Abstract
TEMPI syndrome is a rare disease associated with plasma cell neoplasms. Although previous studies have reported that bortezomib is effective as a first-line treatment for TEMPI syndrome, some cases are refractory to this treatment. Pomalidomide, a kind of immunomodulatory drug, is widely used for the treatment of relapsed or refractory multiple myeloma and could be administered without dose modification in patients with renal dysfunction. We present a case of bortezomib-refractory TEMPI syndrome with renal insufficiency that was successfully treated with a combination of pomalidomide and low-dose dexamethasone with minimal adverse effects, followed by autologous hematopoietic stem cell transplantation (ASCT). To the best of our knowledge, this is the first case of TEMPI syndrome that was successfully treated with pomalidomide. Pomalidomide may be suitable for patients who do not respond to a proteasome inhibitor-based treatment. In addition, a subsequent ASCT could also be effective for achieving a further treatment response.
References
1.
Sykes
DB
, Schroyens
W
, O’Connell
C
. The TEMPI syndrome: a novel multisystem disease
. N Engl J Med
. 2011
;365
(5
):475
–7
. .2.
Zhang
X
, Fang
M
. TEMPI syndrome: erythrocytosis in plasma cell dyscrasia
. Clin Lymphoma Myeloma Leuk
. 2018 Nov
;18
(11
):724
–30
. .3.
Sykes
DB
, O’Connell
C
, Schroyens
W
. The TEMPI syndrome
. Blood
. 2020 Apr 9
;135
(15
):1199
–203
. .4.
Ryden
A
, Wei
K
, Rodriguez
R
, Mahrer
T
. Too much blood: a case of the newly described TEMPI syndrome
. Chest
. 2013
;144
(4
.5.
Kwok
M
, Korde
N
, Landgren
O
. Bortezomib to treat the TEMPI syndrome
. N Engl J Med
. 2012 May 10
;366
(19
):1843
–5
. .6.
Schroyens
W
, O’Connell
C
, Sykes
DB
. Complete and partial responses of the TEMPI syndrome to bortezomib
. N Engl J Med
. 2012 Aug 23
;367
(8
):778
–80
. .7.
Jasim
S
, Mahmud
G
, Bastani
B
, Fesler
M
. Subcutaneous bortezomib for treatment of TEMPI syndrome
. Clin Lymphoma Myeloma Leuk
. 2014 Dec
;14
(6
):e221
–3
. .8.
Liang
SH
, Yeh
SP
. Relapsed multiple myeloma as TEMPI syndrome with good response to salvage lenalidomide and dexamethasone
. Ann Hematol
. 2019 Oct
;98
(10
):2447
–50
. .9.
Miguel
JS
, Weisel
K
, Moreau
P
, Lacy
M
, Song
K
, Delforge
M
, Pomalidomide plus low-dose dexamethasone versus high-dose dexamethasone alone for patients with relapsed and refractory multiple myeloma (MM-003): a randomised, open-label, phase 3 trial
. Lancet Oncol
. 2013 Oct
;14
(11
):1055
–66
.10.
Dimopoulos
MA
, Sonneveld
P
, Leung
N
, Merlini
G
, Ludwig
H
, Kastritis
E
, International myeloma working group recommendations for the diagnosis and management of myeloma-related renal impairment
. J Clin Oncol
. 2016 May
;34
(13
):1544
–57
.11.
Dimopoulos
M
, Weisel
K
, van de Donk
NWCJ
, Ramasamy
K
, Gamberi
B
, Streetly
M
, Pomalidomide plus low-dose dexamethasone in patients with relapsed/refractory multiple myeloma and renal impairment: results from a phase II trial
. J Clin Oncol
. 2018 Jul
;36
(20
):2035
–43
.12.
Viglietti
D
, Sverzut
JM
, Peraldi
MN
. Perirenal fluid collections and monoclonal gammopathy
. Nephrol Dial Transplant
. 2012 Jan
;27
(1
):448
–9
. .13.
Diral
E
, Parma
M
, Renso
R
, Pezzatti
S
, Terruzzi
E
, Perfetti
P
, A fatal case of TEMPI syndrome, refractory to proteasome inhibitors and autologous stem cell transplantation
. Leuk Res
. 2020 Oct
;97
:106441
.14.
Shizuku
T
, Matsui
K
, Yagi
S
, Iwabuchi
S
. The first case of TEMPI syndrome in Japan
. Intern Med
. 2020 Jul 15
;59
(14
):1741
–4
. .15.
Kumar
S
, Paiva
B
, Anderson
KC
, Durie
B
, Landgren
O
, Moreau
P
, International Myeloma Working Group consensus criteria for response and minimal residual disease assessment in multiple myeloma
. Lancet Oncol
. 2016 Aug
;17
(8
):e328
–e46
.16.
