Lymphoma

Research Article

Non-Hodgkin Lymphoma in Children with Primary Immunodeficiencies: Clinical Manifestations, Diagnosis, and Management, Belarusian Experience

Table 2

Characteristics of patients with PID^a and NHL^b.


Patient	Age (years)/sex	Type of PID	Lymphoma	Stage	Involved sites	EBV^c in tumor	EBV in blood (DNA)	Treatment	Outcome

1	0.6/F	SCID^d, atypical	DLBCL^e	III	Lungs, pleura, chest soft tissues^*	POS^f	NEG^g	A^# + R –5 courses R-EPOCH, 2/3 dosage	CR^h 1, alive disease-free for 4.8 years

2	2.2/F	SCID, atypical	DLBCL, immunoblastic	IV	CNS^j (massive brain^* tumor), mediastinum, lung^*	POS	POS	Individual 5 courses with HD MTX or ARA-C + R	Progression after the 5th course, died of disease at 6 months after the diagnosis

3	1.4/F	CID^k, NS^l	PTCL^m, CD8+, NOSⁿ	III	Peripheral LN^o*, tonsils, mediastinum, lungs, inguinal LN, ileac LN, spleen, retroperitoneal LN	ND^p	ND	AA^#, 2/3 dosage	Partial response, death of sepsis after the 1st course

4	1.2/M	CID, NS	DLBCL, centroblastic	III	Peripheral LN^, mediastinum, lungs, abdominal LN, retroperitoneal LN, hepar^, small and large intestines	POS	POS	Dexamethasone only	Sepsis and generalized EBV infection initially, death after 7 days of therapy

5	0.7/F	CID, NS	DLBCL, immunoblastic	III	Peripheral LN^*, mediastinum, lungs, salivary glands, spleen, abdominal LN	POS	POS	Dexamethasone only	Sepsis and generalized EBV infection initially, death after 5 days of therapy

6	1.1/M	CID, NS	DLBCL, immunoblastic	III	Lungs^, hepar, spleen, cervical LN^, abdominal LN	POS	POS	R-EPOCH, 3/4 dosage	Partial response, sepsis initially, death of sepsis after the 1st course of therapy

7	8.3/M	CID, NS	T-LL^r (pro-T)	IV	Peripheral LN^*, abdominal LN, retroperitoneal LN, hepar, spleen, BM^s	ND	ND	NHL-BFM 95, TG1, full dosage	Early relapse (BM) during maintenance, death of sepsis in CR 2 at 2 years after the diagnosis

8	15.5/F	CID, NS	ALCL^t, cutaneous, ALK^u - negative	I	Skin^* of the left upper eyelid	ND	ND	Brachytherapy only	CR 1, alive disease-free for 2.0 years

9	6.3/M	WAS^w	Burkitt	III	Small intestine^, abdominal LN^	ND	ND	AA–BB–AA–BB–AA–BB^#, full dosage	Second malignancy (B-mature leukemia) in 15 years, died at 15.1 years after the first diagnosis

10	10.9/F	NBS^x	DLBCL, T-cell/rich	III	Peripheral LN^*, mediastinum, lungs, spleen, abdominal LN, retroperitoneal LN	ND	ND	A–B^#, full dosage, stop therapy in unconfirmed remission because of parent's refusal	Second NHL in 4.5 years, no treatment, died of disease at 4.6 years after the first diagnosis

11	5.6/M	NBS	T-LL (mature CD8+)	III	Mediastinum, lung, peripheral LN^*	NEG	ND	NHL-BFM-95, TG1: I phase I protocol, HR1-HR2-HR3-HR1-HR2-HR3 (full dosage), nonmyeloablative conditioning, HSCT, unrelated	CR 1, alive disease-free for 1.4 years

12	16.9/M	NBS	PTCL, small cell type, CD8+, high grade	III	Mediastinum (bulky disease), peripheral LN^*	NEG	ND	CHOP–CHOP–ICE–DHAP–DHAP–DexaBEAM, full dosage	Progression after the 5th course, died of disease at 5 months after the diagnosis

13	14.2/M	NBS	T-LL (cortical)	III	Mediastinum^* (bulky disease)	ND	ND	NHL-BFM 95, TG1, ImRG, protocol I, full dosage	Death of sepsis in partial remission after 7 weeks of therapy

14	9.1/F	NBS	ALCL	III	Peripheral LN^*, mediastinum, abdominal LN, retroperitoneal LN, ileac LN	ND	NEG	A^# (no MTX)	Partial response, died of invasive pulmonary aspergillosis after the 1st course

15	15.9/M	Bloom syndrome	ALCL, ALK-negative	III	Peripheral LN^*, mediastinum, lungs, abdominal LN, retroperitoneal LN, ileac LN	NEG	NEG	ALCL99, HRG, full dosage	Secondary hemophagocytic syndrome during maintenance, death of sepsis at 1.3 years after the diagnosis

16	6.0/M	XLPS-1^y	Burkitt	III	Mesentery, jejunum^, abdominal LN^, bladder, abdominal wall	NEG	ND	NHL-BFM 95, TG2, R2, full dosage, +3 doses of R	CR 1, alive disease-free for 5.7 years

Primary immune deficiency disease, ^bnon-Hodgkin lymphoma, ^cEpstein-Barr virus, ^dsevere combined immunodeficiency, ^ediffuse large B-cell lymphoma, ^fpositive, ^gnegative, ^hcomplete remission, ^jcentral nervous system, ^kCombined immunodeficiency, ^lNot specified, ^mPeripheral T-cell lymphoma, ⁿNot other specified, ^oLymph node, ^pno data, ^rlymphoblastic lymphoma, ^sbone marrow, ^tanaplastic large cell lymphoma, ^uanaplastic lymphoma kinase, ^wWiskott-Aldrich syndrome, ^xNijmegen Breakage syndrome, ^yХ-linked lymphoproliferative syndrome-1, ^*site of biopsy, and ^#chemotherapy course, see [5] (Reiter et al. Blood, 1999).