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Year : 2012  |  Volume : 57  |  Issue : 2  |  Page : 157-158
T-cell prolymphocytic leukemia with cutaneous involvement as the presenting feature

1 Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, India
2 Department of Dematology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune, India

Date of Web Publication20-Apr-2012

Correspondence Address:
Amrut V Ashturkar
Department of Pathology, Shrimati Kashibai Navale Medical College and General Hospital, Narhe, Pune
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.94297

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How to cite this article:
Ashturkar AV, Pathak GS, More YE, Bhandare A. T-cell prolymphocytic leukemia with cutaneous involvement as the presenting feature. Indian J Dermatol 2012;57:157-8

How to cite this URL:
Ashturkar AV, Pathak GS, More YE, Bhandare A. T-cell prolymphocytic leukemia with cutaneous involvement as the presenting feature. Indian J Dermatol [serial online] 2012 [cited 2022 Jun 28];57:157-8. Available from:


T-cell prolymphocytic leukemia (T-PLL) is a rare mature post-thymic T-cell malignancy that is usually reported in the elderly. [1] In contrast to B-cell lymphoproliferative disorders, T-cell malignancies show a tendency to specific cutaneous involvement. [2] The clinical course of T-PLL is typically aggressive with poor response to conventional chemotherapy. The only potential curative treatment is allogeneic stem-cell transplantation. [3] We report here an unusual case of T-PLL in which initial presentation was in the form of cutaneous lesions.

A 60-year-old female presented with erythematous papules, macules, and plaques all over the body since 4 months [Figure 1]. On clinical examination she had pallor, axillary lymphadenopathy, and splenomegaly. There was marked leukocytosis (WBC count 93 x 10 3 /ml) and anemia (Hb 8.3 gm/dl). Platelet count was adequate. Peripheral blood smear examination revealed 69% prolymphocytes. These cells were small to medium sized with a high nuclear to cytoplasmic ratio, moderately condensed chromatin, and a single prominent vesicular nucleolus [Figure 2]. The cytoplasm was scanty, basophilic, and agranular with occasional protrusions. Many of the cells had folded nuclei. Peripheral smear also showed significant amount of eosinophilia (22%). Immunophenotyping of the peripheral blood revealed positivity for CD3, CD4, CD5, CD7, and CD43. CD8, terminal deoxynucleotidyl transferase (TdT) and all the B-cell markers were negative. Bone marrow was hypercellular and revealed increased number of prolymphocytes and eosinophilic precursors. Light microscopy of skin biopsy revealed an unremarkable epidermis with aggregates of atypical lymphoid cells and eosinophils in the superficial dermis. The lymphoid cells were small to medium sized with round nucleus and distinct nucleoli. Epidermotropism was absent [Figure 3]. These lymphoid cells on immunohistochemistry showed similar immunoprofile as that of peripheral blood prolymphocytes [Figure 4]. Serology for Human T-cell leukaemia Virus -I (HTLV-1) was negative. Based on the morphologic features and the immunophenotypic profile, a diagnosis of T-cell prolymphocytic leukemia (T-PLL) was offered. The patient was started on CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) regimen, but did not respond to treatment.
Figure 1: Patient showing erythematous papules, macules, and plaques on the lower extremity

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Figure 2: Photomicrograph of peripheral blood smear showing prolymphocytes and eosinophils (H and E stain, 400×). Inset - Showing closer view of prolymphocytes

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Figure 3: Photomicrograph of skin biopsy showing aggregates of lymphoid cells in the superficial dermis without epidermotropism (H and E stain, 40×)

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Figure 4: Lymphoid cells showing strong positivity for CD3 on immunohistochemistry (H and E stain, 100×)

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Common clinical features of T-PLL are marked lymphocytosis, generalized lymphadenopathy, and hepatosplenomegaly. Secondary skin involvement by T-PLL has been reported in about 25% patients [4] and can be the first manifestation of the disease, as in our case.

There was eosinophilia in this case that persisted even after chemotherapy. Possible reason is that T-cells secrete cytokines such as IL-5, IL-4, and IL-13 that are responsible for IgE production and hypereosinophilia. [5] Skin biopsies of T-PLL with cutaneous involvement show a normal epidermis and a dermal infiltrate concentrated around blood vessels and appendages. Epidermotropism has never been described. [6]

T-PLL cells have a mature post-thymic T-cell phenotype and do not express CD1a and terminal deoxynucleotidyl transferase (TdT). The cells are positive for CD2, CD5, CD3, and CD7. A CD4+/CD8- phenotype is seen in approximately two-thirds of the cases, CD4+/CD8+ in about 25% cases and CD4-/CD8+ expression is less often seen. [7]

The differential diagnosis from other T-cell malignancies such as adult T-cell leukemia (ATLL), Sézary syndrome, and large granular cell leukemia is based on laboratory features such as morphology, histology, and immunophenotyping. The expression of CD7 is a consistent feature of TPLL. But this marker is often negative in ATLL, and Sézary syndrome and large granular cell leukemia. [7] In addition, negative serology for HTLV-I ruled out ATLL. [6]

T-PLL is considered to be an aggressive leukemia with a poor prognosis and resistance to conventional chemotherapy, the median survival being less than 7.5 months. [6] Although Campath-1H, an anti-CD52 humanized monoclonal antibody has been found to be effective in T-PLL, [1] allogeneic stem cell transplantation is still the only curative option for T-PLL [3] and should be considered the initial treatment of choice.

   Acknowledgment Top

Millennium Special Pathology Laboratory, NABL accreditated referral Laboratory, Sadashiv Peth, for conducting flow cytometric analysis on the case sample.

   References Top

1.Ghosh S, Advani SH. T-Cell prolymphocytic leukemia: A rare case. Indian J Cancer 2005;42:104-6.  Back to cited text no. 1
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2.Serra A, Estrach MT, Martí R, Villamor N, Rafel M, Montserrat E. Cutaneous involvement as the first manifestation in a case of T-cell prolymphocytic leukaemia. Acta Derm Venereol 1998;78:198-200.  Back to cited text no. 2
3.Dearden CE. T-Cell prolymphocytic leukemia. Clin Lymphoma Myeloma 2009;9:S239-43.  Back to cited text no. 3
4.Mallet RB, Matutes E, Catovsky D, MacLennan K, Mortimer PS, Holden CA. Cutaneous infiltration in T-cell prolymphocytic. Br J Dermatol 1995;132:263-6.  Back to cited text no. 4
5.Punnonen J, Yssel H, de Vries JE. The relative contribution of IL-4 and IL-13 to human IgE synthesis induced by activated CD4+ or CD8+ T cells. J Allergy Clin Immunol 1997;100:792-801.  Back to cited text no. 5
6.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. 6
7.Matutes E. T-cell prolymphocytic leukemia. Cancer Control 1998;5:19-24.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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