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"Granulomatous" lymphoproliferation (1512)
"Granulomatous" lymphoproliferationnew
Subtitle: HEMATO
Type:
Biopsy/surgpath
Sender:
ugnius
2006-01-09 17:06
INCTR - EBMWG Hematopathology Online
44 yrs male presents with neck lymphadenopathy, B symptoms and splenomegaly. The 3 subsequent biopsies of the cervical lymph node were taken. FIRST BIOPSY (photo aren't included): "Macrophageal reaction, suspitious for HL". SECOND BIOPSY (photos B2): "Reactive lymphadenopathy with reaction of epithelioid histiocytes". THIRD BIOPSY (photos B3): Hodgkin's lymhoma (MC variant) with prominent macrophageal reaction. NOTE: All changes identical to previous biopsies. Differential with peripheral T lymphoma (Lennert type) was discussed.  
B2:T zone expansion (CD3+), CD20+ rudimentary B agregates, some CD30+ activated immunoblasts. The moderate cellularity with diffuse proliferation of small- medium T lympho's with definite cytoplasmic borders and irregular nuclei are observed. The clusters of epithelioid histiocytes are obvious.  
B3: Identical B2 findings + scattered mononuclear CD15+ cells and CD30+ activated cell clusters focally (different cells in different places!). Some hypocellular (AILT like) subcortical areas and FULLY DIFFUSE OBLITERATED ARCHITECTURE WITH CLUSTERING OF MACROPHAGES. The SINGLE cortical subsinusoid areas with RS- like giant cells with lobated nuclei and prominent nucleoli, apoptotic cells (CD30/CD15-/CD20+/EBV LMP1+) with some granulocytes. CD21+ FDC network is more displaced than proliferating (like in AILT). Anti HL therapy (ABVD and after BEACOPP)was applied, but lymphadenopathy remains.  
B4: Nowadays (1 year later after first biopsy) the acute perforation of chronic gastric ulcer appears and he underwent resection of stomach and biopsy from liver nodule with clinical diagnosis "metastasis". The infiltrate: CD3(+) 50%, CD20(-), CD43(+) 70%, CD2 (+) 90%, CD5(+)70%, Bcl 2(+) 40%, CD56+ single cells, Granzym B(+) 10%, CD4+ >> CD8+. CD30(+) single activated cells in liver; CD15(-). Ki67 prolif. activity 30% (the larger cells), Bcl6/CyclinD1/CD23/EBV LMP1(-). The admixture of histiocytes in gastric infiltrate is prominent. .  
PROPOSED DIAGNOSIS: Peripheral T lymphoma (Lennert' type) or AILT (Lennert like variant): spread in lymph node, stomach and liver???  
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kunze
2006-01-12 14:54
My provisional diagnosis is a HL-like lymphoproliferative disease with EBV infection (and reactive epitheloid cell proliferation). In my mind the CD20/LMP-positive atypical lymphoid cells are hardly compatible with a PTCL or AILT. Otherwise, the numerous CD30-positive cells seem to me not typically of HRS-cells.  
Are there hints at a collagenous or autoimmune disease in the clinical history?  
Despite a PCR for TCR rearrangement an immunostain for PAX-5 could be helpful, if available.  
I'm curious about findings in liver and stomach.
ugnius
2006-01-13 12:05
NEW PHOTOS: LIVER.
ugnius
2006-01-13 13:19
NEW PHOTO'S: STOMACH. The parafin blocks are not well preserved. EBV LPM1+ or CD30/CD20+ large cells are absent. But there is DEFINITE AGRESSIVE LYMHOHISTYOID INFILTRATE with CD4+ phenotype. Is it possible to find some "lymphoma signout" in previous biopsies of lymph nodes???
kunze
2006-01-14 13:44
Histological and immunohistological findings in liver and stomach support the diagnosis of an angioimmunoblastic T-cell lymphoma. Occasionally, EBV/CD30-positive large atypical B-cells may occur in this type of a peripheral T-cell lymphoma.
ugnius
2006-01-15 16:55
Thank you for diagnostic ideas. One more problem: just imagine having lymph node biopsy ONLY (with exceptional Lennert'oid pattern).
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Last modified: 2006-01-09 17:06:55