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Mycosis Fungoides/Sézary syndrome (MF/SS)

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Definition

Sézary syndrome (SS) and Mycosis Fungoides (MF) are T-cell lymphomas whose primary manifestation is in the skin.  Mycosis Fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time.The name mycosis fungoides is somewhat misleading--it loosely means "mushroom-like fungal disease". The disease, however, is not fungal infection but rather a type of non-Hodgkin's lymphoma. It was so named because the skin tumors of a severe case as having a mushroom-like appearance.

 

Sézary's disease (or "Sézary syndrome") is a type of cutaneous lymphoma characterized by Albert Sézary."Sézary's cells" are T-cells that have pathological quantities of mucopolysaccharides. Sézary's disease is sometimes considered a late stage of mycosis fungoides

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Case Name (click to open) Comments Size
Case 28 Mycosis Fungoides / Seazary Syndrome 702 kB
 
Epidemiology

It is rare for the disease to appear before age 20, and it appears to be noticeably more common in males than females, especially over the age of 50, where the incidence of the disease (the risk per person in the population) does increase. The average age of onset is between 45 and 55 years of age for patients with patch and plaque disease only, but is over 60 for patients who present with tumours, erythroderma (red skin) or a leukemic form (the Sézary syndrome).

Possible causes

HTLV has thought to have been associated with both of the above cutaneous mycoses.  

Morphology

The malignant cells are small to medium in size  with irregular (cerebriform) nuclei.  a minority of larger cells may be present (classical Sézary cells) but only in minority. (see below)

                 

 ./slide015 sezary1.jpg
 ./slide015 sezary2.jpg
 Example morphology of MF/SS
Immunophenotyping

The demonstration of an elevated CD4/CD8 ratio, and increased proportions of CD4+, CD7- T-lymphocytes.  Tumor cells express CD2, CD3, TCRb, CD5 and CD7 (-/+). Most cases are CD4+, rarely is CD8 expressed. Aberrant T-cell phenotypes are common.  

       

  ssmf45ss.jpg
Seazary cells are CD45 bright and low SS, identical to normal lymphocytes.  


 

 ssmf195.jpg  ssmf37.jpg
 ssmf34.jpg  ssmf857.jpg
   The lymphocytes are CD5+, CD19-, CD3+ and CD4+    The lymphocytes express CD3, but CD7 and CD8 are absent (aberrant). CD57 is also negative.
Other relevant tests

Cytochemistry:

Genetics:T-cell receptor genes are clonally rearranged in most cases. Complex karyotypes are present in many cases in advanced stages.            

Flow Diagnosis:MF/SS cells have a mature T-cell phenotype expressing CD3, CD4, CD45RO and often with aberrant expression of at least one pan-T cell antigen.

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Sample Cases

Click here for instructions on how to download the free FCS Express Reader to view and manipulate the sample cases.

                

Case Name
(click on case name to open)
 
Comments  Size
 
case 28
 
Mycosis Fungoides / Seazary Syndrome
This case was kindly provided by the ASCP Press. It is part of Flow Cytometry in Clinical Diagnosis by John Carey, Phil McCoy and David Keren.

 
702 kB
 


 

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Page last modified 16:03, 3 Dec 2014 by admin