22 yrs old male with polyserositis, fever, hemolytic anemia and liver failure. Original diagnosis (LN biopsy): "Peripheral T lymphoma".  
HISTORY (EVENTS): 1. Antibiotic treatment with suspition of odontogenic sepsis (tooth extraction after fever, myalgia and sore throat presentation). 2. Pleuritis, pericarditis, hepatomegaly. Prednisone therapy. 3. Acute hepatorenal insufficiency. Dialysis. 4. Candida+ in blood culture. Antibiotic treatment.  
5. After improvement of symptoms the patient redirected to HEMATOLOGY dept. (biopsy results).  
HISTO (CONSULTATION CASE): The diffuse CD3+ CD2/CD5/CD7+ CD8+>CD4+ GranzymB/Perforin+ medium-large sized pleomorphic T cell infiltration with prominent diffuse apoptosis, high proliferation rate and focal CD68+/MPO+ histio- reaction with crescentic histyocytes. Scattered CD30+, single CD56+ cells. TDT/CD34/ALK1(-). Rudimentary B zones CD20+. NECROSIS IS ABSENT.  
 
WORKING DIAGNOSIS: REACTIVE T CD8+ PROLIFERATION (KIKUCHI LYMPHADENITIS (CELLULAR PHASE)) VS ...  
 
Thank you for collaboration.

tzankov 2007-12-21 15:29	For me, too, Kikuchi. There is an impression that the lymph node architecture is not entirely efaced, the capsulae is intact, the sinus are visible. there are a lot of apoptoses and also presence of MPO+ macrophages. I think that there are almost no alternative diagnoses to Kikuchi lymphadenitis.
ugnius 2007-12-21 15:55	Thanx. We have a lot of Kikuchi's (previous similar cases in iPath)... with different clinical pictures. The patient might be lost easilly with such septic situation...Maybe the clinical picture "aggravate" pathologist in this case.
Mueller-Hermelink 2007-12-21 19:58	The clinical history is completely atypical for Kikuchi LA.I am also not completely convinced b
Mueller-Hermelink 2007-12-21 20:07	It didn't take the whole message :Thereare several features that do not favour Kikuchi's LA :1) the infiltration is nodular or wedge-shaped with a basis at the marginal sinus in Kikuchi's La , whereas here it is a regular enlarged and activated T zone with preserved B cell follicles, 2) At MPO stain I got the impression that there are degtenerating granulocytes.the amount of histiocytes is rather low and not focally increased both features not compatible with K LA. Therefore I would favour a viral lymphadenitis. For the clinical history reported there are very few plasmacells. Is it an immune deficiency ( Ig deficiency) ?
ugnius 2007-12-21 20:18	Thank you for a comment. I've resend clin request. CD138, kappa, lambda, EBV and Ki67 in the progress. Virus status will ve rechecked (EBV markers was negative in previous hospitalisation time). IH I have not any stains with except EBV LMP1 and CMV.
ugnius 2007-12-22 09:58	Immunogramm: CD3+ 0,14x1000 000 000, CD4+ 0,11x10..., CD8+ 0,04x10..., CD19+ 0,004x10..., CD56/16+ 0,004x10..., CD4/CD8-3%. Lymphopenia, relativelly diminished B and NK sunpopulations, absulutelly diminished all lymphopopulations. HIV, CMV, EBV negative.
hurwitz 2007-12-27 16:14	There seems to general agreement that this is a reactive lymphadenopathy. The clinical history makes Kikutchi's lympadenitis less likely. I would like to add lymphadenopathy associated with systemic lupus erythematosus to the DD. A viral lymphadenitis, as suggested by Prof.Müller-Hermelink is an other option.   PS: could you please add a high power of image 2, just to better appreciate the cellular population?
ugnius 2007-12-27 18:04	Please find original Ki67 up to 95% and more HE from my slides (hemophagocytosis?). The quality of the orginal block is suboptimal.
ugnius 2007-12-27 19:24	Some points: atypical medium and large sized cells are some CD4+ and some CD8+; There are cleraly large MPO/CD68+ cells with eccentric nuclei; I've never met before viral lymphadenopathy like that with up to 100% proliferative activity... So Kikuchi like process seems to me the single possibility. Any haematoxylin bodies or necrosis are present (lupus lymphadenitis possibility).
hurwitz 2007-12-29 22:01	Thanks Ugnius for the additional images. There is a clear mixture of CD4 and CD8+ large cells, another argument for a reactive process. I agree that the lack of hematoxillin bodies and the lack of necrosis are argumetns against SLE, however I share Dr.Müller-Hermelinks reservations regarding Kikutchi's LA, mainly because of the fulminant clinical presentation.   What is the present state of the patient ?
anpo 2008-01-03 22:56	In my opinion that does not look like Kikuchi. If all the viral studies were negative I would do PCR to see if there is clonal T-cell population. With this proliferation a possibility of T-cell NHL cannot be excluded. Did the patient get any steroid therapy?
ugnius 2008-01-04 08:15	Thank you. All viral studies are negative. PCR was recomended. steroids was administrated for some time (I will check once more clin info).
ugnius 2008-01-04 09:20	The patient was and is now under steroid therapy.
anpo 2008-01-06 19:59	Steroid therapy could cause clinical improvement even in T-cell NHL.Has bone marrow biopsy been performed? Let's wait for the TCR results before final conclusion.
ugnius 2008-05-19 17:44	Unfortunatelly T clonality was found. Clinically the patient is well and without signs of disease. Interpretation was "infection asociated T lymphoproliferation".
diane.c.farhi 2008-05-20 17:31	I agree with Nina and Prof Mueller-Hermelink, who favor a viral or autoimmune process with marked lymphoid proliferation. The clonality might not provide much help, unless the clonal bands can be quantified in some way (small band with large reactive T cell population, versus large band with little or no reactive T cell background). Clonal T cell populations have been reported in aging, ataxia telangiectasia, autoimmune disease, benzene toxicity, common variable immunodeficiency, erythroderma, graft-versus-host disease, infection, pure red cell aplasia, rheumatoid arthritis, stem cell transplantation, as well as numerous malignancies (lymphoma, multiple myeloma, thymoma). Thus many reactive options are possible despite the clonality. Have serologic tests for SLE, RA, etc been done?
ugnius 2008-05-20 18:56	PCR beta MONOCLONAL. Gama polyclonal. clinically rheumatic diseases were excluded. Thank you once more.
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