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PTCL with some AILT features (423504)

PTCL with some AILT features
Subtitle: B12-17552

Type:
NODE+BM

Sender:
ugnius

2012-05-24 13:17
INCTR - EBMWG Hematopathology Online

64 yrs old male. Threpine biopsy was taken with suspition of lymphoma. LDH 220 (N). Generalized lymphadenopathy, febrility, hepato- splenomegaly. BLOOD WBC 4,36 (N); RBC 4,71 (N); HB 129 ( sligthly dim); PLT 227 (N);

STEP 1: BM: MPN NOS, favor ET was detected (incidentally) and approved by JAK2 V617F+. Additionally heterogenous CD3 >> CD20+ nodular central infiltrates was detected and diagnosis of T NHL: PTCL or AILT was formulated.

STEP 2: NODE BIOPSY: Partially effaced architecture with neoplastic diffuse expanded T zone+ huge hyper=hyperplastic follicles with rimming of CD4+ cells with some follicullothropism.

FULL IH (NODE): CD3/CD5/CD7+100% ; Bcl2(+)(weak) 100%; CD8/Granzym B/CD57(-); Ki67 ~50% (+++)(brand. r-ja); focal irregular CD23/CD21+ proliferation; EBER+ aggregates perifolicularly.

MOLECS (node): pending. BM: not enough DNA (uninformative; focal lesions).

SPECIAL QUEST: variant of T NHL in NODE.

PROPOSED DIAGNOSIS:

BM: MPN, NOS (favor ET)+ T NHL (AILT vs PTCL).

NODE: PTCL with features of AILT.

Thank you for continuous education.

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tzankov 2012-05-24 18:16	the lymph node architecture is not much efaced... i need more arguments for lymphoma, antigenic loss (CD5, CD2, CD7), expression of TFH markers (CD10, VCL6, PD1, CXCL13), atypical proliferative activity in the T-zones (Ki67), clonality!? was the petient given any therapy or immunization soon? antiepileptics? hepatitis (re)immunization? other durgs?
metz 2012-05-26 02:35	I can comment on the haematology only. The marrow is hypercellular with increased megakaryocytes, some abnormally large, and megakaryocyte clusters. Reticulin is not increased. In conjunction with the JAK2+, this is consistent with ET. However, there is discordance with the peripheral blood findings in that the platelet count is apparently normal. The diagnosis of untreated ET cannot be established without sustained thrombocytosis. Presumably the platelet count has been repeated and there is no platelet clumping which could give rise to spuriously low counts. Lymphoid aggregates are not uncommon in the marrow in myeloproliferative neoplasms and are usually regarded as reactive.
ugnius 2012-05-29 17:30	Please find additional photos from additional blocks (blocks 3 and 6 on photos) and some for BM. 1. NODE: focal nodular expansion of T zone eith pleomorphic cells and HEV's= AILT like areas. I have no CD10+Bcl6+ in the tumor population, but hyper-hyperplastic follicles, clearly induced by perofollicular and maybe intrafollicular CD4+ population visible (as in AILT some variants). I have not PD1 or other IH for Th, so there are just my thoughts at minimum about AILT. 2. BM: T cell reach infiltrates has dim Bcl2 reaction and high irregularity, so its not compatible with reactive states (CD3+ small lymphos with prominent Bcl2 reaction). 3. EBER reaction in large cells (maybe B blasts) within and peri- follicular area: aditional argue for AILT. 4. Clonality studies are in progress. I'm sorry for incapability now to present all virtual slides due to strange "security" politics of my dept. Sometimes its difficult to illustrate subtle changes (BLOCK 1).
tzankov 2012-05-30 07:22	well with the additional microphotographs, I agree that the findings are suspicous of a T-cell lymphoma. Did you obsevre an antigenic loss of CD2, CD3, CD5 od CD7? the bone marrow has some myeloproliferative features, consistent with MPN/PMF, but the organomegaly is most probably due to the AITL PS PD-1 is a usefull antibody, you shoudl convince the authorities to buy it :)
tzankov 2012-05-30 07:22	well with the additional microphotographs, I agree that the findings are suspicous of a T-cell lymphoma. Did you obsevre an antigenic loss of CD2, CD3, CD5 od CD7? the bone marrow has some myeloproliferative features, consistent with MPN/PMF, but the organomegaly is most probably due to the AITL PS PD-1 is a usefull antibody, you shoudl convince the authorities to buy it :)
ugnius 2012-05-30 07:28	Thanx. Please find full IH above: nothing special (except wery weak Bcl2 (downreg?): CD3/CD5/CD7+100%. I will try do my best on IH:)
tzankov 2012-05-30 07:35	I still do not see any info on CD2
ugnius 2012-05-30 07:42	I usually believe that CD3=CD2 in general. I'll do that.
hoellers 2012-05-30 11:00	Dear Ugnius, thank you very much for sharing this intersting case with us. Concerning the bone marrow I would be in favour of PMF, because of the clustering of megas, the bulbous chromatin and the hypercellularity of the marrow. If this case would not be JAK2 mutation positive, I would be cautious with the diagnosis of a MPN since T-cell lymphomas (here the suspicion of a T-cell lymphoma) can imitate a MPN. Concerning the lymph node as already Dr. Tzankov mentioned the loss of T-cell antigens is important as well as the CD4/CD8 ratio and the presence of atypia. Also an expression of CD10 and BCL-6 in the T-cell compartment helps. There is no expansion of dendritic cells (CD21 is sometimes more informative than CD23), no great hyperplasia of vessels and no presence of "fried eggs", so besides the presence of EBV positive cells there are not many arguments for AILT. I'm curious of the result of the clonality analysis.
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Last modified: 2012-06-02 17:20:53