Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma γ/δ Subtype (Cutaneous γ/δ T-Cell Lymphoma): Report of a Case and Review of the Literature

Kao, Grace F.; Resh, Brooke; McMahon, Christine M.; Gojo, Ivana; Sun, Chen; Phillips, Daniel; Zhao, Xianfeng

doi:10.1097/dad.0b013e318182c7bf

Cited by 22 publications

(17 citation statements)

References 26 publications

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“…1 In contrast to the majority of T-cell lymphoma that express the ␣␤ TCR, 2,3 human ␥␦ T-cell lymphoma is a rare clinicopathologic entity with an aggressive course and poor prognosis. [4][5][6][7][8][9][10] Patients usually have splenomegaly, hepatomegaly, and/or cutaneous lesions and, paradoxically, this lymphoma often accompanies autoimmune disease. 4,6,7 The etiology and the genes that control the development of ␥␦ T-cell neoplastic cells are unknown.…”

Section: Introductionmentioning

confidence: 99%

Mutation of inhibitory helix-loop-helix protein Id3 causes γδ T-cell lymphoma in mice

Maruyama

Zhang

et al. 2010

Blood

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Human ␥␦ T-cell lymphoma is a rare clinicopathologic entity with aggressive course and poor prognosis. The etiology and pathogenesis of ␥␦ T-cell lymphoma is unknown. We show here that mice with deficiency in inhibitory helix-loop-helix protein Id3 (Id3 ؊/؊ ) developed ␥␦ T-cell lymphoma that resembled human ␥␦ T-cell lymphoma. The Id3 ؊/؊ mice with lymphoma showed splenomegaly, hepatomegaly, and lymphadenopathy with involvement of bone marrow, thymus, kidney, and lungs between 6 and 15 months of age. Phenotypic analysis revealed that lymphomatous cells were cluster of differentiation (CD)3 ؉ , ␥␦ T-cell receptor (TCR) ؉ , and ␣␤ TCR ؊ , and expressed CD8 ؉ CD4 ؊ , CD4 ؉ CD8 ؊ , or a mixture of the two. Id3 ؊/؊ ␥␦ T-cell lymphoma used predominantly V␥1.1, some V␥3, yet no V␥2 TCR, and some showed increased levels of the oncogene c-Myc. Strikingly, adoptive transfer of the ␥␦ T-cell lymphoma into syngeneic Rag1 ؊/؊ mice resulted in aggressive ␥␦ T-cell lymphoma, identical to the Id3 ؊/؊ donor. Thus, our data demonstrate that Id3 regulates the development of ␥␦ T-cell lymphoma in mice, raising a possibility of Id3 gene mutation in human ␥␦ T-cell lymphoma. Our model will provide a tool for studying the molecular mechanisms and development of human ␥␦ T-cell lymphoma. IntroductionHuman ␥␦ T-cell lymphoma represents a subgroup of peripheral T-cell lymphoma expressing ␥␦ T-cell receptors (TCRs). 1 Hepatosplenic T-cell lymphoma (HSTCL) is the prototype peripheral T-cell lymphoma expressing the ␥␦ TCR, although non-HSTCL ␥␦ T-cell lymphoma also exist. 1 In contrast to the majority of T-cell lymphoma that express the ␣␤ TCR, 2,3 human ␥␦ T-cell lymphoma is a rare clinicopathologic entity with an aggressive course and poor prognosis. [4][5][6][7][8][9][10] Patients usually have splenomegaly, hepatomegaly, and/or cutaneous lesions and, paradoxically, this lymphoma often accompanies autoimmune disease. 4,6,7 The etiology and the genes that control the development of ␥␦ T-cell neoplastic cells are unknown. In addition, there is a lack of animal models that resemble human ␥␦ T-cell lymphoma.The helix-loop-helix (HLH) proteins are a family of transcriptional regulatory proteins comprising 2 major subclasses; the basic and inhibitory HLH proteins. [11][12][13] The basic HLH proteins include the mammalian E2A, E2-2, and Hela E-box-binding (HEB) proteins and the Drosophila gene product Daughterless. It has been reported that E2A inactivation in mice leads to the rapid development of a T-cell lymphoma expressing no or low levels of surface TCRs, and cluster of differentiation (CD)4 and CD8. 14 The lymphoma are monoclonal and highly malignant and displayed a cell surface phenotype similar to that of immature thymocytes. 15,16 The inhibitory HLH proteins include 4 mammalian members Id1, Id2, Id3, and Id4, 17-21 which function as inhibitors of the basic HLH proteins, preventing their binding to target DNA. Id3 has been shown to be involved in thymocyte maturation 20 ; playing a role in the restriction of the ␥␦ lineage development ...

