Table 5. Acquisition of POEMS syndrome... | American Society of Hematology

Table 5.

Acquisition of POEMS syndrome features over time

Patient characteristics			Initial POEMS features									Subsequently acquired POEMS features	TTP, y	FU, y	Response to therapy	Current status
No.	Sex	Age, y	P	O	E	M	S	A	B, no.	C	Pap	Subsequently acquired POEMS features	TTP, y	FU, y	Response to therapy	Current status
1	F	66	P			M			Diff			Hypothyroid, hypoadrenal at 126 mo; ascites, pulmonary hypertension at 156 mo	10.5	14.5	V gd	Alive
2	F	34	P			M			2 or 3			Ascites, Budd-Chiari at 149 mo; pleural effusion at 156 mo	12.4	13.4	V gd	Alive
3	F	34	P			M	S		Diff			Detectable monoclonal protein in serum at 39 mo; hypothyroid at 60 mo; ascites and hepatomegaly at 87 mo	5.0	7.8	Stable	Dead
4	M	46	P	O		M		A	Diff			Pulmonary hypertension and effusion at 36 mo; hypothyroid at 52 mo; acrocyanosis at 54 mo	3.0	5.8	Good	Alive
5	F	58	P			M	S	A	Diff		Pap	Hypothyroid at 9 mo; ascites and renal failure at 12 mo	0.8	3.3	Good	Dead
6	M	43	P			M	S		2 or 3			Hypoadrenal at 4 mo	0.3	1.0	Refr	Alive
7	F	50	P			M	S		1			Pericardial effusion at 1 mo	0.1	0.2	Refr	Dead
8	M	38	P		E	M	S		1			CHF, hypogonad, hypothyroid, hypoadrenal, peripheral edema, acrocyanosis, hypertrichosis, hyperpigmentation at 192 mo	16.0	16.4	V gd	Alive
9	F	33	P		E	M	S		1		Pap	Peripheral edema at 90 mo	7.5	9.5	V gd	Alive
10	M	56	P		E	M	S		2 or 3			Diabetes mellitus at 94 mo	7.8	9.1	V gd	Alive
11	F	44	P		E	M	S		1		Pap	Ascites and CHF at 56 mo	4.7	5.0	Good	Dead
12	M	51	P	O		M	S		Diff	C		Clubbing, ascites, and renal failure at 52 mo	4.3	4.9	ND	Dead
13	F	61	P		E	M	S		2 or 3			Ascites at 1 mo	0.1	0.7	ND	Alive
14	F	57	P	O	E	M	S	A	Diff			Ascites at 22 mo; renal failure and oxygen dependence at 117 mo	1.8	10.0	V gd	Dead
15	F	50	P	O	E	M	S		2 or 3		Pap	Ascites at 12 mo; respiratory failure at 66 mo	1.0	5.7	Good	Dead
16	M	51	P	O	E	M	S		2 or 3			Hepatomegaly and hepatic dysfunction at 30 mo	2.5	2.8	Good	Dead
17	M	39	P	O	E	M	S		1			Lymphadenopathy at 17 mo; restrictive lung disease at 36 mo	1.4	1.4	Good	Alive
18	M	64	P	O	E	M	S	A	Diff		Pap	Hypoparathyroid at 84 mo	7.0	14.6	Good	Alive

Patient characteristics			Initial POEMS features									Subsequently acquired POEMS features	TTP, y	FU, y	Response to therapy	Current status
No.	Sex	Age, y	P	O	E	M	S	A	B, no.	C	Pap	Subsequently acquired POEMS features	TTP, y	FU, y	Response to therapy	Current status
1	F	66	P			M			Diff			Hypothyroid, hypoadrenal at 126 mo; ascites, pulmonary hypertension at 156 mo	10.5	14.5	V gd	Alive
2	F	34	P			M			2 or 3			Ascites, Budd-Chiari at 149 mo; pleural effusion at 156 mo	12.4	13.4	V gd	Alive
3	F	34	P			M	S		Diff			Detectable monoclonal protein in serum at 39 mo; hypothyroid at 60 mo; ascites and hepatomegaly at 87 mo	5.0	7.8	Stable	Dead
4	M	46	P	O		M		A	Diff			Pulmonary hypertension and effusion at 36 mo; hypothyroid at 52 mo; acrocyanosis at 54 mo	3.0	5.8	Good	Alive
5	F	58	P			M	S	A	Diff		Pap	Hypothyroid at 9 mo; ascites and renal failure at 12 mo	0.8	3.3	Good	Dead
6	M	43	P			M	S		2 or 3			Hypoadrenal at 4 mo	0.3	1.0	Refr	Alive
7	F	50	P			M	S		1			Pericardial effusion at 1 mo	0.1	0.2	Refr	Dead
8	M	38	P		E	M	S		1			CHF, hypogonad, hypothyroid, hypoadrenal, peripheral edema, acrocyanosis, hypertrichosis, hyperpigmentation at 192 mo	16.0	16.4	V gd	Alive
9	F	33	P		E	M	S		1		Pap	Peripheral edema at 90 mo	7.5	9.5	V gd	Alive
10	M	56	P		E	M	S		2 or 3			Diabetes mellitus at 94 mo	7.8	9.1	V gd	Alive
11	F	44	P		E	M	S		1		Pap	Ascites and CHF at 56 mo	4.7	5.0	Good	Dead
12	M	51	P	O		M	S		Diff	C		Clubbing, ascites, and renal failure at 52 mo	4.3	4.9	ND	Dead
13	F	61	P		E	M	S		2 or 3			Ascites at 1 mo	0.1	0.7	ND	Alive
14	F	57	P	O	E	M	S	A	Diff			Ascites at 22 mo; renal failure and oxygen dependence at 117 mo	1.8	10.0	V gd	Dead
15	F	50	P	O	E	M	S		2 or 3		Pap	Ascites at 12 mo; respiratory failure at 66 mo	1.0	5.7	Good	Dead
16	M	51	P	O	E	M	S		2 or 3			Hepatomegaly and hepatic dysfunction at 30 mo	2.5	2.8	Good	Dead
17	M	39	P	O	E	M	S		1			Lymphadenopathy at 17 mo; restrictive lung disease at 36 mo	1.4	1.4	Good	Alive
18	M	64	P	O	E	M	S	A	Diff		Pap	Hypoparathyroid at 84 mo	7.0	14.6	Good	Alive

A indicates anasarca, edema, ascites, or pleural effusion; B, bone lesions; C, Castleman disease; CHF, congestive heart failure; Diff, diffuse; E, endocrinopathy; FU, follow-up; M, monoclonal plasma cell dyscrasia; ND, no data; O, organomegaly (including hepatomegaly, splenomegaly, and lymphadenopathy); P, peripheral neuropathy; Pap, papilledema; Refr, refractory disease; S, skin changes; TTP, time to progression; and V gd, very good.

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