Demographic and clinical variables of hospitalized patients who died
| Variable . | Alive* . | Death . | P† . | ||
|---|---|---|---|---|---|
| n . | Results . | n . | Results . | ||
| Age, median (IQR), y | 44 | 31 (23-38) | 7 | 53 (39-68) | <.001 |
| Age ≥21 y | 36 (82) | 7 (100) | .58 | ||
| Female sex | 44 | 23 (52) | 7 | 4 (57) | >.9 |
| Hispanic | 44 | 6 (14) | 7 | 1 (14) | >.9 |
| Genotype | 44 | 7 | .66 | ||
| SS/Sβ0 | 30 (68) | 4 (50) | |||
| SC | 11 (25) | 2 (29) | |||
| Sβ+ | 3 (7) | 1 (14) | |||
| Smoking history | 44 | 10 (23) | 7 | 3 (43) | .35 |
| Body mass index, median (IQR), kg/m2 | 43 | 23.9 (20.4-28.0) | 7 | 25.3 (19.8-26.8) | .81 |
| History | |||||
| Venous thromboembolism‡ | 44 | 10 (23) | 7 | 4 (57) | .08 |
| Pulmonary hypertension | 44 | 8 (18) | 7 | 6 (86) | .001 |
| ACS | 44 | 25 (57) | 7 | 6 (86) | .22 |
| Stroke | 44 | 6 (14) | 7 | 4 (57) | .021 |
| CKD§ | 44 | 16 (36) | 7 | 6 (86) | .034 |
| Diabetes | 44 | 3 (7) | 7 | 2 (29) | .13 |
| Splenectomyǁ | 44 | 7 (16) | 7 | 2 (29) | .59 |
| Congestive heart failure | 44 | 5 (12) | 7 | 4 (57) | .013 |
| Presenting symptoms | |||||
| Pain | 44 | 33 (75) | 7 | 2 (29) | .025 |
| Abnormal chest radiograph at presentation | 43 | 24 (56) | 7 | 7 (100) | .035 |
| Fever | 42 | 3 (7) | 7 | 1 (14) | .47 |
| Hypoxia¶ | 42 | 7 (17) | 7 | 0 | .57 |
| Presenting laboratory tests, median (IQR) | |||||
| Hemoglobin, g/dL | 42 | 8.4 (7.5-10.4) | 7 | 5.8 (4.6-9.7) | .07 |
| White blood cells, ×109/L | 42 | 11.3 (6.5-16.1) | 7 | 11.3 (10.6-21.3) | .30 |
| Lymphocyte count, ×109/L | 42 | 2.1 (1.2-3.6) | 7 | 2.2 (1.6-3.0) | .81 |
| NLR | 42 | 3.0 (1.8-5.2) | 7 | 3.5 (2.3-8.0) | .34 |
| Platelet, ×109/L | 42 | 321 (207-376) | 7 | 277 (201-344) | .63 |
| Creatinine, mg/dL | 37 | 0.8 (0.6-1.0) | 7 | 4.1 (2.0-8.5) | <.001 |
| CRP, mg/dL | 26 | 11.9 (4.8-24) | 2 | 29.8 | .20 |
| LDH, U/L | 35 | 431 (313-583) | 7 | 631 (462-1293) | .012 |
| D-dimer, μg/mL | 20 | 1.9 (0.9-3.4) | 5 | 9.2 (2.1-12.7) | .011# |
| Treatment during hospitalization | |||||
| HU | 44 | 16 (36) | 7 | 2 (29) | >.9 |
| Blood transfusion | 44 | 25 (57) | 7 | 5 (71) | .69 |
| Anticoagulation | 44 | 30 (68) | 7 | 2 (29) | .09 |
| Mechanical ventilation | 44 | 2 (5) | 7 | 3 (43) | .015 |
| ICU admission | 44 | 5 (11) | 7 | 1 (14) | >.9 |
| LOS | 44 | 5 (3-10) | 7 | 8 (1-11) | .75 |
| Treatment (chronic) | |||||
| HU | 44 | 18 (41) | 7 | 0 | .042 |
| Chronic transfusion | 44 | 4 (9) | 7 | 0 | >.9 |
| l-glutamine | 44 | 6 (13) | 7 | 0 | .58 |
| Crizanlizumab | 44 | 1 (2) | 7 | 0 | >.9 |
| Voxelotor | 44 | 2 (5) | 7 | 0 | >.9 |
| Any disease modifier | 44 | 24 (55) | 7 | 0 | .011 |
| Variable . | Alive* . | Death . | P† . | ||
|---|---|---|---|---|---|
| n . | Results . | n . | Results . | ||
| Age, median (IQR), y | 44 | 31 (23-38) | 7 | 53 (39-68) | <.001 |
| Age ≥21 y | 36 (82) | 7 (100) | .58 | ||
