case report hemophilia a
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Case ReportHemophilia A
Introduction
Case ReportCPR, male, 3 year 2 months, came to RSHAM on 14th December 2013 with history of bleeding tongue as the main complaint. The patient has been presenting this symptoms for the past 24 hours and presented to the hospital with continous bleeding from his tongue until a few hours before reached RSUP HAM. The patient had accidentally biting his tongue. This led to profuse bleeding from the dorsal surface of his tongue. No other bruising or active bleeding was noticed except extensive bruising over his left scapular area and the presence of hematomes on the chin and right elbow with active oozing since 3 days ago. There was no nose bleeding, hematuria, bloody stool, accompanying hemoptysis, joint pain or swelling. History of fever and trauma were not found. The patient has been having bleeding especially when he falls down or hit on anything since 1 years old age. There was no history of jaundice. The patient’s family history is noteworthy consanguineous parents (his uncle) who also experienced bleeding disorder. On physical examination, the patient was fully alert, with normal blood pressure and no sign of fever. The patient was previously outpatient in hemato-oncology division and diagnosed with hemophilia A. He received the last Koate injection on May 2013.
Follow up14th December 2013
S Hematomes (+), bleeding (-)
O Sensorium: was clear; Temperature: 36,8oCHead : Normal limitEyes : Light reflex (+/+), isochoric pupil, conjunctiva palpebra inferior anemic (-/-), icteric (-/-). E/N/T : normal appearance/normal appearance/purpura on tongueNeck : no lymph nodes enlargement. Hematoma on chinThorax : Symmetrical fusiform, no retraction, heart rate 88 x/minute, regular, no murmur and gallop, respiratory rate 20 x/minute, regular, no rhonchi and wheezing. Abdomen : supple, peristaltic was normal, liver/spleen/renal: not palpable, hematoma on scapular areaExtremities : pulse 88 x/minute, regular, adequate tone and volume,warm acral, CRT < 3”. Hematoma on right elbow
A Hemophilia A
P R/Koate Injection 375 IU/12 hr/iv for 3 days [Requirement : 15 x 50% x 0.5] = 375 IU R/Diet MB 1250 kkal with 30 gr protein
Follow up15th December 2013
S Hematomes (+), bleeding (-)
O Sensorium: was clear; Temperature: 36,8oCHead : Normal limitEyes : Light reflex (+/+), isochoric pupil, conjunctiva palpebra inferior anemic (-/-), icteric (-/-). E/N/T : normal appearance/normal appearance/purpura on tongueNeck : no lymph nodes enlargement. Hematoma on chinThorax : Symmetrical fusiform, no retraction, heart rate 86 x/minute, regular, no murmur and gallop, respiratory rate 24 x/minute, regular, no rhonchi and wheezing. Abdomen : supple, peristaltic was normal, liver/spleen/renal: not palpable, hematoma on scapular areaExtremities : pulse 86 x/minute, regular, adequate tone and volume,warm acral, CRT < 3”. Hematoma on right elbow
A Hemophilia A
P - Koate Injection 375 IU/12 hr/iv for 3 days ( day 1) [Requirement : 15 x 50% x 0.5] = 375 IU- Diet MB 1250 kkal with 30 gr protein
Follow up16th December 2013
S Hematomes (+), bleeding (-)
O Sensorium: was clear; Temperature: 37,0oCHead : Normal limitEyes : Light reflex (+/+), isochoric pupil, conjunctiva palpebra inferior anemic (-/-), icteric (-/-). E/N/T : normal appearance/normal appearance/normal appearanceNeck : no lymph nodes enlargement. Thorax : Symmetrical fusiform, no retraction, heart rate 88 x/minute, regular, no murmur and gallop, respiratory rate 20 x/minute, regular, no rhonchi and wheezing. Abdomen : supple, peristaltic was normal, liver/spleen/renal: not palpable, hematoma on scapular areaExtremities : pulse 88 x/minute, regular, adequate tone and volume,warm acral, CRT < 3”.
