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Table 1 Reported cases of HBSE to date

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

24 y

Hgb: 9

NA

None

Alive

Female

HBS: 69.8

 

HBE: 30.2

Arbefeville et al. [14]

CR

1, USA

12 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

56 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

22 y

Hgb: 10.8

VOC, pain crisis

Transfusion

Alive

Female

HBS: 60

Splenomegaly

HBE: 40

Parvovirus b19

Englestad [18]

CR

1, USA

34 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

23 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

28 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

1 y

Hgb: 11.9

1 VOC

None

Alive

Male

HBS: 70

6 y

HBE: 30

Female

Italia et al. [24]

RS

4, India

20.75 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

17 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

17.8 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

1 y

Male

Hgb: 10

VOC

None

Alive

HBS: 58.9

HBF: 5.2

HBE + HBA2: 37.5

Mishra et al. [26]

CR

2, India

7 y

Hgb: 9.3

Splenomegaly

None

Alive

Male

HBS: 58.05

HA

20 y

HBF: 2.8

 

Female

HBE + HBA2 = 35.4

 

Mukhopadhyay et al. [27]

CR

1, India

26 y

Male

Hgb: NA

NA

None

Alive

HBS: 58.1

HBF:4.3

HBE: 33.2

Pajak et al. [28]

CR

1, USA

55 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

28 y

Female

Hgb: NA

Postpartum endometritis

Transfusion

Alive

HBS: 65.3

HBF: 3.5

HBE: 31.2

Rayburg et al. [30]

CR

1, USA

7 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

4.1 y

Hgb: 11.4

None

Alive

2 Males

HBS: 61.5

2 Females

HBE + HBA2: 30

Schroeder et al. [32]

CR

1, USA

22 y

Hgb: 14.6

Hematuria

None

Alive

Male

HBS: 60

Splenomegaly

HBE + HBA2: 32

Smith et al. [33]

CR

1, USA

52 y

Hgb: 13.4

VOC, HA, Gallstones

Transfusion

Death

Female

HBS: 25

HBE: 8

Tamminga et al. [34]

CR

1, Netherlands

7 y

Hgb: 6.8

Rhabdomyolysis

None

Alive

Male

HBS: 65

HBF: 1.4

HBE: 34

Tay et al. [35]

CR

1, Bangladesh

66 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

18 y

Male

Hgb: 9

VOC

NA

Alive

HBS: 68

HA

HBF: 2.1

Splenomegaly

HBE: 29.9

 
  1. Hgb hemoglobin, HU hydroxyurea, SCT stem cell transplant, VOC vaso-occlusive crisis, HA hemolytic anemia, ACS acute chest syndrome, AVN AVASCULAR necrosis, UTI urinary tract infections, PE pulmonary embolism, RS retrospective study, CR case report, NA not available
  2. aOutcome at the time of the case report/series