Thread by @TristanRutland7, Female, adult, leg. @ZHOUSEH @MattieFarzin @rennmont @DrJPLow @JMGardnerMD @DrGeeONE @Kashifhazim @Divya

Female, adult, leg.

@ZHOUSEH @MattieFarzin @rennmont @DrJPLow @JMGardnerMD @DrGeeONE @Kashifhazim @Divya_Madhala @Meghna0630 @ariella8 @pembeoltulu @MAHoureih @ChristineJKoMD @rdpatho

#dermpath #pathtwitter #pathology

CD20

CD3

CD30

ALK

CD7

CD4

CD8

CD2 CD5

Patient had other lesions on body (mainly trunk).

Patient had Hx of mycosis fungoides.

CD4 on other lesion.

Diagnosis:

Large cell transformation of mycosis fungoides (MF).

#Hemepath

This was a colleague’s case.

1/ Mycosis fungoides (MF) is a cutaneous T-cell lymphoma and has a range of stages:

Patch & plaque stage

indolent

Tumour stage

worse prognosis

2/ It usually expresses mature T cell phenotype CD4 (but v rare CD8 cases can occur).
https://pubmed.ncbi.nlm.nih.gov/10071312

Prognosis in cutaneous T-cell lymphoma by skin stage: long-term survival in 489 patients - PubMed

The relative survival of CTCL patients worsens with increasing skin stage, although stages T3 and T4 had closely similar survivals. The great majority of patients with CTCL do not die of their...

https://pubmed.ncbi.nlm.nih.gov/10071312

2b/ In regards to staging, I have attached an excellent article below.

https://ashpublications.org/blood/article/110/6/1713/24087/Revisions-to-the-staging-and-classification-of

3/ Large cell transformation is defined by greater than 25% large cells (4X size normal lymphocytes) or nodular aggregates in dermis.

4/ It usually arises from tumour stage (rare cases arising from patch stage have occurred).
https://pubmed.ncbi.nlm.nih.gov/10733487/

Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases....

The course of mycosis fungoides (MF) is indolent except when transformation to a large T-cell lymphoma occurs. The diagnosis of transformed MF (T-MF) relies on the presence of more than 25% of large...

https://pubmed.ncbi.nlm.nih.gov/10733487/

5/ CD30 is NOT a marker for transformation (can be found in patch stage), but might provide a target from treatment.
https://pubmed.ncbi.nlm.nih.gov/21047225/

Brentuximab vedotin (SGN-35) for relapsed CD30-positive lymphomas - PubMed

Brentuximab vedotin induced durable objective responses and resulted in tumor regression for most patients with relapsed or refractory CD30-positive lymphomas in this phase 1 study. Treatment was...

https://pubmed.ncbi.nlm.nih.gov/21047225/

6/ Differential diagnosis that need to be considered:

Primary cutaneous anaplastic large cell lymphoma (PCALCL)

Lymphomatoid papulosis (LyP)

Hx is absolutely essential in these situations (ie previous MF).
(?GATA3 may be helpful sometimes?).
https://pubmed.ncbi.nlm.nih.gov/25440432/

Expression of helper T cell master regulators in inflammatory dermatoses and primary cutaneous...

A predominance of T-bet(+) T cells in the epidermis support patch stage MF over dermatitis. A predominance of GATA-3(+) T cells in the dermis support CD30(+) MF with large cell transformation over...

https://pubmed.ncbi.nlm.nih.gov/25440432/

7/ Lastly, 25% of MF can be associated LyP….
https://pubmed.ncbi.nlm.nih.gov/14512906/

Lymphomatoid papulosis associated with mycosis fungoides: a study of 21 patients including analyses...

Findings of this study strengthen the idea that LyP and MF are related T-cell lymphoproliferative disorders.

https://pubmed.ncbi.nlm.nih.gov/14512906/

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