treatment of acute bleeds in acquired haemophilia l j2014.pdf · acquired haemophilia a: results...

Treatment of acute bleeds in Acquired Haemophilia

Dr. Laszlo Nemes

National Haemophilia Center and Haemostasis Department

Medical Centre of Hungarian Defence Forces Budapest Hungary

Slovenien Haematology-Transfusiology Congress 2014

03rd – 04th of October, 2014, Bled

• AH is not known by all of the physicians (rare disease)

• AH is often missed or misdiagnosed (multidisciplinary clinical problem)

• Severe bleeding tendency despite no previous history of abnormal bleeding

• AH can be life-threatening • Progressively prolonged, non-correctible APTT is

the clue for diagnosis (but often not done or ignored)

• After achieving proper diagnosis, we can treat the acute bleeds (treatable condition)

• After achieving proper diagnosis, we can eradicate the inhibitor in most of the cases (curable condition)

The importance of the

condition

Bled, 2014

Data of the existing cohorts

and registries

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• Although data on several AHA cohorts have been collected,

limited information is available on the optimal management of

AHA

• Previous studies:

Rationale

1. Green D, Lechner K. Thromb Haemost 1981; 45:200-3.

2. Delgado J, et al. Br J Haematol 2003; 121:21-35.

3. Collins PW, et al. Blood 2007; 109:1870-7.

Green D,

et al. 19811

Retrospective

survey

215 patients with AHA referred to

specialist centres

Delgado J, et al. 20032

Meta-analysis 249 patients from 21 case series;

published between 1985-2002

Collins PW,

et al. 20073

Prospective

surveillance study

172 patients with AHA who presented in

the UK between 2001-2003

Bled, 2014

Extensive subcutaneous and intramuscular

haematomas in pts. with acquired haemophilia

Nemes L, Pitlik E:Haematologica, 2000; 85: 64-68 Bled, 2014

Retroperitoneal haematoma is a

common bleeding manifestation in AHA

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Dorsal haematoma in idiopathic

acquired haemophilia

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• Austria

• Finland

• France

• Germany

• Greece

• Hungary

• Italy

• The Netherlands

• Portugal

• Spain

• Sweden

• Switzerland

• United Kingdom

EACH2: participating countries

Bled, 2014

Steering Committee

members of EACH2 project

• F. Baudo – Ospedale Niguarda, Milan, Italy

• A. Huth-Kühne – Kurpfalzkrankenhaus, Heidelberg, Germany

• H. Lévesque – Rouen University Hospital, Rouen, France

• P. Marco – Hospital General, Alicante, Spain

• L. Nemes – National Haemophilia Center, Budapest, Hungary

• L. Tengborn – Universitetssjukhuset MAS, Malmö, Sweden

• Advisor: P. Collins – University of Wales College of Medicine,

Cardiff, UK

Bled, 2014

Demographic and clinical data in acquired haemophilia A: Results from the European Acquired Haemophilia

(EACH2) Registry

Paul Knoebl, Pascual Marco, Francesco Baudo, Peter Collins,

Angela Huth-Kühne, László Nemes, Fabio Pellegrini, Lilian Tengborn,

Hervé Lévesque on behalf of the EACH2 registry contributors

J Thromb Haemost 2012;10:622-631

Bled, 2014

• 13 European countries and 117 treatment centres

• Enrolment period: January 2003 – December 2008

• Local authorities in 5 countries (43 centres) restricted

patient recruitment to surviving patients only

• No data entry on individuals who had died

• Finland, Greece, Hungary, Italy, Portugal

• To avoid any bias, data for these centers have been

excluded from certain analyses (e.g. eradication

therapy)

Study design

Bled, 2014

Entire collective All patients

entered*

Patients who

had died not

entered†

P

Demographics

Patients [n (%)] 501 (100) 331 (66.1) 170 (33.9) NA

Median age at

diagnosis

[years (IQR)]

73.9 (61.4-80.4) 75.4 (63.6-81.2) 70.4 (59.3-77.0) <0.0001

Male:Female

[n (%) ratio]

266:235

(53.1:46.9)

1:0.88

179:152

(54.1:45.9)

1:0.85

87:83

(51.2:48.8)

1:0.95

NS

Demographics

Novel finding: Preponderance of males

Data consistent with other large cohorts of patients with AHA

Data are reported as n (%) and median (IQR) for categorical and continuous variables, respectively.

P-values refer to Pearson’s chi-square test or Mann-Whitney U-test.

