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  Table of Contents    
CASE REPORT  
Year : 2021  |  Volume : 64  |  Issue : 4  |  Page : 817-819
Leonine facies: A unique presentation of T-prolymphocytic leukemia


1 Department of Hematology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

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Date of Submission02-Apr-2020
Date of Decision01-May-2020
Date of Acceptance22-Jun-2020
Date of Web Publication20-Oct-2021
 

   Abstract 


We report a 52-year-old man who presented with erythroderma and nodular lesions on face manifesting as “Leonine facies”. He had impaired sensation over the face and was initially diagnosed to have lepromatous leprosy and was treated with antileprosy drugs. Investigations showed a total Leukocyte count of 550 X 109/l with 90% atypical lymphoid cells with prominent central nucleolus suggestive of prolymphocytes. On flow cytometry, these cells were positive for cytoplasmic CD3, CD2, CD5, CD7, CD4, and CD38 (dim) and were negative for CD1a and TdT and diagnosis of T-prolymphocytic leukemia was made.

Keywords: Leonine facies, leukemia cutis, prolymphocytes, T-PLL

How to cite this article:
Hasan F, Parashar Y, Rao RN, Kashyap R. Leonine facies: A unique presentation of T-prolymphocytic leukemia. Indian J Pathol Microbiol 2021;64:817-9

How to cite this URL:
Hasan F, Parashar Y, Rao RN, Kashyap R. Leonine facies: A unique presentation of T-prolymphocytic leukemia. Indian J Pathol Microbiol [serial online] 2021 [cited 2021 Nov 28];64:817-9. Available from: https://www.ijpmonline.org/text.asp?2021/64/4/817/328554





   Introduction Top


T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive mature T-cell neoplasm of post thymic origin characterized by the proliferation of small- to medium-sized lymphocytes.[1] It accounts for less than 2% of mature lymphocytic leukemias in adults. The most frequent chromosomal defect is an inversion of chromosome 14, in 80% of patients followed by t (14; 14) in 10% cases.[2],[3]

T-PLL patients present with fever, weight loss, lymphadenopathy, and hepatosplenomegaly. Cutaneous involvement is seen in one-fourth of patients, in the form of macules, papules, nodules, and erythema over the trunk and limbs. The involvement of the face in the form of purpura and periorbital edema, nodular lesions is rare.[2],[3],[4] We describe a patient with leonine facies (LF) who was initially clinically diagnosed to have leprosy because of the presence of erythematous nodules and papules on the facial skin and impaired skin sensation. Hematological evaluation and skin histopathology confirmed the diagnosis of T-PLL. The classical LF has been very rarely described in patients T-PLL.[5]


   Case Report Top


A 52-year-old male presented with a history of multiple skin nodules on the face and upper limbs for 5 months. The skin lesions were nonpruritic but he had impairment of sensations on the face. He also complained of low-grade intermittent, night sweats, and loss of weight and appetite. He was provisionally diagnosed to have lepromatous leprosy 3 months earlier by a local physician because of the skin lesions and started on antileprosy medications based on the clinical findings.

On physical examination, generalized erythema and nodular lesions were present over the face, trunk abdomen, and upper limbs. The nodules were firm, nontender, and nonpruritic. The facial skin and both the earlobes were thickened with furrowing producing the characteristic “Leonine facies” [Figure 1]. The cervical and axillary lymph nodes were enlarged. The spleen and liver both were enlarged.
Figure 1: Photograph of the patient showing nodules and erythema over the face and thickened ear lobes with furrowing suggestive of leonine facies

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Laboratory investigations revealed the hemoglobin level 10.2 g/dl, total leukocyte count 550 X 109/l, and platelet count 88 X109/l. Peripheral blood smear examination showed 90% atypical medium-sized lymphoid cells with irregular nuclei and prominent nucleoli with prominent cytoplasmic protrusions and blebs with prominent central nucleolus suggestive of prolymphocytes [Figure 2]. Biochemical tests showed elevated levels of serum uric acid 14.6 mg/dl, serum K 6.2 mEq/L, serum phosphorus 5.0 mg/dl, and low serum calcium level 7.1 mg/dl suggestive of tumor lysis. Flow cytometry was performed on the peripheral blood, and 94% of cells were gated in bright CD45 and low-side scatters. The cells which were positive for cytCD3/CD2/CD5/CD7/CD4/CD38 (dim) were negative for CD1a, sCD3, CD8, and TdT. The cells were negative for B-cell markers CD 10, CD19, CD25, myeloid lineage markers CD13, CD 33, CD 117, CD79a, CD 116, and MPO suggestive of T-PLL. A skin biopsy performed from the facial skin nodules, showed normal keratinized stratified squamous epithelium with diffuse infiltration of dermal, peri-adnexal, and subcutaneous adipose tissue by atypical lymphoid cells. The cells were small- to medium-sized with high nuclear–cytoplasmic ratio, round to irregular hyperchromatic nuclei, conspicuous nucleoli, and a scant amount of cytoplasm and presence of frequent mitotic figures (8–10/hpf) suggestive of leukemic infiltration [Figure 3]a and [Figure 3]b. Bone marrow aspiration and biopsy showed hypercellular marrow with the near-total replacement of the normal marrow components by small- to medium-sized lymphoid cells with morphological features of prolymphocytes. Serological tests for hepatitis B and C, HIV, and HTLV-1 infections were negative.
Figure 2: Photomicrograph of peripheral blood smear showing typical T-PLL medium-sized lymphocytes with a high nuclear-cytoplasmic ratio and deeply basophilic cytoplasm without granules

