Table 1. Patient characteristics and... | American Society of Hematology

Table 1.

Patient characteristics and initial COVID-19 disease course

Patient	Sex and age	CTLA4 variant	CTLA4 manifestations	Baseline ALC (cells per mL)	Auto-Abs to type 1 IFNs	Immunomo-dulatory treatment	Initial SARS-CoV-2 testing	Manifestations of COVID-19	COVID-19 severity	Treatment start, d∗	COVID-19 treatment	Symptom resolution
1	M 20 y	c.223C>T (R75W)	AIHA, ITP CNS infiltrate CVID Enteritis GH deficiency HSM ILD LAD	810	Neg	Everolimus Abatacept IVIG	PCR	Nasal congestion Anosmia	Mild	D 6	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose Azithromycin 250 mg daily for 7 d	D 7
2	F 34 y	c.151C>T (R51X)	AI CVID Lymphopenia Neutropenia PA	410	Neg	Sirolimus IVIG	Antigen	Nasal congestion Anosmia Nausea Sore throat Cough Muscle pain Joint pain	Mild	D 4	Bamlavinimab 700 mg 1 dose	D 7
3	M 27 y	c.436G>A (G146R)	AI Arthritis Bronchiectasis CVID Enteritis HSM HT ILD Neutropenia, ITP PA	690	Neg	Sirolimus Abatacept IVIG	Antigen	Nasal congestion Anosmia Sore throat Cough SOB Fever Night sweats Fatigue Pulmonary infiltrates Hypoxemia	Severe	D 7	Remdesevir 200 mg 1 dose followed by 100 mg daily for 2 d Meropenem 1g q 8 h for 3 d Azithromycin 250 mg daily for 7 d Dexamethasone ASA 81 daily	D 30
4	M 20 y	c.410C>T (P137L)	ILD LAD Neutropenia Subclinical HT	1520	Neg	Everolimus	PCR	Fever Sore throat Headache	Mild	D 2	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 4
5	F 19 y	c.436G>A (G146R)	AIHA, ITP Neutropenia Chronic Norovirus enteritis CNS infiltrate CVID HSM ILD LAD	540	Neg	IVIG	PCR	Fatigue Sore throat Cough Fever	Mild	D 3	Bamlavinimab 700mg 1 dose	D 7
6	F 54 y	c.436G>A (G146R)	Hypogammaglobulinemia	2710	Neg	None	PCR	Anosmia Fatigue Fever Headache Nasal congestion Sore throat	Mild	NA	None	D 7
7	M 36 y	c.151C>T (p.R51X)	Arthritis CVID ITP T1DM	2740	Neg	IVIG	PCR	Chest pain Cough Fever Muscle pain	Mild	D 5	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 13
8	M 38 y	c.151C>T p.Arg51Ter	AIHA, ITP CNS infiltrates CVID	1211	Unk	Abatacept SQIG	PCR	Cough Diarrhea Nasal congestion Nausea	Mild	None	None	D 5
9	M 27 y	c.1A>G p.Met1	Aplastic anemia CVID Enteropathy	570	Neg	Sirolimus IVIG	PCR	Cough Diarrhea Headache Nasal congestion	Mild	D 13	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	Unk
10	M 24 y	c.223C>T p.Arg75Trp	AIHA, ITP CNS infiltrates CVID Enteropathy HT ILD LAD	350	Neg	Abatacept IVIG	PCR	Fever Headache Nasal congestion	Mild	D 2	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 18‡
11	M 44 y	75delG (L28FfS†44)	AIHA, ITP CNS infiltrates CVID Enteropathy HSM ILD LN	760	Neg	Abatacept	PCR	Fever Muscle pain Sore throat	Mild	D 5	Sotrovimab 500 mg Remdesevir 200 mg 1 dose followed by 100 mg daily for 2 d	D 7
12	M 20 y	410C>T (P137L)	CNS infiltrates Enteropathy ITP Neutropenia LN	1310	Neg	Abatacept Sirolimus	PCR	Fever Headache Sore throat		D 1	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 2
13	F 5 y	410C>T (P137L)	HT	1500	Unk	None	PCR	Fatigue	Mild	D 11	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 13†
14	F 48 y	410C>T (P137L)	Asymptomatic	1960	Unk	None	PCR	Fatigue Cough Nasal congestions	Mild	1	Remdesevir 200 mg 1 dose followed by 100 mg daily for 4 d	D 30
15	M 27 y	223C>T (R75W)	CNS infiltrates CVID Enteropathy HSM ILD	2760	Unk	IVIG Prednisone	PCR	Nasal congestion	Mild	NA	None	D 15
16	M 19 y	567+5G>C	AIHA CNS infiltrates CVID Eczema Enteropathy HSM ILD	890	Unk	IVIG Sirolimus	PCR	Nasal congestion	Mild	4	Sotrovimab 500 mg 1 dose	D 6
17	M 51 y	223C>T (R75W)	Asymptomatic	2610	Unk	None	PCR	Asymptomatic		NA	None	D 11
18	F 45 y	c.371_372insG (L126TfsTer28)	AI Arthritis Colitis CVID Eczema HT ITP LN PA Vitiligo	7960	Unk	IVIG Abatacept Prednisolone Budesonide	PCR	Cough Fever	Mild	D 4	Remdesevir 200 mg 1 dose followed by 100 mg daily for 4 d	D 7
19	F 32 y	c.416A>G (Y139C)	Alopecia Eczema HT	Unk	Unk	None	PCR	Anosmia Cough SOB	Moderate	NA	None	D 30
20	F 8 mo	c.416A>G (Y139C)	Arthritis	Unk	Unk	Prednisone	PCR	Fever Nasal congestion	Mild	NA	None	D 8
21	F 19 y	c.487delC (L163SfsTer24)	AIHA Colitis CVID ITP Psoriasis	410	Unk	IVIG Prednisolone	PCR	Anosmia Cough Fatigue Fever Headache Muscle Ache	Mild	D 2	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 8
22	F 34 y	c.280 G>T (p.Glu94X)	Arthritis CVID Enteropathy HT	950	Unk	Sirolimus Abatacept IVIG	Antigen	Fatigue	Mild	None	None	D 20