IQR, inter-quartile range; BU, Bethesda Units; Hb, Haemoglobin; NA, not applicable; NS, not significant; and chi², Pearson’s chi-square test.

*Centres could enter all patients. †Centres could not enter patients who had died.

Bled, 2014

Age at diagnosis according to

gender Median age at

diagnosis:

73.9 years

Peripartum and

autoimmune-

associated

FVIII inhibitors

Median age:

33.9 years

30

25

20

15

10

5

0

Female

Male

Nu

mb

er

of p

atie

nts

13

–1

4

15

–1

6

17

–1

8

19

–2

0

21

–2

2

23

–2

4

25

–2

6

27

–2

8

29

–3

0

31

–3

2

33

–3

4

35

–3

6

37

–3

8

39

–4

0

41

–4

2

43

–4

4

45

–4

6

47

–4

8

49

–5

0

51

–5

2

53

–5

4

55

–5

6

57

–5

8

59

–6

0

61

–6

2

63

–6

4

65

–6

6

67

–6

8

69

–7

0

71

–7

2

73

–7

4

75

–7

6

77

–7

8

79

–8

0

81

–8

2

83

–8

4

85

–8

6

87

–8

8

89

–9

0

91

–9

2

93

–9

4

95

–9

6

97

–9

8

99

–1

00

10

1–

10

2

Age range (years)

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Impact of diagnostic delay

Time

bleeding

to definite diagnosis

No. of

patients(%)

Median

FVIII

activity

[U/dL (IQR)]

Median

inhibitor

titer

[BU/mL (IQR)]

Hb

[g/dL (IQR)]

Severe

bleeding [n (%)]

Median time to start of

haemostatic therapy

[days (IQR)]

Median time to bleeding

resolved [days (IQR)]

0-1 day 174

(38.2)

2

(1-4)

14

(6-58)

9.4

(7.6-9.4)

121

(70.0)

1

(0-3)

4

(2-11)

2-7 days 121

(26.5)

2

(1-5)

15

(5-41)

8.5

(7.1-10.7)

95

(78.5)

4

(2-5)

4

(2-9)

>7 days 161

(35.3)

2

(0-6)

7

(2-30)

8.9

(7-11.3)

108

(67.1)

20

(12-43)

5

(2-13)

P* NA NS NS NS NS <0.0001 NS

Data are reported as n (%) and median (IQR) for categorical and continuous variables, respectively.

*Kruskal-Wallis test.

Diagnostic delay increases the interval between onset of bleeding

and start of haemostatic therapy

Bled, 2014

Disorder No. of

patients (%)

Idiopathic (no underlying

disorder reported) 260 (51.9)

Malignancy 59 (11.8)

Autoimmune diseases 58 (11.6)

Pregnancy 42 (8.4)

Infections 19 (3.8)

Drug-induced 17 (3.4)

MGUS 13 (2.6)

Polymyalgia rheumatica 11 (2.2)

Dermatology 7 (1.4)

Blood product transfusion 4 (0.8)

Others disorders 41 (8.2)

Underlying disorders

34.5% rheumatoid arthritis

67.8% solid tumours

MGUS, monoclonal gammopathy of undetermined significance.

Distribution consistent

with previous reports

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Underlying disorders

autoimmune

17%

postpartum

6%

neoplastic

10% psoriasis

4%

MGUS

6%

TTP

2%

idiopathic

55%

Nemes L. et al: Haematologica, 2003; 88: 106-110

Bled, 2014

Acquired haemophilia:

rheumathoid arthritis

Bled, 2014

Giant dorsal haematoma in

PSS-associated AH

Nemes L, Pitlik E:Haematologica, 2000; 85: 64-68 Bled, 2014

Nemes L, Pitlik E:Haematologica, 2000; 85: 64-68

Retroperitoneal haematoma in

paraneoplastic acquired haemophilia

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EACH2: Initial inhibitor titer

0

5

10

15

20

25

30

35

0 1 10 100 1000

FVIII:inhibitor titer (BU/mL)

Knoebl P. et al: JTH, 2012; 10:622-631 Bled, 2014

EACH2: Initial FVIII:C

0

20

40

60

80

100

120

140

0 5 10 15 20 25 30 35 40

FVIII:activity (U/dL)

Knoebl P. et al: JTH, 2012; 10:622-631 Bled, 2014

EACH2: probability of survival

0 1 2 3 4 5 6 7 yrs.