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Section: Introductionmentioning

confidence: 99%

Mutation of inhibitory helix-loop-helix protein Id3 causes γδ T-cell lymphoma in mice

Maruyama

Zhang

et al. 2010

Blood

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“…Since 2008, CGD-TCL has been included in the WHO classification as a rare sub-type of primary cutaneous peripheral T-cell lymphoma3. Lesions of this sub-type resemble those of panniculitis; therefore, it was previously known as the γ/δ sub-type of subcutaneous panniculitis-like T-cell lymphoma (SPTCL)4,5. Based on cell surface receptors on neoplastic T-cells, SPTCL was further subdivided into a α/β sub-type and a γ/δ sub-type2,5.…”

Section: Discussionmentioning

confidence: 99%

“…Lesions of this sub-type resemble those of panniculitis; therefore, it was previously known as the γ/δ sub-type of subcutaneous panniculitis-like T-cell lymphoma (SPTCL)4,5. Based on cell surface receptors on neoplastic T-cells, SPTCL was further subdivided into a α/β sub-type and a γ/δ sub-type2,5. Histopathologic findings, immunohistochemical findings, and prognosis between these 2 sub-types showed considerable difference6.…”

Section: Discussionmentioning

confidence: 99%

Fatal Cutaneous γ/δ T-Cell Lymphoma with Central Nerve System Metastasis

et al. 2011

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Cutaneous γ/δ T-cell lymphoma is an extremely rare and highly aggressive tumor that is often resistant to treatment, such as polychemotherapy and radiotherapy. Due to the complexity of clinical, pathologic, and immunohistochemical features of this disease entity, the physician should perform a careful evaluation; however, treatment should be rapid and aggressive. We present a case of fatal cutaneous γ/δ T-cell lymphoma of a 55-year old woman who died after recurrence with central nerve system metastasis.

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“…A variety of diseases, especially dermatological and rheumatic conditions, may have an appearance and presentation similar to SPTL. These include conditions such as septal panniculitis (erythema nodosum), lobular panniculitides (erythema induratum, nodular vasculitis, and lupus panniculitis), lobular and septal panniculitis (alpha-1 antitrypsin deficiency panniculitis), infectious panniculitis, pyoderma gangrenosum, systemic vasculitis and inflammatory myopathies [2][3][4][5]. Histopathologic examination shows subcutaneous infiltrates containing a mixture of neoplastic pleomorphic cells of varying sizes and macrophages and rimming of individual fat cells by neoplastic cells are features of SPTL (Fig.…”

Section: Discussionmentioning

confidence: 99%

Fatal Case of Subcutaneous Panniculitis T-Cell Lymphoma Mimicking Polyarteritis Nodosa: A Case Report and Review of Literature

Sehgal¹,

Yachoui²,

Cutlan³

2016

J Hematol

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Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare subtype of cutaneous T-cell lymphoma. These cases can remain undiagnosed and are often treated with prolonged and ineffective immunosuppressive agents before a diagnosis of T-cell lymphoma is made. A 76-year-old white male presented with a 3-month history of fatigue, weight loss, night sweats, low grade fevers, gradual cognitive decline, and new onset skin lesions. Skin examination revealed mildly tender, indurated, dull red subcutaneous nodules on face, chest, back, upper, and lower extremities, most numerous on the chest and back. A punch biopsy from one of the skin nodules revealed small and medium vessel vasculitis with associated neutrophilic lobular panniculitis. A full thickness skin biopsy showed inflammatory infiltrate composed predominantly of atypical small to medium sized lymphocytes with hyperchromatic nuclei. A diagnosis of T-cell lymphoma was made on clinical presentation, histopathology, immunohistochemical and molecular studies. SPTL can mimic autoimmune diseases including primary vasculitis disorder in presentation. A superficial skin biopsy may be sufficient in diagnosing SPTL; however, presence of vasculitis on a superficial punch biopsy specimen should be followed by deep incisional biopsy (sometimes multiple) in all cases with atypical presentation or those who do not respond appropriately to treatment.

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Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma γ/δ Subtype (Cutaneous γ/δ T-Cell Lymphoma): Report of a Case and Review of the Literature

Cited by 22 publications

References 26 publications

Mutation of inhibitory helix-loop-helix protein Id3 causes γδ T-cell lymphoma in mice

Mutation of inhibitory helix-loop-helix protein Id3 causes γδ T-cell lymphoma in mice

Fatal Cutaneous γ/δ T-Cell Lymphoma with Central Nerve System Metastasis

Fatal Case of Subcutaneous Panniculitis T-Cell Lymphoma Mimicking Polyarteritis Nodosa: A Case Report and Review of Literature

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