Surgical management of monomorphic epitheliotropic intestinal T-cell lymphoma followed by chemotherapy and stem-cell transplant: A case report and review of the literature

Table 1 Patient’s laboratory results of blood chemistry

Item	Result	Reference value
White blood cell count (× 109/L)	7.8	3.5-9.5
Neutrophils (%)	88.2	40.0-75.0
Eosinophils (%)	0.6	0.4-0.8
Basophils (%)	0.3	0.0-1.0
Lymphocytes (%)	0.58	1.10-3.20
Monocytes (%)	0.28	0.10-0.60
Red blood cell count (× 1012/L)	3.87	4.30-5.80
Hemoglobin (g/L)	113	130-175
Mean corpuscular volume (fL)	88.6	82.0-100.0
Platelet count (× 109/L)	380	125-350
Hematocrit (%)	0.34	0.11-0.28
Lactate dehydrogenase (IU/L)	180	120-250
C-reactive protein (mg/L)	163.5	< 6.0
Direct bilirubin (umol/L)	2.7	0.0-4.0
Indirect bilirubin (umol/L)	7.90	0.00-22.00
Creatine kinase (U/L)	45	50-310
Total protein (g/L)	67.7	65.0-85.0
Albumin (g/L)	36.2	40.0-55.0
Globulin (g/L)	31.4	25.0-35.0
Glucose (mmol/L)	6.34	4.30-5.90

Table 2 Tumor markers

Marker	Result	Reference value
CA211	1.05	0.0-3.3
SCC	1.08	0.0-1.5
CA724	1.36	0.0-6.9
CA242	4.89	0.0-20.0
CA125	31.51	< 35.0
CA-153	12.87	< 25.0
CEA	1.97	0.0-5.0
CA19-9	18.57	< 37.0

Table 3 Panel of immunohistochemical stains

IHC stain	Result
CD3	+
CD4	+
CD5	-
CD7	+
CD8	+
CD30	-
CD56	+
CD117	-
CD138	-
Ki-67	80%
Kappa	+
Lambda	+
PD-1	-
CK-PAN	-
TIA-1	+
Granzyme B	+
EBER ISH	-

IHC: Immunohistochemistry; EBER: Epstein-Barr virus-encoded RNA.

Table 4 The Lugano staging system

Stage		Features
I		Tumor confined to small bowel: Single or multiple primary lesions
II	II	Para-intestinal nodal involvement
	II-1	Involving mesenteric, aortic, caval, pelvic, or inguinal nodes
	II-2	With penetration of serosa involving adjacent organs or tissues
	E (IIE, II-1E, II-2E)	Tumor extending into abdomen from primary small bowel site
III		NO stage III
IV		Disseminated extranodal sites or supra-diaphragmatic nodal involvement