0.0

0.2

0.4

0.6

0.8

1.0

inhibitor eradicated, off therapy (n=366)

inhibitor not eradicated, on immunisuppression (n=63)

inhibitor not eradicated, off therapy (n=47)

p<0.0001 (log-rank test)

Pro

bability o

f surv

ival (a

ll p

ts.)

Knoebl P. et al: JTH, 2012; 10:622-631

Bled, 2014

All Severe Non severe P*

Total no. of bleeding

episodes [n (%)] 474 333 (70.3) 137 (28.9) NA

Cause [n (%)]

Spontaneous 367 (77.4) 250 (76.0) 113 (83.7) NS

Trauma 40 (8.4 ) 33 (10.0) 7 (5.2) NS

Surgery 39 (8.2) 30 (9.1) 9 (6.7) NS

Peripartum 17 (3.6) 14 (4.3) 2 (1.5) NS

Other 13 (2.7) 8 (2.4) 4 (3.0) NS

Initial bleeding events: cause

Bled, 2014

All Severe Non severe P*

Total no. of bleeding

episodes [n (%)] 474 333 (70.3) 137 (28.9) NA

Site/type [n (%)]

Skin 252 (53.2) 152 (46.2) 97 (71.9) <0.0001

Deep 238 (50.2) 214 (65.0) 21 (15.6) <0.0001

Mucosa 150 (31.6) 113 (34.4) 35 (25.9) NS

Haemarthrosis 23 (4.9) 17 (5.2) 6 (4.4) NS

CNS 5 (1.1) 5 (1.5) 0 (0) NS

Initial bleeding events:

site/type

Deep; musculoskeletal or retroperitoneal

CNS; central nervous system

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Total [n] 474

1 bleeding episode without relapse [n (%)] 315 (66.5%)

> 1 bleeding episodes [n (%)] 159 (33.5%)

2 bleeding events 108

3 bleeding events 35

4 – 7 bleeding events 16

Median time between 1st and 2nd bleeding

episode

28 days (IQR 14-69)

Further bleeding events

Bled, 2014

Management of hemorrhage:

General and adjunctive measures

• Avoidance of anticoagulants, ASA and NSAIDS

• Rest, immobilisation, cold application (RICE)

• Topical haemostatic agents (thrombin, fibrin sealants, topical gelatin, local antifirinolytic solutions, collagen)

• Antifibrinolytics (tranexamic acid, aminocaproic acid)

• Contraindication: DIC, haematuria, concurrent FEIBA administration

Bled, 2014

Management of

hemorrhage: DDAVP

• Well-documented use in vWD, and in uncomplicated mild haemophilia A

• Dose: 1-2 x 0,3-0,4 microg/bwkg/day

• Case reports in low-titer autoantibody pts with measurable FVIII:C1,2

• Not useful in life-threatening bleeding situations, major operations or in high titer inhibitors

• Test dose and APTT- FVIII:C monitoring is necessary

Bled, 2014

1de la Fuente: Br J Haemat 1985, 2Vincente V: Br J Haemat 1985


High-dose human FVIII

• Inhibitor titer should be less than 5 BU

• With no anamnestic response

• The choice of FVIII:

PD vs. rFVIII and HP vs vWF-containing?

• Dose: 2-4x20 IU/kg for each BU + 40 IU/kg

• Unpredictable FVIII recovery and half-life in

autoantibody pts – close laboratory follow up is

necessary

Bled, 2014


poFVIII

• Autoantibodies usually show low porcine cross-reactivity

• Laboratory monitoring: cross-reactivity, APTT correction, FVIII:C recovery is strongly recommended1

• 80 % of AH pts have good-to-excellent clinical response2

• In October, 2004 the production was discontinued (no viral inactivation, po parvovirus contamination)

• rBDD-po-FVIII formulation is in clinical development

• Efficacy? Immunogenicity?

Bled, 2014

1Morrison et al, Blood, 1993;81:1513-20 2Kessler et al, Semin Hematol, 1993;30:22-7


APCC - FEIBA

• APCC-s are more effective than PCC-s (alloantibody data)

• Uncontrolled retrospective case collections1

• Dose 2-3 x 50-100 IU/bwkg/day

• Bolus dose > 200 IU/bwkg increases the risk of thrombotic side-effects (DIC)

• First line APCC in 34 AH pts: CR – 100 % in moderate and 76 % in severe bleeding episodes2

• Anamnestic response (traces of FVIII in the product) is of no significance in AH

Bled, 2014

1Goudemand et al: Haemophilia 2004;10:14 2Sallah S et al: Haemophilia 2004;10:169-73


rFVIIa - NovoSeven

• Dose 90 microg/kg/2-3h (megadose: 270 microg/kg?)

