jones D, Dearden C, Keating M, O’Brien S: Mature T-cell leukemias. Cancer 2005;104:1808–1818.
11.
Sugimoto T, Imoto S, Matsuo Y, Kojima K, Yasukawa M, Murayama T, Kohfuku J, Mizuno I, Yakushijin K, Sada A, Nishimura R, Koizumi T: T-cell receptor gamma delta T-cell leukemia with the morphology of T-cell prolymphocytic leukemia and a post-thymic immunophenotype. Ann Hematol 2001;80:749–751.
12.
Toyota S, Nakamura N, Dan K: Small cell variant of T-cell prolymphocytic leukemia with a gammadelta immunophenotype. Int J Hematol 2005;81:66–68.
13.
Pekarsky Y, Hallas C, Isobe M, Russo G, Croce CM: Abnormalities at 14q32.1 in T cell malignancies involve two oncogenes. Proc Natl Acad Sci USA 1999;96:2949–2951.
14.
Brito-Babapulle V, Catovsky D: Inversions and tandem translocations involving chromosome 14q11 and 14q32 in T-prolymphocytic leukemia and T-cell leukemias in patients with ataxia telangiectasia. Cancer Genet Cytogenet 1991;55:1–9.
15.
Hetet G, Dastot H, Baens M, Brizard A, Sigaux F, Grandchamp B, Stern MH: Recurrent molecular deletion of the 12p13 region, centromeric to ETV6/TEL, in T-cell prolymphocytic leukemia. Hematol J 2000;1:42–47.
16.
Brito-Babapulle V, Hamoudi R, Matutes E, Watson S, Kaczmarek P, Maljaie H, Catovsky D: p53 allele deletion and protein accumulation occurs in the absence of p53 gene mutation in T-prolymphocytic leukaemia and Sezary syndrome. Br J Haematol 2000;110:180–187.
17.
Costa D, Queralt R, Aymerich M, Carrio A, Rozman M, Vallespi T, Colomer D, Nomdedeu B, Montserrat E, Campo E: High levels of chromosomal imbalances in typical and small-cell variants of T-cell prolymphocytic leukemia. Cancer Genet Cytogenet 2003;147:36–43.
18.
Maljaei SH, Brito-Babapulle V, Hiorns LR, Catovsky D: Abnormalities of chromosomes 8, 11, 14, and X in T-prolymphocytic leukemia studied by fluorescence in situ hybridization. Cancer Genet Cytogenet 1998;103:110–116.
19.
Herling M, Patel KA, Teitell MA, Konopleva M, Ravandi F, Kobayashi R, Jones D: High TCL1 expression and intact T-cell receptor signaling define a hyperproliferative subset of T-cell prolymphocytic leukemia. Blood 2008;111:328–337.
20.
Stilgenbauer S, Schaffner C, Litterst A, Liebisch P, Gilad S, Bar-Shira A, James MR, Lichter P, Dohner H: Biallelic mutations in the ATM gene in T-prolymphocytic leukemia. Nat Med 1997;3:1155–1159.
21.
Vorechovsky I, Luo L, Dyer MJ, Catovsky D, Amlot PL, Yaxley JC, Foroni L, Hammarstrom L, Webster AD, Yuille MA: Clustering of missense mutations in the ataxia-telangiectasia gene in a sporadic T-cell leukaemia. Nat Genet 1997;17:96–99.
22.
Le Toriellec E, Despouy G, Pierron G, Gaye N, Joiner M, Bellanger D, Vincent-Salomon A, Stern MH: Haploinsufficiency of CDKN1B contributes to leukemogenesis in T-cell prolymphocytic leukemia. Blood 2008;111:2321–2328.
23.
Röth A, Dürig J, Himmelreich H, Bug S, Siebert R, Dührsen U, Lansdorp PM, Baerlocher GM: Short telomeres and high telomerase activity in T-cell prolymphocytic leukemia. Leukemia 2007;21:2456–2462.
24.
Garand R, Goasguen J, Brizard A, Buisine J, Charpentier A, Claisse JF, Duchayne E, Lagrange M, Segonds C, Troussard X, Flandrin G: Indolent course as a relatively frequent presentation in T-prolymphocytic leukaemia. Groupe Français d’Hématologie Cellulaire. Br J Haematol 1998;103:488–494.
25.
Cavazzini F, Cuneo A, Bardi A, Castoldi G: Indolent T-cell prolymphocytic leukemia: a case report and a review of the literature. Am J Hematol 2003;74:145–147.
26.
Dearden CE: T-cell prolymphocytic leukemia. Med Oncol 2006;23:17–22.
27.
Lorand-Metze I, Oliveira GB, Aranha FJ: Treatment of prolymphocytic leukemia with cladribine. Ann Hematol 1998;76:85–86.
28.
