Life-threatening bleeding in patients with hemophilia (PWH): a 10-year cohort study in Dakar, Senegal

Figures & data

Table 1. General characteristics of study population.

	Number (n)	Percentage %
Type and severity of hemophilia
Hemophilia A
Severe	141	51.5
Moderate	68	24.8
Minor	32	11.6
Hemophilia B
Severe	25	9.2
Moderate	7	2.5
Minor	1	0.4
Age of patients
0–4 years	28	10.2
5–13 years	108	39.4
14–18 years	43	15.7
19–44 years	84	30.6
>45 years	11	4.1
Family history of hemophilia	151
Treatment
No treatment	57	20.8
On demand treatment	161	58.8
Prophylaxis
Continuous	24	8.7
Intermittent	32	11.7
Inhibitors (n = 13)
Low responders	8	2.9
High responders	5	1.8

Table 2. Type of hemophilia, bleeding circumstances and bleeding sites for patients with life-threatening bleeding.

Variables	Number	%
Type, severity of hemophilia and treatment regimen
Severe HA
Prophylaxis	3	13.7
On-demand	13	59.1
Moderate HA
Prophylaxis	0	0
On-demand	4	18.2
Severe HB
Prophylaxis	0	0
On-demand	1	4.5
Moderate HB
Prophylaxis	0	0
On-demand	1	4.5
Bleeding circumstances
Spontaneous	13	41.9
Post-trauma	18	58.1
Site of bleeds
Cerebral	13	41.9
Spinal	1	3.2
Gastro-intestinal	8	25.8
Intra-abdominal	3	9.7
Muscular	3	9.7
Facial	2	6.5
Purse	1	3.2

Figure 1. Cerebral hematoma. (a and c) subacute hemorrhagic stroke with falcorial engagement in an 11-month-old infant; (b) left hemispherical multiple acute extradural hematoma associated with hyperdensities of the subarachnoid spaces; (d) left hemispheric acute subdural hematoma.

Table 3. Management strategies and outcomes for life-threatening bleeding.

Variables	Number	%
Treatment
Yes	16	72.7
No	6	27.3
Number of severe bleeding treated	25	80.6
Type of treatment
CFCs	25	80.6
EHL products	14	56
SHL products	11	44
Surgical drainage	1	3.2
Electrocoagulation during FOGD	1	3.2
Status
Alive	13	59.1
Complete remission	11	50
Partial remission with sequelae	2	9.1
Dead	9	40.9
Secondary prophylaxis
On-demand treatment to LDP	4	30.7
On-demand treatment to Emicizumab	3	23.1
LDP to Emicizumab	3	23.1
On-demand treatment	3	23.1
Death
Cerebral bleeding	7	31.8
Intra-abdominal bleeding	1	4.5
Septic stroke	1	4.5

Table 4. Characteristics of deceased patients.

Patients	Type and severity of hemophilia	Therapeutic regimen	Presence of inhibitor	Emergency treatment	Age of death	Cause of death
1	A severe	On-demand	Yes (4.8UB)	Yes	34 years	Gastrointestinal bleeding
2	A severe	On- demand	No	No	37 years	Cerebral Bleeding
3	A severe	On-demand	No	Yes	16 years	Septic shock
4	A severe	On-demand	No	No	16 years	Cerebral bleeding
5	A severe	On-demand	No	No	5 years	Cerebral bleeding
6	A severe	On-demand	No	No	7 years	Cerebral bleeding
7	A severe	On-demand	No	No	8 years	Cerebral bleeding
8	B moderate	On-demand	No	Yes	4 months	Cerebral bleeding
9	A severe	On-demand	No	No	8 years	Cerebral bleeding

Data availability statement

The data that support the findings of this study are available from the corresponding author upon reasonable request.