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Hairy, Scary Disease in a Blood Filled Organ

Dr Sampurna Roy  MD


 

Hairy Cell Leukaemia in Spleen

         


 

Hairy Cell Leukaemia is a chronic leukaemia and is characterized by cells with fine villous surface projections and bean shaped nuclei and pale cytoplasm, which are seen in the circulation, in the bone marrow and in the red pulp of the Spleen.

The most characteristic histological changes are observed in the Spleen.

Clinically, adults present with splenomegaly and pancytopenia. The disease tends to follow an indolent course.

In paraffin sections a distinctive pattern of markers can be detected and these may be of diagnostic value (specially when marrow shows a low level of infiltration).

In addition to pan-B-cell markers such as CD20 and CD79a, neoplastic cells often express CD68 (as cytoplasmic dots) and are labelled by antibody DBA44.  Acid Phosphatase isoenzyme 5 (Tartarate - resistant) -TRAP is another useful marker in the identification of hairy cell leukaemia.

See the images below:

 

 

The hairy projections are visible in well-prepared blood smear.

 
 

Hairy cells demonstrate strong positivity for tartrate-resistant acid phosphatase (TRAP) staining.

 
 

Hairy cell Leukaemia in the Spleen. The splenic cords are widened by diffuse infiltration of leukaemic cells.

 
 

Hairy cell Leukaemia in the Spleen. Hematoxylin and Eosin stain.

 
 

DBA 44 is a highly sensitive marker of Hairy Cell Leukaemia and is particularly useful in the identification of small amounts of disease in bone marrow biopsies of patients following therapy.

Although this antibody stains upto 100% of cases of hairy cell leukaemia it is not specific and is positive in a wide range of other lymphomas. - Example: Lymphoplasmacytic Lymphoma, Mantle cell Lymphoma, and Follicular cell lymphoma.

 

 
 

Hairy cell Leukaemia in the Spleen -  Acid Phosphatase isoenzyme 5 (Tartarate - resistant) -TRAP is another useful marker in the identification of hairy cell leukaemia and can be detected by immunohistochemistry using a monoclonal antibody reactive in formalin-fixed paraffin-embedded material.

 


 

 

 

 

Dr  Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 

 

          

 

 

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