Blood Res 2021; 56(3):
Published online September 30, 2021
https://doi.org/10.5045/br.2021.2020289
© The Korean Society of Hematology
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
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.
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
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.
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
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.
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 hemorrhage | Hemophilia A | ||||
---|---|---|---|---|---|
Lower- dose practice pattern | Higher- dose practice pattern | ||||
Peak factor level (IU/dL) | Treatment duration (d) | Peak factor level (IU/dL) | Treatment duration (d) | ||
Joint | 10–20 | 1–2a) | 40–60 | 1–2 | |
Superficial muscle/no NV compromise (except iliopsoas) | 10–20 | 2–3a) | 40–60 | 2–3a) | |
Iliopsoas or deep muscle with NV injury or substantial blood loss | |||||
Initial | 20–40 | 1–21 | 80–100 | 1–2 | |
Maintenance | 10–20 | 3–5b) | 30–60 | 3–5b) | |
Intracranial | |||||
Initial | 50–80 | 1–3 | 80–100 | 1–7 | |
Maintenance | 20–40 | 8–14 | 50 | 8–21 | |
30–50 | 4–7 | - | - | ||
Throat and neck | |||||
Initial | 30–50 | 1–3 | 80–100 | 1–7 | |
Maintenance | 10–20 | 4–7 | 50 | 8–14 | |
Gastrointestinal | |||||
Initial | 30–50 | 1–3 | 80–100 | 7–14 | |
Maintenance | 10–20 | 4–7 | 50 | ||
Renal | 20–40 | 3–5 | 50 | 3–5 | |
Deep laceration | 20–40 | 5–7 | 50 | 5–7 | |
Surgery (major) | |||||
Pre-op | 60–80 | 80–100 | |||
Post-opc) | 30–40 | 1–3 | 60–80 | 1–3 | |
20–30 | 4–6 | 40–60 | 4–6 | ||
10–20 | 7–14 | 30–50 | 7–14 | ||
Surgery (minor) | |||||
Pre-op | 40–80 | 50–80 | |||
Post-opd) | 20–50 | 1–5 | 30–80 | 1–5 |
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..
Year | N of admitted case | N of admitted patient | Type of hemophilia severity | Mean age on admission (yr) | Mean admission days |
---|---|---|---|---|---|
2018 | 6 | 6 | S 4 | 20.5 | 5.83 |
M 2 | |||||
2017 | 7 | 5 | S 1 | 14.57 | 4.71 |
M 4 | |||||
2016 | 10 | 8 | S 5 | 24 | 11.1 |
M 3 | |||||
2015 | 5 | 5 | S 2 | 27.8 | 8.8 |
M 3 | |||||
2014 | 12 | 10 | S 7 | 28.17 | 9.25 |
M 3 | |||||
2013 | 9 | 7 | S 5 | 33.67 | 21.11 |
M 2 | |||||
2012 | 11 | 8 | S 6 | 29.55 | 24.63 |
M 2 | |||||
2011 | 12 | 11 | S 9 | 22.58 | 6 |
M 2 | |||||
2010 | 10 | 10 | S 7 | 25.82 | 11.18 |
M 3 | |||||
2009 | 8 | 7 | S 4 | 23.5 | 12.38 |
M 2 | |||||
m 1 | |||||
2008 | 12 | 6 | S 3 | 49.33 | 13.58 |
M 3 | |||||
2007 | 8 | 5 | S 2 | 33 | 8.25 |
M 2 | |||||
m 1 | |||||
2006 | 16 | 9 | S 4 | 35.06 | 13.88 |
M 4 | |||||
m 1 | |||||
2005 | 4 | 4 | S 2 | 35.25 | 9.25 |
M 2 | |||||