• Efficacy as frontline Tx: 100 %, rescue Tx: 75%1

• Early treatment is important1

• Case presentations of arterial thrombosis associated with the use of rFVIIa in AH

• AMI in AH during the treatment course with rFVIIa2

• 10/139 (7.2 %) (arterial) thromboembolic events in AH pts treated with rFVIIa3

Bled, 2014

1Hay CRM et al. TH 1997;78:1463 2 Guillet B et al: TH 2002;88:698-9 3Sumner MJ et al:Haemophilia 2007;13:451-61


rFVIIa - NovoSeven

• Retrospective analysis of 139 pts treated with rFVIIa during 182 bleeding episodes1

• First-line Tx: 103 bleeding episodes: effective/partially effective in 95 %

• Second-line Tx: effective/partially effective in 80 %

• Surgeries: 57 effective/partially effective in 86 %

• Mean duration of Tx: 61 pts 6 days (range 1-33)

Bled, 2014

1Sumner MJ et al:Haemophilia 2007;13:451-61

Algorithm for treatment of acute

bleeds in acquired haemophilia

autoantibody

0,6-5 BU 5BU-

HD-Hu-FVIII 2-3x200IU/kg

APCC 2-3x50-100 IU/kg

Or rFVIIa

90microg/kg/2-3h

APCC 2-3x50-100 IU/kg

Or rFVIIa

90microg/kg/2-3h

Extracorporal plasmapheresis immunoadsorption

Bled, 2014

Kessler CM, Acs P, Mariani G: The immune coagulopathies. In: Hemostasis and Thrombosis Vth ed.

International recommendations

Haematologica 2009; 94:566-575. doi:10.3324/haematol.2008.001743

Bled, 2014

• Active severe bleeding:

~at least 24-72 h of treatment

first line: by-passing agents

– rFVIIa 90 microg/kg/2-3 h or

– APCC 50-100 IU/kg/8-12 h (<200IU/kg/day)

if unavailable:

– PD- or rFVIII or DDAVP

second line – immunoadsorption and/or plasmapheresis

International recommendations

on the Dx and Tx of pts with AH

Bled, 2014

Huth-Kühne et al: Haematologica 2009;94:566-75

Management of bleeding in acquired hemophilia A (AHA): Results from the

European Acquired Haemophilia (EACH2) Registry

Francesco Baudo, Peter Collins, Angela Huth-Kuehne, Hervé Lévesque, Pascual Marco, László Nemes, Fabio Pellegrini, Lilian Tengborn, Paul

Knoebl on behalf of the EACH2 registry contributors;

Blood 2012;120:39-46

Bled, 2014

• Only first bleeding episodes were analysed

• Primary endpoint:

• Control of first bleeding episode treated with first-line haemostatic therapy

• Baseline characteristics were matched using propensity score (PS) methodology1,2

• Two treatment comparisons:

1. Bypassing agents (rFVIIa or aPCC) vs. strategies to increase FVIII levels (FVIII concentrate and DDAVP)

2. rFVIIa vs. aPCC

Study analysis

1. Parsons LS. Reducing bias in a propensity score matched-pair sample using greedy matching techniques. In: Proceedings of

the Twenty-Sixth Annual SAS Users Group International Conference. Cary, NC: SAS Institute; 2004..

2. D'Agostino RB, Jr. Stat Med 1998; 17:2265-81.

Bled, 2014

Patient disposition

EACH2 cohort

n=501

At least one bleeding episode

n=482

No bleeding episodes

n=19

Initiated haemostatic/

ancillary treatment

n=338

Not treated

n=144

Ancillary therapy only

n=31

Main outcome available

(bleeding resolved vs.

not resolved)

n=288

Main outcome not available

n=19

Treated with rFVIIa, aPCC,

FVIII or DDAVP

n= 307

Bled, 2014

First-line haemostatic treatment

174

63

56

14

First-line haemostatic treatment (n=307)

rFVIIa

aPCC

FVIII

DDAVP

Bled, 2014

First-line haemostatic therapy for all first

bleeding episodes

IQR, interquartile range

BU, Bethesda Units

Therapy n Baseline FVIII

level IU/dL

Baseline inhibitor titre

BU/mL

Initial dose µg/kg or U/kg

Initial dosing interval

h

Total doses per patient

n

Total dose per patient

rFVIIa 174 2.0

(0.0-32.0) 15.5

(1.0-2765) 90

(84.71-102.86) 3

(2-6) 12

(3-35) 84 mg

(24-216)

aPCC 63 1.0

(0.0-40.0) 18.0

(0.1-1700) 66.67

(52.63-82.19) 12

(12-12) 8

(3-15) 30,000 U

(12,000-56,000)

