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Original Article

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Blood Res 2021; 56(3):

Published online September 30, 2021

https://doi.org/10.5045/br.2021.2020289

© The Korean Society of Hematology

Clinical Analysis of Hospitalized Patients with Hemophilia A: Single-hemophilia Treatment Center Experience in Korea over 10 years

Ju Young Kim, Se Jin Park, Chur Woo You

Author Info. +

Department of Pediatrics, Daejeon Eulji Medical Center, Eulji University School of Medicine, Daejeon, Korea

Correspondence to : Chur Woo You, Ph.D.
Department of Pediatrics, Daejeon Eulji Medical Center, Eulji University School of Medicine, 95 Dunsanseo-ro, Seo-gu, Daejeon 35233, Korea
E-mail: YCW1@eulji.ac.kr

Received: November 17, 2020; Revised: March 30, 2021; Accepted: July 8, 2021

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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Background
There is lack of data on admitted hemophilia patients in Korea. For this reason, this study was intended to analyze the hospitalization data of hemophilia patients in a regional Hemophilia Treatment Center (HTC) for the first time in Korea.
Methods
In this retrospective study, we surveyed hospitalized patients with Hemophilia A (HA) in a HTC for 14 years. Medical records of these hemophiliacs were reviewed and data regarding demographic characteristics, cause of admissions and their outcomes in each patient were obtained. In addition, the data of admitted days, type and amount of Coagulation factor concentrate (CFC) used, treatments other than CFC infusion during the admission days were also obtained from the medical record of each patient.
Results
A total 107 patients with hemophilia A were admitted during 14 years. Annual rate of admission of patients with HA was 8%. Mean age on admission was 29.63±19.51 years old and mean admission days were 11.28±5.46 days. Most admissions were occurred in severe and moderate hemophilia patients. The most common cause of admission was bleed control followed by surgery and other reasons. With modified WFH CFC supplementation guideline, all the bleeds were successfully controlled and all surgeries were also successfully conducted with less total CFC consumption compared to the consumed dose of other reports.
Conclusion
These results suggest that it is necessary to develop more specified regimens different from WFH

Keywords Hemophilia A, Admitted patient, Analysis, Factor VIII concentrate consumption, Korea

Article

Original Article

Blood Res 2021; 56(3): 141-149

Published online September 30, 2021 https://doi.org/10.5045/br.2021.2020289

Copyright © The Korean Society of Hematology.

Clinical Analysis of Hospitalized Patients with Hemophilia A: Single-hemophilia Treatment Center Experience in Korea over 10 years

Ju Young Kim, Se Jin Park, Chur Woo You

Department of Pediatrics, Daejeon Eulji Medical Center, Eulji University School of Medicine, Daejeon, Korea

Correspondence to:Chur Woo You, Ph.D.
Department of Pediatrics, Daejeon Eulji Medical Center, Eulji University School of Medicine, 95 Dunsanseo-ro, Seo-gu, Daejeon 35233, Korea
E-mail: YCW1@eulji.ac.kr

Received: November 17, 2020; Revised: March 30, 2021; Accepted: July 8, 2021

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background
There is lack of data on admitted hemophilia patients in Korea. For this reason, this study was intended to analyze the hospitalization data of hemophilia patients in a regional Hemophilia Treatment Center (HTC) for the first time in Korea.
Methods
In this retrospective study, we surveyed hospitalized patients with Hemophilia A (HA) in a HTC for 14 years. Medical records of these hemophiliacs were reviewed and data regarding demographic characteristics, cause of admissions and their outcomes in each patient were obtained. In addition, the data of admitted days, type and amount of Coagulation factor concentrate (CFC) used, treatments other than CFC infusion during the admission days were also obtained from the medical record of each patient.
Results
A total 107 patients with hemophilia A were admitted during 14 years. Annual rate of admission of patients with HA was 8%. Mean age on admission was 29.63±19.51 years old and mean admission days were 11.28±5.46 days. Most admissions were occurred in severe and moderate hemophilia patients. The most common cause of admission was bleed control followed by surgery and other reasons. With modified WFH CFC supplementation guideline, all the bleeds were successfully controlled and all surgeries were also successfully conducted with less total CFC consumption compared to the consumed dose of other reports.
Conclusion
These results suggest that it is necessary to develop more specified regimens different from WFH

Keywords: Hemophilia A, Admitted patient, Analysis, Factor VIII concentrate consumption, Korea

Table 1 . WFH guidelines, practice patterns: peak plasma factor levels and duration of administration [5]..