Bazari
H
, Attar
EC
, Dahl
DM
, Uppot
RN
, Colvin
RB
. Case records of the Massachusetts General Hospital. Case 23-2010. A 49-year-old man with erythrocytosis, perinephric fluid collections, and renal failure
. N Engl J Med
. 2010 Jul 29
;363
(5
):463
–75
. .17.
Rosado
FG
, Oliveira
JL
, Sohani
AR
, Schroyens
W
, Sykes
DB
, Kenderian
SS
, Bone marrow findings of the newly described TEMPI syndrome: when erythrocytosis and plasma cell dyscrasia coexist
. Mod Pathol
. 2015 Mar
;28
(3
):367
–72
.18.
Lipsker
D
. Monoclonal gammopathy of cutaneous significance: review of a relevant concept
. J Eur Acad Dermatol Venereol
. 2017 Jan
;31
(1
):45
–52
. .19.
Fermand
JP
, Bridoux
F
, Dispenzieri
A
, Jaccard
A
, Kyle
RA
, Leung
N
, Monoclonal gammopathy of clinical significance: a novel concept with therapeutic implications
. Blood
. 2018 Oct 4
;132
(14
):1478
–85
.20.
Kenderian
SS
, Rosado
FG
, Sykes
DB
, Hoyer
JD
, Lacy
MQ
. Long-term complete clinical and hematological responses of the TEMPI syndrome after autologous stem cell transplantation
. Leukemia
. 2015 Dec
;29
(12
):2414
–6
. .21.
Belizaire
R
, Sykes
DB
, Chen
YB
, Spitzer
TR
, Makar
RS
. Difficulties in hematopoietic progenitor cell collection from a patient with TEMPI syndrome and severe iatrogenic iron deficiency
. Transfusion
. 2015 Sep
;55
(9
):2142
–8
. .22.
Pascart
T
, Herbaux
C
, Lemaire
A
, Soncin
F
, Hachulla
E
, Hatron
PY
, Coexistence of rheumatoid arthritis and TEMPI syndrome: new insight in microangiogenic-related diseases
. Joint Bone Spine
. 2016 Oct
;83
(5
):587
–8
.23.
Mohammadi
F
, Wolverson
MK
, Bastani
B
. A new case of TEMPI syndrome
. Clin Kidney J
. 2012 Dec
;5
(6
):556
–8
. .24.
Delgado
C
, Baweja
M
, Crews
DC
, Eneanya
ND
, Gadegbeku
CA
, Inker
LA
, A unifying approach for GFR estimation: recommendations of the NKF-ASN task force on reassessing the inclusion of race in diagnosing kidney disease
. Am J Kidney Dis
. 2022 Feb
;79
(2
):268
–288.e1
.25.
Sykes
DB
, Schroyens
W
. Complete responses in the TEMPI syndrome after treatment with daratumumab
. N Engl J Med
. 2018 Jun 7
;378
(23
):2240
–2
. .26.
van de Donk
NWCJ
, Pawlyn
C
, Yong
KL
. Multiple myeloma
. Lancet
. 2021 Jan 30
;397
(10272
):410
–27
. .27.
Attal
M
, Lauwers-Cances
V
, Hulin
C
, Leleu
X
, Caillot
D
, Escoffre
M
, Lenalidomide, bortezomib, and dexamethasone with transplantation for myeloma
. N Engl J Med
. 2017 Apr 6
;376
(14
):1311
–20
.28.
Gay
F
, Oliva
S
, Petrucci
MT
, Conticello
C
, Catalano
L
, Corradini
P
, Chemotherapy plus lenalidomide versus autologous transplantation, followed by lenalidomide plus prednisone versus lenalidomide maintenance, in patients with multiple myeloma: a randomised, multicentre, phase 3 trial
. Lancet Oncol
. 2015 Dec
;16
(16
):1617
–29
.29.
Fermand
JP
, Katsahian
S
, Divine
M
, Leblond
V
, Dreyfus
F
, Macro
M
, High-dose therapy and autologous blood stem-cell transplantation compared with conventional treatment in myeloma patients aged 55 to 65 years: long-term results of a randomized control trial from the Group Myelome-Autogreffe
. J Clin Oncol
. 2005 Dec 20
;23
(36
):9227
–33
.30.
Child
JA
, Morgan
GJ
, Davies
FE
, Owen
RG
, Bell
SE
, Hawkins
K
, High-dose chemotherapy with hematopoietic stem-cell rescue for multiple myeloma
. N Engl J Med
. 2003 May 8
;348
(19
):1875
–83
.31.
Yoshifuji
K
, Toya
T
, Adachi
H
, Fujita
M
, Wada
A
, Konuma
R
, Successful hematopoietic stem-cell mobilization with plerixafor plus granulocyte-colony stimulating factor in multiple myeloma patients treated with pomalidomide
. Int J Hematol
. 2019 Jul
;110
(1
):115
–8
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