Table 1.
Clinical features of GATA2-deficient patients
Kindred | Code | No. | Mutation | Sex | Origin | Vital status | Mycobacterial species | Other microorganisms | Other clinical features | Transplantation |
---|---|---|---|---|---|---|---|---|---|---|
A | II. 1 | P1 | p.M388T/WT | Female | Brazil | Alive | M. tuberculosis, M. kansasii | S. aureus, HPV | Hypothyroidism, basal cell carcinoma, intermittent anemia, chronic sinusitis | - |
B | II. 4 | P2A | p.R398W/WT | Female | Brazil | Alive | M. kansasii | K. pneumoniae, HPV | Lymphedema | - |
C | II. 1 | P3 | c.915_916del/WT | Male | Colombia | Dead | M. tuberculosis | P. aeruginosa, HPV, Candida spp. | Deafness, cellulitis, spinocellular carcinoma | - |
D | I.1 | P4B | p.R398Q/WT | Female | France | Alive | Mycobacterium spp. | H. influenzae, S. pneumoniae | Mouth ulcers, pneumonia, cellulitis, hemophagocytic syndrome | HSCT |
E | II. 2 | P5B | c.1023del/WT | Female | France | Dead | M. avium, M. kansasii | HPV, Candida spp., Aspergillus spp. | Lymphedema, psoriasis, sinusitis | - |
E | II. 3 | P6B | c.1023del/WT | Female | France | Alive | M. kansasii | HPV | Lymphedema | - |
F | II. 1 | P7 | p.R398W/WT | Male | Germany | Dead | M. kansasii | - | - | - |
G | II. 1 | P8 | p.R396Q/WT | Female | Mexico | Alive | M. kansasii | C. difficile | Obesity, subclavian thrombosis, hepatomegaly | - |
H | II. 1 | P9 | c.599dup/WT | Female | Portugal | Dead | M. avium complex | EBV, Candida spp. | Granulomatous hepatitis | - |
H | II. 2 | P1 0 | c.599dup/WT | Female | Portugal | Dead | Mycobacterium spp. | HPV, HHSV-2 | Mouth ulcers, hemochromatosis | Liver |
I | II. 1 | P11 | p.R330*/WT | Mal e | Spain | Dead | Mycobacterium spp. | Candida spp. | Lymphedema, monoclonal gammapathy, sarcoidosis | - |
J | II. 2 | P12 | p.M388T/WT | Female | Spain | Alive | M. avium intracellulare | S. salivarius, A. fumigatus | Deafness | HSCT |
K | II. 1 | P13 | c.1035_1036insTC TGGCC/WT | Male | Spain | Dead | M. kansasii | C. freundii, Staphylococcus spp., Candida spp. | Inferior vena cava thrombosis, deafness | - |
L | I.1 | P14 | c.599dup/WT | Female | Tunisia | Alive | M. tuberculosis | HPV | Lymphedema, thrombosis | - |
L | II. 1 | P15 | c.599dup/WT | Male | Tunisia | Dead | M. tuberculosis | HPV | Lymphedema | - |
M | II. 1 | P16 | p.R396W/WT | Male | Turkey | Alive | M. tuberculosis | HSV 1, HPV, Molluscum contagiosum | Hepatosplenomegaly, psoriasis, sarcoidosis | HSCT |
N | II. 1 | P17 | c.1099dup/WT | Female | USA | Alive | M. avium intracellulare | C. difficile, S. pneumoniae, H. capsulatum, Brucella spp. | Mouth ulcers | - |
O | II. 1 | P18 | c.1143+2T>A/WT | Female | France | Alive | M. avium | Undetermined | Erythema nodosum | - |
HSCT, Hematopoietic stem cell transplantation; HPV, Human papilloma virus; HSV, Herpes simplex virus; EBV, Epstein-Barr virus; -, Not present; patients previously reported by Jobim et al.60 (P2A) or Donadieu et al.36 (P4-P6B).