2004 | 7 | 6 | S 4 | 33.14 | 9.28 |
M 2 | |||||
Total | 137 | 107 | S 65 (61%) | 29.63±19.51 | 11.28±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 bleed | 26.3±20.1 | S 5 | 10 | 36.27±59.4 | 579.5±494.0 | 213.7±114.9 | 202±176.4 |
M 5 | |||||||
GI bleed | 45.3±12.2 | S 2 | 17 | 5.6±2.6 | 975.2±714.1 | 191.4±103.5 | 77.9±74.3 |
M 13 | |||||||
m 2 | |||||||
Iliopsoas bleed | 25.1±13.2 | S 4 | 7 | 11.3±13.1 | 473.1±300.6 | 179.6±160.1 | 188.2±129.9 |
M 3 | |||||||
Compartment syndrome | 42±1.4 | S1 | 2 | 19.0±16.9 | 353.0±79.2 | 188.2±129.9 | 98.5±25.7 |
M 1 | |||||||
Opthalmic hemorrhagea) | 32.9±9.1 | S 3 | 7 | 8.6±4.7 | 488.1±481.2 | 152.0±120.6 | 135.3±128.9 |
M 4 | |||||||
Major bleeds total/average | 35.1±2.7 | S 15 | 43 | 15.4±9.98 | 460.3±66.4 | 187.2±114.8 | 142.7±139.6 |
M 26 | |||||||
m 2 | |||||||
Moderate bleeds | |||||||
Dental bleed | 22.8±17.7 | S 5 | 12 | 3.2±1.6 | 127.2±79.2 | 121.3±68.7 | - |
M 7 | |||||||
Joint bleed | 32.0±21.7 | S 8 | 14 | 9.1±7.7 | 359.2±300.8 | 262.2±208.9 | 107.1±95.5 |
M 6 | |||||||
Intramuscular bleedb) | 28.9±23.4 | S 5 | 9 | 8.2±6.9 | 524.7±513.7 | 181.7±59.8 | 96.7±86.1 |
M 4 | |||||||
Large ecchymosis | 15.4±7.1 | S 5 | 5 | 5.6±3.05 | 214.2±154.0 | 136.2±71.7 | 74.4±72.1 |
Moderate bleeds total/average | 26.8±3.1 | S 23 | 40 | 6.7±6.1 | 334.4±54.9 | 183.3±145.0 | 68.5±60.7 |
M 17 | |||||||
0.30 | 0.021 | 0.042 | 0.885 | 0.021 |
a)Vitreous hemorrhage, Hematoma of eyeball. b)Intramuscular bleed, excluding: iliopsoas bleed. c)
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 case | Hospitalized days (day) | Amount of CFCs per kg (IU/kg) | |||
---|---|---|---|---|---|---|---|
Total | First 3ds | After 3ds | |||||
Major operation | |||||||
ORIF | 30±8.2 | S 1 | 3 | 34.7±15.8 | 359.2±110.2 | 123.8±41.2 | 123.6±20.8 |
M 2 | |||||||
Synovectomy | 20.4±13.3 | S 4 | 7 | 17.4±10.2 | 979.5±519.5 | 267.7±140.8 | 211.1±95.1 |
M 3 | |||||||
Spinal stenosis operation | 68 | S 1 | 1 | 50 | 1,040 | 325 | 150 |
Open fracture operation | 28.5±12.2 | S 2 | 4 | 20.25±5.4 | 601.6±116.6 | 222.7±138.5 | 140±22.8 |
M 2 | |||||||
Major operation | 27.7±15.9 | S 8 | 15 | 23.8±14.9 | 758.7±733.3 | 209.2±131.7 | 174.1±74.7 |
M 7 | |||||||
Minor operation | |||||||
Endoscopic synovectomy | 19±17.3 | S 2 | 3 | 4.3±3.2 | 165.7±59.5 | 69.6±24.2 | 78 |
M 1 | |||||||
Closed fracture operation | 7.3±2.3 | S 3 | 3 | 3.3±0.6 | 123.4±537 | 121.3±53.8 | - |
Minor operation total/average | 13.2±12.8 | S 5 | 6 | 5.8±4.9 | 143.5±56.2 | 95.5±46.9 | 78 |
M 1 | |||||||
0.48 | 0.018 | 0.040 | 0.020 | 0.527 |
a)
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 case | Hospitalized day | Amount of CFCs per kg (IU/kg) | |||
