Experience of primary intestinal lymphangiectasia in adults: Twelve case series from a tertiary referral hospital

Table 1 Demographic information in patients with primary intestinal lymphangiectasia

Demographic information
Total number of patients	12
Age, yr	48 (32, 63)
Male/female	7/5 (58/42)
BMI	22 (21, 23)
Comorbidities
Cerebrovascular disease	3 (25)
Hypertension	2 (17)
Cardiovascular disease (unstable angina, MI, CHF)	1 (8)
Chronic obstructive pulmonary disease	0 (0)
Diabetes mellitus	3 (25)
Chronic kidney disease	1 (8)
Cancer	3 (25)
Follow-up duration, mo	30 (24, 37)

Categorical variables were presented as numbers and percentages, whereas continuous variables were represented as median and interquartile range. BMI: Body mass index; CHF: Congestive heart failure; MI: Myocardial infarction.

Table 2 Diagnostic findings in patients with primary intestinal lymphangiectasia

Diagnostic findings
Symptoms
Peripheral edema	12/12 (100)
Diarrhea	9/12 (75)
Abdominal pain	4/12 (33)
Nausea/vomiting	0 (0)
Laboratory findings
Leukocyte, 109/L	4.8 (3.9, 6.6)
Lymphocyte, 109/L	1.1 (0.7, 1.4)
Lymphocytopenia, < 1.0 × 109/L	5/12 (42)
Hemoglobin, g/dL	11.9 (10.7, 13.4)
Albumin, g/dL	1.8 (1.7, 2.4)
IgG, mg/dL	391 (343, 474)
Ferritin, ng/mL	130 (54, 256)
Ferritin < 30 (male) or 13 (female) ng/mL (two missing value)	1/10 (10)
CRP, mg/dL	0.16 (0.08, 0.49)
Alpha-1 antitrypsin clearance, mL/24 h	566 (211, 704)
Alpha-1 antitrypsin clearance > 27 mL/24 h (two missing value)	10/10 (100)
Radiologic findings
Small bowel wall thickening and edema	8/12 (67)
Ascites	7/12 (58)
Pleural effusion	3/12 (25)
Ileus	0/12 (0)
Endoscopic findings
Snowflake appearance	9/12 (75)
Pathological findings
Dilation of the lymphatics in mucosa or submucosa	12/12 (100)

Categorical variables were presented as numbers and percentages, whereas continuous variables were represented as median and interquartile range. CRP: C-reactive protein.

Table 3 Medical treatment and interventions during the follow-up of adult patients with primary intestinal lymphangiectasia

Case number	Age/sex	Albumin	Octreotide	Tranexamic acid	Steroid	Sirolimus	Everolimus	Outcome of treatment	Lng mediastinum MRI (reason)	Lymphangiography	Lymphatic embolization
		Periodic injection intravenously	Subcutaneous; Dose; Frequency; Duration	Oral; Dose; Frequency; Duration	Oral; Dose; Frequency; Duration	Oral; Dose; Frequency; Duration	Oral; Dose; Frequency; Duration		No/yes (reason)	No/yes (reason)	No/yes (reason)
1	30/M	Yes	(5) 100-200 mcg qd-bid for 6 month	(4) 500 mg tid for 3 month	(2) 40 mg tapered for 2 month	(3)-(4) 1-2 mg qd for 6 month	(1) 2.5 mg qd for 2 month	Improvement and observation after stopping medical treatment
2	70/M	Yes	(1)-(3) 50-100 mcg qd-bid for continuously	(2) 500 mg tid for 3 month		(3) 1 mg qd for 6 month		Octreotide continuously
3	63/F	Yes						Unable to treat appropriately due to underlying colon cancer
4	54/M	Yes				(1) 2-3 mg qd for continuously		Sirolimus continuously
5	28/F	Yes	(1)-(2) 100 mcg qd for 6 month			(2) 1-2 mg qd for continuously		Sirolimus continuously
6	67/F	Yes	(1) 100 mcg bid for 3 month	(2) 250 mg tid for continuously		(2) 2 mg qd for continuously		Tranexamic acid and sirolimus continuously	Yes (pleural effusion)	Yes (pleural effusion)	Yes (pleural effusion)
7	32/M	Yes						Regular albumin replacement	No	No	No
8	65/M	Yes				(1) 1-2 mg qd for continuously		Sirolimus continuously	No	No	No
9	41/F	Yes			(1) 40 mg tapered for 4 wk	(2) 1-2 mg qd for 1.5 yr		Improvement and observation after stopping medical treatment	Yes (pleural effusion)	No	No
10	35/M	Yes	(1)-(2) 50-200 mcg qd-bid for 2 yr	(4) 250 mg tid for 6 month	(3) 30 mg tapered for 1 month	(2)-(4) 1 mg qd for continuously		Sirolimus continuously	Yes (pleural effusion)	Yes (pleural effusion)	No
11	32/M	Yes	(2) 100 mcg qd-bid for continuously			(1)-(2) 1-4 mg qd for continuously		Octreotide and sirolimus continuously	No	No	No
12	76/F	Yes	(2) 100 mcg qd-bid for continuously			(1) 1 mg qd-bid for 6 month		Octreotide continuously	No	No	No

bid: Bis in die (two times a day); F: Female; M: Male; tid: Ter in die (three times a day); qd: Quaque die (once a day); qid: Quarter in die (four times a day).

Table 4 Complications and prognosis during the follow-up of adult patients with primary intestinal lymphangiectasia

Case number	Age/sex	Hospitalization after diagnosis, frequency per year	Any infection, no/yes (disease)	Any thromboembolism, no/yes (disease)	Mortality, no/yes (cause)
1	30/M	14	Yes (invasive pulmonary aspergillosis, zoster, tuberculosis)	Yes (pulmonary thromboembolism)	No
2	70/M	2	No	No	No
3	63/F	6	Yes (pneumonia, cellulitis, urinary tract infection)	No	Yes (underlying cancer)
4	54/M	1	No	No	No
5	28/F	1	No	No	No
6	67/F	6	Yes (pneumonia)	Yes (cerebral infarction)	Yes (primary intestinal lymphangiectasia)
7	32/M	2	No	No	No
8	65/M	1	No	No	No
9	41/F	2	Yes (zoster)	No	No
10	35/M	7	Yes (catheter-related infection)	Yes (pulmonary thromboembolism)	No
11	32/M	0	No	No	No
12	76/F	3	Yes (spontaneous bacterial peritonitis, pneumonia)	No	No

F: Female; M: Male.