Table 1. Characteristics, therapy, and... | American Society of Hematology

Table 1.

Characteristics, therapy, and outcome of pediatric PTCL after SOT

Patient number	Age/sex	Graft	Time to PTLD (y)	Location	Tumor cell morphology	EBV	Immunophenotype	Molecular results	Reported diagnosis	Treatment	Outcome
Patient 1¹⁶	21-y male	Liver	5.0	Lymphadenopathy	N/A	Positive	CD3⁺, CD4^–, CD8⁺, CD30 subset⁺	N/A	PTCL, NOS	CHOP	In remission 9 mo later
Patient 2²⁵	7-y male	Liver	7.0	Gastrointestinal erosions	N/A	Negative	CD3⁺, CD4⁺, CD8^–, CD5⁺, CD7⁺, CD103⁺, CD56^–	Clonal TCR	PTCL, NOS	Reduced immunosuppressive	In remission
Patient 3⁹	3-y female	Lung	3.0	Mesenteric and retroperitoneal mass	N/A	N/A	CD3 dim⁺, CD4⁺, CD8^–, CD7 partial loss	Clonal TCR	PTCL, NOS	ALL-like (vincristine, daunorubicin, asparaginase, and prednisone)	In remission 20 mo later
Patient 4⁹	9-y female	Liver	5.0	Hepatosplenomegaly and lymphadenopathy	N/A	N/A	CD3⁺, CD4⁺, CD8^–, CD7 partial loss	Clonal TCR	PTCL, NOS	ALL-like	In remission 10 mo later
Patient 5¹⁰	2-y male	Liver	1.5	Lymph node and graft	small to medium tumor cells	N/A	CD3⁺, CD4⁺, CD8^–	N/A	PTCL, NOS	N/A	Died of disease in 1 mo
Patient 6¹⁰	1-y male	Liver	0.2	Lymph nodes and pleural fluid	N/A	N/A	N/A	N/A	PTCL, NOS	N/A	Alive 48 mo follow-up
Patient 7²⁶	4-y female	Liver	3.5	Abdominal mass and bowel perforation	N/A	Negative	CD3 dim⁺, CD4⁺, CD8^–, CD5 dim⁺, CD7^–	Clonal TCR	PTCL, NOS	EPOCH and ICE × 2 cycles, HSCT	In remission 3.5 y follow-up
Patient 8¹³	6-y female	Heart	5.4	Lung, small bowel and lymph node	N/A	N/A	N/A	N/A	PTCL, NOS	ALL-like	Alive at 22 mo follow-up
Patient 9¹⁵	4.7-y female	Heart	1.4	Retroperitoneal lymph nodes and bowel	N/A	Negative	CD4⁺	Clonal TCR	PTCL, NOS	Chemotherapy	In remission at 4 mo follow-up; died of heart rejection 3 y later
Patient 10	12-y female	Heart	11	Lymphadenopathy	Medium to large tumor cells	Negative	CD3⁺, CD4⁺, CD8^–, CD7^–, CD5⁺, CD2⁺, CD30⁺;Ki67 20%;CD34^–;TCR delta^–	Clonal TCR; KRAS and DICER1 mutations	PTCL, NOS	Chemotherapy (GDP-BV)	In remission; relapsed 8 mo later
Patient 11	3-y female	Liver, intestine, and pancreas	2.0	Stomach and lung mass	Small to medium tumor cells	Negative	CD3⁺, CD4⁺, CD8^–, CD5⁺, CD7⁺; TP53^–, MYC^–, TdT^–, CD34^–; Ki67 10%	Clonal TCR	PTCL, NOS	Chemotherapy	Died of disease in 5 mo
Patient 12	15-y female	Kidney	11	Ovary and pelvis mass	Small to medium tumor cells	Positive (by PCR)	CD3⁺, CD4⁺, CD8^–, CD5⁺, TdT^–, CD34^–	Clonal TCR	PTCL, NOS	Chemotherapy	Died of disease in 1 y
Patient 13	5-y female	Liver	4.0	Lymphadenopathy	Small to medium tumor cells	N/A	CD3⁺, CD4⁺, CD8^–, CD5 dim, CD2 dim⁺, CD7 dim⁺	Clonal TCR; Chr18q aberrancy	PTCL, NOS	Chemotherapy	Died of disease in 1 y
Patient 14	4-y male	Liver	4.0	Chest wall and pleural effusion	Small to medium tumor cells	Positive	CD3⁺, CD4⁺, CD5 dim, CD7 loss, CD30^–, Ki67 50%-60%	Clonal TCR	PTCL, NOS	Chemotherapy (CHOP)	Died of disease in 3 mo
Patient 15	4-y male	Heart	3.0	Duodenum	Small to medium tumor cells	Negative	CD3⁺, CD4⁺ (>>CD8), CD5 and CD7 partial loss, CD30 patchy⁺, BF1⁺	Clonal TCR	PTCL, NOS	Chemotherapy (BV-CHP)	Died of disease in 13 mo
Patient 16	12-y male	Heart	11	Bone marrow, splenomegaly, and mediastinal/subcarinal adenopathy	Small to medium tumor cells	Positive	CD3⁺, CD2⁺, CD8⁺, CD4^–, CD5 ⁺, CD7^– ; BF1^–; CD56 partial⁺; CD34^–	Clonal TCR	PTCL, NOS	Chemotherapy (anakinra, dexamethasone; CHOP)	Died of disease in 3 mo