FVIII 56 3.0

(0.0-34.0) 7.5

(0.8-180) 52.91

(40.00-81.97) 12

(8-12) 5

(2-10) 20,000 U

(9,000-49,500)

DDAVP 14 3.5

(0.0-17.0) 8.0

(0.3-200) 0.3

(0.3-0.3) 12

(8-24) 2.5

(1-3) 40 µg

(21-64)

Patients treated with bypassing agents had lower FVIII levels and higher

inhibitor titres.

Bled, 2014

Bleeding control

PS, propensity score; rFVIIa, recombinant activated factor VII; aPCC, activated prothrombin complex concentrate;

FVIII, coagulation factor VIII; DDAVP, 1-desamino-8-D-arginine-vasopressin

Bleeding control was significantly higher in patients treated with

bypassing agents vs. FVIII/DDAVP and similar between rFVIIa and aPCC.

Unmatched groups PS-matched groups

Bypassing

agent

91.8%

Replacement

therapy

69.6%

rFVIIa

91.2%

aPCC

93.3%

Bypassing

agent

93.3%

Replacement

therapy

68.3%

rFVIIa

93.0%

aPCC

93.0%

FVIII

70.1%

DDAVP

64.3%

Bled, 2014

• Bleeding was controlled in 269 patients overall

• Responders vs. non-responders

• Difference in delay in time to treatment

(median 1.00 vs. 4.00 days; P=0.0155)

• No difference in

FVIII levels at time of first bleeding episode

Inhibitor titre

Bleeding cause

Bleeding site

Severity

• Recurrence

• 68 patients

• Median time to recurrence of 14 days

Bleeding control

Bled, 2014

• Thrombotic events were significantly associated with age (79.4 vs.

68.3, P=0.0341), but not with underlying clinical condition (P=0.6302)

Thrombotic events

Bled, 2014

Thrombotic events and

haemostatic therapy

n Thrombotic event

n (%)

Patients with ≥1 bleeding episode 482 13 (2.7)

No haemostatic treatment 144 2 (1.4)

First-line haemostatic treatment 307 11 (3.6)

FVIII 56 0

DDAVP 14 0

rFVIIa 174 5 (2.9)

aPCC 63 3 (4.8)

Thrombotic events were reported in 3.6% of patients treated with a

haemostatic agent with a similar incidence between rFVIIa and aPCC.

It is not possible to draw any definite conclusions about the causal

relationship between the haemostatic treatment and the thrombotic event

from the data available in the registry.

Bled, 2014

• 16/482 patients who experienced at least one bleeding episode

and 10/307 patients treated with haemostatic therapy died as a

result of bleeding (mortality 3.3%)

• 4/16 deaths occurred on the first day of therapy

• Median time from therapy to death 23 days

Mortality related to bleeding

1. Collins PW, et al. Blood 2007; 109:1870-7.

The registry reports a mortality rate of 3.3%, much lower than the current

literature.1

Bled, 2014

• Optimal treatment of bleeding in AHA comprises

bypassing agents (rFVIIa or aPCC)

• Can be expected to resolve bleeding in >90% of

cases

• Bleeding control similar with rFVIIa and aPCC

• The data support caution in the use of bypassing

agents due to an possible association with

thrombotic events

Key conclusions of EACH2

regarding acute treatment

Bled, 2014

How I manage? BJH review

British Journal of Haematology, 2013, 161, 157–165

Bled, 2014

UKHCDO AHA guideline

British Journal of Haematology, 2013, 162, 758–773 Bled, 2014

UKHCDO AHA guideline

British Journal of Haematology, 2013, 162, 758–773

Bled, 2014

FEIBA in acquired

haemophilia: patient cohort

• 43 consecutive non-haemophiliac FVIII inhibitor patients treated in a single center with our ITI protocol

• 6 historical control patients treated with traditional immunosuppression Tx (st.+/-CPH)