Table 5 Details of previously published case reports

Ref.	Gender/age	Chief complaint	Treatment	Prognosis
Chen et al[29]	M/60	Abdominal pain	Emergency surgery followed by CHOP + IVE/MTX + SCT, followed by ASCT	CR; liver recurrence 2.5 years later refractory to GDP regimen. Passed away 2 wk after recurrence
Ishibashi et al[30]	M/60	Diarrhea and 10 kg weight loss in 17 mo	CHASE followed by SCT	3 years
	F/40	Diarrhea and 6 kg weight loss in 3 mo	THP-COP followed by surgery 10 mo later	2 mo after surgery
	F/50	Abdominal distension	CHOP + high-dose MTX + SCT	9 mo
	M/70	Nausea	SMILE	9 mo
Aiempanakit et al[31]	M/67	Diarrhea for 4 mo and 15 kg weight loss over 3 mo	Anthracycline-based regimen	2 mo
Antoniadu et al[32]	M/76	Severe dyspnea	N/A	5 d
Aoyama et al[33]	M/85	Fever and diarrhea	CHOP followed by DeVIC	Not stated but deceased subsequently due to progressive disease
Pan et al[34]	F/67	Abdominal pain	1 cycle of CEOP	3.7 mo
Liu et al[35]	F/48	Abdominal pain, distension, vomiting, watery diarrhea, weight loss	Unspecified chemotherapy	1 mo after chemotherapy initiation
Ozaka et al[36]	F/68	Melena and mild anemia	8 cycles of CHOP	Achieved complete remission and was still alive at the time of publication (68 mo after diagnosis)
Kasinathan et al[37]	F/70	Abdominal pain and vomiting for 4 wk	2 cycles of CHOP, followed by 2 cycles of GDP	Developed gastrointestinal bleeding and succumbed 4 wk after initiation of GDP
Mago et al[38]	M/59	SOB for 1 mo, abdominal distension for 2 wk	1 cycle of CHOEP	Passed away within few days after tumor lysis syndrome
Nato et al[39]	F/43	Abdominal distension, 2 mo history of early satiety and nausea	4 cycles of GDP achieving a PR, CR was achieved after CBT conditioned with total body irradiation, cyclophosphamide, and cytarabine	Cognitive impairment (7 mo post transplantation) was improved after 3 cycles of MPV and whole brain radiotherapy and passed away 6 mo later
Pan et al[40]	M/63	Diffuse abdominal pain for 1 mo	Emergency surgery followed by 2 cycles of CHOP	2 mo
	M/47	Diarrhea, dyspnea, orthopnea, weight loss for 1 year	1 dose of L-asparaginase, etoposide, and decadron regimen followed by emergency surgery, adjuvant chemotherapy included etoposide, methylprednisolone, high-dose cytarabine, and cisplatin	9 mo
Umino et al[41]	M/41	Diarrhea and epigastric pain for 1 mo	3 neoadjuvant cycles of ICE followed by autologous SCT	13 mo
Ferran et al[42]	F/45	Cutaneous lesions followed by abdominal perforation after chemotherapy initiation	6 neoadjuvant cycles of CHOP and 1 cycle of SMILE followed by surgery. 1 adjuvant cycle L-GEMOX	8 mo
Aoki et al[43]	F/77	Abdominal discomfort, night sweats, and fever for 1 mo	EPOCH for 6 mo	Still alive 1 year after diagnosis
Soardo et al[44]	M/65	2-wk history of weight gain, increased abdominal volume with progressive mild dyspnea, and fever in the last 2 d	Emergency laparotomy	1 mo postoperatively
Liu et al[45]	M/61	Upper abdominal pain and black stool for 2 mo	Partial excision of small intestine and chidamide-based combination regimen	15 mo
	F/35	Abdominal distension for 1 mo	Sigmoid colostomy followed by chidamide-based combination therapy	17 mo
Samuel et al[46]	M/62	Hypovolemic shock secondary to severe chronic diarrhea and 100 pounds lost over a year	Chemotherapy	1 mo
Ikeda et al[47]	M/61	3 episodes of ileal strangulation within 4 mo of gastrectomy	Ileal resection followed by 2 cycles of CHOP and 1 cycle of ICE	3 mo
Lenti et al[48]	F/63	Diarrhea and 10 kg weight loss in 6 mo	Surgery followed by a single course of CHOP	27 mo
	M/58	Diarrhea and 5 kg weight loss	Surgery	4 mo
Broccoli et al[49]	M/65	Petechiae at both limbs, acute abdominal pain, diarrhea, and clinical signs of bowel perforation	Emergency resection of 9 cm of small bowel	6 mo