---|---|---|---|---|---|---|---|
Total | First 3ds | After 3ds | |||||
Major operation | |||||||
Appendectomy | 16 | S 1 | 1 | 4 | 314.3 | 208.3 | 124 |
Splenectomy | 23 | S 1 | 1 | 31 | 231.9 | 139.1 | 139 |
Craniotomy & closed drainage | 38.75±12.1 | S 4 | 4 | 67.5±53.9 | 1069.9±507.4 | 337±87.1 | 371.5±153.0 |
Adenoidectomy | 3 | S 1 | 1 | 6 | 712.5 | 437.5 | 393 |
Tooth extraction (>3) | 47.7±1.2 | M 3 | 3 | 3.7±0.6 | 178.1±48.7 | 178.1±48.7 | - |
Major operation total/average | 34.5±16.7 | S 7 | 10 | 32.2±24.3 | 578.9±487.1 | 217.3±143.3 | 271.4±166.8 |
M 3 | |||||||
Minor operation | |||||||
Hemorrhoidectomy | 25.6±4.6 | S 3 | 3 | 4 | 288.3±89.0 | 196.9±8.9 | 78 |
Removal of lipoma | 39 | M 1 | 1 | 6 | 120 | 60 | 60 |
Tooth extraction (≤2) | 23.0±14.7 | S 1 | 3 | 1.3±0.6 | 68.6±33.4 | 68.6±33.4 | - |
M 2 | |||||||
Tympanoplasty | 44 | S 1 | 1 | 13 | 73.8 | 73.8 | - |
Colonoscopic resection or Bx | 25.7±7.6 | M 3 | 3 | 3.3±1.2 | 182.1±38.6 | 182.1±38.6 | - |
Minor operation total/average | 31.6±11.5 | S 5 | 11 | 4.5±3.5 | 179.0±112.8 | 137.6±66.8 | 98.0±28.3 |
M 6 | |||||||
0.09 | 0.04 | 0.000 | 0.005 | 0.198 |
a)
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 cases | Hospitalized days | Amount of CFCs per kg (IU/kg) | |
---|---|---|---|---|---|
DM | 36 | Severe 1 | 1 | 6 | 266.1 |
Pneumonia | 6.0±7.0 | Severe 2 | 2 | 5.5±2.1 | 100.0±70.7 |
Pancreatitis | 42 | Mild 1 | 1 | 15 | 140.2 |
Liver cirrhosis | 41.3±0.6 | Mild 3 | 3 | 7.6±6.4 | 98.0±68.3 |
Colitis | 20 | Severe 1 | 1 | 5 | 44.1 |
APN | 22.5±12.0 | Severe 2 | 2 | 4±1.4 | 70.9±41.1 |
Chemotherapy | 69 | Severe 1 | 1 | 38 | 465 |
Dialysis | 70 | Severe 2 | 2 | 17.5 | 326.6±85.5 |
PT | 30.7±22.9 | Severe 5 | 5 | 21.5±17.8 | 338.4±197.9 |
OBS | 7.6±7.8 | Severe 5 | 5 | 6.6±5.5 | 667.7±609.4 |
Total/average | 29.0±23.4 | Severe 19 | 23 | 12.5±11.4 | 324.0±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 case | Hospitalized 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.±15.9 | S 15 | 25 | 23.7±3.9 | 541.7±432.2 | 209.5±134.0 | 173.5±92.3 | |
M 10 | |||||||
Minor operation (orthopedic and non-orthopedic surgeries) | 21.0±13.8 | S 9 | 16 | 8.0±7.4 | 276.8±188.9 | 117.9±61.8 | 91.3±23.1 |
M 7 | |||||||
0.683 | 0.028 | 0.018 | 0.090 | 0.072 |
a)
Sung Eun Kim, Ji Yoon Kim, Jeong A Park, Chuhl Joo Lyu, Seung Min Hahn, Jung Woo Han and Young Shil Park
Blood Res 2024; 59():Valentina Perrone, Melania Leogrande, Maria Cappuccilli and Luca Degli Esposti
Blood Res 2024; 59():Young-Shil Park, Ki-Young Yoo, Sang Kyu Park, Taiju Hwang, Aeran Jung and Eun Jin Choi
Blood Res 2024; 59():