Severe Reversible Pulmonary Hypertension in Smoldering Multiple Myeloma: Two Cases and Review of the Literature

Immune thrombocytopenic purpura is an autoimmune disorder retained after elimination of other causes of low platelets’ rate. It is mostly seen with B cell lymphoproliferative disorders. Immune thrombocytopenic purpura’s association with plasma cell neoplasms is possible but extremely rare. Although several pathophysiological mechanisms have been proposed, the causal link between these two conditions is not yet clearly understood. Therapeutic management is not standardized and depends mainly on the type of gammopathy and the chronology of onset of immune thrombocytopenic purpura compared to multiple myeloma. Our case is about an 81-year-old male diagnosed with concurrent smoldering multiple myeloma and immune thrombocytopenic purpura who was started on steroids without anti-neoplastic therapy for multiple myeloma with partial platelet response. We also review the few reported cases of simultanious immune thrombocytopenic purpura and smoldering multiple myelom or multiple myeloma. Keywords: ITP; thrombocytopenia; smoldering multiple myeloma; corticosteroids.

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Pulmonary hypertension as a dominant clinical picture in a case of amyloidosis and smoldering multiple myeloma

International Journal of Cardiology ◽

10.1016/j.ijcard.2006.07.059 ◽

2007 ◽

Vol 115 (1) ◽

pp. E29-E30 ◽

Cited By ~ 7

Author(s):

Jukka Lehtonen ◽

Pasi Kettunen

Keyword(s):

Multiple Myeloma ◽

Pulmonary Hypertension ◽

Clinical Picture ◽

Smoldering Multiple Myeloma

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Faculty Opinions recommendation of Lenalidomide plus dexamethasone for high-risk smoldering multiple myeloma.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.718056499.793484851 ◽

2013 ◽

Author(s):

Wee Joo Chng

Keyword(s):

Multiple Myeloma ◽

High Risk ◽

Smoldering Multiple Myeloma

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Faculty Opinions recommendation of Smoldering multiple myeloma.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.725419665.793522184 ◽

2016 ◽

Author(s):

Parameswaran Hari ◽

Binod Dhakal

Keyword(s):

Multiple Myeloma ◽

Smoldering Multiple Myeloma

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What Is New in the Treatment of Smoldering Multiple Myeloma?

Journal of Clinical Medicine ◽

10.3390/jcm10030421 ◽

2021 ◽

Vol 10 (3) ◽

pp. 421

Author(s):

Niccolo’ Bolli ◽

Nicola Sgherza ◽

Paola Curci ◽

Rita Rizzi ◽

Vanda Strafella ◽

...

Keyword(s):

Multiple Myeloma ◽

Clinical Trials ◽

High Risk ◽

Early Treatment ◽

Individual Case ◽

Symptomatic Disease ◽

Plasma Cell Neoplasm ◽

Smoldering Multiple Myeloma ◽

Wide Range ◽

Critical Issues

Smoldering multiple myeloma (SMM), an asymptomatic plasma cell neoplasm, is currently diagnosed according to the updated IMWG criteria, which reflect an intermediate tumor mass between monoclonal gammopathy of undetermined significance (MGUS) and active MM. However, SMM is a heterogeneous entity and individual case may go from an “MGUS-like” behavior to “early MM” with rapid transformation into symptomatic disease. This wide range of clinical outcomes poses challenges for prognostication and management of individual patients. However, initial studies showed a benefit in terms of progression or even survival for early treatment of high-risk SMM patients. While outside of clinical trials the conventional approach to SMM generally remains that of close observation, these studies raised the question of whether early treatment should be offered in high-risk patients, prompting evaluation of several different therapeutic approaches with different goals. While delay of progression to MM with a non-toxic treatment is clearly achievable by early treatment, a convincing survival benefit still needs to be proven by independent studies. Furthermore, if SMM is to be considered less biologically complex than MM, early treatment may offer the chance of cure that is currently not within reach of any active MM treatment. In this paper, we present updated results of completed or ongoing clinical trials in SMM treatment, highlighting areas of uncertainty and critical issues that will need to be addressed in the near future before the “watch and wait” paradigm in SMM is abandoned in favor of early treatment.

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Telomere Architecture Correlates with Aggressiveness in Multiple Myeloma

Cancers ◽

10.3390/cancers13081969 ◽

2021 ◽

Vol 13 (8) ◽

pp. 1969

Author(s):

Aline Rangel-Pozzo ◽

Pak Yu ◽

Sadhana LaL ◽

Yasmin Asbaghi ◽

Luiza Sisdelli ◽

...

Keyword(s):

Multiple Myeloma ◽

Disease Progression ◽

Genomic Instability ◽

Clinical Decision Making ◽

Three Dimensional ◽

Clinical Decision ◽

Response To Treatment ◽

Average Intensity ◽

Patient Response ◽

Smoldering Multiple Myeloma

The prognosis of multiple myeloma (MM), an incurable B-cell malignancy, has significantly improved through the introduction of novel therapeutic modalities. Myeloma prognosis is essentially determined by cytogenetics, both at diagnosis and at disease progression. However, for a large cohort of patients, cytogenetic analysis is not always available. In addition, myeloma patients with favorable cytogenetics can display an aggressive clinical course. Therefore, it is necessary to develop additional prognostic and predictive markers for this disease to allow for patient risk stratification and personalized clinical decision-making. Genomic instability is a prominent characteristic in MM, and we have previously shown that the three-dimensional (3D) nuclear organization of telomeres is a marker of both genomic instability and genetic heterogeneity in myeloma. In this study, we compared in a longitudinal prospective study blindly the 3D telomeric profiles from bone marrow samples of 214 initially treatment-naïve patients with either monoclonal gammopathy of undetermined significance (MGUS), smoldering multiple myeloma (SMM), or MM, with a minimum follow-up of 5 years. Here, we report distinctive 3D telomeric profiles correlating with disease aggressiveness and patient response to treatment in MM patients, and also distinctive 3D telomeric profiles for disease progression in smoldering multiple myeloma patients. In particular, lower average intensity (telomere length, below 13,500 arbitrary units) and increased number of telomere aggregates are associated with shorter survival and could be used as a prognostic factor to identify high-risk SMM and MM patients.

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