Type of hemorrhageHemophilia A
Lower- dose practice patternHigher- dose practice pattern
Peak factor
level (IU/dL)		Treatment
duration (d)		Peak factor
level (IU/dL)		Treatment
duration (d)
Joint10–201–2a)40–601–2
Superficial muscle/no NV compromise (except iliopsoas)10–202–3a)40–602–3a)
Iliopsoas or deep muscle with NV injury or substantial blood loss
Initial20–401–2180–1001–2
Maintenance10–203–5b)30–603–5b)
Intracranial
Initial50–801–380–1001–7
Maintenance20–408–14508–21
30–504–7--
Throat and neck
Initial30–501–380–1001–7
Maintenance10–204–7508–14
Gastrointestinal
Initial30–501–380–1007–14
Maintenance10–204–750
Renal20–403–5503–5
Deep laceration20–405–7505–7
Surgery (major)
Pre-op60–8080–100
Post-opc)30–401–360–801–3
20–304–640–604–6
10–207–1430–507–14
Surgery (minor)
Pre-op40–8050–80
Post-opd)20–501–530–801–5

In this table, the desired peak factor levels of CFC replacement shown for treatment of hemorrhages at different anatomical sites represent the ranges in global practice patterns depending on available resources. Importantly, it should be recognized that the goal of such treatment is effective control of bleeding and should be the same everywhere in the world. Lower CFC replacement levels require much closer observation for effectiveness of bleeding control, with a potentially greater chance of requiring additional CFC replacement to achieve the target plasma level as well as the hemostatic and musculoskeletal outcomes..

a)May be longer if response is inadequate. b)Sometimes longer as secondary prophylaxis during physical therapy. c)The duration of treatment refers to sequential days post-surgery. Type of CFC and patient's response to CFC should be taken into account. d)Depending on procedure; the number of doses would depend on the half-life of the CFC used..

Abbreviations: CFC, clotting factor concentrate; NV, neurovascular..

Table 2 . The annual number of admitted cases..

YearN of admitted caseN of admitted patientType of hemophilia severityMean age on admission (yr)Mean admission days
201866S 420.55.83
M 2
201775S 114.574.71
M 4
2016108S 52411.1
M 3
201555S 227.88.8
M 3
20141210S 728.179.25
M 3
201397S 533.6721.11
M 2
2012118S 629.5524.63
M 2
20111211S 922.586
M 2
20101010S 725.8211.18
M 3
200987S 423.512.38
M 2
m 1
2008126S 349.3313.58
M 3
200785S 2338.25
M 2
m 1
2006169S 435.0613.88
M 4
m 1
200544S 235.259.25
M 2
200476S 433.149.28
M 2
Total137107S 65 (61%)29.63±19.5111.28±5.46
M 39 (36%)
M 3 (3%)

Abbreviations: m, mild hemophilia A; M, moderate hemophilia A; S, severe hemophilia A..

Table 3 . Bleeds needed for hospitalization and used amount of CFCs..

Mean age
(yr)		Severity
(N of cases)		N of
case		Hospitalized
days (day)		Amount of CFCs per kg (IU/kg)
Total First 3ds After 3ds
Major bleeds
CNS bleed26.3±20.1S 51036.27±59.4579.5±494.0213.7±114.9202±176.4
M 5
GI bleed45.3±12.2S 2175.6±2.6975.2±714.1191.4±103.577.9±74.3
M 13
m 2
Iliopsoas bleed25.1±13.2S 4711.3±13.1473.1±300.6179.6±160.1188.2±129.9
M 3
Compartment syndrome42±1.4S1219.0±16.9353.0±79.2188.2±129.998.5±25.7
M 1
Opthalmic hemorrhagea)32.9±9.1S 378.6±4.7488.1±481.2152.0±120.6135.3±128.9
M 4
Major bleeds total/average35.1±2.7S 154315.4±9.98460.3±66.4187.2±114.8142.7±139.6
M 26
m 2
Moderate bleeds
Dental bleed22.8±17.7S 5123.2±1.6127.2±79.2121.3±68.7-
M 7
Joint bleed32.0±21.7S 8149.1±7.7359.2±300.8262.2±208.9107.1±95.5
M 6
Intramuscular bleedb)28.9±23.4S 598.2±6.9524.7±513.7181.7±59.896.7±86.1
M 4
Large ecchymosis15.4±7.1S 555.6±3.05214.2±154.0136.2±71.774.4±72.1
Moderate bleeds total/average26.8±3.1S 23406.7±6.1334.4±54.9183.3±145.068.5±60.7
M 17
Pc)0.300.0210.042 0.8850.021

a)Vitreous hemorrhage, Hematoma of eyeball. b)Intramuscular bleed, excluding: iliopsoas bleed. c)P-value less than 0.05 is regarded as statistically significant in comparison with Major and moderate bleeds..

