Table 2.

Population characteristics at the time of TPO-RAs introduction

Patient and sexITP duration, moAge at diagnosis of PID/ITP/introduction of TPO-RA, yIgG/IgA/IgM levels at PID diagnosis, mg/dL∗ Type of PIDRecurrent infections†/IRTOther PID-related manifestations
1 M 294 16/5/29 321/37/12 LOCID Yes Yes Lymphadenopathy, splenomegaly, GLILD, bronchiectasis, AIHA, HPV infections, PSVD 
2 F <1 19/19/19 250/15/28 CVID Yes No Splenomegaly, autoimmunity, hepatomegaly 
3 M 168 24/9/23 150/0/500 HIGM Yes No Lymphadenopathy, granulomas, MALT lymphoma 
4 M 295 33/12/37 85/0/4 LOCID Yes Yes Lymphadenopathy, splenomegaly, granulomas, bronchiectasis, HPV infections, PSVD 
5 F 3 49/48/48 300/18/8 CVID Yes No Lymphadenopathy, splenomegaly, GLILD 
6 M 5 22/22/23 460/33/39 CVID Yes No Autoimmunity, enteropathy 
7 F 127 14/14/25 250/50/50 CVID Yes Yes Lymphadenopathy, splenomegaly, AIHA 
8 M <1 28/30/30 <500/‡ /‡  CVID Yes Yes Lymphadenopathy, splenomegaly, granulomas, autoimmunity 
9 M <1 16/29/29 <500/‡/‡  CVID Yes Yes Lymphadenopathy, splenomegaly, granulomas, bronchiectasis, autoimmunity, enteropathy 
10 F ‡  33/34/34 320/5/25 CVID Yes Yes Splenomegaly, bronchiectasis 
11 F 55 68/68/72 438/146/33 CVID Yes No Lymphoma, bronchiectasis, ANCA associated vasculitis 
12 F 110 25/26/35 223/6/48 LOCID Yes Yes Splenomegaly, GLILD, lymphoid hyperplasia, AIHA, AIN, allergies, basal cell carcinoma, enteropathy, PSVD 
13 M 188 ‡/32/47 <500/‡/‡  CVID No No AIHA 
14 F 167 46/31/44 486/40/46 CTLA4 Yes No Lymphadenopathy, psoriasis, AIHA, allergies, HPV infections and related cancer, celiac disease 
15 M 64 20/19/25 450/42/47 CVID Yes No Lymphadenopathy, splenomegaly, autoimmunity 
16 M 62 26/26/31 450/47/‡  CVID Yes No Lymphoid hyperplasia, granulomas, AIHA, enteropathy 
17 F 27 32/31/34 430/26/45 CVID Yes No Lymphadenopathy, granulomas, psoriasis, AIHA, B12 deficiency 
18 F 191 50/50/66 426/104/109 CVID Yes Yes Lymphadenopathy, GLILD, LGL leukemia, bronchiectasis, AIHA and AIN 
19 M 21 43/43/45 350/20/45 CVID Yes Yes Allergies, AIN 
20 M 1 27/34/35 130/0/37 CVID Yes No Lymphadenopathy, splenomegaly, AIHA 
21 M 249 51/33/54 187/32/126 PI3KD No No AIHA and AIN 
22 F 1 18/18/18 396/25/33 CVID No No Lymphadenopathy, splenomegaly 
23 F 48 ‡/55/59 197/28/17 CVID Yes Yes - 
24 M 4 42/42/42 452/32/40 CVID No No - 
25 M 54 52/53/57 302/6/17 CVID No No Splenomegaly, autoimmunity 
26 M 47 36/39/43 <500/‡/‡  CVID No No - 
27 M 13 56/58/59 330/69/30 CVID Yes No - 
28 M 111 15/13/22 398/17/60 Kabuki No Yes AIHA and AIN, cholesteatoma 
Patient and sexITP duration, moAge at diagnosis of PID/ITP/introduction of TPO-RA, yIgG/IgA/IgM levels at PID diagnosis, mg/dL∗ Type of PIDRecurrent infections†/IRTOther PID-related manifestations
1 M 294 16/5/29 321/37/12 LOCID Yes Yes Lymphadenopathy, splenomegaly, GLILD, bronchiectasis, AIHA, HPV infections, PSVD 
