59 yrs female with clinical diagnosis of B-CLL/SLL. Previous histology (original diagnoses):  
2000: SPLEEN: B-SLL/CLL (without IH).  
2007: Threpine: B mantle cell lymphoma (CyclinD1+).  
The patient was treated as B mantle cell lymphoma.  
 
Revised and IH typed/retyped data:  
2000: SPLEEN: HCL vs SMzL?. The diffuse B infiltrate: CD20+/CD79a+; HCL+; CD23-; CD43-; CD5-; Single CyclinD1+ cells; IgM/IgD+. The cleariness of cytoplasm as in HCL.  
2007: Low grade B NHL, most probably MzL: The diffuse B infiltrate: CD20+; CD23-; CD43-; CD5-; Single CyclinD1+ cells; IgM/IgD+; single HCL+ cells. FDC CD23+ rudimentary network (folliculotropism). There are any histo signs of HCL.  
2008: Threpine: Low grade B NHL: HCL vs MzL?: In part HCL "clear" difuse infiltration. The same immunophenotype with stronh HCL/CD20+ positivity, CD23/CD5(-). FDC CD23+ rudimentary network (folliculotropism).  
 
The question/problem: 1. In 2007 and in part in 2008 threpine the classic HCL histopicture is absent. 2. Bcl6+ in 2008 threpine. 3. The possibility of primary splenic marginal zone lymphoma ("with willous lymphos") with HCL and (aberant) cyclinD1+ expression???  
Thank you for continuous support.  

bvrugt 2008-08-25 18:56	In my view the morphology of the cells in the HPF images of the trephin from 2008 favor HCL (I presume no trephin biopsy was taken in 2000?). I find the histology of the spleen difficult to interpret, but the focal expression of the cells for cyclin-D1 in both the spleen and the trephin, in combination with DBA.44 reactivity in a part of the tumor cell population support HCL. As far as I know cyclin-D1 expression has been described in HCL, and may occur in a small minority of tumor cell population, whereas the expression in MZL is generally more extensive, but absent in SMZL. In summary, I think that the proliferations in spleen and trephin are related to infiltration wth HCL (with Cyclin-D1 expression).
tzankov 2008-08-26 14:23	for me too, a classcial HCL... the positivity of Cyclin D1 and DBA44 as well as the diffuse BM (but not sinusoidal, like in SMZL) infiltration are all in favor of the diagnosis.
hurwitz 2008-08-27 13:28	I agree with the previous comments, the morphology in particular is characteristic for HCL. I also agree that the DD between SMZL and HCL can be difficult if only splenic tissue is available.   DBA44 is generally regarded as a marker for hairy cells, but it is not entirely specific. Cyclin D1 expression in HCL has been described {PMID: 10456674}, {PMID: 12231535}
ugnius 2008-08-27 13:34	Thank you a lot.
hurwitz 2008-09-03 22:54	I think we can summarize the case as:   Spleen and bone marrow biopsy with infiltrates of HCL with aberrant cyclin D1 expression.     Thanks to all the participants.
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