Abbreviations: m, mild hemophilia A; M, moderate hemophilia A; S, severe hemophilia A..

Table 4 . Orthopedic surgeries and used amount of CFCs..

Mean age (yr)Severity
(N of cases)		N of caseHospitalized days (day)Amount of CFCs per kg (IU/kg)
TotalFirst 3dsAfter 3ds
Major operation
ORIF30±8.2S 1334.7±15.8359.2±110.2123.8±41.2123.6±20.8
M 2
Synovectomy20.4±13.3S 4717.4±10.2979.5±519.5267.7±140.8211.1±95.1
M 3
Spinal stenosis operation68S 11501,040325150
Open fracture operation28.5±12.2S 2420.25±5.4601.6±116.6222.7±138.5140±22.8
M 2
Major operation27.7±15.9S 81523.8±14.9758.7±733.3209.2±131.7174.1±74.7
M 7
Minor operation
Endoscopic synovectomy19±17.3
S 234.3±3.2165.7±59.569.6±24.278
M 1
Closed fracture operation7.3±2.3S 333.3±0.6123.4±537121.3±53.8-
Minor operation total/average13.2±12.8S 565.8±4.9143.5±56.295.5±46.978
M 1
Pa)0.480.0180.0400.0200.527

a)P-value less than 0.05 is regarded as statistically significant in comparison with major and minor operation..

Abbreviations: M, moderate hemophilia A; ORIF, open reduction and internal fixation; S, severe hemophilia A..

Table 5 . Other surgeries and used amount of CFCs..

Mean age (yr)Severity
(N of cases)		N of caseHospitalized dayAmount of CFCs per kg (IU/kg)
TotalFirst 3dsAfter 3ds
Major operation
Appendectomy16S 114314.3208.3124
Splenectomy23S 1131231.9139.1139
Craniotomy & closed drainage38.75±12.1S 4467.5±53.91069.9±507.4337±87.1371.5±153.0
Adenoidectomy3S 116712.5437.5393
Tooth extraction (>3)47.7±1.2M 333.7±0.6178.1±48.7178.1±48.7-
Major operation total/average34.5±16.7S 71032.2±24.3578.9±487.1217.3±143.3271.4±166.8
M 3
Minor operation
Hemorrhoidectomy25.6±4.6S 334288.3±89.0196.9±8.978
Removal of lipoma39M 1161206060
Tooth extraction (≤2)23.0±14.7S 131.3±0.668.6±33.468.6±33.4-
M 2
Tympanoplasty44S 111373.873.8-
Colonoscopic resection or Bx25.7±7.6M 333.3±1.2182.1±38.6182.1±38.6-
Minor operation total/average31.6±11.5S 5114.5±3.5179.0±112.8137.6±66.898.0±28.3
M 6
Pa)0.090.040.0000.0050.198

a)P-value less than 0.05 is regarded as statistically significant in comparison with major and minor operation..

Abbreviations: M, moderate hemophilia A; S, severe hemophilia A..

Table 6 . Cause of admissions unrelated to acute bleeds and surgeries..

Mean age (yr)Severity (N of cases)N of casesHospitalized daysAmount of CFCs per kg (IU/kg)
DM36Severe 116266.1
Pneumonia6.0±7.0Severe 225.5±2.1100.0±70.7
Pancreatitis42Mild 1115140.2
Liver cirrhosis41.3±0.6Mild 337.6±6.498.0±68.3
Colitis20Severe 11544.1
APN22.5±12.0Severe 224±1.470.9±41.1
Chemotherapy69Severe 1138465
Dialysis70Severe 2217.5326.6±85.5
PT30.7±22.9Severe 5521.5±17.8338.4±197.9
OBS7.6±7.8Severe 556.6±5.5667.7±609.4
Total/average29.0±23.4Severe 192312.5±11.4324.0±317.3
Mild 4

Abbreviations: APN, acute pyelonephritis; OBS, hospitalized for observation; PT, physical therapy..

Table 7 . Comparison of all major operations and minor operations..

Mean age (yr)Severity
(N of cases)		N of caseHospitalized days(day)Amount of CFCs per kg (IU/kg)Amount of CFCs first 3 days Amount of CFCs After 3 days
Major operation (orthopedic and non-orthopedic surgeries)
25.5.±15.9S 152523.7±3.9541.7±432.2209.5±134.0173.5±92.3
M 10
Minor operation (orthopedic and non-orthopedic surgeries)21.0±13.8S 9168.0±7.4276.8±188.9117.9±61.891.3±23.1
M 7
Pa)0.6830.0280.0180.0900.072

a)P-value less than 0.05 is regarded as statistically significant in comparison with major and minor operation..
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