PATIENT: 60-year-old woman with many concomitant diseases (coronary heart disease, hypertension, hypertensive cardiomyopathy, congestive heart failure, pulmonary hypertension, type 2 diabetes, dyslipidemia).  
 
CLINICAL HISTORY: Three years ago, due to the suspition of cardiac amyloidosis, the patient underwent bone marrow aspiration and trephine biopsy, which showed reactive changes (mixed lymphoid hyperplasia) and normal polyclonal plasma cells. Peripheral blood showed mild leukocytosis (10,54 *109/L, normal range beeing up to 9,8 *109/L) with mild lymphocytosis (4,1 *109/L, normal range beeing up to 4,0). Although immunofixation showed no monoclonal protein, there was a significantly increase in Ig kappa chains (up to 367 mg/L, normal range beeing 6,7-22,4) and mild increase in Ig lambda chains (up to 36,50 mg/L , normal range beeing 8,3-27,0). Also increased was beta2 microglobuline 5,74 mg/L (normal range 0,97-2,64). Abdominal fat aspiration biopsy was negative for amyloid.  
One year ago the patient suffered a stroke.  
Recently the patient experienced weight loss (10 kg per 7 months).  
PET-CT scan shows multiregional lymphadenopathy with low metabolic activity.  
 
CORE NEEDLE BIOPSY OF A RIGHT AXILLA LYMPH NODE (Fig.1-21): primary and secondary lymphoid follicles without FDCs expansion, paracortex with monotonous small-to-medium CD3/CD2/CD5/CD7+, CD4+ >> CD8+ T cells and scattered larger CD30+/EBER(-) cells with conspicuous nucleoli. Increased vascularity and focally open subcapsular sinus. Clonality evaluation by PCR showed clonal IG profile (IGH FR1-3) and no TCR clonality.  
 
THE SUBSEQUENT BONE MARROW TREPHINE BIOPSY (Fig.22-28) showed nonspecific reactive lymphoid hyperplasia.  
 
EXCISIONAL LEFT AXILLARY LYMPH NODE BIOPSY (Fig.29-67): retained architecture with Castleman-like secondary lymphoid follicles with vessels penetrating fibrosed (IgM positive??) germinal centers (”lollipop”) . Increased vascularity of paracortex. Clonality evaluation by PCR revealed (the same as previously seen in another lymph node) clonal IG profile (IGH FR1-3) and no TCR clonality.  
 
PROPOSED DIAGNOSIS:  
- Reactive paracortical hyperplasia? (B clonal??)  
- AITL?  
- Other lymphoproliferative disorder?  
 
Thanks so much for Your valuable insights!!

tzankov 2021-07-07 06:44	Very difficult case, indeed!   Would you be so kind to stain the lymphadenectomy for CD34, EGFR, Kappa and Lambda, IgA and IgG?   Any chance to sequence some genes such as IDH2R172K, RHOAG17V, DNMT3A, TET2, CD28, CTNNB1, FYN, GTF2I, MYD88, PI3K and PLCG1?   Any chance to perform FCM of the blood to look for pathologic or clonal B-cells?
ugnius 2021-07-11 22:27	I added IgG, IgA, Kappa, Lambda, EGFR and CD34. In my view, all of it look nonspecific. Also, staining with IgG4 showed no positive plasma cells. MYD88 sequencing (from the core needle biopsy material) was negative for mutation.   About peripheral blood FCM - not sure if it will be possible to perform (still pending..).   I hope this can help You a bit.
tzankov 2021-07-12 06:57	Well, with the clearly polytypic but massive plasmacytosis, with the neoangiogenic component, the CD4 predominance and the distribution of the ICOS+ cells as well as the present CD30+ immunoblastoid cells and the "roulghly AILD syndrome", I favor AITL pattern 1 (inspite or irrespective of the lacking clonality). This remains yet to be proven by demonstrating one of the listed typical mutations.
ugnius 2021-07-12 08:11	Thank You for the oppinion!!
tzankov 2021-07-12 08:52	What is your oppinion?
ugnius 2021-07-12 11:08	For now I would still prefer to call it smth like "atypical paracortical hyperplasia with polytypic plasmacytosis", possibly related to autoimmunity(?). In my view, there is not enought specific histological changes that would be seen in AITL, exept for vascular proliferation. That would be possible in AITL I pattern but IH (especially PD1) shows staining pattern characteristic for reactive Tfh proliferation. Also, Tfh itself is monomorphic.   For sure, this could also be an early changes of neoplastic process (AITL?) and the patient should be followed up.   By the way, this is Ugnius' apprentice Gintarė. Ugnius might have other oppinion.
	» Add comment (Login)