Kantarjian HM, Childs C, O’Brien S, Huh Y, Beran M, Schachner J, Koller C, Keating MJ: Efficacy of fludarabine, a new adenine nucleoside analogue, in patients with prolymphocytic leukemia and the prolymphocytoid variant of chronic lymphocytic leukemia. Am J Med 1991;90:223–228.
29.
Mercieca J, Matutes E, Dearden C, MacLennan K, Catovsky D: The role of pentostatin in the treatment of T-cell malignancies: analysis of response rate in 145 patients according to disease subtype. J Clin Oncol 1994;12:2588–2593.
30.
Montillo M, Tedeschi A, O’Brien S, Di Raimondo F, Lerner S, Ferrajoli A, Morra E, Keating MJ: Phase II study of cladribine and cyclophosphamide in patients with chronic lymphocytic leukemia and prolymphocytic leukemia. Cancer 2003;97:114–120.
31.
Keating MJ, Cazin B, Coutré S, Birhiray R, Kovacsovics T, Langer W, Leber B, Maughan T, Rai K, Tjønnfjord G, Bekradda M, Itzhaki M, Hérait P: Campath-1H treatment of T- cell prolymphocytic leukemia in patients for whom at least one prior chemotherapy regimen has failed. J Clin Oncol 2002;20:205–213.
32.
Ravandi F, O’Brien S, Jones D, Lerner S, Faderl S, Ferrajoli A, Wierda W, Garcia-Manero G, Thomas D, Koller C, Verstovsek S, Giles F, Cortes J, Herling M, Kantarjian H, Keating M: T-cell prolymphocytic leukemia: a single-institution experience. Clin Lymphoma Myeloma 2005;6:234–239.
33.
Khot A, Dearden C: T-cell prolymphocytic leukemia. Expert Rev Anticancer Ther 2009;9:365–371.
34.
Gilleece MH, Dexter TM: Effects of CAMPATH-1H antibody on human haemopoietic progenitors in vitro. Blood 1993;82:807–812.
35.
Ginaldi L, De Martinis M, Matutes E, Farahat N, Morilla R, Dyer MJ, Catovsky D: Levels of expression of CD52 in normal and leukemic B and T cells: correlation with in vivo therapeutic responses to Campath-1H. Leuk Res 1998;22:185–191.
36.
Dungarwalla M, Matutes E, Dearden CE: Prolymphocytic leukaemia of B- and T-cell subtype: a state-of-the-art paper. Eur J Haematol 2008;80:469–476.
37.
Ravandi F, Aribi A, O’Brien S, Faderl S, Jones D, Ferrajoli A, Huang X, York S, Pierce S, Wierda W, Kontoyiannis D, Verstovsek S, Pro B, Fayad L, Keating M, Kantarjian H: Phase II study of alemtuzumab in combination with pentostatin in patients with T-cell neoplasms. J Clin Oncol 2009;27:5425–5430.
38.
Hopfinger G, Busch R, Eichhorst B, Cramer P, Kandler G, Fingerle-Rowson G, Fink AM, Stilgenbauer S, Hallek M: TPLL-1 protocol of the German CLL Study Group (GCLLSG) – a prospective phase II trial of fludarabine phosphate, mitoxantrone and cyclophosphamide (FMC) followed by alemtuzumab consolidation in T-PLL. ASH Annual Meeting Abstracts 2007;110:2039.
39.
Curtin NJ, Schwarer AP: Nonmyeloablative peripheral blood stem cell transplant for T-cell prolymphocytic leukaemia complicated by fulminant haemolysis and acute renal failure at engraftment secondary to minor ABO incompatibility. Clin Lab Haematol 2005;27:206–208.
40.
Ghobrial IM, Bunch TJ, Caplice NM, Edwards WD, Miller DV, Litzow MR: Fatal coronary artery disease after unrelated donor bone marrow transplantation. Mayo Clin Proc 2004;79:403–406.
41.
Collins RH, Piñeiro LA, Agura ED, Fay JW: Treatment of T prolymphocytic leukemia with allogeneic bone marrow transplantation. Bone Marrow Transplant 1998;21:627–628.
42.
Tanimoto TE, Hirano A, Nagafuji K, Yamasaki S, Hashiguchi M, Okamura T, Kamezaki K, Takase K, Numata A, Miyamoto T, Fukuda T, Harada M: Mismatched unrelated cord blood transplantation in a patient with T-cell prolymphocytic leukemia. Leukemia 2005;19:679–681.
43.
Garderet L, Bittencourt H, Kaliski A, Daniel M, Ribaud P, Socié G, Gluckman E: Treatment of T-prolymphocytic leukemia with non-myeloablative allogeneic stem cell transplantation. Eur J Haematol 2001;66:137–139.
44.
de Lavallade H, Faucher C, Fürst S, El-Cheikh J, Vey N, Coso D, Bouabdallah R, Stoppa AM, Gastaut JA, Blaise D, Mohty M: Allogeneic stem cell transplantation after reduced-intensity conditioning in a patient with T-cell prolymphocytic leukemia: graft-versus-tumor effect and long-term remission. Bone Marrow Transplant 2006;37:709–710.
