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Mycosis Fungoides, tumor stage (5618)
Mycosis Fungoides, tumor stageclosed
Subtitle: B07-8957, B07-14111, B08-10187
Type:
skin
Sender:
ugnius
2008-05-05 17:48
INCTR - EBMWG Hematopathology Online
65 yrs female with clinical suspition of mycosis fungoides.  
HISTORY: 2005 06: multiple erythematous confluent eruption in the skin of truncus, back, face, neck, hands and legs. Biopsy from from BREAST skin. Histo: spongiotic dermatitis. Clininal: parapsoriasis. Photodynamic therapy and topicals. Regression of symptoms. NO PHOTOS ARE INCLUDED.  
2006 08: The same eruption from 2006 02. Biopsy from BACK skin. Histo: chronic contact dermatitis. Clininal: parapsoriasis. Follow up (mycosis suspition). Photodynamic therapy and topicals. Regression of symptoms. NO PHOTOS ARE INCLUDED.  
2007 03: Biopsy of the face skin: T lymphoma, most probable Mycosis fungoides (BIOPSY 1- IN PHOTOS).  
2007 04: Tumor in the cheek skin: 9x6cm. Biopsy (BIOPSY 2- IN PHOTOS). Histo: Primary ALCL diagnosed. Radiotherapy to residual tumor. Interferon therapy.  
2008 04: Rebiopsy (BIOPSY 3- IN PHOTOS) from the HAND skin. Extracorporal phoresis and Alemtuzumab- without efect. Now: Intereferon and Acitretin. NO EFFECT.  
Threpine biopsy in 2007 was normal.  
HISTO: 1. Small- medium sized lymphocytic nodular infiltrate in dermis with epidermothropism. 2. More intensive infiltrate with more prominent syringothropism. 3. More deep, more pleomorphic subcutaneous infiltrate and the rest of CD4+ superficial infiltrate (esp in CD4 stain).  
IH: 1. CD3+; CD4+ 10%; GranzymB+ 10%; CD30+ <5%; CD56-.  
IH 2: CD3+; CD2+; CD4+; CD30+ 40%; CD56-; Ki67 90%; GranzymB+ 30%.  
IH 3: CD4+; CD30-; GranzymB+ 5%.  
Proposal/question: Definite nosology of T-NHL: Mycosis fungoides (CD4+ GranB+ CD30+) (tumor stage) in transformation?  
 
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tzankov
2008-05-07 07:57
considering the images and the information given, MF tumor stage with large cell transformation (biopsy 2) would be the most proper designation of what we are facing. would you please specify on which biopsy did you perfom the Ki-67 staining? were the biopsies taken form different sites? were there CD30+ blasts in the 3rd biopsy?
ugnius
2008-05-07 08:35
Appologies: Ki67 from largest infiltrate (biopsy 2). Cd30+ was absent in Biopsy 3 (it seems like postterapy regression a little).
hurwitz
2008-05-09 18:03
I agree with the diagnosis of mycosis fungoides, tumor stage in transformation. The expression of cytotoxic granules is seen in advanced disease.  
The expression of CD30, seems to be linked to a better prognosis  
{PMID: 17573879}
hurwitz
2008-05-11 16:09
Dear Ugnius, I am well aware of your difficulties in obtaining clinical information, but sometimes it helps to insist.  
It is important to be able to include clinical findings into diagnostic considerations.  
 
schulze
2008-05-13 11:18
I fully agree with the diagnosis of specific skin infiltration of systemic epidermotropic T cell lymphoma, that clinically fits well to tumor stage of mycosis fungoides.  
However, secondary CD30-expression (well documented in second biopsy in comparison to first biopsy) in combination with increase in lymphoblastic cells, high proliferative activity and expression of GranB-cytotoxic granules (in biopsy 2) as well as partial loss of epidermotropism and CD4-expression (compare biopsy 3 with biopsy 1) speek for transient transformation, i.e. progress with poorer prognosis than expected in primary CD30-positive mycosis fungoides.
ugnius
2008-05-13 16:33
I fully accept the Prof. Nina critics about clinical data. Please find excerpt from clinical records. Long history and the large tumor in the cheek in the context of diffuse erythematous eruptions.
ugnius
2008-05-13 16:43
P.S. The full data will be appended from hemato dept. I'm appologize once more for inconvenience.
ugnius
2008-05-13 18:31
All done are placed in.
hurwitz
2008-05-15 22:00
Thanks Ugnius for the clinical data.  
 
Please regard Dr.Schulzes comment as the final diagnosis
ugnius
2008-05-16 09:02
Thank you for support.
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Last modified: 2008-05-05 17:48:04