Table 1. Clinical characteristics of... | American Society of Hematology

Table 1.

Clinical characteristics of European patients with CD40 ligand deficiency

Case	Age at Tx, y	PCP	Respiratory status	Liver histology	Cryptosporidium infection
1	1	+	Normal	Not evaluated	−
2	1.2	+	Normal	Normal	−
3	2	+	Normal	Not evaluated	−
4	2	+	Normal	Not evaluated	−
5	3	−	Normal	Not evaluated	−
6	3.5	+	Normal	Not evaluated	−
7	3.75	−	Normal	Sclerosing cholangitis	+
8	4	−	Normal	Not evaluated	−
9	4	−	Normal	Not evaluated	−
10	4	−	Normal	Cirrhosis	+
11	4	+	Bronchiectasis	Not evaluated	−
12	4.5	+	Normal	Not evaluated	+
13	4.5	−	Normal	Normal	−
14	4.5	−	Normal	Not evaluated	−
15	7	−	Normal	Sclerosing cholangitis	+
16	7	+	Normal	Normal	−
17	7.3	−	Normal	Sclerosing cholangitis	+
18	8	+	Normal	Sclerosing cholangitis	+
19	8	−	Bronchiectasis	Portal inflammation	+
20	8.7	−	Normal	Sclerosing cholangitis	+
21	9.5	−	Normal	Normal	−
22	9.5	+	Normal	Normal	+^*
23	10	+	Normal	Sclerosing cholangitis	−
24	10	−	Normal	Sclerosing cholangitis cirrhosis	+
25	11	+	Normal	Sclerosing cholangitis	+
26	11.5	−	Normal	Sclerosing cholangitis	+
27	11.5	+	Bronchiectasis	Sclerosing cholangitis cirrhosis	+
28	11.5	−	Normal	Not evaluated	−
29	13	+	Bronchiectasis	Portal inflammation	+
30	13	−	Normal	Sclerosing cholangitis	+
31	14	−	Normal	Fibrosis	+
32	14	+	Bronchiectasis	Sclerosing cholangitis	+
33	15	−	Normal	Normal	−
34	15.7	−	Normal	Sclerosing cholangitis	+
35	16	+	Intrapulmonary calcification	Sclerosing cholangitis atypical sarcoma	−
36	16.5	−	Normal	Sclerosing cholangitis	+^*
37	18	−	Normal	Sclerosing cholangitis cirrhosis	−
38	19	−	Bronchiectasis	Not evaluated	−

Case	Age at Tx, y	PCP	Respiratory status	Liver histology	Cryptosporidium infection
1	1	+	Normal	Not evaluated	−
2	1.2	+	Normal	Normal	−
3	2	+	Normal	Not evaluated	−
4	2	+	Normal	Not evaluated	−
5	3	−	Normal	Not evaluated	−
6	3.5	+	Normal	Not evaluated	−
7	3.75	−	Normal	Sclerosing cholangitis	+
8	4	−	Normal	Not evaluated	−
9	4	−	Normal	Not evaluated	−
10	4	−	Normal	Cirrhosis	+
11	4	+	Bronchiectasis	Not evaluated	−
12	4.5	+	Normal	Not evaluated	+
13	4.5	−	Normal	Normal	−
14	4.5	−	Normal	Not evaluated	−
15	7	−	Normal	Sclerosing cholangitis	+
16	7	+	Normal	Normal	−
17	7.3	−	Normal	Sclerosing cholangitis	+
18	8	+	Normal	Sclerosing cholangitis	+
19	8	−	Bronchiectasis	Portal inflammation	+
20	8.7	−	Normal	Sclerosing cholangitis	+
21	9.5	−	Normal	Normal	−
22	9.5	+	Normal	Normal	+^*
23	10	+	Normal	Sclerosing cholangitis	−
24	10	−	Normal	Sclerosing cholangitis cirrhosis	+
25	11	+	Normal	Sclerosing cholangitis	+
26	11.5	−	Normal	Sclerosing cholangitis	+
27	11.5	+	Bronchiectasis	Sclerosing cholangitis cirrhosis	+
28	11.5	−	Normal	Not evaluated	−
29	13	+	Bronchiectasis	Portal inflammation	+
30	13	−	Normal	Sclerosing cholangitis	+
31	14	−	Normal	Fibrosis	+
32	14	+	Bronchiectasis	Sclerosing cholangitis	+
33	15	−	Normal	Normal	−
34	15.7	−	Normal	Sclerosing cholangitis	+
35	16	+	Intrapulmonary calcification	Sclerosing cholangitis atypical sarcoma	−
36	16.5	−	Normal	Sclerosing cholangitis	+^*
37	18	−	Normal	Sclerosing cholangitis cirrhosis	−
38	19	−	Bronchiectasis	Not evaluated	−

Tx indicates transplantation; PCP, Pneumocystis carinii pneumonia.

Positive on PCR testing only.