45.
Okamura K, Ikeda T, Shimakura Y, Yoshiba F, Kishi K, Ando K, Hotta T: Allogeneic bone marrow transplantation for chemotherapy-resistant T-prolymphocytic leukemia (in Japanese). Rinsho Ketsueki 2005;46:527–531.
46.
Krishnan B, Else M, Tjonnfjord GE, Cazin B, Carney D, Carter J, Ketterer N, Catovsky D, Ethell M, Matutes E, Dearden CE: Stem cell transplantation after alemtuzumab in T-cell prolymphocytic leukaemia results in longer survival than after alemtuzumab alone: a multicentre retrospective study. Br J Haematol 2010;149:907–910.
47.
Kalaycio ME, Kukreja M, Woolfrey AE, Szer J, Cortes J, Maziarz RT, Bolwell BJ, Buser A, Copelan E, Gale RP, Gupta V, Maharaj D, Marks DI, Pavletic SZ, Horowitz MM, Arora M: Allogeneic hematopoietic cell transplant for prolymphocytic leukemia. Biol Blood Marrow Transplant 2010;16:543–547.
48.
Hong A, Lee CS: Kaposi’s sarcoma: clinico-pathological analysis of human immunodeficiency virus (HIV) and non-HIV associated cases. Pathol Oncol 2002;8:31–35.
49.
Hiatt KM, Nelson AM, Lichy JH, Fauburg-Smith JC: Classic Kaposi sarcoma in the United States over the last two decades: a clinicopathologic and molecular study of 438 non-HIV-related Kaposi sarcoma patients with comparison to HIV-related Kaposi sarcoma. Mod Pathol 2008;21:572–582.
50.
Vitale F, Briffa DV, Whitby D, Maida I, Grochowska A, Levin A, Romano N, Goedert JJ: Kaposi’s sarcoma herpes virus and Kaposi’s sarcoma in the elderly populations of 3 Mediterranean islands. Int J Cancer 2001;91:588–591.
51.
Cole HN, Crump ES: Report of two cases of idiopathic hemorrhagic sarcoma (Kaposi), the first complicated with lymphatic leukemia. Arch Dermatol Syph 1920;1:283–295.
52.
Vey N, Camerlo J, Xerri L, Petit N, Dermeche S, Maraninchi D: Simultaneous occurrence of Kaposi sarcoma and chronic myelogenous leukemia. Leuk Lymphoma 2001;41:425–428.
53.
Licci S, D’Antonio A, Boscaino A, Morelli L, Piscioli F, Abbate I, Donnorso RP, Del Nonno F: Non-Hodgkin lymphomas concurrent with HHV8-associated Kaposi’s sarcoma in the same lymph node in AIDS and non-AIDS patients. Acta Haematol 2007;118:47–52.
54.
Escalon MP, Hagemeister FB: AIDS-related malignancies; in Kantarjian HM, Wolff RA, Koller CA (eds): MD Anderson Manual of Medical Oncology. New York, McGraw-Hill, 2006, pp 903–910.
55.
Hjalgrim H, Frisch M, Pukkala E, Tulinius H, Ekbom A, Dictor M, Langmark F, Hardarson S, Melbye M: Risk of second cancers in classical Kaposi’s sarcoma. Int J Cancer 1997;73:840–843.
56.
Biggar RJ, Curtis RE, Cote TR, Rabkin CS, Melbye M: Risk of other cancers following Kaposi’s sarcoma: relation to acquired immunodeficiency syndrome. Am J Epidemiol 1994;139:362–368.
57.
Franceschil S, Arnianil S, Balzi D, Geddes M: Survival of classic Kaposi’s sarcoma and risk of second cancer. Br J Cancer 1996;74:1812–1814.
58.
Koreishi A, Saenz AJ, Arcila ME, Hedvat C, Fleming S, Teruya-Feldstein J: Synchronous follicular lymphoma, Kaposi sarcoma, and Castleman’s disease in a HIV-negative patient with EBV and HHV-8 coinfection. Int J Surg Pathol 2011;19:685–691.
59.
Royle JS, Baade P, Joske D, Fritschi L: Risk of second cancer after lymphohematopoietic neoplasm. Int J Cancer 2011;129:910–919.
60.
Raulet DH, Guerra N: Oncogenic stress sensed by the immune system: role of natural killer cell receptors. Nat Rev Immunol 2009;9:568–580.
61.
Caccialanza M, Marca S, Piccinno R, Eulisse G: Radiotherapy of classic and human immunodeficiency virus-related Kaposi’s sarcoma: results in 1,482 lesions. J Eur Acad Dermatol Venereol 2008;22:297–302.
62.
Di Lorenzo G: Update on classic Kaposi sarcoma therapy: new look at an old disease. Crit Rev Oncol Hematol 2008;68:242–249.
© 2012 S. Karger AG, Basel
2012
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.