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Table 1 Characteristics of patients with association of AHA and plasma cell neoplasms – a systematic review

From: Acquired hemophilia A and plasma cell neoplasms: a case report and review of the literature

Patient no.

Author, year [reference]

Sex

Age (years)

Diagnosed first

Bleeding

FVIII:C (%)

FVIII inhibitor (BU/ml)

Hemostatic treatment

Treatment AHA inhibitor eradication

AHA outcome

Paraprotein

Treatment PCN

PCN outcome

Alive/died

1

Glueck et al., 1965 [5]

M

70

PCN

MC, RT

NA

NA

NA

C

NA

NA

NA

NA

NA

2

Loftus et al., 1994 [6]

F

58

PCN

MC, A

8

36

FVIII, pFVIII

S, C

Bleeding continued

Lambda light chain

M

NA

Died of intra-abdominal bleeding

3

Stricker et al., 1994a [7]

M

52

PCN

I

2

17.8

FVIII, plasma

S, PEX

Normal APTT and FVIII:C

Kappa light chain

IFN-α, ASCT

CR

Died of sudden cardiac death

4

Sallah et al., 2000 [8]

F

58

AHA

MC

< 1

28

pFVIII, APCC

S, PEX

Inhibitor persisted

NA

M

Died

Died of acute renal failure/hemorrhage

5

Holme et al., 2005 [9]

M

58

AHA

I

6

20

APCC

S, C

NA

NA

NA

PR

Alive

6

Sari et al. 2009 [10]

F

43

AHA

I

6

10

No treatment

No treatment

Normal coagulation

IgG kappa

VinOD, ASCT

CR

NA

7

Decaux et al. 2009 [11]

F

44

PCN

MC

6

29

rFVIIa

R

NA

IgA kappa

NA

NA

NA

8

Muzaffar et al. [12]

M

65

PCN

PE, HA

< 5

9.5

APCC, plasma

IVIG, R

FVIII:C 22%, no FVIII inhibitor

Lambda light chain

VTD

CR

Alive

9

Saburi et al. 2015a [13]

F

67

PCN

NA

2

4.9

No treatment

S, C

Normal APTT, FVIII inhibitor 4.85 BU/ml

NA

VD, LCD

CR

NA

10

Ross et al. [14]

F

64

AHA

MC, HP

17

5

rFVIIa

S

Normal coagulation

IgM kappa

VTD

NA

NA

11

Innao et al. [15]

M

67

PCN

NA

28

NA

FVIII

No treatment

Normal coagulation

IgG kappa, kappa light chain

VMP, ASCT

CR

Alive

12

Brás, et al. [16]

M

87

PCN

MC, IM

1.4

18.4

APPC

S, C, B

Normal APTT, FVIII:C 36%, FVIII inhibitor 0.8 BU/ml

IgG kappa

MTP, VD

PR

NA

13

Napolitano et al. 2017 [17]

F

59

AHA

MC, HA, A

12

70

rFVIIa, APCC

S, R

Normal coagulation

IgG lambda

VMP

CR

Alive

14

Kawashima et al. 2018 [18]

M

52

PCN

IM

17

1

rFVIIa

No treatment

Normal coagulation

IgA kappa

VD, VCD, VTD, LD, ASCT, allo-HCT

CR

NA

15

Sourdeau et al. 2019 [19]

M

78

PCN

ST

< 1

19

NA

NA

NA

NA

VCD

NA

NA

16

Our patient

M

77

AHA

IM

2

102

rFVIIab

S, C, IA

Normal APTT, normal FVIII

IgG kappa

VRD, RD

VGPR

Alive

  1. Abbreviation: AHA acquired hemophilia, PCN plasma cell neoplasm, NA not available, n.d. not done
  2. Bleeding: MC mucocutaneous bleeding (epistaxis, gingiva, soft tissue, gastro-intestinal, gynecological), I iatrogenic (postoperative, after biopsy or dental procedure), A intra-abdominal, HA hemarthrosis, PE pericardial bleeding, HP hemoptysis, RT retinal bleeding, IM intramuscular
  3. Hemostatic treatment: rFVIIa recombinant activated factor VII, aPCC activated prothrombin complex concentrate, FVIII factor VIII (human plasma or recombinant), pFVIII porcine factor VIII, plasma fresh frozen plasma or cryoprecipitate
  4. Other treatment: allo-HCT allogenic stem cell transplantation, ASCT autologous stem cell transplantation, C cyclophosphamide, CR complete remission, D dexamethasone, IA immunoadsorption, INF-a interferon alpha, IVIG intravenous immunoglobulin, L lenalidomide, M melphalan, O doxorubicin, P prednisone, PEX plasma exchange, PR partial remission, R Rituximab, S steroids, T thalidomide, V bortezomib, Vin vincristine, VGPR very good partial remission
  5. aAHA considered a side effect of plasma cell disease treatment (discussed in text)
  6. bHemostatic treatment only for interventions (bone marrow biopsy, surgery)