From: Manifestations of HbSE sickle cell disease: a systematic review
Author | Study type | Patients (N) and nationality | Age (years) | Hgb (g/dL) and Hb electrophoresis (%) | Manifestations/complications | Treatment | Outcomea |
---|---|---|---|---|---|---|---|
Sex | |||||||
Acipayam et al. [10] | RS | 20, Turkey | 21.45 ± 15 y | Hgb: 12.06 | 3 VOC (1 stroke) | HU = 1 | Alive |
8 Males | HBS: 59.5 | ||||||
12 Females | HBF: 1.9 | 17 asymptomatic | |||||
HBE: 34.5 | |||||||
Aksoy [11] | CR | 1, Turkey | 63 y | Hgb: 8.4 | Splenomegaly | None | Alive |
Female | HBF: 0.8 | ||||||
Altay et al. [12] | CR | 1, Georgia | 20 y | Hgb: 12.7 | NA | None | Alive |
Female | HBS: 64.2 | ||||||
HBF: 1.3 | |||||||
HBE + HBA2: 34.6 | |||||||
Andino et al. [13] | CR | 1, USA | 24Â y | Hgb: 9 | NA | None | Alive |
Female | HBS: 69.8 | ||||||
 | HBE: 30.2 | ||||||
Arbefeville et al. [14] | CR | 1, USA | 12Â y | Hgb: NA | VOC causing ischemia and cardiopulmonary collapse | None | Death |
Male | HBS: 57.4 | ||||||
HBE: 34.2 | |||||||
Baciu et al. [15] | CR | 1, USA | 56Â y | Hgb: 13.3 | Retinopathy | None | Alive |
Male | HBS: 64.1 | ||||||
HBF: 1.2 | |||||||
HBE: 34.7 | |||||||
Bird et al. [16] | CR | 1, South Africa | NA | HBE: 28 | NA | None | Alive |
Eichhorn et al. [17] | CR | 1, Turkey | 22Â y | Hgb: 10.8 | VOC, pain crisis | Transfusion | Alive |
Female | HBS: 60 | Splenomegaly | |||||
HBE: 40 | Parvovirus b19 | ||||||
Englestad [18] | CR | 1, USA | 34Â y Female | Hgb: 10 | VOC, pain crisis, chronic pain, hemolytic anemia, pneumonia, acute splenic sequestration, functional asplenia, splenomegaly, retinopathy | Transfusion, splenectomy | Alive |
HBS: 60 | |||||||
HBF: 40 | |||||||
Ganesh et al. [19] | CR | 1, Omani | 23Â y Male | HBS: 66 | None | Alive | |
HBF: 0.5 | |||||||
HBE: 33.5 | |||||||
George et al. [20] | CR | NA | NA | NA | Avascular necrosis | NA | NA |
Gupta et al. [21] | CR | 1, Pakistani | 28Â y Female | Hgb: 12.9 | NA | None | Alive |
HBS: 60 | |||||||
HBF: 4 | |||||||
HBE: 36 | |||||||
Gürkan [22] | CR | 1, USA | 1 y Male | Hgb: 10 | VOC, pain crisis | None | Alive |
HBS: 58.9 | |||||||
HBF: 5.2 | |||||||
HBE + HBA2: 37.5 | |||||||
Hardy et al. [23] | CR | 2, Saudi Arabia | 1Â y | Hgb: 11.9 | 1 VOC | None | Alive |
Male | HBS: 70 | ||||||
6Â y | HBE: 30 | ||||||
Female | |||||||
Italia et al. [24] | RS | 4, India | 20.75Â y | Hgb: 11.05 | 3 VOC, 1 HA, 1 splenomegaly, 1 chronic pain, pneumonia, ACS | None | Alive |
1 Male | HBS: 58.55 | ||||||
