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Table of Contents 
CASE REPORT
Year : 2017  |  Volume : 62  |  Issue : 2  |  Page : 200-202
CD3+, CD56+, CD4−, CD8−, CD20−, CD30− Peripheral T-Cell Non-Hodgkin's Lymphoma: A Rare Case Report


Department of Skin and VD, B.J. Medical College and Civil Hospital, Ahmedabad, Gujarat, India

Date of Web Publication9-Mar-2017

Correspondence Address:
Ashish Jagati
Room No. 139, First Floor, Wing No 3, OPD Building, Civil Hospital, Asarwa, Ahmedabad, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_440_16

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   Abstract 

Cutaneous T-cell lymphoma (CTCL) commonly presents as mycosis fungoides or Sezary syndrome, both having CD4 positivity. A subset of CTCL which lacks CD4 surface marker is classified as cutaneous g and d–T-cell lymphoma (CGD-TCL). Because of its rarity and inability to study large number of patients, the impact of immunophenotype on the clinical outcome of primary CTCL in patients is limited. We report a case of primary CGD-TCL in a 71-year-old male because of this rarity and to emphasize its aggressive nature.


Keywords: Epidermotropism, immunohistochemistry, primary cutaneous gamma/delta–T-cell lymphoma


How to cite this article:
Jagati A, Shah BJ, Tibrewal S, Gajjar T. CD3+, CD56+, CD4−, CD8−, CD20−, CD30− Peripheral T-Cell Non-Hodgkin's Lymphoma: A Rare Case Report. Indian J Dermatol 2017;62:200-2

How to cite this URL:
Jagati A, Shah BJ, Tibrewal S, Gajjar T. CD3+, CD56+, CD4−, CD8−, CD20−, CD30− Peripheral T-Cell Non-Hodgkin's Lymphoma: A Rare Case Report. Indian J Dermatol [serial online] 2017 [cited 2017 Mar 25];62:200-2. Available from: http://www.e-ijd.org/text.asp?2017/62/2/200/201762

What was known?
Peripheral T cell lymphomas(PTCL) are a heterogeneous group of clinically aggressive diseases which are associated with a poor outcome. PTCLs remain largely unexplored and the optimal treatment remains ill-defined due to the disease rarity and biological heterogeneity. Classifying CTCLs based on immunohistochemistry markers hints towards the clinical behavior and prognosis of the lymphoma.



   Introduction Top


Cutaneous T-cell lymphoma (CTCL) is classified as a subtype of non-Hodgkin's lymphoma. It is a heterogeneous group of T-cell malignancies which are not defined by any of the recognized clinicopathologic subsets. Several subsets based on characteristic clinical, pathologic, and immunophenotypic features have been described. Full staging investigations are required to exclude a systemic nodal/extranodal peripheral T-cell lymphoma, and multiagent chemotherapy is recommended. In our best knowledge, only forty cases of primary cutaneous gamma/delta–T-cell lymphoma (CGD-TCL) have been described so far.


   Case Report Top


A 71-year-old male patient presented with asymptomatic, multiple papulonodular lesions over body for the past 5 months without any constitutional symptom. The initial site affected was the right leg, and then, he developed lesions over the lips, face, abdomen, and trunk.

Physical examination revealed few erythematous tender nodules over the face. There were multiple erythematous tender nodules and plaques, few with eschar over the abdomen, trunk, and extremities [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d. There was no associated lymphadenopathy. Systemic examination was insignificant.
Figure 1: (a-d) Multiple erythematous tender nodules and plaques, few with eschar over the abdomen, trunk, and extremities

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All the baseline investigations were normal except slightly raised white blood cell count and mean corpuscular hemoglobin. Peripheral blood smear and bone marrow biopsy showed no abnormality.

On skin biopsy, epidermis showed epidermotropic lymphoid cell [Figure 2]a. Dense infiltrate of medium to large lymphoid cell with vesicular nuclei, prominent nucleoli, and moderate cytoplasm was present in the dermis [Figure 2]b and [Figure 2]c on hematoxylin and eosin stain.
Figure 2: (a) Epidermotropism of lymphoid cell (×40, H and E). (b) Low magnification (×10, H and E). Dense infiltrate of medium to large lymphoid cell with vesicular nuclei, prominent nucleoli, and moderate cytoplasm was present in the dermis. ( c) High magnification (×40, H and E). Dense infiltrate of medium to large lymphoid cell with vesicular nuclei, prominent nucleoli, and moderate cytoplasm was present in the dermis

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Immunohistochemistry done twice showed CD2+, CD3+ [Figure 3]a and [Figure 3]c, CD56+ [Figure 3]b, CD4−, CD8−, CD20−, CD30−, and Ki67+ [Figure 3]d; 90% of the cells showed MIB1 positivity.
Figure 3: (a) CD3 positivity in lymphoid cell, (b) CD56 positivity in lymphoid cell, (c) CD3 positivity in epidermotropic lymphoid cell, (d) Ki67-positive cell