Patient number	Age/sex	Graft	Time to PTLD (y)	Location	Tumor cell morphology	EBV	Immunophenotype	Molecular results	Reported diagnosis	Treatment	Outcome
Patient 1¹⁶	21-y male	Liver	5.0	Lymphadenopathy	N/A	Positive	CD3⁺, CD4^–, CD8⁺, CD30 subset⁺	N/A	PTCL, NOS	CHOP	In remission 9 mo later
Patient 2²⁵	7-y male	Liver	7.0	Gastrointestinal erosions	N/A	Negative	CD3⁺, CD4⁺, CD8^–, CD5⁺, CD7⁺, CD103⁺, CD56^–	Clonal TCR	PTCL, NOS	Reduced immunosuppressive	In remission
Patient 3⁹	3-y female	Lung	3.0	Mesenteric and retroperitoneal mass	N/A	N/A	CD3 dim⁺, CD4⁺, CD8^–, CD7 partial loss	Clonal TCR	PTCL, NOS	ALL-like (vincristine, daunorubicin, asparaginase, and prednisone)	In remission 20 mo later
Patient 4⁹	9-y female	Liver	5.0	Hepatosplenomegaly and lymphadenopathy	N/A	N/A	CD3⁺, CD4⁺, CD8^–, CD7 partial loss	Clonal TCR	PTCL, NOS	ALL-like	In remission 10 mo later
Patient 5¹⁰	2-y male	Liver	1.5	Lymph node and graft	small to medium tumor cells	N/A	CD3⁺, CD4⁺, CD8^–	N/A	PTCL, NOS	N/A	Died of disease in 1 mo
Patient 6¹⁰	1-y male	Liver	0.2	Lymph nodes and pleural fluid	N/A	N/A	N/A	N/A	PTCL, NOS	N/A	Alive 48 mo follow-up
Patient 7²⁶	4-y female	Liver	3.5	Abdominal mass and bowel perforation	N/A	Negative	CD3 dim⁺, CD4⁺, CD8^–, CD5 dim⁺, CD7^–	Clonal TCR	PTCL, NOS	EPOCH and ICE × 2 cycles, HSCT	In remission 3.5 y follow-up
Patient 8¹³	6-y female	Heart	5.4	Lung, small bowel and lymph node	N/A	N/A	N/A	N/A	PTCL, NOS	ALL-like	Alive at 22 mo follow-up
Patient 9¹⁵	4.7-y female	Heart	1.4	Retroperitoneal lymph nodes and bowel	N/A	Negative	CD4⁺	Clonal TCR	PTCL, NOS	Chemotherapy	In remission at 4 mo follow-up; died of heart rejection 3 y later
Patient 10	12-y female	Heart	11	Lymphadenopathy	Medium to large tumor cells	Negative	CD3⁺, CD4⁺, CD8^–, CD7^–, CD5⁺, CD2⁺, CD30⁺;Ki67 20%;CD34^–;TCR delta^–	Clonal TCR; KRAS and DICER1 mutations	PTCL, NOS	Chemotherapy (GDP-BV)	In remission; relapsed 8 mo later
Patient 11	3-y female	Liver, intestine, and pancreas	2.0	Stomach and lung mass	Small to medium tumor cells	Negative	CD3⁺, CD4⁺, CD8^–, CD5⁺, CD7⁺; TP53^–, MYC^–, TdT^–, CD34^–; Ki67 10%	Clonal TCR	PTCL, NOS	Chemotherapy	Died of disease in 5 mo
Patient 12	15-y female	Kidney	11	Ovary and pelvis mass	Small to medium tumor cells	Positive (by PCR)	CD3⁺, CD4⁺, CD8^–, CD5⁺, TdT^–, CD34^–	Clonal TCR	PTCL, NOS	Chemotherapy	Died of disease in 1 y
Patient 13	5-y female	Liver	4.0	Lymphadenopathy	Small to medium tumor cells	N/A	CD3⁺, CD4⁺, CD8^–, CD5 dim, CD2 dim⁺, CD7 dim⁺	Clonal TCR; Chr18q aberrancy	PTCL, NOS	Chemotherapy	Died of disease in 1 y
Patient 14	4-y male	Liver	4.0	Chest wall and pleural effusion	Small to medium tumor cells	Positive	CD3⁺, CD4⁺, CD5 dim, CD7 loss, CD30^–, Ki67 50%-60%	Clonal TCR	PTCL, NOS	Chemotherapy (CHOP)	Died of disease in 3 mo
Patient 15	4-y male	Heart	3.0	Duodenum	Small to medium tumor cells	Negative	CD3⁺, CD4⁺ (>>CD8), CD5 and CD7 partial loss, CD30 patchy⁺, BF1⁺	Clonal TCR	PTCL, NOS	Chemotherapy (BV-CHP)	Died of disease in 13 mo
Patient 16	12-y male	Heart	11	Bone marrow, splenomegaly, and mediastinal/subcarinal adenopathy	Small to medium tumor cells	Positive	CD3⁺, CD2⁺, CD8⁺, CD4^–, CD5 ⁺, CD7^– ; BF1^–; CD56 partial⁺; CD34^–	Clonal TCR	PTCL, NOS	Chemotherapy (anakinra, dexamethasone; CHOP)	Died of disease in 3 mo

ALL, acute lymphoblastic leukemia; BV-CHP, brentuximab vedotin, cyclophosphamide, doxorubicin, prednisone; CHOP, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and the steroid hormone prednisone; EPOCH, etoposide, vincristine, doxorubicin, cyclophosphamide, and prednison; GDP-BV, gemcitabine, dexamethasone, cisplatin, and brentuximab vedotin; HSCT, hematopoietic stem cell transplantation; ICE, ifosfamide, carboplatin, and etoposide phosphate; N/A, not applicable; PCR, polymerase chain reaction; PTLD, posttransplant lymphoproliferative disorder.

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