Table 2.

Clinical characteristics of somatic ALPS patients

Patient	Original ALPS classification	Sex	Age at presentation	LN size*	Age at splenectomy, y	Cytopenias†	Apoptosis‡
43	Phenotype	M	3 mo	3	6	A,T,N	77
84	Phenotype	M	6 mo	2	—	A,N	61
135	Phenotype	F	1 y	4	—	A,T,N	55
228	Phenotype	F	6 mo	3	—	A,AH,N	102
235	Phenotype	M	48 y	2	—	A	58
241	Phenotype	F	43 y	4	—	N	53
108	Type III	M	1 y	4	5	A,T,N	18
164	Type III	M	17 y	3	—	N	39
256	Type III	M	11 y	4	—	HSP-AG	49
262	Type III	M	12 y	4	—	A	23
263	Type III	M	1 y	3	6 (partial)	A,T,N	18
276	Type III	M	10 y	4	—	A	41

— indicates not applicable.

^*LN size grading: 1 = presence of few shotty nodes; 2 = presence of multiple 1- to 2-cm nodes; 3 = more than 2-cm nodes; and 4 = visible LN or more than 4 cm by CT.

^†A = anemia; T = thrombocytopenia; AH = autoimmune hepatitis; N = neutropenia; HSP-AG = Henoch-Schonlein Purpura-acute glomerulonephritis.

^‡Percent of normal control; less than 50% is considered defective.