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Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 950-953

T-cell prolymphocytic leukemia presenting with leukemic serous effusion in a prostate cancer patient

1 Department of Heamatology, School of Medicine, Akdeniz University, Antalya, Turkey
2 Department of Medical Oncology, School of Medicine, Akdeniz University, Antalya, Turkey
3 Department of Medical Biology and Genetics, School of Medicine, Akdeniz University, Antalya, Turkey

Date of Web Publication15-Feb-2016

Correspondence Address:
Ozan Salim
Akdeniz Üniversitesi Tip Fakültesi, Tibbi Hematoloji Bölümü, Konyaalti, Antalya 07600
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.163707

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 > Abstract 

T-cell prolymphocytic leukemia (T-PLL) is highly aggressive mature postthymic lymphoproliferative disorder, which is characterized by several clinical features. Leukemic prolymphocytes are found in the peripheral blood, bone marrow, lymph nodes, spleen, liver, and sometimes skin. T-PLL and solid tumor coincidence was reported by only four previous cases. Solid tumor components included breast cancer, classic Kaposi sarcoma, gastric cancer, and lung cancer in those cases. We report the first case of T-PLL, an extremely rare disease, presented with serous effusion in an elderly prostate cancer patient in literature.

Keywords: Cancer, serous effusion, T-cell prolymphocytic leukemia

How to cite this article:
Salim O, Salim DK, Berker S, Undar L. T-cell prolymphocytic leukemia presenting with leukemic serous effusion in a prostate cancer patient. J Can Res Ther 2015;11:950-3

How to cite this URL:
Salim O, Salim DK, Berker S, Undar L. T-cell prolymphocytic leukemia presenting with leukemic serous effusion in a prostate cancer patient. J Can Res Ther [serial online] 2015 [cited 2020 Aug 4];11:950-3. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/950/163707

 > Introduction Top

T-cell prolymphocytic leukemia (T-PLL) is mature lymphocytic leukemia with aggressive behavior, which is often resistant to conventional chemotherapy. [1] T-PLL is rare leukemia typically affecting older people and occurs more frequently in males. Patients usually have splenomegaly, generalized lymphadenopathy, skin infiltration, and lymphocytosis over 100 × 10 9 /L at presentation. Morphologically, the predominant cell population in the peripheral blood (PB) is nucleolated prolymphocytes. An accurate diagnosis can be made by means of typical morphologic features and advanced immunophenotyping methods. [1] Before the current treatment approaches, such as alemtuzumab and hematopoietic stem cell transplantation (HSCT), the median survival was less than a year. [2],[3]

T-PLL cases with widespread presenting clinical features have previously been reported. [3] Serous effusions may develop during the course of the disease and also are seen in 15% at diagnosis as in this case. [2],[3] Clinically, significant effusion is observed more frequently in aggressive T-PLL cases than that in indolent T-PLL cases. [4]

As far as we know, T-PLL and solid tumor coincidence was reported by only four previous cases. [5],[6],[7],[8] Solid tumor components included breast cancer, classic Kaposi sarcoma, gastric cancer, and lung cancer in those cases.

Here, we report the first case of T-PLL, a rare disease, presented with serous effusion in an elderly prostate cancer patient in literature.

 > Case report Top

A 72-year-old man was referred to the medical oncology department with complaints of dyspnea and abdominal discomfort lasting for 2 months. His past medical history included radical prostatectomy due to prostate adenocarcinoma in 2004. He had leuprolide acetate and bicalutamide for 5 years. His family history was noncontributory. A physical examination and computed tomography scan revealed ascites, pleural effusion, and splenomegaly (152 mm in diameter) with no lymphadenopathy, so clinical diagnosis of peritonitis carcinomatosa was made. There was no evidence of any other visceral metastases and prostate specific antigen (PSA) progression. The white blood cell count was 314 × 10 9 /L with 79% lymphocytes. PB smear showed a predominant population of medium-sized cells with agranular cytoplasm, slightly eccentric nucleus and a single prominent nucleolus representing prolymphocytes [Figure 1]a]. Morphologic examination of pleural and peritoneal fluids [Figure 1]b] and bone marrow (BM) aspirate were similar.
Figure 1: (a) Peripheral blood smear. MGG, ×1200. (b) Peritoneal fluid smear. MGG, ×1200. Abbreviation: MGG = May-Grünwald Giemsa

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Immunophenotyping of lymphocytes were strongly positive for CD2, CD3, CD4, CD5, CD7, and negative for CD8, TdT, HLADR, CD20, CD34, MPO, and CD56 [Figure 2]a-d]. No cells in metaphase were obtained in the cytogenetic analysis of BM aspirate. There were abnormalities of chromosome 8 (trisomy 8 in 47% of cells and tetrasomy 8 in 48% of cells) by fluorescence in situ hybridization (FISH) analysis [Figure 3]a and b]. The anti-HTLV-1 antibody was negative. Based on the morphologic features and immunophenotypic profile, a diagnosis of CD4 + /CD8 T-PLL was made. He was treated with three courses of fludarabine, cyclophosphamide (FC) and two courses of cyclophosphamide, vincristine, adriamycin and dexamethasone (CVAD) regimens by which a partial response was achieved. After compassionate use approval was obtained, he received 12 doses of subcutaneous alemtuzumab. At 4 th week of the treatment, cytomegalovirus (CMV) reactivation was detected under appropriate antimicrobial prophylaxis. During CMV infection treatment with ganciclovir, alemtuzumab could not be continued. In case of CMV infection was controlled PLL progression developed, and the patient died at a 12 th month from diagnosis. When he died, his PSA levels were in castrated levels, and he was disease free for prostate cancer.
Figure 2: (a) Peripheral blood immunophenotyping. (b) Peritoneal fluid immunophenotyping. (c) Pleural fluid immunophenotyping. (d) Bone marrow immunophenotyping

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Figure 3: CEP 8 single color cytocell probe was used. (a) Abnormal signaling cell with three green signaling 8th chromosome. (b) Abnormal signaling cell with four green signaling 8th chromosome

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 > Discussion Top

In this case, we report pleural and peritoneal effusion revealing T-PLL in a hormone responsive prostate cancer patient.

