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

Kawamura, Satoshi; Tamaki, Masaharu; Nakamura, Yuhei; Kawamura, Masakatsu; Takeshita, Junko; Yoshino, Nozomu; Misaki, Yukiko; Yoshimura, Kazuki; Matsumi, Shimpei; Gomyo, Ayumi; Okada, Yosuke; Akahoshi, Yu; Kusuda, Machiko; Kameda, Kazuaki; Tanihara, Aki; Kimura, Shun‐ichi; Nakasone, Hideki; Kako, Shinichi; Kanda, Yoshinobu

doi:10.1159/000525056

Cited by 5 publications

(2 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Patients with TEMPI syndrome generally have a dramatic response to plasma cell‐directed treatment which has included bortezomib, lenalidomide, and daratumumab [5–9]. As with our case, some authors have reported that treatment with ASCT has yielded long‐term responses [10].…”

Section: Figuresupporting

confidence: 61%

Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation

Nappi

Tauber

Sykes

2022

eJHaem

View full text Add to dashboard Cite

The TEMPI syndrome is a very rare paraneoplastic syndrome associated with plasma cell dyscrasia and monoclonal gammopathy. First described in 2011, the pathophysiology of TEMPI syndrome is still unknown. Essential for diagnosis is to recognize the five clinical findings: telangiectasias, erythrocytosis and elevated serum erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting. Here we report a case of a woman with the coexistence of TEMPI andThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

show abstract

Section: Figuresupporting

confidence: 61%

Coexistence of TEMPI syndrome and leukocytoclastic vasculitis successfully treated with autologous stem cell transplantation

Nappi

Tauber

Sykes

2022

eJHaem

View full text Add to dashboard Cite

show abstract

“…Bone marrow biopsy typically shows findings consistent with those of MGUS or smoldering myeloma [ 92 ]. Initial treatment with daratumumab or bortezomib is recommended, but other drugs and autologous stem cell transplants are also effective [ 93 , 94 , 95 , 96 , 97 ]. The increase of serum erythropoietin or the M-protein, as well as the reemergence of telangiectasias, may be used as markers of relapse.…”

Section: Mgcs As a Global And Unifying Conceptmentioning

confidence: 99%

Monoclonal Gammopathies of Clinical Significance: A Critical Appraisal

Ríos-Tamayo

Lahuerta

Martínez‐López

et al. 2022

Cancers

View full text Add to dashboard Cite

Monoclonal gammopathies of clinical significance (MGCSs) represent a group of diseases featuring the association of a nonmalignant B cells or plasma cells clone, the production of an M-protein, and singularly, the existence of organ damage. They present a current framework that is difficult to approach from a practical clinical perspective. Several points should be addressed in order to move further toward a better understanding. Overall, these entities are only partially included in the international classifications of diseases. Its definition and classification remain ambiguous. Remarkably, its real incidence is unknown, provided that a diagnostic biopsy is mandatory in most cases. In fact, amyloidosis AL is the final diagnosis in a large percentage of patients with renal significance. On the other hand, many of these young entities are syndromes that are based on a dynamic set of diagnostic criteria, challenging a timely diagnosis. Moreover, a specific risk score for progression is lacking. Despite the key role of the clinical laboratory in the diagnosis and prognosis of these patients, information about laboratory biomarkers is limited. Besides, the evidence accumulated for many of these entities is scarce. Hence, national and international registries are stimulated. In particular, IgM MGCS deserves special attention. Until now, therapy is far from being standardized, and it should be planned on a risk and patient-adapted basis. Finally, a comprehensive and coordinated multidisciplinary approach is needed, and specific clinical trials are encouraged.

show abstract