Difficulties in hematopoietic progenitor cell collection from a patient with TEMPI syndrome and severe iatrogenic iron deficiency

Belizaire, Roger; Sykes, David B.; Chen, Yi-Bin; Spitzer, Thomas R.; Makar, Robert S.

doi:10.1111/trf.13125

Cited by 10 publications

(6 citation statements)

References 23 publications

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“…Due to the pathogenesis of TEMPI syndrome, the treatment strategies are generally similar to those for multiple myeloma. Bor is one of the most frequently used treatments for TEMPI syndrome [1,[4][5][6][7][20][21][22]. Although most cases can be successfully treated with Bor, some cases are refractory to PI [8,13].…”

Section: Discussionmentioning

confidence: 99%

Successful Treatment of the TEMPI Syndrome with Pomalidomide Plus Dexamethasone Followed by Autologous Stem Cell Transplantation

Kawamura¹,

Tamaki²,

Nakamura³

et al. 2022

Acta Haematol

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TEMPI syndrome is a rare disease associated with plasma cell neoplasms. Although previous studies have reported that bortezomib is effective as a 1st-line treatment for TEMPI syndrome, some cases are refractory to this treatment. Pomalidomide, a kind of immunomodulatory drugs, 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. 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.

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Section: Discussionmentioning

confidence: 99%

Successful Treatment of the TEMPI Syndrome with Pomalidomide Plus Dexamethasone Followed by Autologous Stem Cell Transplantation

Kawamura¹,

Tamaki²,

Nakamura³

et al. 2022

Acta Haematol

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“…Some patients with TEMPI syndrome have required wheelchairs and continuous supplemental oxygen because of their progressive hypoxia ( 5 , 6 ). However, some patients with TEMPI syndrome have not had right-to-left shunting at their first presentation ( 7 , 8 ), possibly because the characteristic manifestations progress slowly over several years.…”

Section: Discussionmentioning

confidence: 99%

The First Case of TEMPI Syndrome in Japan

et al. 2020

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TEMPI syndrome, a disease entity comprising telangiectasia, erythrocytosis with high erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting, was first described by Sykes et al. in 2011. To our knowledge, only 15 cases have been reported worldwide, none of which were in Japan. We herein report a 47-year-old man who had intractable ascites for 2 and a half years and was referred to our department for a peritoneovenous shunt. In addition to ascites, he had telangiectasia, high erythropoietin, monoclonal gammopathy, and perinephric fluid collection. Thus, this is the first case of TEMPI syndrome in Japan.

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“…13,14 Of note, we found all the 3 patients had the clinical characteristics of peritoneal fluid and pleural effusion (supplemental Table 5), and previous studies also showed that 5 patients with TEMPI syndrome exhibited peritoneal fluid or pleural effusion. [15][16][17][18][19] In this study, we also found that the level of serum MIF in patient 1 was significantly decreased after treatment with the VCD regimen, which was changed in accordance with the downtrend of PCs, M-protein, hemoglobin, erythropoietin, and the improvement of telangiectasias, perinephric fluid collections, and intrapulmonary shunting (Figure 2E). These studies revealed a possible role of MIF in the development of intrapulmonary shunting, telangiectasias, elevated erythropoietin levels, and perinephric fluid collections, and furthermore, with the association between MIF levels and response to treatment in our patient, supporting that the duplication of 22q11.23 and upregulation of MIF in the monoclonal PCs may be involved in the pathophysiology of TEMPI syndrome.…”

Section: Resultsmentioning

confidence: 65%

Whole-genome sequencing suggests a role of MIF in the pathophysiology of TEMPI syndrome

Sun

Zhang

et al. 2021

Blood Advances

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TEMPI syndrome (telangiectasias, elevated erythropoietin level and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting) is a newly defined multisystemic disease with its pathophysiology largely unknown. Here, we report the whole-genome sequencing (WGS) analysis on the tumor-normal paired cells from a patient with TEMPI syndrome. WGS revealed somatic nonsynonymous single-nucleotide variants, including SLC7A8, NRP2, and AQP7. Complex structural variants of chromosome 2 were found, particularly within regions where some putative oncogenes reside. Of potential clinical relevance, duplication of 22q11.23 was identified, and the expression of the located gene macrophage migration inhibitory factor (MIF) was significantly upregulated in 3 patients with TEMPI syndrome. Importantly, the level of serum MIF in one patient with TEMPI syndrome was significantly decreased in accordance with the downtrend of plasma cells, M-protein, hemoglobin, and erythropoietin and the improvement of telangiectasias, perinephric fluid collections, and intrapulmonary shunting after treatment with plasma cell–directed therapy. In conclusion, our study provides insights into the genomic landscape and suggests a role of MIF in the pathophysiology of TEMPI syndrome.

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Difficulties in hematopoietic progenitor cell collection from a patient with TEMPI syndrome and severe iatrogenic iron deficiency

Cited by 10 publications

References 23 publications

Successful Treatment of the TEMPI Syndrome with Pomalidomide Plus Dexamethasone Followed by Autologous Stem Cell Transplantation

Successful Treatment of the TEMPI Syndrome with Pomalidomide Plus Dexamethasone Followed by Autologous Stem Cell Transplantation

The First Case of TEMPI Syndrome in Japan

Whole-genome sequencing suggests a role of MIF in the pathophysiology of TEMPI syndrome

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