17 yrs old male with neck lymphadenopathy and Lyme disease *under treatment). 2 biopsies were obtained. Both original reports: "Reactive changes". History of multiple purulent tonsilitis. Blood: ESR 65; WBC 13.6x10(9); LDH 617 U/l; Hb 106g/l; BM aspirate: leukemoid reaction.  
HISTO: Some expanded parakortical areas with large and highly atypical (binucleated) immunoblasts with basophilic macronucleoli with CD20+ EBV LMP1- and CD3+? immunophenotype. Activated CD30+dim cells. CD30+CD15+ typical RS or H cells are absent. Ki67 activity ~30% in the nidus. Eos and granulocytes in open sinus. Focal immature histyocytosis in the sinuses.  
PICTURES: Nidus 1 (with more prominent CD20+ immunoblasts) and Nidus 2 (more closed to sinus). CD3 is right-sided in "CD3 CD20 SCAN".  
 
PROPOSAL: Reactive lymphadenopathy with prominent immunoblastic reaction.

tzankov 2008-04-07 08:10	The lymph node zonation is retained. The paracortical and parafollicular zones are enlarged and have a pale character due to a hidge amount of parafollicularly activated immunoblasts. My working hypothesis is "parafollicular blast activation", I would perform CD138 and ALK1, sinc eI expect no ALK-positivity and partial positivity for CD138 in these immunoblsts. a clinical work-up, considering the drug anamnesis could be helpful.
ugnius 2008-04-07 08:58	Thanx. CD138+ in plasma cells only. ALK1 in progress.
Mueller-Hermelink 2008-04-07 14:10	I agree with Dr. Tzankovs comment . This is a typical case of so-called extrafollicular activation of plasma cell precursors . In CD79a stain more B cells will be seen . Light chain staining will be polytypic.Pathogenetic consideration usually are bacterial infection in the afferent regions ( which is also compatible with the clinical situation)
hurwitz 2008-04-07 15:56	There is little doubt that at present this is a reactive predominantly paracortical proliferation of immunoblasts. However, in case of clinically proven borreliosis, the association between borreliosis and subsequent lymphoma has to be considered, and the follow-up of the patient planned accordingly   {PMID: 15370236},{PMID: 16112357},{PMID: 11972710}
ugnius 2008-04-07 19:07	In the deeper serial sections Nidus 2 (closed to capsule) reveals abscess with granulocytes. Some folicullar centres merge with immunoblastic proliferation without mantle zone, creating the pattern of FC "destruction" (pictures "CONFLUENT").
ugnius 2008-04-07 19:18	Some T nodules with S100+ CD1a+ reaction like in dermatopathic lymphadenopathies.
diane.c.farhi 2008-04-08 18:24	I agree with the comments so far. What could be the etiology of this process? It seems too acute for chronic Lyme disease, and I don't think we have the characteristic features of toxoplasmosis, cat scratch disease, syphilis, or Kikuchi disease. If the boy is on therapy for Lyme, he should be taking a broad-spectrum antibiotic, and I'm surprised he still has such an acutely inflamed lymph node. Was the biopsy submitted for culture? Can we be sure it's not EBV?
ugnius 2008-04-08 18:35	EBV (clin) is negative. The immature histyocytosis is visible. Some new IH stains will be apended without any substantial add info.
hurwitz 2008-04-09 20:17	The S-100 and CD1a+ nodules are features of dermatopathic lymphadenitis. Could you please find out if there are sings of a skin affection in the area drained by this node?
hurwitz 2008-04-17 14:22	Final diagnosis     Paracortical and parafollicular hyperplasia with prominent immunoblastic proliferation, secondary to bacterial infection.   (Borreliosis, according to clinical information)   No evidence of lymphoproliferative disease)     Thanks for this interesting case and discussion)
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