T/natural killer–cell lymphomas

Karan, Kalpita R; Waguespack-LaBiche, Jennifer; Rapini, Ronald P.; Jordon, Robert E.; Raimer, S.; Tyring, Stephen K.

doi:10.1016/j.jaad.2004.12.010

Cited by 3 publications

(7 citation statements)

References 11 publications

(7 reference statements)

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“…More recently, it has been established that the cell of origin is a plasmacytoid/dendritic cells, conesquently the term "blastic NK-like T-cell lymphomas has been supplanted by the term "CD4+ CD56+ hematodermic neoplasm" in the WHO/EORTC classification but is now termed blastic plasmacytoid dendritic cell neoplasm(BPDCN) [2,3]. Blastic NK-like T-cell lymphoma can arise at any age including childhood, but it tends to present in middle aged or elderly patients with a median age of 52 years [3][4][5]. It affects predominantly man than woman with a sex ratio 3:1 [5], there is no racial predilection [6].…”

Section: Discussionmentioning

confidence: 99%

“…Cutaneous lesions may be solitary or multiple nodules or tumors, or have petechial appearance witch tend to be generalized [3,[7][8][9]. Skin biopsy reveals a dense dermal infiltration by medium malignant cells without epidermal involvement with focal angiocentric accentuation and necrosis [2,4,7]. Immunophenotypically, blastic NK-cell lymphoma is typically CD45+, CD2+, surface CD3−, cytoplasmic CD3+, CD4+, CD56+, CD5−, CD7+/−, TdT+/−TCR genes are in germline configuration [2,7].…”

Section: Discussionmentioning

confidence: 99%

“…Immunophenotypically, blastic NK-cell lymphoma is typically CD45+, CD2+, surface CD3−, cytoplasmic CD3+, CD4+, CD56+, CD5−, CD7+/−, TdT+/−TCR genes are in germline configuration [2,7]. The absence of positivity for myeloperoxidase, CD13 and CD33 in bone marrow cells excluded the diagnosis of CD56+ myeloid leukemia [4,10]. In contrast with aggressive NK-cell leukemia and extranodal NK/T-cell lymphomas, EBV DNA in tumor cells is negative, so the possibility of extranodal NK/ T-cell lymphoma nasal type is excluded and the positivity of EBV might serve as a key point for differential diagnosis from mature NK cell malignancies [8,9].…”

Section: Discussionmentioning

confidence: 99%

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CD4+/CD56+ Hematodermic Neoplasm Presenting in the Skin: A Tunisian Case Report and Current Review of the Literature

Youssef¹,

Sayed²,

Zahra³

et al. 2012

OJBD

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The CD4+/CD56+ hematodermic neoplasm is a rare aggressive systemic neoplasm for which effective therapies have not yet been established, it is clinically characterized by cutaneous involvement with spread to bone marrow, blood and poor prognosis with current chemotherapy regimens. Our objective is to report diagnosis and treatment difficulties of CD4+/CD56+ hematodermic neoplasm. We describe here a Tunisian man who presented with subcutaneous ulcerated lesion localized in the right leg and multiples generalized nodules. Skin biopsy showed an atypical lymphoid cell infiltration with an angiocentric pattern and extensive necrosis by immuno-histochemical analysis, these cells were positive for CD4, CD56, granzyme B and negative for CD8, CD123, CD20 and CD30. T-cell rearrangement and Epstein-Barr-virus (EBV) in situ hybridation studies were negative. The patient underwent 5 cycles chemotherapy SMILE regimen monthly sandwiched with radiotherapy on the residual lesions of the right leg with great tolerance but he relapsed within 8months with skin, blood, bone marrow, lung, and cerebrospinal involvement. Based on these findings, the patient was diagnosed with CD4+/CD56+ hematodermic neoplasm (blastic NK-like T-cell lymphoma) treated with one course of hyper-CVAD regimen, he died within 20 days with a septic chok. Despite the use of L-Asparaginase and radiotherapy the prognosis is very poor; we suggest the exploration for highly active drugs, hematopoietic stem cell transplantation (HSCT) is crucial to improve survival.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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CD4+/CD56+ Hematodermic Neoplasm Presenting in the Skin: A Tunisian Case Report and Current Review of the Literature

Youssef¹,

Sayed²,

Zahra³

et al. 2012

OJBD

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“…There is generally no necrosis or angioinvasion, and inflammatory cells are absent. 5,7,8 The tumour cells usually have a CD4+, CD56+, CD8 − , CD7 − /+, CD2 − /+, CD45RA+ phenotype, but do not express surface and cytoplasmatic CD3 or cytotoxic proteins. TdT and CD68 may be positive.…”

Section: Discussionmentioning

confidence: 99%

“…10 T-cell receptor genes are in a germline configuration 5 and oncogenic transformation is not associated with EBV. 2,7 CD4+/CD56+ haematodermic neoplasm has an aggressive course and has proved fatal, with rapid development of disseminated disease in the majority of patients. The median survival is 14 months, and no significant differences have been found between patients presenting with skin lesions with or without concurrent extracutaneous disease.…”

Section: Discussionmentioning

confidence: 99%

CD4+/CD56+ haematodermic neoplasm: a preculsor haematological neoplasm that frequently first presents in the skin

Martín

Nicolau

Galán

et al. 2006

Acad Dermatol Venereol

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CD4+/CD56+ hematodermic neoplasm, formerly known as blastic NK cell lymphoma, is an aggressive and rare preculsor hematologic neoplasm recently recognized by the WHO-EORTC classification consensus for cutaneous lymphomas. The neoplasm tends to affect elderly patients, who usually present with skin lesions but often have a disseminated disease, including bone marrow involvement. Although the lesions are composed of cells with a lymphoblast-like morphology and an NK-cell phenotype, exhibiting a CD4+, CD56+ positive immunophenotype, recent studies support a relationship to plasmacytoid dendritic cells. Because of the rarity of this disease, we describe two patients suffering a CD4+/CD56+ hematodermic neoplasm.

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An atypical presentation of a blastic plasmacytoid dendritic cell tumors

López¹,

Martí²,

Ferrández

et al. 2010

J Cutan Pathol

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Blastic plasmacytoid dendritic cell tumor is a rare lymphoma, which has been included as an independent entity in the World Health Organization (WHO) 2008 classification for cutaneous lymphomas. This neoplasm usually affects middle-aged or elderly patients with predominant skin or soft tissues involvement. Although it usually has a good initial response to chemotherapy, relapses are the rule and they occur rapidly. We report a new case of an 81-year-old woman with a blastic plasmacytoid dendritic cell tumors with an unusual presentation.

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T/natural killer–cell lymphomas

Cited by 3 publications

References 11 publications

CD4+/CD56+ Hematodermic Neoplasm Presenting in the Skin: A Tunisian Case Report and Current Review of the Literature

CD4+/CD56+ Hematodermic Neoplasm Presenting in the Skin: A Tunisian Case Report and Current Review of the Literature

CD4+/CD56+ haematodermic neoplasm: a preculsor haematological neoplasm that frequently first presents in the skin

An atypical presentation of a blastic plasmacytoid dendritic cell tumors

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