case 5 afula mcu compressed - hematology.org.il
TRANSCRIPT
4/17/2017
1
Case 5A
A 16 year old female underwent renal transplant for a diagnosis of focal segmental glomerlulosclerosis (FSGS).
One year later she presented with symptoms of an acute abdomen and had jejunal perforation.
CD20
CD20
MIB-1
EBER
4/17/2017
2
Additional studies
• Infiltrating cells positive for CD20, CD30, CD45, with kappa light chain restriction
• Proliferative rate: moderate to high
• Focal staining for LMP-1
Dx: Post-transplant lymphoproliferative disease, polymorphic type
Rx: R-CHOP with CR
Follow-up: 4 years later presented with PTLD-plasmacytoma type, involving laryngeal mucosa
Case 5B
• 57 year old male presented with abdominal pain, 10 years after right kidney transplant for polycystic kidney disease and renal failure
• CT scan showed small bowel obstruction with ulceration, focal perforation
Small bowel ulceration
Focal extension to muscularis propria –mainly by small lymphocytes
Scattered atypical cells in a rich inflammatory background
4/17/2017
3
CD20
CD3
CD20
CD30 CD15
CD30
PAX5
Bcl-2MUM1
CD79a
EBER
LMP1
Polyclonal by PCR
Fr 2, Fr 3, IgK
Treatment & Follow up : 4x RituximabReduction of Mycophenolate acid from 720mg to 360mg No evidence of disease with 10 months follow-up
Diagnosis: EBV+ mucocutaneous ulcer, polymorphic post-transplant lymphoproliferative disorder (PTLD)-related
Comparison with 5A:
• Scattered HRS-like cells in a marked inflammatory background, numerous T-cells, histiocytes
• Atypical cells do not sheet out
• No evidence of clonality
4/17/2017
4
Mucocutaneous Ulcer (Dojcinov et al. AJSP 2010)
Sites: Oropharyngeal mucosa (70%); Skin (25%); GI tract (5%)
Median Age 77 (42-101)– Age related only, Med 79 (64-101)
– Age + iatrogenic immunosuppression, Med 72 (42-80)
• Waxing and waning clinical course, may regress spontaneously
• Clinical options, local radiation therapy, rituximab
• No disease related deaths
Mucocutaneous ulcerGingival Involvement common
85 y.o. male
EBV+ Mucocutaneous Ulcer
EBER
CD30
EBER
CD30CD15
PAX5 CD20
CD30 CD15
PAX5Phenotype often mimicsClassical Hodgkin lymphoma
Some cases probably misclassified as cutaneous CHL in the past
4/17/2017
5
Spontaneous resolution of MCU in a patient with RA on MTX over the course of 8 weeks following withdrawal of drug
75 y.o. femaleLesion of arm
CD15
CD30CD20
Curves for MCU & RH overlap at 100%
Age related EBV LPD – Dojcinov et al Blood 2011
• 7 cases in series of 70 transplant recipients
5 renal, 1 heart, 1 lung• Involved oral mucosa in 4 and GI tract in 3• Patients treated with reduced
immunosuppression or rituximab or both • No patient recurred or developed another
PTLD• Conservative management is sufficient
EBV-related Lymphoproliferative B-cell Disorders
• Infectious mononucleosis• Post-Transplant and other iatrogenic B-cell LPD’s• Lymphomatoid granulomatosis • EBV+ DLBCL with chronic inflammation • Burkitt lymphoma (subset)• Classical Hodgkin’s lymphoma
– Mainly Mixed cellularity and Lymphocyte depleted• Primary effusion lymphoma (HHV-8 & EBV)• Germinotropic B-LPD • Plasmablastic lymphoma• EBV + large B-cell lymphoma, NOS • Mucocutaneous ulcer – provisional entity in WHO 2016
4/17/2017
6
Patterns of reactive B‐cell hyperplasia
Aggressive B‐cell lymphoma
B‐LPD of varied malignant potential
Indolent B‐cell lymphoma
Hyperplasia
Lymphoma
EBV+ B‐Cell Spectrum