Patient	Sex and age	CTLA4 variant	CTLA4 manifestations	Baseline ALC (cells per mL)	Auto-Abs to type 1 IFNs	Immunomo-dulatory treatment	Initial SARS-CoV-2 testing	Manifestations of COVID-19	COVID-19 severity	Treatment start, d∗	COVID-19 treatment	Symptom resolution
1	M 20 y	c.223C>T (R75W)	AIHA, ITP CNS infiltrate CVID Enteritis GH deficiency HSM ILD LAD	810	Neg	Everolimus Abatacept IVIG	PCR	Nasal congestion Anosmia	Mild	D 6	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose Azithromycin 250 mg daily for 7 d	D 7
2	F 34 y	c.151C>T (R51X)	AI CVID Lymphopenia Neutropenia PA	410	Neg	Sirolimus IVIG	Antigen	Nasal congestion Anosmia Nausea Sore throat Cough Muscle pain Joint pain	Mild	D 4	Bamlavinimab 700 mg 1 dose	D 7
3	M 27 y	c.436G>A (G146R)	AI Arthritis Bronchiectasis CVID Enteritis HSM HT ILD Neutropenia, ITP PA	690	Neg	Sirolimus Abatacept IVIG	Antigen	Nasal congestion Anosmia Sore throat Cough SOB Fever Night sweats Fatigue Pulmonary infiltrates Hypoxemia	Severe	D 7	Remdesevir 200 mg 1 dose followed by 100 mg daily for 2 d Meropenem 1g q 8 h for 3 d Azithromycin 250 mg daily for 7 d Dexamethasone ASA 81 daily	D 30
4	M 20 y	c.410C>T (P137L)	ILD LAD Neutropenia Subclinical HT	1520	Neg	Everolimus	PCR	Fever Sore throat Headache	Mild	D 2	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 4
5	F 19 y	c.436G>A (G146R)	AIHA, ITP Neutropenia Chronic Norovirus enteritis CNS infiltrate CVID HSM ILD LAD	540	Neg	IVIG	PCR	Fatigue Sore throat Cough Fever	Mild	D 3	Bamlavinimab 700mg 1 dose	D 7
6	F 54 y	c.436G>A (G146R)	Hypogammaglobulinemia	2710	Neg	None	PCR	Anosmia Fatigue Fever Headache Nasal congestion Sore throat	Mild	NA	None	D 7
7	M 36 y	c.151C>T (p.R51X)	Arthritis CVID ITP T1DM	2740	Neg	IVIG	PCR	Chest pain Cough Fever Muscle pain	Mild	D 5	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 13
8	M 38 y	c.151C>T p.Arg51Ter	AIHA, ITP CNS infiltrates CVID	1211	Unk	Abatacept SQIG	PCR	Cough Diarrhea Nasal congestion Nausea	Mild	None	None	D 5
9	M 27 y	c.1A>G p.Met1	Aplastic anemia CVID Enteropathy	570	Neg	Sirolimus IVIG	PCR	Cough Diarrhea Headache Nasal congestion	Mild	D 13	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	Unk
10	M 24 y	c.223C>T p.Arg75Trp	AIHA, ITP CNS infiltrates CVID Enteropathy HT ILD LAD	350	Neg	Abatacept IVIG	PCR	Fever Headache Nasal congestion	Mild	D 2	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 18‡
11	M 44 y	75delG (L28FfS†44)	AIHA, ITP CNS infiltrates CVID Enteropathy HSM ILD LN	760	Neg	Abatacept	PCR	Fever Muscle pain Sore throat	Mild	D 5	Sotrovimab 500 mg Remdesevir 200 mg 1 dose followed by 100 mg daily for 2 d	D 7
12	M 20 y	410C>T (P137L)	CNS infiltrates Enteropathy ITP Neutropenia LN	1310	Neg	Abatacept Sirolimus	PCR	Fever Headache Sore throat		D 1	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 2
13	F 5 y	410C>T (P137L)	HT	1500	Unk	None	PCR	Fatigue	Mild	D 11	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 13†
14	F 48 y	410C>T (P137L)	Asymptomatic	1960	Unk	None	PCR	Fatigue Cough Nasal congestions	Mild	1	Remdesevir 200 mg 1 dose followed by 100 mg daily for 4 d	D 30
15	M 27 y	223C>T (R75W)	CNS infiltrates CVID Enteropathy HSM ILD	2760	Unk	IVIG Prednisone	PCR	Nasal congestion	Mild	NA	None	D 15
16	M 19 y	567+5G>C	AIHA CNS infiltrates CVID Eczema Enteropathy HSM ILD	890	Unk	IVIG Sirolimus	PCR	Nasal congestion	Mild	4	Sotrovimab 500 mg 1 dose	D 6
17	M 51 y	223C>T (R75W)	Asymptomatic	2610	Unk	None	PCR	Asymptomatic		NA	None	D 11
18	F 45 y	c.371_372insG (L126TfsTer28)	AI Arthritis Colitis CVID Eczema HT ITP LN PA Vitiligo	7960	Unk	IVIG Abatacept Prednisolone Budesonide	PCR	Cough Fever	Mild	D 4	Remdesevir 200 mg 1 dose followed by 100 mg daily for 4 d	D 7
19	F 32 y	c.416A>G (Y139C)	Alopecia Eczema HT	Unk	Unk	None	PCR	Anosmia Cough SOB	Moderate	NA	None	D 30
20	F 8 mo	c.416A>G (Y139C)	Arthritis	Unk	Unk	Prednisone	PCR	Fever Nasal congestion	Mild	NA	None	D 8
21	F 19 y	c.487delC (L163SfsTer24)	AIHA Colitis CVID ITP Psoriasis	410	Unk	IVIG Prednisolone	PCR	Anosmia Cough Fatigue Fever Headache Muscle Ache	Mild	D 2	Casirivimab 1200 mg Imdevimab 1200 mg 1 dose	D 8
22	F 34 y	c.280 G>T (p.Glu94X)	Arthritis CVID Enteropathy HT	950	Unk	Sirolimus Abatacept IVIG	Antigen	Fatigue	Mild	None	None	D 20