A Hemophilia A
P - Koate Injection 375 IU/12 hr/iv for 3 days (day 2) [Requirement : 15 x 50% x 0.5] = 375 IU- Diet MB 1250 kkal with 30 gr protein
Laboratory Result, 16th December 2013 Complete blood count(CBC)
Normal Value Result Value Unit
Hemoglobin (HGB) 11.3-14.1 6.80 g%
Eritrosit (RBC) 4.40-4.48 2.83 106/ mm3
Leukosit (WBC) 6.0-17.5 13.79 103/ mm3
Hematokrit 37-41 22.30 %
Trombosit (PLT) 217-497 346 103/ mm3
MCV 81-95 78.80 fL
MCH 25-29 24.00 Pg
MCHC 29-31 30.50 g%
RDW 11.6-14.8 18.70 %
MPV 10.00 Fl
PCT 0.35 %
PDW 10.5 Fl
WBC Count
Neutrofil 37 – 80 39.00 %
Limfosit 20 – 40 37.60 %
Monosit 2 – 8 9.90 %
Eosinofil 1 – 6 12.70 %
Basofil 0 – 1 0.800 %
Faal Hemostasis
Normal Value Result Value Unit
Faktor VIII 55 - 150 5.2 %
Faktor IX 70 - 140 152.0 %
Follow up17th December 2013
S Hematomes (-), bleeding (-), pale (+)
O Sensorium: was clear; Temperature: 36,8 oCHead : Normal limitEyes : Light reflex (+/+), isochoric pupil, conjunctiva palpebra inferior anemic (+/+), icteric (-/-). E/N/T : normal appearance/normal appearance/normal appearanceNeck : no lymph nodes enlargement. Thorax : Symmetrical fusiform, no retraction, heart rate 90 x/minute, regular, no murmur and gallop, respiratory rate 20 x/minute, regular, no rhonchi and wheezing. Abdomen : supple, peristaltic was normal, liver/spleen/renal: not palpable,Extremities : pulse 90 x/minute, regular, adequate tone and volume,warm acral, CRT < 3”.
A Hemophilia A
P - Koate Injection 375 IU/12 hr/iv for 3 days (day 3) [Requirement : 15 x 50% x 0.5] = 375 IU- PRC transfusion (75 cc from total 252 cc) [PRC requirement : 4 x ( 11 – 6.8 ) x 15 = 252 cc]- Diet MB 1250 kkal with 30 gr protein
Follow up18th December 2013
S Hematomes (-), bleeding (-), pale (+)
O Sensorium: was clear; Temperature: 36,8 oCHead : Normal limitEyes : Light reflex (+/+), isochoric pupil, conjunctiva palpebra inferior anemic (+/+), icteric (-/-). E/N/T : normal appearance/normal appearance/normal appearanceNeck : no lymph nodes enlargement. Thorax : Symmetrical fusiform, no retraction, heart rate 92 x/minute, regular, no murmur and gallop, respiratory rate 24 x/minute, regular, no rhonchi and wheezing. Abdomen : supple, peristaltic was normal, liver/spleen/renal: not palpable,Extremities : pulse 92 x/minute, regular, adequate tone and volume,warm acral, CRT < 3”.
A Hemophilia A
P - PRC transfusion (75 cc from total 252 cc) [PRC requirement : 4 x ( 11 – 6.8 ) x 15 = 252 cc]- Diet MB 1250 kkal with 30 gr protein
Follow up19th December 2013
S Hematomes (-), bleeding (-), pale (-)
O Sensorium: was clear; Temperature: 37,0 oCHead : Normal limitEyes : Light reflex (+/+), isochoric pupil, conjunctiva palpebra inferior anemic (-/-), icteric (-/-). E/N/T : normal appearance/normal appearance/normal appearanceNeck : no lymph nodes enlargement. Thorax : Symmetrical fusiform, no retraction, heart rate 96 x/minute, regular, no murmur and gallop, respiratory rate 22 x/minute, regular, no rhonchi and wheezing. Abdomen : supple, peristaltic was normal, liver/spleen/renal: not palpable,Extremities : pulse 96 x/minute, regular, adequate tone and volume,warm acral, CRT < 3”.
A Hemophilia A
P - PRC transfusion (75 cc from total 252 cc) [PRC requirement : 4 x ( 11 – 6.8 ) x 15 = 252 cc]- Diet MB 1250 kkal with 30 gr protein
• Patient discharge on 20th December 2013 afternoon
DISCUSSION
Table 1 : Bleeding manifestations with different severities of hemophilia A
Type of bleedingFactor Level Deficiency
Severe (<1%) Moderate (1-5%) Mild (5-40%)Age of onset <1 years 1-2 years >2 years-adultHamarthroses Spontaneous ++++ ++ -
Following minor trauma
++++ +++ -
Musle hematoma ++++ ++ -Surgery ++++ +++ ++Dental extraction ++++ +++ ++Trauma to soft tissue Mild ++++ ++ -
Significant ++++ +++ +
Hematuria ++++ ++ -
Central nervous system ++ + -
Man
Woman
Patient
Carrier