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Figure 3: ( a) The section from skin biopsy shows unremarkable keratinized stratified squamous epithelium. The dermis and subcutaneous adipose tissue show peri-adnexal diffuse infiltration by atypical lymphoid cells (H and E x10 magnification). (b) The section shows small- to medium-sized atypical lymphoid cells with high N-C ratio, round to irregular hyperchromatic nuclei, conspicuous nucleoli, and a scant amount of cytoplasm. Frequent mitotic figures are noted (8–10/hpf) (H and E x 40 magnifications)

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The patient was managed for tumor lysis syndrome. The patient and his relatives were counselled on the natural disease history, available novel therapies, and prognosis. The patient was treated with systemic CHOP (Cyclophosphamide 750 mg/m2 i.v on day 1, doxorubicin 50 mg/m2 i.v on day 1, vincristine 1.5 mg/m2 i.v on day 1, and prednisolone 100 mg per oral days 1-5) chemotherapy. After two cycles of chemotherapy, there was a significant amelioration of symptoms with the reduction in the size of the skin nodules, lymph nodes, and hepatosplenomegaly. Given financial constraints the patient discontinued further treatment.


   Discussion Top


T- Prolymphocytic leukemia is a rare and aggressive neoplasm of post thymic origin T-lymphocytes characterized by the involvement of peripheral blood, bone marrow, liver spleen, and skin. It is a disease of the elderly aged 65 years and older and has a male predominance.[2],[3],[4] T-PLL was first recognized in the year 1973 by Catovsky et al. in four patients presenting with hyper-leukocytosis and prolymphocytic morphology but was E rosette positive.[6] T-PLL patients typically present with a history of fever, weightloss, and lymphadenopathy of short duration. Small subsets of 10–15% of patients have an indolent course and may remain asymptomatic for several years.[7] In one of the large series of T-PLL published by Matutes et al., splenomegaly was seen in 73% of cases, lymphadenopathy in 53%, and hepatomegaly in 40% of patients. Cutaneous involvement was observed in 27% of patients with T-PLL, mainly in the form of a maculopapular rash, nodules, and occasional erythroderma.[4] A total leukocyte count >100 x 109/L was observed in 75% of patients and upto 50% of them also had associated thrombocytopenia and anemia. T-PLL may be associated with pleural effusion, ascites, and CNS involvement.[3],[4]

LF or facial features of lion occur due to the thickening of the facial skin by infiltrative disease. The skin nodules and papules fuse resulting in convexities and furrowed creases on the face to produce this appearance. LF has been described in patients with lepromatous leprosy, Leishmaniosis, sarcoidosis, amyloidosis, and cutaneous T-cell lymphomas (CTCL).[8],[9],[10] Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common forms of CTCL associated with skin involvement, and LF has been well documented in them. It has also been reported in patients with acute lymphoblastic leukemia and acute myeloid leukemia.[10],[11] Brown et al. reviewed 1338 patients with MF treated at their center over 28 years and found that among them 10 patients had LF. They observed that LF was more common in males and was associated with stage IV CTCL. LF was more frequently seen with folliculotropic MF followed by classical MF and SS.[11] It has rarely been described in patients with T-PLL. Sivaramakrishnan et al. have described a 75-year-old male who presented with scaly erythematous papules and plaques on the trunk and limbs. Five years after the initial presentation, he developed LF and erythroderma. Peripheral blood smear examination, immunophenotype, and molecular genetic studies confirmed the diagnosis of T-PLL[5]

T-PLL has to be differentiated from B-PLL, SS and adult T-cell leukemia-lymphoma (ATLL) which have mature T-cell immunophenotype and overlapping morphological features. Skin lesions and lymphadenopathy are less common in B-PLL compared to T-PLL. In SS and ATLL, the total leukocyte count is usually <100 X 109/L, and massive splenomegaly is rare. Erythroderma with pruritus is more common in SS, and hypercalcemia is a hallmark of ATLL.[3],[12] Morphologically, the T-prolymphocytes are smaller, have more basophilic cytoplasm and nuclear irregularity compared to B-PLL. In SS, the cells have characteristic cerebriform nuclei and ATLL is characterized by the presence of flower cells with multilobulated nuclei.[3],[12]