3 Females | HBF: 3.6 | ||||||
HBE: 26.2 | |||||||
Ibrahim K et al. [25] | CR | 1, Qatar | 17Â y | Hgb: 13.3 | HU, transfusion | Alive | |
Male | HBS: 67.1 | ||||||
HBF: 2.2 | |||||||
HBE: 28.7 | |||||||
Knox-Macaulay et al. [7] | RS | 12, Oman | 17.8Â y | Hgb: 12 | 1 ACS | None | Alive |
8 Males | HBS: 63.5 | 2 VOC | |||||
4 Females | HBF: 2.3 | 1 frontal bossing | |||||
HBE: 32.7 | 1 recurrent UTIs | ||||||
Masiello et al. [9] | CR | 1, USA | 1Â y Male | Hgb: 10 | VOC | None | Alive |
HBS: 58.9 | |||||||
HBF: 5.2 | |||||||
HBE + HBA2: 37.5 | |||||||
Mishra et al. [26] | CR | 2, India | 7Â y | Hgb: 9.3 | Splenomegaly | None | Alive |
Male | HBS: 58.05 | HA | |||||
20Â y | HBF: 2.8 | Â | |||||
Female | HBE + HBA2 = 35.4 |  | |||||
Mukhopadhyay et al. [27] | CR | 1, India | 26Â y Male | Hgb: NA | NA | None | Alive |
HBS: 58.1 | |||||||
HBF:4.3 | |||||||
HBE: 33.2 | |||||||
Pajak et al. [28] | CR | 1, USA | 55Â y Female | Hgb: 11.7 | HA, VOC, splenomegaly, functional asplenia | Splenectomy | Alive |
HBS: 68.6 | |||||||
HBF: 1.4 | |||||||
HBE: 26.3 | |||||||
Ramahi et al. [29] | CR | 1, USA | 28Â y Female | Hgb: NA | Postpartum endometritis | Transfusion | Alive |
HBS: 65.3 | |||||||
HBF: 3.5 | |||||||
HBE: 31.2 | |||||||
Rayburg et al. [30] | CR | 1, USA | 7Â y | Hgb: 11.4 | VOC, HA, bone marrow infection with parvovirus b19, bone marrow embolism, PE, Splenomegaly asymptomatic | None | Death |
Female | HBS: 55 | ||||||
HBF: 1.3 | |||||||
HBE: 31 | |||||||
Rey et al. [31] | RS | 4, Haiti | 4.1Â y | Hgb: 11.4 | None | Alive | |
2 Males | HBS: 61.5 | ||||||
2 Females | HBE + HBA2: 30 | ||||||
Schroeder et al. [32] | CR | 1, USA | 22Â y | Hgb: 14.6 | Hematuria | None | Alive |
Male | HBS: 60 | Splenomegaly | |||||
HBE + HBA2: 32 | |||||||
Smith et al. [33] | CR | 1, USA | 52Â y | Hgb: 13.4 | VOC, HA, Gallstones | Transfusion | Death |
Female | HBS: 25 | ||||||
HBE: 8 | |||||||
Tamminga et al. [34] | CR | 1, Netherlands | 7Â y | Hgb: 6.8 | Rhabdomyolysis | None | Alive |
Male | HBS: 65 | ||||||
HBF: 1.4 | |||||||
HBE: 34 | |||||||
Tay et al. [35] | CR | 1, Bangladesh | 66Â y Female | Hgb: 9.3 | HA, VOC, ACS, AVN | Transfusion, HU | Alive |
HBS: 61.2 | |||||||
HBF: 7.2 | |||||||
Thornburg et al. [36] | CR | 1, USA | 2 Male | Hgb: 10.2 | NA | NA | Alive |
HBS: 68 | |||||||
HBE: 32 | |||||||
Vishwanathan et al. [47] | CR | 1, India | 18Â y Male | Hgb: 9 | VOC | NA | Alive |
HBS: 68 | HA | ||||||
HBF: 2.1 | Splenomegaly | ||||||
HBE: 29.9 | Â |