• In summary 49 pts were included in the analysis

• 37 pts received FEIBA

• 3 pts did not need any TX for acute bleeding

• 3 LR pts received HD-Hu or PoFVIII (Hyate:C)

• 6 pts was treated by rFVIIa (NovoSeven)

Bled, 2014

FEIBA in AHA – single

center experiences (I.) initials sex F/M age at Dx underlying condition initial bleeding res.FVIII:C(%)initial BIA FEIBA dose

E. B M 58 psoriasis pharyngeal 1 60 2x75

K. L. F 75 idiopathic femoral haematoma 14 28 2x45

S. B. M 69 idiopathic pharyngeal 5 20 2x50

J. B. F 69 idiopathic pharyngeal 5 20 2x50

M. K. F 60 PSS brachial, epistaxis 16 10 2x90

L. L. F 65 idiopathic thoracic w all 2 30 2x45

K. B. M 22 TTP, pheresis sc.,musc.haematomas 6 4,7 2x100

S. H M 75 idiopathic retroperitoneal 1 14 2x100

I. F. F 77 Sjögren sy. femoral haematoma 7 5 1x80

B. F. F 65 RA Hhaemarthros gen lu. 2 118 2x50

F. R. M 74 gastric cc. gluteal haematoma 1,4 19 1x100

A. P. M 60 idiopathic femoral haematoma 2 58 1x75

I. J. F 65 psoriasis retroperitoneal 1 1128 2x75

E. N. F 64 idiopathic thoracic w all 1 9 1x100

S. D. M 78 MGUS femoral haematoma 8 30 1x75

I. T. F 53 idiopathic sc.musc.haemat. 4 20 1x75

Gy. Sz. M 55 renal cc+IFN alfa retroperit.+central line 5 320 2x100

J. P. F 30 postpartum fem.haemat., GU 1 33 2x75

P. F. M 79 gastric cc. retroperitoneal 4 22 1x75

J. T. M 80 idiopathic femoral + GI 6 2,2 2x75

J. H. F 36 PSS dorsal haematoma 1 3200 2x75

Bled, 2014

A. Cs. M 62 idiopathic femoral, pharyngeal 1 437 2x75

J. A F 60 SLE retroperitoneal, sc. 3 134 2x60

L. V. F 73 idiopathic femoral, sc. 1 205 1x100

Gy. K M 70 idiopathic abdominal, sc. 6 223 1x100

L. P. F 70 RA thoracic w all 7 245 1x75

I. M. M 60 idiopathic femoral 8 189 1x75

B. P. M 80 mycoplasma pneumonia brachial, sc. 1 18 1x50

A. K. F 24 postpartum femoral, brachial 5 245 1x75

S. L. F 70 colol cc. femoral, brachial 1 7 2x75

Z. L. M 49 idiopathic retroperitoneal 3 109 2x75

J. P. F 60 idiopathic femoral, brachial 1 19 2x65

L. Sz. F 80 RA gluteal haematoma 1 14 2x75

P. Cs. M 69 idiopathic femoral, brachial 1 145 1x100

Mean M/F VIIIF:C % BU BU IU/bwkg

20/17 61 4 198 105


center experiences (II.)

Bled, 2014

• FEIBA was used in 37 high titer AH pts

• Average daily dose of FEIBA: 105 IU/bwkg

• Efficacy has been excellent (35/37 excellent/good response)

• 2 failures occurred (central line insertion operation) in which cases the patients were switched to either PoFVIII or rVIIa

• No thromboembolic or allergic complications were noted (large doses were slowly infused)

• The mean dose in AH was less than the suggested maximal daily dose of the drug


center experiences (III.)

Bled, 2014

FEIBA prophylaxis in AH –

case presentation

Bled, 2014

• Prophylactic APCC administration can be a valuable

therapeutic alternative to on demand treatment

- in patients with acquired haemophilia

- who have failed eradication and

- experience serious dangerous bleeding episodes

3 pts, 2 female, 1 male

2 idiopathic, 1 postpartum AH

Average dose: 2x85 IU/bwkg/week

In our patients, FEIBA prophylaxis decreased the average

bleeding frequency from 1,5/month to 0,12/month

without paying the price of higher costs

FEIBA prophylaxis in AH –

conclusions

Bled, 2014

Thank you for your attention!

Slovenien Haematology-Transfusiology Congress 2014

03rd – 04th of October, 2014, Bled

Treatment of acute bleeds in Acquired Haemophilia

treatment of acute bleeds in acquired haemophilia l j2014.pdf · acquired haemophilia a: results...

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