J Hematol

Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc

Journal website https://www.thejh.org

Review

Volume 11, Number 4, August 2022, pages 123-130

Treatment of Light Chain Deposition Disease: A Systematic Review

Figure 2. Hematological response comparison based on treatment regimen. *No response patients not included in the graph: three patients with ASCT and five patients with thalidomide-based therapies. ASCT: autologous stem cell transplant; BorD: bortezomib and dexamethasone; Bor: bortezomib; no.: number; CR: complete response; VGPR: very good partial response; PR: partial response; PD: progression of disease.

Figure 3. Renal response comparison based on treatment regimen. No.: number; ASCT: autologous stem cell transplant; BorD: bortezomib and dexamethasone; Bor: bortezomib-based regimens.

**Table 1.** Comparison of Hematologic and Organ Responses With Various Regimens for Light Chain Deposition Disease
First author, year	Evaluable patients (LCDD only), n (%)	Primary treatment	Conditioning regimen if ASCT	Median age in years (range)	Hematologic response (VGPR or greater)	Biopsy-proven renal disease	Renal response, n (%)	Survival outcomes
LCDD: light chain deposition disease; ASCT: autologous stem cell transplant; BorD: bortezomib + dexamethasone; Dex: dexamethasone; VAD: vincristine + adriamycin + dexamethasone; VGPR: very good partial response; n: number; NA: not available; mos: months; f/u: follow-up.
Lorenz et al, 2008 [11]	3/6 (50%)	Dex (67%) followed by ASCT	Melphalan 200 mg/m² or reduced	39 (33 - 43)		100%	1 (33%)	66% alive after median f/u of 46.2 mos
Kimura et al, 2018 [12]	3/3 (100%)	Prior BorD (33%) followed by lenalidomide-based regimen		60 (59 - 69)	100%	100%	0 (0%)
Jimenez-Zapeda et al, 2012 [13]	6/6 (100%)	BorD (50%) or Dex (50%) followed by ASCT	Melphalan 140 - 200 mg/m²	NA	83%		NA	Median f/u 23 mos
Kastritis et al, 2009 [14]	4/4 (100%)	Various prior (VAD or cyclophosphamide/prednisone) followed by BorD then ASCT (75%)	High-dose melphalan	52 (46 - 56)	67%	100%	3 (100%)	100% alive after f/u of 10 - 18 mos
		BorD only (25%)		67	0%		1 (100%)
Lessi et al, 2012 [15]	4/4 (100%)	BorD only (25%)		44.5 (37 - 64)	67%	75%	4 (100%)
		BorD followed by ASCT (75%)	Melphalan 140 mg/m²		0%
Minarik et al, 2012 [16]	3/3 (100%)	BorD		48 (33 - 56)	NA	100%	1 (33%)
Sayed et al, 2015 [17]	25/53 (47%)	Thalidomide based (n = 11)		56 (29 - 78)	45%	100%	NA	Median f/u 74.4 mos
		Bortezomib based (n = 9)			89%
		ASCT (n = 4)	Melphalan		100%
		Lenalidomide based (n = 1)			0%
Telio et al, 2012 [18]	5/8 (63%)	BorD (20%) or Dex (60%) followed by ASCT	Melphalan 140 - 200 mg/m²	48 (40 - 55)	20%	100%	4 (80%)	Median f/u 29 mos
Tovar et al, 2012 [19]	3/3 (100%)	BorD followed by ASCT (100%)	Melphalan 140 - 200 mg/m²	48 (36 - 63)	100%	100%	2 (67%)	100% alive after a median f/u of 34 mos
Weichman et al, 2006 [20]	4/6 (67%)	ASCT	Melphalan 140 - 200 mg/m²	45 (36 - 51)	100%	100%	2 (50%)	100% alive after a median f/u of 12 mos
Hassan Zafar et al, 2011 [21]	4/20 (20%)	BorD		NA	25%	100%
Kastritis et al, 2021 [22]	6/25	Daratumumab		67.5 (59 - 83)	67%			Median f/u 25 mos

Table 1. Comparison of Hematologic and Organ Responses With Various Regimens for Light Chain Deposition Disease

First author, year

Evaluable patients (LCDD only), n (%)

Primary treatment

Conditioning regimen if ASCT

Median age in years (range)

Hematologic response (VGPR or greater)

Biopsy-proven renal disease

Renal response, n (%)

Survival outcomes

LCDD: light chain deposition disease; ASCT: autologous stem cell transplant; BorD: bortezomib + dexamethasone; Dex: dexamethasone; VAD: vincristine + adriamycin + dexamethasone; VGPR: very good partial response; n: number; NA: not available; mos: months; f/u: follow-up.

Lorenz et al, 2008 [11]

3/6 (50%)

Dex (67%) followed by ASCT

Melphalan 200 mg/m² or reduced

39 (33 - 43)

100%

1 (33%)

66% alive after median f/u of 46.2 mos

Kimura et al, 2018 [12]

3/3 (100%)

Prior BorD (33%) followed by lenalidomide-based regimen

60 (59 - 69)

100%

0 (0%)

Jimenez-Zapeda et al, 2012 [13]

6/6 (100%)

BorD (50%) or Dex (50%) followed by ASCT

Melphalan 140 - 200 mg/m²

83%

Median f/u 23 mos

Kastritis et al, 2009 [14]

4/4 (100%)

Various prior (VAD or cyclophosphamide/prednisone) followed by BorD then ASCT (75%)

High-dose melphalan

52 (46 - 56)

67%

100%

3 (100%)

100% alive after f/u of 10 - 18 mos

BorD only (25%)

1 (100%)

Lessi et al, 2012 [15]

4/4 (100%)

BorD only (25%)

44.5 (37 - 64)

67%

75%

4 (100%)

BorD followed by ASCT (75%)

Melphalan 140 mg/m²

Minarik et al, 2012 [16]

3/3 (100%)

BorD

48 (33 - 56)

100%

1 (33%)

Sayed et al, 2015 [17]

25/53 (47%)

Thalidomide based (n = 11)

56 (29 - 78)

45%

100%

Median f/u 74.4 mos

Bortezomib based (n = 9)

89%

ASCT (n = 4)

Melphalan

100%

Lenalidomide based (n = 1)

Telio et al, 2012 [18]

5/8 (63%)

BorD (20%) or Dex (60%) followed by ASCT

Melphalan 140 - 200 mg/m²

48 (40 - 55)

20%

100%

4 (80%)

Median f/u 29 mos

Tovar et al, 2012 [19]

3/3 (100%)

BorD followed by ASCT (100%)

Melphalan 140 - 200 mg/m²

48 (36 - 63)

100%

2 (67%)

100% alive after a median f/u of 34 mos

Weichman et al, 2006 [20]

4/6 (67%)

ASCT

Melphalan 140 - 200 mg/m²

45 (36 - 51)

100%

2 (50%)

100% alive after a median f/u of 12 mos

Hassan Zafar et al, 2011 [21]

4/20 (20%)

BorD

25%

100%

Kastritis et al, 2021 [22]

6/25

Daratumumab

67.5 (59 - 83)

67%

Median f/u 25 mos