Abs, antibodies; AI, adrenal insufficiency; AIHA, autoimmune hemolytic anemia; ALC, absolute lymphocyte count; CNS, central nervous system; CVID, common variable immunodeficiency; F, female; GH, growth hormone; HSM, hepatosplenomegaly; HT, hypothyroidism; ILD, interstitial lung disease; ITP, immune thrombocytopenic purpura; LAD, lymphadenopathy; M, male; N/A, not applicable; Neg, negative; PA, pernicious anemia; PCR, polymerase chain reaction; SOB, shortness of breath; SQIG, subcutaneous immunoglobulin; Tx, treatment; Unk, unknown; q, daily.

∗

Day 0 was defined as the first day of symptoms.

†

Based on a previously described ordinal scale: 1, not hospitalized and no limitations of activities; 2, not hospitalized, with limitation of activities, home oxygen requirement, or both; 3, hospitalized, not requiring supplemental oxygen and no longer requiring ongoing medical care (used if hospitalization was extended for infection-control or other nonmedical reasons); 4, hospitalized, not requiring supplemental oxygen but requiring ongoing medical care (related to COVID-19 or to other medical conditions); 5, hospitalized, requiring any supplemental oxygen; 6, hospitalized, requiring noninvasive ventilation or use of high-flow oxygen devices; 7, hospitalized, receiving invasive mechanical ventilation or extracorporeal membrane oxygenation; and 8, death.¹⁴

‡

Persistent fatigue after symptom resolution.

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