2 F <1 19/19/19 250/15/28 CVID Yes No Splenomegaly, autoimmunity, hepatomegaly 
3 M 168 24/9/23 150/0/500 HIGM Yes No Lymphadenopathy, granulomas, MALT lymphoma 
4 M 295 33/12/37 85/0/4 LOCID Yes Yes Lymphadenopathy, splenomegaly, granulomas, bronchiectasis, HPV infections, PSVD 
5 F 3 49/48/48 300/18/8 CVID Yes No Lymphadenopathy, splenomegaly, GLILD 
6 M 5 22/22/23 460/33/39 CVID Yes No Autoimmunity, enteropathy 
7 F 127 14/14/25 250/50/50 CVID Yes Yes Lymphadenopathy, splenomegaly, AIHA 
8 M <1 28/30/30 <500/‡ /‡  CVID Yes Yes Lymphadenopathy, splenomegaly, granulomas, autoimmunity 
9 M <1 16/29/29 <500/‡/‡  CVID Yes Yes Lymphadenopathy, splenomegaly, granulomas, bronchiectasis, autoimmunity, enteropathy 
10 F ‡  33/34/34 320/5/25 CVID Yes Yes Splenomegaly, bronchiectasis 
11 F 55 68/68/72 438/146/33 CVID Yes No Lymphoma, bronchiectasis, ANCA associated vasculitis 
12 F 110 25/26/35 223/6/48 LOCID Yes Yes Splenomegaly, GLILD, lymphoid hyperplasia, AIHA, AIN, allergies, basal cell carcinoma, enteropathy, PSVD 
13 M 188 ‡/32/47 <500/‡/‡  CVID No No AIHA 
14 F 167 46/31/44 486/40/46 CTLA4 Yes No Lymphadenopathy, psoriasis, AIHA, allergies, HPV infections and related cancer, celiac disease 
15 M 64 20/19/25 450/42/47 CVID Yes No Lymphadenopathy, splenomegaly, autoimmunity 
16 M 62 26/26/31 450/47/‡  CVID Yes No Lymphoid hyperplasia, granulomas, AIHA, enteropathy 
17 F 27 32/31/34 430/26/45 CVID Yes No Lymphadenopathy, granulomas, psoriasis, AIHA, B12 deficiency 
18 F 191 50/50/66 426/104/109 CVID Yes Yes Lymphadenopathy, GLILD, LGL leukemia, bronchiectasis, AIHA and AIN 
19 M 21 43/43/45 350/20/45 CVID Yes Yes Allergies, AIN 
20 M 1 27/34/35 130/0/37 CVID Yes No Lymphadenopathy, splenomegaly, AIHA 
21 M 249 51/33/54 187/32/126 PI3KD No No AIHA and AIN 
22 F 1 18/18/18 396/25/33 CVID No No Lymphadenopathy, splenomegaly 
23 F 48 ‡/55/59 197/28/17 CVID Yes Yes - 
24 M 4 42/42/42 452/32/40 CVID No No - 
25 M 54 52/53/57 302/6/17 CVID No No Splenomegaly, autoimmunity 
26 M 47 36/39/43 <500/‡/‡  CVID No No - 
27 M 13 56/58/59 330/69/30 CVID Yes No - 
28 M 111 15/13/22 398/17/60 Kabuki No Yes AIHA and AIN, cholesteatoma 

AIHA, autoimmune hemolytic anemia; AIN, autoimmune neutropenia; ANCA, anti-neutrophil cytoplasmic antibodies; F, female; GLILD, granulomatous-lymphocytic interstitial lung disease; HPV, human papillomavirus; IRT, immunoglobulin replacement therapy; LGL, large granular lymphocytic; M, male; MALT, mucosa-associated lymphoid tissue.

∗

Normal values (ranges): IgG, 650 to 1400 mg/dL; IgA, 70 to 320 mg/dL; IgM, 50 to 350 mg/dL.

†

Recurrent sino-pulmonary infections.

‡

Missing data.

View Large
Close Modal
This Feature Is Available To Subscribers Only

or Create an Account

Close Modal
Close Modal