Tabata et al[50]	M/72	Ileum perforation…severe constipation after 21 mo in CR	Emergency resection followed by anthracycline-based regimen chemotherapy (CR for 21 mo), paltrexate therapy was administered during recurrence	In CR after 52 mo
Fisher et al[51]	F/60	Abdominal pain, diarrhea, and 30 pounds of weight loss over 3 mo	EOCH chemotherapy (subsequently developing a large lymphoma 6 mo after therapy initiation)	N/A
Tian et al[8]	M/58	Abdominal pain, diarrhea, and weight loss over 3 mo	1 course of CHOP	Died subsequently after the first cycle due to bone marrow suppression
	F/64	Abdominal pain and diarrhea for 5 years	5 wk of adjuvant chemotherapy consisting of romidepsin with Revlimid followed by laparotomy involving small bowel bypass	3 mo
Kubota et al[52]	M/41	Diarrhea for 1 mo and intermittent abdominal pain	Resection followed by CHOP and 3 cycles of ICE resulted in CR	Repeated intrathecal chemotherapy and high-dose chemotherapy followed by ASCT achieved CR
Gentille et al[53]	F/70	Intermittent abdominal pain, nausea, vomiting and diarrhea for 14 mo. 50 pounds of weight loss	Right hemicolectomy followed by 5 cycles of EPOCH (with PEG-asparaginase added in the last cycle)	Developed abdominal pain 15 mo after initial therapy, subsequently passing away around 20 mo after initial diagnosis
Sato et al[54]	F/52	Diarrhea and anorexia for 8 wk + 6 kg weight loss	CHOP followed by stem-cell transplant	Unknown
Kakugawa et al[55]	M/65	Watery diarrhea for 14 mo	8 cycles of CHOP followed by 5 cycles of ESHAP	Still alive 67 mo post chemotherapy
Felipe-Silva et al[56]	M/78	Diarrhea for 2 mo + 20 kg weight loss	Surgical resection followed by 2 cycles of CHOP, which was changed to COP	6 mo
Okumura et al[57]	F/66	Abdominal distension for 1 mo presenting with acute abdomen	Surgical resection followed by high dose chemotherapy and SCT	Still alive at the time of publication, in complete remission
Yang et al[58]	M/39	Acute onset of lower abdominal pain and diffuse peritonitis	Surgical resection	Unknown
Fukushima et al[59]	M/60	Severe diarrhea	CHOP	1 year
Liong et al[60]	M/50	Diarrhea for 6 mo, presenting with acute abdomen due to intestinal perforation	Surgical resection followed by CHOP	4 mo
Noh et al[61]	M/68	Nausea and vomiting for 6 mo + 25 kg weight loss	Surgical resection followed by chemotherapy (unspecified)	Unknown
Hashimoto et al[62]	M/64	Diarrhea for several months	Chemotherapy (unspecified)	Unknown
Liu et al[63]	F/43	Upper abdominal pain and weight loss for 3 mo	4 cycles of CHOEP and 2 cycles of DHAP followed by surgery	11 mo after diagnosis, 1 d after surgery due to septic shock
Fukushima et al[64]	F/68	Upper abdominal pain and nausea	Laparoscopic intestinal resection followed by auto-peripheral blood SCT	22 mo without recurrence; passed away 1 mo after duodenal recurrence in 23rd mo

M: Male; F: Female; CHOP: Cyclophosphamide, doxorubicin, vincristine, prednisolone; IVE: Ifosfamide, vincristine, and etoposide; MTX: Methotrexate; SCT: Stem cell transplant; CHASE: Cyclophosphamide, cytarabine, etoposide, dexamethasone; GDP: Gemcitabine, dexamethasone and cisplatin; THP-COP: Pirarubicin, cyclophosphamide, vincristine and prednisolone; SMILE: Dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide; DeVIC: Etoposide, ifosfamide, and carboplatin; CEOP: Cyclophosphamide, epirubicin, vincristine, and prednisolone; SOB: Shortness of breath; CHOEP: Cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone; PR: partial response; CR: Complete response; CBT: Cord blood transplantation; MPV: Methotrexate, procarbazine, vincristine; ICE: Ifosfamide, carboplatin, and etoposide; L-GEMOX: Gemcitabine, oxaliplatin, asparaginase, dexamethasone; EPOCH: Etoposide, prednisolone, oncovin, cyclophosphamide, hydroxydaunorubicin; ICE: Ifosfamide, carboplatin and etoposide; ESHAP: Etoposide, methylprednisolone, cytarabine, cisplatin; DHAP: Dexamethasone, high dose cytarabine, platinol.