| Female sex | 44 | 23 (52) | 7 | 4 (57) | >.9 |
| Hispanic | 44 | 6 (14) | 7 | 1 (14) | >.9 |
| Genotype | 44 | 7 | .66 | ||
| SS/Sβ0 | 30 (68) | 4 (50) | |||
| SC | 11 (25) | 2 (29) | |||
| Sβ+ | 3 (7) | 1 (14) | |||
| Smoking history | 44 | 10 (23) | 7 | 3 (43) | .35 |
| Body mass index, median (IQR), kg/m2 | 43 | 23.9 (20.4-28.0) | 7 | 25.3 (19.8-26.8) | .81 |
| History | |||||
| Venous thromboembolism‡ | 44 | 10 (23) | 7 | 4 (57) | .08 |
| Pulmonary hypertension | 44 | 8 (18) | 7 | 6 (86) | .001 |
| ACS | 44 | 25 (57) | 7 | 6 (86) | .22 |
| Stroke | 44 | 6 (14) | 7 | 4 (57) | .021 |
| CKD§ | 44 | 16 (36) | 7 | 6 (86) | .034 |
| Diabetes | 44 | 3 (7) | 7 | 2 (29) | .13 |
| Splenectomyǁ | 44 | 7 (16) | 7 | 2 (29) | .59 |
| Congestive heart failure | 44 | 5 (12) | 7 | 4 (57) | .013 |
| Presenting symptoms | |||||
| Pain | 44 | 33 (75) | 7 | 2 (29) | .025 |
| Abnormal chest radiograph at presentation | 43 | 24 (56) | 7 | 7 (100) | .035 |
| Fever | 42 | 3 (7) | 7 | 1 (14) | .47 |
| Hypoxia¶ | 42 | 7 (17) | 7 | 0 | .57 |
| Presenting laboratory tests, median (IQR) | |||||
| Hemoglobin, g/dL | 42 | 8.4 (7.5-10.4) | 7 | 5.8 (4.6-9.7) | .07 |
| White blood cells, ×109/L | 42 | 11.3 (6.5-16.1) | 7 | 11.3 (10.6-21.3) | .30 |
| Lymphocyte count, ×109/L | 42 | 2.1 (1.2-3.6) | 7 | 2.2 (1.6-3.0) | .81 |
| NLR | 42 | 3.0 (1.8-5.2) | 7 | 3.5 (2.3-8.0) | .34 |
| Platelet, ×109/L | 42 | 321 (207-376) | 7 | 277 (201-344) | .63 |
| Creatinine, mg/dL | 37 | 0.8 (0.6-1.0) | 7 | 4.1 (2.0-8.5) | <.001 |
| CRP, mg/dL | 26 | 11.9 (4.8-24) | 2 | 29.8 | .20 |
| LDH, U/L | 35 | 431 (313-583) | 7 | 631 (462-1293) | .012 |
| D-dimer, μg/mL | 20 | 1.9 (0.9-3.4) | 5 | 9.2 (2.1-12.7) | .011# |
| Treatment during hospitalization | |||||
| HU | 44 | 16 (36) | 7 | 2 (29) | >.9 |
| Blood transfusion | 44 | 25 (57) | 7 | 5 (71) | .69 |
| Anticoagulation | 44 | 30 (68) | 7 | 2 (29) | .09 |
| Mechanical ventilation | 44 | 2 (5) | 7 | 3 (43) | .015 |
| ICU admission | 44 | 5 (11) | 7 | 1 (14) | >.9 |
| LOS | 44 | 5 (3-10) | 7 | 8 (1-11) | .75 |
| Treatment (chronic) | |||||
| HU | 44 | 18 (41) | 7 | 0 | .042 |
| Chronic transfusion | 44 | 4 (9) | 7 | 0 | >.9 |
| l-glutamine | 44 | 6 (13) | 7 | 0 | .58 |
| Crizanlizumab | 44 | 1 (2) | 7 | 0 | >.9 |
| Voxelotor | 44 | 2 (5) | 7 | 0 | >.9 |
| Any disease modifier | 44 | 24 (55) | 7 | 0 | .011 |
Data are presented as n (%) unless otherwise identified. Bold P values indicate statistically significant results (P < .05).
CRP, C-reactive protein; ICU, intensive care unit; LDH, lactate dehydrogenase.
Limited to hospitalized patients.
P value comparing patients who died vs those who survived.
Includes pulmonary embolism.
Defined according to local guidelines at each institution.
Indicates surgical splenectomy.
Indicated by oxygen saturation <92%.
P value from the permutation Student t test.