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T lymphoproliferation vs Langerhans' cell histiocytosis (1362)
T lymphoproliferation vs Langerhans' cell histiocytosisnew
Subtitle: DERMA
Type:
BIOPSY
Sender:
ugnius
2005-11-19 18:33
INCTR - EBMWG Hematopathology Online
27 yrs old woman presents with multiple confluent painless nodules in skin (breast, back, trunck, buttocks). Excisional biopsy was performed (consultation case).  
HISTO: Irregular confluent nodular perivascular and periadnexal dermal infiltrate consist of small- medium lymphocytes with irregular, wrincled, cleaved nuclei (T) and admixture of histyocytes and eosinophils. The epidermothropism, adnexothropism are absent (single CD3+ cells in epidermis). Mitoses and atypical forms are present. Exulceration is present. The infiltrate is irregular wedge shaped with basis in upper part.  
IMMUNOHISTO (in progress): CD3+ infiltrate with mixture CD4+, CD8+ cells (ratio CD4/CD8 about 3-4/1), CD68+ macrophages. There are some CD56+ cells or CD30+ interstitial large cells. The multinucleated HL like cells are absent.  
PROPOSED DIAGNOSIS 1: Cutaneous T pseudolymphoma (drugs? insect bites?) >>> cutaneous T lymphoma?.  
PROPOSED DIAGNOSIS AFTER IH 2: Langerhans' cell histiocytosis?  
Thank you for comments in advance.  
FINAL DRAFT DIAGNOSIS: T pseudolymphoma with reactive LCH-like lession?  
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hurwitz
2005-11-28 20:22
There is a very heavy lymphoid infiltrate in the dermis. It is sharply demarkated, adnexal structures are well preserved. There is some atypia, but in general the cells are rather uniform. There is a muxture of CD4+ and CD8+ cells, as well as a few CD30+ activated cells.  
I would strongly favour the diagnosis of a reactive lesion, drugs?, insect bite?. I do not think that this is a neoplastic lesion. Could you try to obtain more clinical information regarding drugs, or insect bites?
ugnius
2005-11-29 08:04
Dear Prof.N.Hurwitz, there is any evindence of tick bites and drugs. The eruption was treates with topicals (steroids etc.) with no effect. Any signs of lymphadenopathy, B symptoms are present. The lessions are multiple.
hurwitz
2005-11-29 09:39
This is very interesting. I will ask others for their opinion. Probably it will be necessary to do PCR for TCR rearrangement. But lets wait for additional opinions.
ugnius
2005-11-29 10:11
Additional question: in case like this with a huge and deep infiltrate with T phenotype what definite criteria you prefer to differential of T lymphoma and pseudo T? Thanx.
ugnius
2005-11-29 10:12
And what to do with some clear and pleomorphic cells with mitotic activity?
ugnius
2005-11-30 12:29
I've got information about patient: she presents with larger nodules in skin and comes to my city hospital. Diagnosis I hope will be elludicated, maybe we've get a node.  
Tick bites, drugs are excluded. So maybe we ca formulate this diagnosis of the T infiltrate like suspitious only?  
I CANNOT prove TCR monoclonality.
hurwitz
2005-11-30 14:06
I discussed this case with Prof.Bettina Borisch, last night. The summary of our discussion is that it is difficult to exclude a T-cell lymphoma in this case. Bettina Borisch suggested to perform several biopsies of the skin lesions (2-3)and see if they are clonal, and if there is one or more clones in the lesions. I think this is a brilliant idea. Since this is a young patient the situation has to be clarified soon in order to treat her correctly.
ugnius
2005-11-30 14:12
Thank you. But it's impossible to do PCR for TCR in my Centre.
ugnius
2005-12-01 11:51
NEW DATA: Additional stains for CD1a and S100 reveal PROMINENT nodular and difusse network of Langerhans cells. It seems I've overlook it at first. Infiltrate looks a little bluish and clear in HE. And there are some cells with definitelly wrincled or reniform nuclei.  
Maybe it's pushing us to formulate Lagerhans cell histicytosis diagnosis one more time (prev case)?  
It's strange, that the infiltrate is so deep and practically eithout Eo's.  
I've waiting upcomming comments...
dirnhofer
2005-12-02 15:44
to me, this is well compatible with lch and in all likelyhood a reactive lmphoid infiltrate.  
 
nonetheless, to rule out lch-associated t-cell lymphoma you can send me the block for clonality analysis if you woul like to.
kunze
2005-12-02 20:15
It's a teaching case for the DD. of reactive lymphocytic skin infiltrates. I admit that I didn't think of LHC. Seeing the additional stains for CD1a and S100 the scales fell from my eyes.
Went
2005-12-06 09:21
To demonstrate an T-NHL, an aberrant phenotype with loss of CD7 (or CD5) can be helpful at times. In this case, without epidermotropism of T-cells, I would also favour LCH.
ugnius
2005-12-06 09:34
Thank you all for comments and support in this a little bit "uncomfortable" case.
hurwitz
2005-12-11 19:45
For the sake of the young lady the results of molecular studies are urgently needed.  
Did you accept Dr.Dirnhofer's offer to do molecular studies on this case?
ugnius
2006-01-09 07:30
The case was sent to dr.S.Dirnhofer who kindly accept it for external consultation. Thank you ofr help.
ugnius
2006-01-12 10:41
Some news: Hum Pathol (2006) 37, 32-39 article "Lesions resembling LCH..." by L.J.Christie et all.  
Maybe this case fit to category "Reactive LCH-like lession accompaning T pseudolymphoma"?  
The single hope that Dr.S.Dirnhofer will try to answer with T monoclonality evaluation. The truth is somewhere beyond...
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Last modified: 2005-11-19 18:33:40