Spontaneous splenic rupture associated with multiple myeloma: a serious and unusual case

Mello, Denise Fabri Rezende Engracia; Costa, Mauro José Brandão da; Febrônio, Eduardo Miguel; Landell, Gláucia Aparecida Magnani; Queiroz, Rodolfo Mendes

doi:10.1016/j.htct.2019.01.011

Cited by 4 publications

(5 citation statements)

References 7 publications

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“…At present, treatment options include splenectomy, chemotherapy and autologous stem cell transplantation according to reported The clinical features of the newly diagnosed MM with spleen involvement. [4][5][6][7][8][9][10][11] Author Perry-Thornton et al [4] Sherwood et al [5] Ho et al [6] Magnoli et al [7] Peng et al [8] Abreu et al [9] Mello et al [10] Liu et al [11] Present experiences. Similar to a study of relapsed/refractory EMM, the present case appears to benefit more from chemotherapy than new drug therapy.…”

Section: Discussionmentioning

confidence: 99%

“…[3] In particular, spleen involvement in MM at the time of initial diagnosis is extremely rare, and to our knowledge, as few as 8 cases have been published in the literature to date. [4–11] Most of these cases were reported as splenic rupture secondary to MM, with overall survival <1 year or even several days. [5–7] Herein, we describe an unusual case of spleen infiltration in a newly diagnosed MM patient whose initial manifestation was megalosplenia, an atypical manifestation of MM.…”

Section: Introductionmentioning

confidence: 99%

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Megalosplenia as an initial manifestation of multiple myeloma with a novel CYLD gene mutation: A case report and literature review

Zhang,

Zhang

2024

Medicine

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Introduction: Megalosplenia in newly diagnosed multiple myeloma (MM) is extremely rare, posing diagnostic and therapeutic challenges due to its unusual location and clinical manifestations and lack of optimal therapeutic strategies. Case presentation: A 65-year-old female who was previously healthy presented with a history of ecchymosis on her right leg accompanied by progressive fatigue for 2 weeks. She was admitted to our center in July 2019 due to thrombocytopenia. The patient presented with megalosplenia, anemia, monoclonal protein (λ-light chain type) in the serum and urine, and 45.6% malignant plasma cells in the bone marrow. Splenectomy was performed due to persistent splenomegaly after 3 cycles of the bortezomib plus dexamethasone regimen, and immunohistochemistry results indicated λ-plasmacytoma of the spleen. The same cytogenetic and molecular abnormalities, including t(14;16), 14q32 amplification, 16q32 amplification, 20q12 amplification, and a novel CYLD gene mutation, were identified using fluorescence in situ hybridization and next-generation sequencing in both bone marrow and spleen samples. Therefore, a diagnosis of MM (λ-light chain type, DS III, ISS III, R-ISS III, high-risk) with spleen infiltration was proposed. The patient did not achieve remission after induction treatment with bortezomib plus lenalidomide and dexamethasone or salvage therapy with daratumumab plus ixazomib and dexamethasone. However, she ultimately did achieve very good partial remission with a regimen of bendamustine plus lenalidomide and dexamethasone. Unfortunately, she died of pneumonia associated with chemotherapy. Conclusion: To our knowledge, only 8 cases of spleen plasmacytoma at MM diagnosis have been described previously. Extramedullary myeloma patients with spleen involvement at diagnosis are younger and that the condition is usually accompanied by splenic rupture with aggressive clinical features and poor prognosis. Further studies are needed to explore pathogenesis and effective therapies to prolong the survival of such patients.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Megalosplenia as an initial manifestation of multiple myeloma with a novel CYLD gene mutation: A case report and literature review

Zhang,

Zhang

2024

Medicine

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“…Splenic injury commonly results from abdominal trauma, most frequently from motor vehicle accidents. The incidence of splenic injury in multiple myeloma is very rare, with only four cases of splenic rupture having been reported in the literature [ 5 , 6 ]. Splenomegaly can be a manifestation of multiple myeloma as well, but this often manifests with thrombocytopenia which was not present in our patient [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Seurat Spleen: A Pathognomonic Sign in Angiography

Newcomer¹,

Gabriel²,

Raissi³

2021

Cureus

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Splenic injury commonly occurs following abdominal trauma and can result in severe complications and death if it goes unrecognized. The Seurat spleen is a term used to describe the angiographic appearance of splenic injury following blunt trauma, given its resemblance to the pointillistic artwork of French neo-impressionist Georges Seurat. We present a case of a 43-year-old man who presented following a motor vehicle collision and was found to have multiple punctate foci of contrast extravasation in the spleen consistent with the Seurat spleen angiographic sign. This angiographic pattern can be used as a pathognomonic sign to identify splenic injury, with early identification crucial to preventing further complications of the injury.

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“…These extramedullary sites, in decreasing order of prevalence, were: kidneys (27.3%); skin (23%); lymph nodes (17.3%); central nervous system (10.1%); lungs and respiratory tract (6.5%); liver and gastrointestinal tract (5.8%); pleura and heart (5%), and; spleen, ovaries and testicles (5.3%). Spleen infiltration, followed by its pathological rupture as the initial presentation of multiple myeloma, is extremely rare, with only three cases reported so far, 6 , 7 , 8 two of them associated with fatal outcomes.…”

Section: Literature Reviewmentioning

confidence: 99%

Spontaneous spleen rupture: an unusual presentation of extramedullary multiple myeloma

Abreu

Farias

Lima

et al. 2020

Hematology, Transfusion and Cell Therapy

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Spontaneous splenic rupture associated with multiple myeloma: a serious and unusual case

Cited by 4 publications

References 7 publications

Megalosplenia as an initial manifestation of multiple myeloma with a novel CYLD gene mutation: A case report and literature review

Megalosplenia as an initial manifestation of multiple myeloma with a novel CYLD gene mutation: A case report and literature review

Seurat Spleen: A Pathognomonic Sign in Angiography

Spontaneous spleen rupture: an unusual presentation of extramedullary multiple myeloma

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