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Computed tomography (CT) scan of the lower lip showed heterogeneously enhancing soft-tissue lesion [Figure 4]a; CT scan of the chest and abdomen showed Multiple heterogeneously enhancing, soft-tissue density (nodular) lesions over the anterior chest wall [Figure 4]b, anterior abdominal wall [Figure 4]c, largest 40 mm × 16 mm. Multiple, bilateral axillary lymph nodes, largest 12 mm × 10 mm right, and 14 mm × 8 mm left; few small nodes in bilateral level IA and level IB; and well-defined sclerotic lesions in bilateraliliac bones [Figure 4]d.
Figure 4: (a) Heterogeneously enhancing soft-tissue lesion over the lip, (b) M/L heterogeneously enhancing, soft-tissue density (nodular) lesions over the anterior chest wall, (c) M/L heterogeneously enhancing, soft-tissue density (nodular) lesions over the abdomen, (d) Well- defined sclerotic lesions in B/L iliac bones

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Bone marrow biopsy showed normocellular marrow (Myeloid : Erythroid ratio=3.5 : 1) uninvolved by primary/secondary malignancy. No evidence of atypical cells or granuloma.

Since immunohistochemistry showed CD2+, CD3+, CD56+, CD4−, CD8−, CD20−, and CD30−, 90% of the cells showed MIB1 positivity, and on the basis of biopsy findings, he was diagnosed as high-grade (CD2+, CD3+, CD4−, CD8−) peripheral T-cell non-Hodgkin's lymphoma.


   Discussion Top


About 10%–15% of non-Hodgkin's lymphomas have been diagnosed as peripheral T-cell lymphoma.[1] The most common presentations of CTCL are mycosis fungoides (MF) or sezary syndrome (SS), both show CD4 surface positivity. A subset of CTCL which lacks CD4 surface marker is classified as CGD-TCL. Occasionally, CGD-TCL can show epidermotropism which is a prominent feature of MF/SS.[2] Clinically aggressive CTCL is classified as below:[3]

  1. Sezary syndrome
  2. Adult T-cell leukemia/lymphoma
  3. Extranodal NK/T-cell lymphoma, nasal type
  4. Primary cutaneous peripheral T-cell lymphoma, unspecified
  5. Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma (provisional)
  6. CGD-positive TCL (provisional).


Clinically, CGD-TCL presents with sudden appearance of multiple cutaneous plaques, nodules, and tumors, which have tendency to ulcerate, without any preceding patches or plaques. Although mucosal and extranodal involvement is common, it can involve subcutaneous tissue also.[2]

The distinct histological presentation of CGD-TCL has been in literature; epidermotropic, dermal, and subcutaneous and more than one of these three can be present in the same patient within the same biopsy specimen or in different biopsy specimens.[4],[5] These tumor cells show surface marker for CD2, CD3, and CD56 but negative CD5, CD4, and CD8.

To our best knowledge, only forty cases of primary CGD-TCL have been reported till date.[6]

Because of its aggressive nature and resistance to multidrug chemotherapy and radiation therapy, median survival rate of only 15 months has been reported in one study.[5]

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Jaffe ES, Krenacs L, Raffeld M. Classification of T-cell and NK-cell neoplasms based on the REAL classification. Ann Oncol 1997;8 Suppl 2:17-24.  Back to cited text no. 1
    
2.
Berti E, Cerri A, Cavicchini S, Delia D, Soligo D, Alessi E, et al. Primary cutaneous gamma/delta T-cell lymphoma presenting as disseminated pagetoid reticulosis. J Invest Dermatol 1991;96:718-23.  Back to cited text no. 2
    
3.
Willemze R, Jaffe ES, Burg G, Cerroni L, Berti E, Swerdlow SH, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005;105:3768-85.  Back to cited text no. 3
    
4.
Burg G, Dummer R, Wilhelm M, Nestle F, Ott MM, Feller A, et al. A subcutaneous delta-positive T-cell lymphoma that produces interferon gamma. N Engl J Med 1991;325:1078-81.  Back to cited text no. 4
    
5.
Toro JR, Liewehr DJ, Pabby N, Sorbara L, Raffeld M, Steinberg SM, et al. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood 2003;101:3407-12.  Back to cited text no. 5
    
6.
Koch R, Jaffe ES, Mensing C, Zeis M, Schmitz N, Sander CA. Cutaneous gamma/delta T-cell lymphoma. J Dtsch Dermatol Ges 2009;7:1065-7.  Back to cited text no. 6
    

What is new?
When CTCL is suspected in the patients, biopsy with immunohistochemistry holds a very important place. It helps in the classification of the lymphoma and early identification of the clinically aggressive CTCL. Patient can thus be explained the prognosis and management with multidrug chemotherapy and radiation can be started at the earliest, to prolong the survival.


    Figures

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



 

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