Table 1.

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

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

Abbreviation: AHA acquired hemophilia, PCN plasma cell neoplasm, NA not available, n.d. not done

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

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

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

^aAHA considered a side effect of plasma cell disease treatment (discussed in text)

^bHemostatic treatment only for interventions (bone marrow biopsy, surgery)