In 20% of T-PLL cases, the cells are small in size, nuclei round, and nucleoli less prominent and is referred to as “small cell variant” of T-PLL. In 5% cases, the T-PLL cells have irregular nuclei and resemble the cerebriform nuclei of SS cells. Both these morphological subtypes can be easily diagnosed by immunophenotyping by FCM. The T-PLL cells show co-expression of CD4, CD8, strong CD7, CD52, and CD2 positivity (with negative CD1a and TdT (post thymic neoplastic T-cells cells). Cytoplasmic CD 3 is always present and membrane CD 3 may be absent. Strong Cd 7 expression and CD 25 expression absence help to distinguish T-PLL from adult T-cell leukemia and SS.[3],[12] The histopathological examination of skin biopsy in T-PLL shows infiltration of the dermis, appendages, and the subcutis by the leukemic, but unlike SS, there is no epidermotropism, thereby differentiating the two entities.[3]

The disease has a very aggressive course with a median survival of 1–2 years with no curative therapy. CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisone) has shown a 30% response rate with early disease recurrence and mortality. The introduction of alemtuzumab, a monoclonal antibody against CD52 which is expressed in high density in these neoplasms, has shown promising results with higher cure rates.[13]

In conclusion, T-PLL is an extremely rare T-cell leukemia with a myriad of clinical presentation. LF is a manifestation of leukemic skin infiltration and the disease may be misdiagnosed especially in patients with an indolent form of the disease. A high degree of clinical suspicion and laboratory investigations are necessary for its diagnosis because in a developing country like India where the prevalence of Leprosy is still high, many patients with underlying hematological malignancies are often misdiagnosed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Galton DA, Goldman JM, Wiltshaw E, Catovsky D, Henry K, Goldberg GJ. Prolymphocytic leukemia. Br J Hematol 1974;27:7-23.  Back to cited text no. 1
    
2.
Swerdlow SH, Campo E, Pilen S, Harris NL, Stein H, Sibert R, et al. The 2016 revision of the. World Health Organization classification of lymphoid neoplasms. Blood 2016;127:2375-90.  Back to cited text no. 2
    
3.
Sud A, Dearden C. T-cell Prolymphocytic leukemia. Hematol Oncol Clin N Am 2017;31:273-83.  Back to cited text no. 3
    
4.
Matutes E, Brito-Babapulle V, Swansbury J, Ellis R, Morilla C, Dearden A, et al. Clinical and laboratory features of 78 cases of T-cell prolymphocytic leukemia. Blood 1991;78:3269-74.  Back to cited text no. 4
    
5.
Sivaramakrishnan M, Kerr R, Evans A, Affleck AL. A rare presentation of cutaneous pseudolymphoma as a preleukemic state. J Leuk 2013;1:1-4.  Back to cited text no. 5
    
6.
Catovsky D, Galetto J, Okos A, Galton DA, Wiltshaw E, Stathopoulos G. Prolymphocytic leukemia of B and T cell type. Lancet 1973;2:232-4.  Back to cited text no. 6
    
7.
Garand R, Goasguen J, Brizard A, Buisine J, Charpentier A, Classie JF, et al. Indolent course as a relatively frequent presentation in T-prolymphocytic leukemia. Groupe Francais d'Hematologic cellulaire. Br J Hematol 1998;103:488-94.  Back to cited text no. 7
    
8.
Jindal N, Jindal P, Kumar J, Gupta S. Animal eponyms in dermatology. Indian J Dermatol 2014;59:631.  Back to cited text no. 8
    
9.
Naseem S, Kashyap R, Awasthi NP, Krishnani N, Kumari N. Sezary syndrome presenting with 'leonine facies'. Australas J Dermatol 2009;50:285-8.  Back to cited text no. 9
    
10.
Ratnam KV, Khor CJL, Su WPD. Leukemia cutis. Dermatol Clin 1994;12:419-31.  Back to cited text no. 10
    
11.
Brown DN, Wieser I, Wang C, Dabaja BS, Duvic M. Leonine facies (LF) and mycosis fungoides (MF): A single centre study and systematic review of literature. J Am Acad Dermatol 2015;73:976-86.  Back to cited text no. 11
    
12.
Naseem S, Gupta R, Kashyap R, Nityanand S. T-cell prolymphocytic leukemia: A report of two cases with review of the literature. Indian J Hematol Blood Transf 2008;24:178-81.  Back to cited text no. 12
    
13.
Dearden C. How I treat prolymphocytic leukemia. Blood 2015;120:538-51.  Back to cited text no. 13
    

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Correspondence Address:
Rajesh Kashyap
Department of Hematology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow . 226 014, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_301_20

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