New term request: gamma-delta T-cell lymphoma

[mbernste]

Hi, I would like to request a term for 'gamma-delta T-cell lymphoma'. See this paper: https://www.nature.com/articles/nrclinonc.2009.169

"γδ TCLs originate from γδ T cells, a small subset of peripheral T cells with direct antigen recognition capability acting at the interface between innate and adaptive immunity."

Matt

[lschriml]

Thank you Matt, I will take a look and get this added to DO. Cheers, Lynn

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On Apr 18, 2018, at 12:52 PM, Matthew Bernstein notifications@github.com wrote:

Hi, I would like to request a term for 'gamma-delta T-cell lymphoma'. See this paper: https://www.nature.com/articles/nrclinonc.2009.169

"γδ TCLs originate from γδ T cells, a small subset of peripheral T cells with direct antigen recognition capability acting at the interface between innate and adaptive immunity."

Matt

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[lschriml]

The requested term has been added to the DO. primary cutaneous gamma-delta T-cell lymphoma

[rare T-cell lymphomas]

 hepatosplenic T-cell lymphoma (HSTL). (HSTCL)
              NCI:C8459
              --> [definition]:  An extranodal, mature T-cell non-Hodgkin lymphoma that originates from cytotoxic T-cells, usually of gamma/delta T-cell type. It is characterized by the presence of medium-size neoplastic lymphocytes infiltrating the hepatic sinusoids. A similar infiltrating pattern is also present in the spleen and bone marrow that are usually involved at the time of the diagnosis.

https://www.ncbi.nlm.nih.gov/labs/pmc/articles/PMC7596851/

 --> add as a child of : DOID:0050743  mature T-cell and NK-cell lymphoma

============================================= [DOID:0060061] cutaneous T cell lymphoma --> update disease name: Primary Cutaneous T-Cell Non-Hodgkin Lymphoma, PCTCL --> add NCI:C3467.

[new child term]

 **primary cutaneous gamma-delta T-cell lymphoma** (PCGD-TCL)    [NCI:C45340]
      [synonyms] 
            (cutaneous gamma/delta T-cell lymphoma).  (GD-TCL)
                        https://www.ncbi.nlm.nih.gov/labs/pmc/articles/PMC3471805/
            (Primary Cutaneous Gamma-Delta T Cell Lymphomas). (PCGDTCLs)
                         https://www.ncbi.nlm.nih.gov/labs/pmc/articles/PMC8628721/

      [definition]
--> characterised by the clonal cutaneous proliferation of activated mature gamma-delta T cells with a cytotoxic phenotype.

HSTL is a well-characterized extranodal lymphoma that has a disguised onset, secondary to intrasinusoidal infiltration of the spleen, liver and bone marrow, has a rapidly progressive course that is poorly responsive to chemotherapy, and often ensues in the setting of immune system suppression.

PCGD-TCL can present with prominent epidermal involvement or with a panniculitis-like clinical picture that can be complicated by a concurrent hemophagocytic syndrome;

Cutaneous T-cell lymphomas (CTCLs) are a group of disorders characterized by an abnormal accumulation of cancerous T-cells (a type of white blood cells) in the skin resulting in an itchy, red rash that can thicken or form a tumor