T-PLL is mostly the disease of elderly males with median age 65 years, [1] and our patient was at a natural risk for this disease. Through a literature review, T-PLL has not been described earlier in relation to prostate cancer or its hormonal therapy. Furthermore, the absence of cytotoxic therapy history and no family history of any malignancies weighs on the possibility of a genetic abnormality for such a co-occurrence. T-PLL is a heterogeneous disease with a high frequency of chromosomal alterations, and also many genetic alterations have been shown in prostate cancer patients. The mostly reported cytogenetic abnormalities in T-PLL patients are in v 14, t(14;14), del 11q23 and abnormalities of chromosome 8. [9] In our case, trisomy 8 and tetrasomy 8 were detected by FISH analysis, which may suggest the common molecular genetics of prostate cancer and T-PLL co-occurrence.

T-PLL can easily be distinguished from B-PLL by immunophenotyping. T-PLL cases typically express T-cell differentiation antigens CD2, CD3, CD5, and CD7 but not CD1, HLA-DR, or TdT. In two-third of those, the cells are CD4 + /CD8 .

The careful diagnostic approach is needed to distinguish T-PLL from other mature T-cell leukemias that may have a similar presentation. In this case, negative serology for human T-cell lymphotropic virus, normal calcium level, and CD7 immunophenotyping exclude the diagnosis of adult T-cell leukemia/lymphoma. Sezary syndrome was ruled out because this patient has no cutaneous involvement and CD7 immunophenotyping. T-cell large granular lymphocyte leukemia was ruled out by the lack of large granular lymphocytes and absence of neutropenia.

Although it is a rare disease, it is usually aggressive with low response rates to conventional chemotherapies. [2],[3] Alemtuzumab has been shown to be effective with 74% and 91% response rates in previously treated and untreated patients, respectively. [10] However, it is not curative, and HSCT in the first remission should be an option for transplant eligible patients.

Our patient was responsive and asymptomatic for prostate cancer, so androgen deprivation treatment was continued, along with FC and CVAD regimens with the best response of partial response. Then he received 12 doses of alemtuzumab over 4 weeks till CMV reactivation. Unfortunately, with the discontinuation of alemtuzumab, his T-PLL progressed after an initial stable disease of 2 months and he died at a 12 th month from diagnosis.

Correct diagnosis of T-PLL is important due to its poor prognosis and nonspecific clinical characteristics which make diagnosis difficult. This case demonstrates the feasibility and efficacy of morphological and immunophenotypical investigation of abnormal body fluids in the diagnosis of T-PLL. In addition, it illustrates the rare simultaneous occurrence of prostate cancer and T-PLL.

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Conflicts of interest

There are no conflicts of interest.

 > References Top

Swerdlow SH, Campo E, Hornis NL, Jaffe ES, Piteri SA, Stein H, et al. WHO Classification of Tumors of Hematopoetic and Lymphoid Tissues. 4 th ed. Lyon: International Agency for Research on Cancer; 2008. p. 720-21.  Back to cited text no. 1
Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, et al. Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia. Blood 1991;78:3269-74.  Back to cited text no. 2
Dungarwalla M, Matutes E, Dearden CE. Prolymphocytic leukaemia of B- and T-cell subtype: A state-of-the-art paper. Eur J Haematol 2008;80:469-76.  Back to cited text no. 3
Garand R, Goasguen J, Brizard A, Buisine J, Charpentier A, Claisse JF, et al. Indolent course as a relatively frequent presentation in T-prolymphocytic leukaemia. Groupe français d'hématologie cellulaire. Br J Haematol 1998;103:488-94.  Back to cited text no. 4
Kameoka J, Takahashi N, Noji H, Murai K, Tajima K, Kameoka Y, et al. T-cell prolymphocytic leukemia in Japan: Is it a variant? Int J Hematol 2012;95:660-7.  Back to cited text no. 5
Yokohama A, Saitoh A, Nakahashi H, Mitsui T, Koiso H, Kim Y, et al. TCL1A gene involvement in T-cell prolymphocytic leukemia in Japanese patients. Int J Hematol 2012;95:77-85.  Back to cited text no. 6
Paul RN, Alizadeh L, Ajayi OI, Karpurapu H, Ganesan C, Taddesse-Heath L, et al. A case report of T cell prolymphocytic leukemia and Kaposi sarcoma and a review of T cell prolymphocytic leukemia. Acta Haematol 2012;127:235-43.  Back to cited text no. 7
Singhal M, Raina V, Gupta R, Das P. T cell-prolymphocytic leukemia detected in a patient of breast cancer at the time of recurrence: A case report. Cases J 2010;3:4.  Back to cited text no. 8
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;103:110-6.  Back to cited text no. 9
Dearden CE, Matutes E, Cazin B, Tjønnfjord GE, Parreira A, Nomdedeu B, et al. High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H. Blood 2001;98:1721-6.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]


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